腦血管疾病學 the Tingling Sensation and Limbs Weakness Recurred and She Was Referred 1 to Our Hospital for Further Survey

Poster Session performed one hour later was negative. 壁報論文 Her symptoms recovered gradually after 8 hours of staying in hospital. Nevertheless, five hours after discharge, 腦血管疾病學 the tingling sensation and limbs weakness recurred and she was referred 1 to our hospital for further survey. 個案研究:以急性全身刺痛與無力表現 Neurological examination revealed the 的雙內側延髓中風 muscle power was 3/5 in all four limbs 朱海瑞湯頌君鄭建興 with bilateral extensor type of plantar 國立臺灣大學醫學院附設醫院神經部 response. The gag reflex was trance Bilateral medial medullary infarction and tongue could not be protruded. Ten presenting as acute fluctuating tingling hours after arriving in our emergency sensation and weakness over all limbs room, the muscle strength deteriorated and trunk: a case report Hai-Jui Chu, Sung-Chun Tang, Jiann-Shing Jeng to 2/5 in upper limbs and 1/5 in lower Department of Neurology, National Taiwan University Hospital ones. Magnetic resonance imaging revealed bilateral MMI infarction with Background Bilateral medial heart shape sign. Digital subtraction medullary infarction (MMI) is extremely angiography showed normal rare among patients with acute ischemic appearance of bilateral vertebral stroke. Here we reported a case of arteries but suspected an azygos bilateral MMI presenting as an unusual anterior spinal artery mainly derived course of acute fluctuating tingling from the left vertebral artery. For the sensation and weakness in all limbs and consequently respiratory distress, she trunk for 2 days. was intubated. Heparinization was given for 2 days and was shifted to Case Report A 41-year-old female had Clopidogrel 1 tablet per day. Three newly diagnosed hypertension but did not months later, her muscle strength take regular medication for several improved significantly and she could months. The day before the symptom walk with a quadricane well. onset, she went to bed as usual. Around midnight, she was awakened by Conclusion Although rare, bilateral generalized tingling and burning MMI needs to be considered in the sensation. It first started from tips of all differential diagnosis for patients with fingers and soon ascended to both arms, acute motor and sensory symptoms of head, neck and whole body. She also all limbs and trunk plus bulbar complained four limbs weakness symptoms. although she could elevate her four limbs symmetrically. Head CT which was

1 2 size. Carotid stenosis was associated 腦出血病人頸動脈內膜厚度與再中風 positively with old age, hypertension, 的關聯性 and inversely with hemorrhage size. 江宜蓁陳怡君林口長庚紀念醫院神經內科 Total cholesterol was the only independent predictor (OR 1.03 and Carotid intima-media thickness and 95% CI 1.00-1.06, P = 0.04) of incidence of recurrent stroke in significant stenosis. The follow-up patients with first-ever spontaneous intracerebral hemorrhage (ICH) time was 4.30 ± 2.61 years for Yi-Chen Chiang, Yi-Chun Chen first-ever ICH patients with CCD, with Department of Neurology, Chang Gung Memorial Hospital, Lin-Kou Medical Center time to recurrent stroke 2.41 ± 2.02 years. Recurrent stroke was 15.1 %, Objectives Increase in carotid with 12.6 % for infarction and 2.5% intima-media thickness (IMT) and for ICH. Multivariable Cox stenosis are shown to be independent proportional hazards models did not predictors of primary ICH, but their find any association of carotid association with recurrent stroke after thickness or stenosis with recurrent primary ICH is less clear. This study stroke. Increasing carotid IMT was examined the correlation of carotid associated with a hazard ratio of ultrasonographic findings with first-ever recurrent ICH of 0.49 (95% CI, ICH and its role in recurrence of ICH and 0.08-3.0) and a hazard ratio of infarction. recurrent infarct of 1.01 (95% CI, 0.63-1.64). Increasing carotid stenosis Methods This retrospective study was associated with a hazard ratio of included 508 patients with first-ever recurrent ICH of 0.99 (95% CI, spontaneous ICH. Among them, 166 0.90-1.08) and a hazard ratio of patients had examination of carotid recurrent infarct of 1.00 (95% CI, color-coded duplex sonography (CCD). 0.97-1.03).

Results CCD was performed more Conclusion The clinical impact of frequently in those with hypertension, carotid IMT and stenosis on recurrent higher Glasgow Coma Scale and smaller stroke in primary ICH cases remains ICH size due to better prognosis. insignificant. This conclusion can be Ultrasonographic findings were further applied for the population of patient divided into 3 subtypes: carotid thickness, with higher Glasgow Coma Scale and carotid stenosis, and significant stenosis smaller ICH size. (>50%). Carotid thickness was correlated positively with old age, hypertension, and DM, and inversely with hemorrhage

2 3 The results of attitudes showed tending 探討花蓮地區緊急救護技術員之急性 to "agree". The results of prehospital 腦中風到院前救護行為意圖 care intention of acute stroke showed 江俊宜 1 胡益進 2 李明憲 3 陳俊安 4 李俊泰 4 1 國軍花蓮總醫院內科部 2 國立台灣師範大學健 "possible to do it ". 康促進與衛生教育學系 3 國立東華大學課程設 2. The relationship between 4 計與潛能開發學系三軍總醫院神經科部 knowledge, attitudes, self-efficacy,

Prehospital care intention of acute cues to action and health behavior stroke related factors among showed positive correlations. emergency medical technicians in the 3. Among the background variables Hualien area Chun-I Chiang1, Yih-Jin Hu 2, Ming-Shinn Lee3, “experience of acute stroke care”, Chun-An Chen4, Jiunn-Tay Lee4 "cues to action”, “self-efficacy” on 1 Department of Internal Medicine, Hualien “prehospital care of acute stroke" could Armed Force General Hospital 2 Department of Health Education, National effectively predict health behavior. The Taiwan Normal University most effective variable was 3 Department of Curriculum Design and Human Potentials Development Graduate Institute of “self-efficacy”. However, “having Compulsory Education, National Dong Hwa advanced cardiovascular life support University, Hualien, 4 Department of Neurology, Tri-Service General training” became a negative effect. Hospital Conclusion Case-based learning for

EMS stroke education may be an Background Emergency medical effective method under the positive services (EMS) transport and hospital variables “experience of acute stroke prenotification are not been strengthened care”, “cues to action”, and in Taiwan. The main purpose of this “self-efficacy”. study is to understand the status of the knowledge, cues to action, attitudes, 4 self-efficacy and intention and related 經動脈血管栓塞術改善顱內動靜脈 factors of prehospital care of acute stroke 廔管引起之癲癇重積症 among emergency medical technicians in 江星逸 1 劉濟弘 1 廖洺鋒 1 林秀娜 1 黃浩輝 2 林口長庚紀念醫院 1 神經內科 2 放射診斷科 the Hualien area.

Transarterial Embolization Methods The target group for this Improves Refractory Status study focused on first line technicians Epilepticus in a Patient with Intracranial Dural Arteriovenous working in the Hualien area. Fistula Hsing-I Chiang1, Chi-Hung Liu1, Ming-Feng 1 1 2 Results The research tool was based on Liao , Siew-Na Lim , Ho-Fai Wong 1Department of Neurology and 2Department of a structured questionnaire among a total Medical Imaging and Intervention, Chang Gung of 109 valid questionnaires. Memorial Hospital, Linkou Medical Center

1. The results of knowledge on acute Background Status epilepticus (SE) stroke showed an upper middle level.

3 is an infrequent manifestation of Conclusion The treatments of this intracranial dural arteriovenous fistula patient suggest the TAE with Onyx (DAVF). Endovascular treatment is an may effectively alleviate the refractory option when aggressive symptoms SE by means of improvement of develop. We report our experience using vasogenic edema and venous endovascular embolization in the hypertension. treatment of DAVF with SE refractory to medications. 5 平板電腦與智慧型手機於急性缺血 Case Report An 82-year-old woman 性腦中風處置之臨床運用何旭華 was admitted due to SE with seizure 虎尾天主教若瑟醫院神經內科 focus at left temporal area. Brain magnetic resonance imaging (MRI) The Applications of Tablet Computer and Smart Phone in Acute showed venous hypertension and Ischemic Stroke vasogenic edema at left occipital and Hsu-Hua Ho Department of Neurology, Saint Joseph's temporal lobes. Digital subtraction Hospital angiography confirmed Cognard type II a + b DAVF at left lateral sinus supplied by 背景以往，我們對於急性缺血性中 middle meningeal artery with reflux into 風期的處置治療，大多是書面記錄於 left posterior cortical veins and left 紙本上，再輸入於電腦，進行後置的 transverse sinus stenosis. Midazolam 人工作業。這種處置與記錄的過程， continuous infusion (0.21mg/kg/hr) and 不僅延長了急性處置的時效，並且增 oral antiepileptic drugs (AED) including 加了後續統計與分析工作的複雜性。 levetiracetam (3000 mg/day), 隨著智慧型手機與平板電腦的普及 oxcarbazepine (1200 mg/day), topiramate 化和軟體程式的革新和進步。我們藉 (400 mg/day), and valproic acid (1600 此發展急性缺血性中風處置流程的 mg/day) failed to control the SE but led 關鍵軟體程式，自動將所得檢驗數據 to unstable vital signs. We arranged 進行符合條件比對、透過數位化記錄 transarterial embolization (TAE) with 各項臨床神經學檢查量表、計算所需 Onyx through middle meningeal artery 施打藥物劑量、同時也追蹤和立即反 and the feeding artery was obliterated. 映出處置進行的時間與流程準確性。 The SE subsided and the electroencephalogram showed burst 方法我們使用 Android 手機作業系 suppression after TAE. Follow-up MRI 統，它是一個以 Linux 為基礎的半開 showed improvement of vasogenic 放原始碼作業系統，由於它支援多工 edema. Meanwhile, we successfully 與多重語言，並且內置 SQLite 小型 discontinued midazolam infusion and 關聯式資料庫管理系統來負責存儲 tapered the total doses of AED. 數據；同時也支援所有的雲端和無線

4 網路服務器訊息；因此非常適合用來開 6 發小型的醫療影像與數據的儲傳資料鑑別診斷洗腎病人之人工血管造成庫。所使用開發應用程式的編程語言為之鎖骨下動脈竊血症候群吳令治 J AVA 和 XML。藉由無線網路系統，財團法人台灣基督教門諾會醫院神經內科將病人基本資料與各項檢驗數據由系統帶入，並進行是否符合條件之判斷。 Can we use carotid duplex to differentiate subclavian steal caused 各項臨床評估量表( NIHSS、巴氏量 by vascular access for hemodialysis 表、和雷氏量表)都于以數位和資料庫 from by subclavian stenosis? 化。量表施測的同時，系統也啟動計時 Ling Chih Wu Neurologic department Mennonite Christian 功能，計算出檢查所花費的時間。施測 hospital 的結束，電腦根據預先設定的流程公式，快速的計算出總分數，多次評估後 Purpose Subclavian steal 作縱橫向的參考和比對。並根據病人體 phenomenum can be caused by 重與單位注射劑量，顯示 r-tPA 治療的 stenosis of subclavian artery or by the 總量與分次施打的劑量。每一筆記錄皆 high and low-resistant flow of vascular 顯明記錄時間，所耗時間，是否符合步 access for patients under hemodialysis. 驟程序，以及運算結果，藉由無線網路 We described a simple method to 系統，自動輸入關聯性資料庫中。 differentiate the different causes by duplex. We want to see if there is any 結果由於檢查醫師，可將神經學評估 difference between these two 各項分數，經由點選智慧型手機、存入 conditions in carotid duplex. 和演算在資料庫中。並藉由電腦與網路資料庫系統的輔助，大大的節省了各項 Method We performed a prospective 報告收集與數據填寫所需花費的時 study and collected patients from 間。電腦自動進行符合條件比對、監控 December 2010 to July 2011 with 篩選步驟、計算藥物使用劑量、和避免 informed consent for all patients with 人為疏乎，讓醫師能夠進行準確的判 vascular access. We have 5 patients 讀。根據臨床的實際操作，這一電腦的 with subclavian steal without vascular 輔助應用，較傳統的紙筆記錄約略節省 access in this period of time and, 五到八分鐘的時間。 according to patient’s will to participate in this study, 11 patients 結論總而言之，訓練有素的專業團 with vascular access in the forearm. 9 隊，良好的部門默契與合作協調，醫病 patients with vascular access were 關係的互信溝通，再加上新的電腦科技 excluded from our study for there is no 與網路資料庫應用，將提供給病患更好 subclavian steal in the ipsilateral side 的醫療品質，把握住急性腦中風黃金治 of vascular access. For every patient, 療的時機。同時對於日後資料的查詢、 we compressed the arm to stop the 統計和分析也提供詳實記錄的參考。 flow by cuff (we only studied patients whose vascular access was located in

5 1 the forearm to avoid direct compression Department of Neurology and Stroke Center, NTUH; 2 Department of Neurology, NTUH, of the vascular access) during duplex Yun-Lin branch examination and studied the flow pattern of subclavian and vertebral artery before Objective To investigate the and during the cuff test determinants of stroke evolution and roles of thrombolytic therapy in large Result No significant changes of flow anterior choroidal artery (AChA) pattern for either vertebral or subclavian infarct. artery during the cuff test for all 5 patients without vascular access. On the Background Large AChA infarct is contrary, flow for both subclavian and highly associated with stroke evolution; vertebral artery change to nearly normal however, the risk and preventing pattern when the blood flow is blocked in factors for stroke evolution are still the arm of these 2 patients with unclear. In addition, although the subclavian steal and with vascular access. benefits of thrombolytic therapy are well known in acute ischemic stroke, Discussion From this study, it seemed the effects of thrombolytic therapy in that duplex findings of subclavian steal AChA infarct are rarely reported. are different between patients without and with vascular access. We can use this Methods From January 2009 through simple method to differentiate the July 2012, we studied 118 consecutive different causes of subclavian steal adult patients with acute large AChA especially for patients with end-stage infarct in National Taiwan University renal disease who often have Hospital (NTUH) and NTUH, Yun-Lin atherosclerosis and are vulnerable to branch. The diagnosis of large AChA contrast used in angiography infarct was confirmed as abnormal hyperintensity in at least three MRI 7 slices (thickness, 5 mm) at DWI within 血栓溶解治療避免前脈絡叢動脈梗塞 typical AChA vascular regions. The 之中風惡化並增進長期預後 definition of stroke evolution was 吳孟真 1 蔡力凱 1,2 葉馨喬 1,2 湯頌君 1 鄭建興 1 1 neurological deterioration with 台大醫院神經部暨腦中風中心 2 台大醫院雲林分院神經部 increase of NIHSS by or more than two in 7 days after onset of stroke.

Thrombolytic Therapy Prevents Stroke Evolution and Improves Results In 118 patients with large Long-term Outcome in Large Anterior AChA infarct, 50 cases (42.4%) Choroidal Artery Infarct developed stroke evolution with an Meng-Chen Wu, MD, 1 Li-Kai Tsai, MD, PhD,1,2 Sing-Joe Yeh, MD, 1,2 Sung-Chun Tang, MD, 1 increase of NIHSS by 3.7±2.1. Among Jiann-Shing Jeng1

6 various demographical, clinical, and Ya-Chun Wu, Jung Lung Hsu Department of neurology, Shin Kong Wu neuroimaging factors, only thrombolytic Ho-Su Memorial Hospital therapy was reversely associated with stroke evolution (P = 0.02). Using Background Stroke of the multivariate analysis, thrombolytic hypothalamus are vanishingly rare, as therapy was still a protective determinant the hypothalamus has the most for stroke evolution (odds ratio, 0.19; luxuriant blood supply in the brain, 95% confident interval, 0.04 to 0.99). befitting a site that is absolutely critical Additionally, thrombolytic therapy was to maintain life. significantly associated with lower mRS at 3 months after onset of stroke (P = Case report A 64-year-old man with 0.04), despite of higher initial NIHSS underlying hypertension, Type 2 DM (<0.001). Earlier treatment (P = 0.009) and coronary artery disease had and higher dosage (P = 0.02) of received CABG (coronary artery thrombolytic therapy were related to bypass graft) surgery on 2012/9/28. An greater improvement. The independent acute ischemic stroke was noted right determinants for functional outcome after the operation. Brain MRI on 10/2 included age (odds ratio, 1.08; 95% showed acute infarction on the left confident interval, 1.04 to 1.13), initial basal ganglion, left mesial temporal NIHSS (1.79; 1.37 to 2.34), occurrence lobe and left hypothalamus. Brain MRI of stroke evolution (4.32; 1.59 to 11.69), also incidentally found a pituitary and thrombolytic therapy (0.15; 0.03 to macroadenoma. While the patient’s 0.94). muscle power got totally recovered afterwards, cognitive problem Conclusion Two-fifth of patients with remained. The patient had got very large AChA infarct got stroke evolution. poor memory, and he failed on the Thrombolytic therapy not only prevented registration of the 3 objects test. stroke evolution, but independently Besides, he also had verbal improved long-term outcome in large communication problem. The language AChA infarct symptom included agraphia, alexia and anomia. He is also noted with 8 significant poor appetite after the 下視丘缺血性中風引起嚴重記憶缺失 stroke, and he persistently has very 及食慾不振 little intake. The patient received 吳雅純徐榮隆 pituitary tumor excision on 11/9. 新光吳火獅紀念醫院神經內科

Hypothalamus ischemic infarction Conclusion The patient’s significant responsible for the severe memory poor appetite should be due to the impairment and poor appetite

7 lateral hypothalamic area infarction, and Hospital). All consecutive stroke his severe memory impairment could be patients treated with intravenous tPA explained by the involvement of the within 4.5 hours of symptom onset columns of fornix. were classified into two groups - treated before 3 h of onset (group A) 9 and treated between 3 and 4.5 h of 超過三小時之靜脈栓溶治療 onset (group B). 1 2 3 4 5 宋昇峯陳志弘林慧娟陳右緯曾美君 1 戴德森醫療財團法人嘉義基督教醫院內科部神經內科 Results Between January 2007 and 2 國立成功大學附設醫院神經部 June 2012, a total of 624 patients 3 奇美醫院神經內科壢新醫院 4 神經內科 5 院長室特聘研究員 (group A: 548, group B: 76) were included in this study. The Intravenous Thrombolysis Beyond the demographic and stroke risk factors 3-Hour Time Window 1Sheng-Feng Sung, 2Chih-Hung Chen, 3Huey-Juan Lin, were similar between the two groups. 4 5 Yu-Wei Chen, Mei-Chiun Tseng The baseline National Institutes of 1Division of Neurology, Department of Internal Medicine, Ditmanson Medical Foundation Chia-Yi Health Stroke Scale (NIHSS) scores Christian Hospital 2Department of Neurology, National Cheng Kung were comparable (group A: median 13, University Hospital 3Department of Neurology, Chi-Mei Medical Center interquartile range 8-20, group B: 4Department of Neurology, Landseed Hospital median 13 interquartile range 7-17, 5Senior Researcher, Landseed Hospital P=0.359). The median Background The recommended onset-to-treatment time was 125 treatment time window of intravenous minutes for group A and 197 minutes tissue plasminogen activator (tPA) has for group B. There were no differences been expanded to 4.5 hours according to between the two groups regarding the various guidelines. Because of off-label rates of any intracerebral hemorrhage use, there is limited experience in (group A: 23%, group B: 20%, thrombolyzing stroke patients between P=0.503) and symptomatic 3-4.5 hours in Taiwan. We hemorrhage (group A: 7%, group B: retrospectively assessed the safety and 4%, P=0.280). The chances of marked short-term efficacy of intravenous improvement at discharge (defined as a thrombolysis in this time window. reduction of ≥10 in the total NIHSS score or a total NIHSS score of 0 or 1) Methods We analyzed the were 35% in group A and 34% in prospectively collected data in the stroke group B. registries of 4 stroke centers (National Cheng Kung University Hospital, Conclusion Our study implicates that Chi-Mei Medical Center, Chia-Yi patients with acute ischemic stroke Christian Hospital, and Landseed may be successfully treated with

8 intravenous thrombolysis shortly after (n=1). Spontaneous intracranial the 3-hour treatment window. Patients dissections involved the right who arrive near the end of the 3-hour paraclinoid internal carotid artery window but bear a chance to be treated (n=1), left middle cerebral artery (n=2) before 4.5 hours of onset probably should and right middle cerebral artery (n=1). not be excluded from treatment. Dissections were treated with balloon-expandable stent in one patient 10 and self-expandable Neuroform stents 以顱內支架置放治療進行性顱內血管 in three patients. Mean dissection 剝離–亞東醫院之經驗 stenosis was 82% before the 李少白 1 賴彥君 2 邱奕華 1 唐志威 1 鄭又禎 1 黃俐文 1 郭冠宏 2 朱耀棠 1 intervention and 18% after stent 亞東紀念醫院 1 神經科 2 影像醫學科 placement. There were no procedure-related complications. The Stenting in Symptomatic and Progressive Intracranial Dissections: range of follow-up time was from 3 Experience at Far Eastern Memorial months to 29 months. All patients Hospital reported clinically improvement Siu-Pak Lee1, Yen-Jun Lai2, Yih-Hwa Chiou1, Chih-Wei Tang1, Yu-Chen Cheng1, Lin-Wen without recurrent stroke. 1 2 1 Huang , Kuei-Hong Kuo , Yiu-Tong Chu 1 Division of Neurology, 2Division of Medical Imaging, Far Eastern Memorial Hospital Conclusion Stent placement seems to be safe and technically effective in the Objective To evaluate endovascular endovascular management of treatment with stent deployment in intracranial dissections, with favorable patients with intracranial arterial clinical outcomes. dissections 11 Method Retrospective review of data at 顱內硬腦膜動靜脈廔管併發可逆性非 Far Eastern Memorial Hospital between 血栓性上矢狀竇阻塞及重積性癲癇 2009 and 2011 of four patients (two men, 李君右湯頌君鄭建興國立台灣大學醫學院附設醫院神經內科 two women; mean age: 45 years) with intracranial arterial dissections treated Intracranial Dural Arteriovenous with stent placement were identified Fistula Complicating Reversible Non-thrombotic Occlusion of the Superior Sagittal sinus and status Result All patients were symptomatic epilepticus and had clinical deterioration shortly after admission and that prompted Chun-Yu Lee, Sung-Chun Tang, Jiann-Shing Jeng aggressive angiographic examinations. Department of Neurology, National Taiwan University Hospital Diagnosis was made based on cerebral angiographic findings including intimal Background Seizure is an uncommon flap (n-3) and “string and bead sign”

9 presentation of dural arteriovenous fistula patient regained clear consciousness. (dAVF) and usually associated with the Trans-arterial embolization (TAE) of existence of cerebral venous sinus dAVF was performed smoothly five thrombosis. days after angioplasty. GTCS recurred Here we reported a rare case of once shortly after TAE but it resolved intracranial dAVF who had status soon after titrating up midazolam epilepticus resulting from acute dosage. Continuous right shoulder reversible non-thrombotic occlusion of twitching with preserved the superior sagittal sinus (SSS). consciousness was noted while tapering midazolam again. Status Case Report A 49-year-old man with partialis continua was impressed and past history of diabetes mellitus was midazolam was titrated up again. After found lying at home with generalized adjusting anti-epileptic drugs, no more tonic clonic seizure(GTCS) by family. He seizures developed thereafter and the was sent to a local community hospital patient recovered well. while the convulsion sustained. Intravenous phenytoin was given but in Conclusion Our study demonstrated vain. Under the impression of status that intracranial dAVF may cause epilepticus, continuous infusion of non-thrombotic occlusion of the midazolam was started for seizure affected sinus and induce status control. CT angiography showed SSS epilepticus. The diagnosis of dAVF occlusion with venous hypertension, needs to be considered in patients with causing multiple small intracerebral status epilepticus, even without hemorrhage and brain edema. He was cerebral venous sinus thrombosis. transferred to our hospital for further management. Digital subtraction 12 angiography was performed, which 硬膜外移植人類誘導性多能幹細胞 revealed dAVF draining into SSS and 分化的神經先驅細胞促進中風成鼠的功能恢復 non-visualization of the posterior part of 李怡慧 1,2,黃相碩 3,莊靜玉 4,蘇育世 2,廖可熏 SSS. However, no thrombus was detected 1,莊嘉琪 1,林宏叡 5,謝睿瑜 5,蘇琡涵 5,李光 in the sinus during the examination. 申 6,7,郭紘志 4,王學偉 5,6,8 1 台北榮民總醫院神經內科, 2 陽明大學腦科 External compression of the dural sinus 學研究所, 3 中山醫學大學藥理研究所, 4 中 lumen by abnormal vessels within the 央研究院基因體研究中心, 5 陽明大學微免 6 7 sinus walls was suspected. Balloon 研究所, 陽明大學臨床醫學研究所, 台北榮民總醫院骨科部, 8 榮陽基因體研究中心 dilatation and angioplasty to SSS were performed and then patency of SSS was Epidural grafts of human iPS restored. Midazolam was tapered cell-derived neural progenitors improve post-stroke recovery via a gradually after the procedure and the unique secretome

10 1, 2 3 I-Hui Lee , Shiang-Suo Huang , Ching-Yu microarrays. Moreover, iPSC-NPCs or Chuang4, Yu-Shih Su2, Ko-Hsun Liao1, Chai-Chi Chuang1, Hung-Jui Lin5, Jui-Yu Hsieh5, Shu-Han vehicle were delivered via epidural 5 7 Su , Oscar Kuang-Sheng Lee6, , Hung-Chih fibrin matrix over the infarct cortex 1 Kuo4, Hsei-Wei Wang5, 6, 8 1 Department of Neurology, Neurological Institute, week following permanent middle Taipei Veterans General Hospital, Taiwan 2 Institute of Brain Science, National Yang-Ming cerebral artery occlusion (MCAO) to University, Taipei, Taiwan compare behavioral and histological 3 Department of Pharmacology and Institute of Medicine, Chung-Shan Medical University, changes. Taichung, Taiwan 4 Stem Cell Program, Institute of Cellular and Organismic Biology and Genomics Research Results Over 80% Pax6+/Sox1+ Center, Academia Sinica, Taipei, Taiwan; iPSC-NPCs were obtained after 5 Institute of Microbiology and Immunology, National Yang-Ming University, Taipei, Taiwan differentiation and mechanical 6 Institute of Clinical Medicine, National Yang-Ming University, Taipei, Taiwan enrichment. In the transmembranous 7 Department of Orthopedics and Traumatology, co-cultures, the iPSC-NPCs, in contrast Taipei Veterans General Hospital, Taiwan 8 VGH-YM Genome and Cancer Research to mesenchymal stem cells from bone Centers, National Yang-Ming University, Taipei, marrow and umbilical cord Wharton’s Taiwan. jelly, enhanced neuronal growth and Objective and background Neural mitigated astrogliosis via a unique progenitor cells derived from induced secretome. The iPSC-NPC- pluripotent stem cells (iPSC-NPCs) show transplanted rats showed significant promise for tailor-made cell therapy in improvement in their paretic forelimb experimental stroke; however, their usage and grip strength from 10 days translational implantation is hampered by post-transplantation (dpt) onwards propensity of tumorigenesis. Little is compared with the vehicle-transplanted known about their secretory mechanisms rats. iPSC-NPCs homed into the following transplantation. We peri-infarct cortex through the leaky investigated the secretome of human blood brain barrier within 1 dpt, but iPSC-NPCs and their extraparenchymal poorly survived without formation of a implantation via an epidural fibrin matrix teratoma at 21 dpt. The cell-treated rats in adult stroke rats. had significantly reduced infarct/atrophy volumes, ameliorated Methods A transmembranous inflammatory infiltration, astrogliosis, co-culture system with rat cortical cells and apoptosis, as well as augmented subjected to oxygen-glucose deprivation angiogenesis, oligodendrocyte (OGD) was used to characterize the progenitors, and white matter tract iPSC-NPC secretome in comparison with integrity. human mesenchymal stem cells utilizing a systems biology approach including Conclusions These results suggest cytokine arrays and whole-genome that the epidural biopolymer-delivered

11 iPSC-NPCs may not only reduce complaints of acute onset of weakness tumorigenic risk but also promote and numbness in his right limbs during peri-infarct cellular survival and his work. Right central type facial functional regeneration primarily through palsy, mild dysarthria, and right bystander effects. hemiparesis were observed. Head CT showed no evidence of hemorrhage. 13 Under the impression of acute 缺鐵性貧血相關之年輕男性缺血性 ischemic stroke with an NIH stroke 中風 scale of 9 scored by a neurologist, he 李俊賢 1,2,3 李君右 1, 鄭文誠 4 1 台大醫院神經部,2 國家衛生研究院環境衛 received treatment with tissue 生與職業醫學組,3 台大醫院環境暨職業醫學 plasminogen activator (tPA) 0.8 mg/Kg, 4 部, 台大醫學院檢驗醫學科，台大醫院檢驗醫 and was admitted to the stroke unit. 學部 Brain MRI revealed a small area of Iron deficiency anemia as a rare cause diffusion restriction at left of ischemic stroke in a young man: a periventricular white matter, which case report Lukas Jyuhn-Hsiarn Lee1,2,3, Chung-Yo Lee1, indicates acute infarct. He had 4 Wern-Cherng Cheng significant neurological improvement, 1 Department of Neurology, National Taiwan University Hospital with NIHSS of 2 as he was discharged 2 Division of Environmental Health and Occupational Medicine, National Health at day 6 after the stroke. Research Institutes His past medical history was 3 Department of Environmental and Occupational Medicine, National Taiwan University Hospital remarkable for anemia without any 4 Laboratory Medicine, College of Medicine, treatment. The hemogram revealed National Tawian University and Department of Laboratory Medicine, National Taiwan microcytic anemia (RBC 5.57 M/μL, University Hospital Hb 11.3 g/dL, hematocrit 37.2%, MCV

66.8 fL, MCH 20.3 pg/cell), and iron Background The cause of young stroke profile showed iron of 20 μg/dL, TIBC remains underdetermined in about 30% of 373 μg/dL, and serum ferritin level of cases. Iron deficiency is a rare cause was 3.76 μg/L, which was compatible of pediatric stroke, mainly due to central with iron deficiency anemia and venous thrombosis, and some associated thalassemia trait. The coagulation with thrombocytosis. Prevention of acute profiles and autoimmune surveys were stroke among young people needs within normal limits. Neurosonography identification of such modifiable risk of carotid arteries were normal; the factors. transcranial cerebral duplex revealed Case Report A 37-year-old man, a high flow resistance index at the left driver of tourist bus, who overworked for middle cerebral artery, which more than 16 hours per day, presented to suggested left MCA distal M1 stenosis. the emergency department with Tracing his occupational history, he has

12 been a driver for 10 years, with habits of intravenous-intra-arterial thrombolytic tobacco smoking 1 pack per day for 15 therapy. years, and betel nut chewing 3 packs per day for 10 years. Background Although intravenous (IV) recombinant tissue plasminogen Conclusion Iron deficiency is a rare activator (rtPA) had proven efficacy for cause of young stroke. Chronic exposure to betel nuts and tobacco are traditional patients with acute ischemic stroke risk factors that increase the risk of (AIS), treatment failure may be cerebral arterial atherosclerosis through observed in patients with large vessels oxidative stress. High calcium oral intake from a great amount of betel nuts occlusions. Additional aggressive consumption may interfere iron therapeutic approaches like bridging absorption in the gastrointestinal tract. intravenous-intra-arterial thrombolytic Low oxygen delivery and possibly reactive thrombosis secondary to iron therapy after IV tPA may be suitable deficiency anemia may trigger the for such patients. premature onset of acute ischemic stroke. Iron supplement therapy with adequate Results Case 1 was a 45-year-old nutrition, and quitting tobacco and betel nuts are helpful for secondary stroke hypertensive man who presented with prevention. left hemiplegia for 80 minutes. The initial National Institute of Health 14 Stroke Scale (NIHSS) was 15 and the 銜接靜脈與動脈血栓溶解治療: 社區 patient received IV rtPA at 177 minutes 醫院與醫學中心的合作經驗 after onset. Due to deterioration of 李政翰 1 胡孟綺 2 謝鎮陽 2,3 陳志弘 4 1 成大醫院心臟內科 2 台南新樓醫院腦中風中 NIHSS to 18, he was transferred to a 3 心暨神經內科國立成功大學臨床藥學與藥物 medical center 2.2 km near-by and 科技研究所 4 成大醫院腦中風中心暨神經部 received intra-arterial thrombolytic Bridging Intravenous-intra-arterial therapy for an occlusion of proximal Thrombolytic Therapy: Experience of right middle cerebral artery. The Cooperation between Community NIHSS was 1 at discharge 8 days later Hospital and Medical Center Cheng-Han Lee1, Monica Hu2,Cheng-Yang and the modified Rankin scale at 3 Hsieh2,3, Chih-Hung Chen4 1 Division of Cardiology, Department of Internal months was 0. Case 2 was a Medicine, National Cheng Kung University 63-year-old man with hypertension, Hospital, Tainan, Taiwan 2 Stroke Center and Department of Neurology, diabetes, and old stroke, though his Tainan Sin Lau Hospital baseline activity was independent. He 3 Institute of Clinical Pharmacy and Pharmaceutical Sciences, National Cheng Kung presented to our ER due to right

University hemiplegia for 90 minutes. The initial 4 Stroke Center and Department of Neurology, National Cheng Kung University Hospital NIHSS was 14 and the clinical diagnosis was basilar artery occlusion. Objective To report two cases who He received IV tPA at 173 minutes received bridging

13 after onset but still deteriorated and was impression of acute ischemic stroke. intubated due to poor respiratory pattern. Her muscle power, linguistic function, We had recanalized his occluded basilar and muscle powers improved on the artery successfully at 420 minutes after second day. Her blood pressure, onset, but he remained comatous after glucose, lipid profiles were normal. that and expired 4 days later. She also had normal RPR, antinuclear Ab, homocysteine, and anti-cardiolipin Conclusion Our experience of bridging Ab results. Her first brain MRI scan intravenous-intra-arterial therapy was showed acute embolic infarct involving limited and the outcome of treatment was left MCA posterior parietal territory diverse. However, such multimodal and bilateral PCA territory occipital therapeutic approaches should still give and bilateral cerebellum regions. The benefit for carefully selected AIS MRA study showed no obvious patients. stenosis lesion. The echocardiogram showed no intracardiac thrombus or 15 vegetation. She was placed on 腦血管事件於一新診斷之胰臟癌患者 antiplatelet therapy after thrombolysis 1 2 周志和陳瑋芬 since the second day. 1 奇美醫院神經內科 2 台南新樓醫院神經內科 th On the 12 day, she had mild fever and Fatal cerebrovascular events in a her consciousness level and right patient with newly-diagnosed hemiplegia worsened. Brain CT shows pancreatic tail tumor: A case report 1 Department of Neurology, Chi Mei Medical new lesions at left temporal, left Center corona radiata, and right parietal 2 Department of neurology, Sin-Lau Hospital regions, and grade 2 hemorrhagic Background Among young stroke transformation in the previous cases, the proportion of uncommon and infarction. Her chest X ray shows th undetermined etiologies is significantly bilateral pneumonia. On the 14 day, higher. We reported a case with fatal abdominal untrasounography shows a cerebrovascular events associated with pancreatic tail tumor, and abdominal her concealed pancreatic tumor CT further indicated that malignancy invaded into left adrenal gland, and Case report The 44 year-old widowed splenic artery and vein, and multiple female was brought to ER due to acute metastatic lesion in the liver and right hemiplegia, linguistic dysfunction, stomach. Regional lymphadenopathy and dysarthria. Other physical was also noted. Her tumor markers, examinations findings were including CEA, CA-125, and CA 19-9 unremarkable. Acute intravenous were high. She received thrombolysis was performed under the broad-spectrum antibiotic treatments

14 but the response was poor. Biopsy was disorder, which requires prompt not done due to unstable vital signs and recognition and appropriate thrombocytopenia. Her consciousness intervention to prevent a devastating level, as well as her respiratory pattern, outcome. Intrasinus thrombolysis is an deteriorated in the following days, she alternative and effective method to was transferred to palliative team, and anticoagulant therapy for the treatment she died on the 21th day. of CVST, but is rarely used in Taiwan.

Discussion After Armand Trousseau’s Case Report A 46-year-old man description in 1865, the association presented with a one-week history of between cancer and hypercoagulation is intractable headache and progressive well recognized. The incidence of weakness of his right lower limb. The thromboembolism in patients with characteristic of increased of malignancy ranges from 1% to 11%. The intracranial pressure were obviously underlying mechanisms include tissue and magnetic resonance venography of factor, tumor-associated cysteine the brain confirmed a diagnosis of proteinase, oncogene activation, extensive venous sinus thrombosis. carcinomas mucins, and others. It is still The patient was successfully treated by uncertain whether anticoagulant or direct intrasinus thrombolysis with antiplatelet is the most optimal treatment urokinase with a dramatic relief of the for those cerebral infarction caused by headache and the neurologic deficits Trousseau’s syndrome. resolved gradually. No hemorrhagic transformation or other complication 16 occurred in this treatment strategy. The 嚴重性腦靜脈竇栓塞使用靜脈竇內血 patient was further received 栓溶解治療之案例報告 anticoagulants therapy and no 林文守高鴻文薛俊仁陳俊安國軍高雄總醫院神經內科三軍總醫院神經放 neurologic deficit occurred anymore, 射科三軍總醫院神經內科國防醫學院 though the brain magnetic resonance venography showed Intrasinus Thrombolysis for Severe transverse-sigmoid sinuses remained Cerebral Venous Sinus Thrombosis - A Case Report occluded, but restoration of the Wen-Sou Lin, MD1, Hung-Wen Kao, MD2, superior sagittal and straight sinuses. Chun-Jen Hsueh, MD2, Chung-An Chen, MD3 1 Department of Neurology, Kaohsiung Armed Forces General Hospital, 2Department of Conclusion This case highlights that Radiology, 3Department of Neurology, early IST can provide rapid 2,3 Tri-Service General Hospital, National symptomatic relief because the Defense Medical Center, Taipei, Taiwan. associated symptoms, such as headache, seizure, and intracranial Background Cerebral venous and sinus hypertension, are primarily caused by thrombosis (CVST) is a rare stroke sinus occlusion.

15 17 patients were divided into two 急性缺血性中風病患靜脈注射過多 subgroups: patients with rt-PA dose of t-PA 並未增加症狀性腦出血:一醫學中 less than 0.9mg/Kg and those greater 心經驗 than 0.9mg/Kg. SICH was defined as 林典佑 1,3 謝函潔 2,3 1 國立成功大學醫學院附設醫院神經部 any deterioration of NIHSS by NINDS 2 國立成功大學附設醫院斗六分院內科部 criteria. 神經內科 3 國立成功大學附設醫院中風中心 Results During the study period, 275 Above Standard t-PA Dose Did Not patients received rt-PA within 3 hours. Increase Symptomatic Intracerebral 80 patients (29.1%) received rt-PA Hemorrhage after Stroke Thrombolysis: Experience from a dose greater than 0.9mg/Kg and 3 Medical Center patients had SICH. 195 patients Tien-Yu Lin1, Han-Chieh Hsieh2 1 (70.9%) received tPA dose less than Department of Neurology, National Cheng Kung University Hospital 0.9mg/Kg, 9 patients had SICH. The 2 Division of Neurology, Department of Internal SICH rate was not significantly Medicine, Dou-Liou Branch, National Cheng Kung University Hospital different between the two groups 3 Stroke Center, National Cheng Kung University (3.8% versus 4.6%). Hospital

Background and purpose According to Conclusion The results of our study the results of NINDS trial, thrombolytic showed similar SICH rate whether the therapy with intravenous recombinant patients received rt-PA dose greater t-PA improves the outcome of acute than 0.9mg/Kg or not. To expedite the ischemic stroke onset within 3 hours. The rt-PA administration, using body recommended dose of rt-PA infusion is weight reported by patient or family is 0.9mg/Kg. However, body weight may a safe alternate if bed scale is not be over-reported by patient or family and available. the dose of rt-PA might be higher than 0.9mg/Kg. We aimed to know whether 18 overdosage of intravenous rt-PA may 血栓溶解劑治療用於住院中急性腦 increase symptomatic intracerebral 栓塞病人之成效林楙勛陳志弘 hemorrhage (SICH) rate or not. 國立成功大學附設醫院神經內科

Methods We retrospectively reviewed Thrombolytic therapy for in-hospital patients with acute ischemic stroke the medical records of all patients Mao-Hsun Lin , Chih-Hung Chen received IV rt-PA from NCKUH with a Department of Neurology, National Cheng Kung University Hospital diagnosis of acute ischemic stroke onset within 3 hours, from September 2006 to Background Intravenous tissue December 2012. According to rt-PA dose, plasminogen activator (IV-tPA) has

16 been used broadly in the treatment of p=0.441) after treatment with IV-tPA. acute ischemic strokes. Sometimes in-hospital patients are candidates for Conclusion Compared to out-of IV-tPA. We analyzed the efficiency of hospital patients, in-hospital stroke procedures before IV-tPA treatment and patients treated with IV-tPA were outcomes between in-hospital and out-of associated with less time delay before hospital stroke patients. receiving brain CT and starting IV-tPA, but there were no significant difference Methods Chart review for patients in outcomes. treated with IV-tPA during the period from September, 2006 to August, 2012 in 19 National Cheng Kung University 乙狀靜脈竇狹窄併搏動性耳鳴:個案 Hospital was done. The length of time 報告邱奕華 1 賴彥君 2 before receiving brain computed 亞東紀念醫院 1 內科部神經科 2 影像醫學部 tomography (CT) scan, the time to start Pulsatile tinnitus associated with IV-tPA, and stroke outcomes were Sigmoid sinus stenosis : A Case Report compared between in-hospital and Yih-Hwa Chiou1, Yen-Jun Lai2 out-of-hospital patients. 1 Section of Neurology, Department of Internal Medicine; Far Eastern Memorial Hospital 2 Department of Radiology; Far Eastern Results There were 220 patients Memorial Hospital enrolled in this study, including 10 in-hospital (IH) and 210 out-of hospital Introduction Pulsatile tinnitus is (OH) patients. The characteristics of usually due to a vascular anomaly patients between the two groups were around the skull base. Clinical and similar. Time delay to receive brain CT radiological work-up may reveal treatable causes, which can affect (IH 73.3±46.7, OH 21.2±12.2 minutes, cerebral arterial or venous p<0.01) and to start IV-tPA (IH 158.2± compartment. Understanding the 53.5, OH 69.3±23.3 minutes, p<0.01) of in-hospital patients were significant. physiopathologic mechanism Outcomes after strokes were defined by underlying the vascular anomaly is modified Rankin scale (mRS) in both one crucial for orienting management and month (mRS1) and three months (mRS3) treatment of this condition. Our after treatment of IV-tPA. There were no observations were made on a patient statistical differences in outcomes complaining of a high-pitched between in-hospital and out-of hospital unilateral pulsatile tinnitus associated patients in either one month (mRS1: IH with unilateral stenosis of sigmoid sinus. A single-stage endovascular 3.6±2.2, OH 2.7±2.0, p=0.978) or three procedure (sigmoid sinus stenting) was month (mRS3: IH 3.5±2.5, OH 2.4±2.1, carried out for treating the venous

17 anomaly, which relived the pulsatile 邱瑋婷陳龍台北醫學大學署立雙和醫院神經科 tinnitus successfully. Is intracranial aneurysm an absolute Case Report A 35-year-old woman, contraindication for r-TPA treatment in acute ischemic stroke? denied previous major systemic diseases, Wei-Ting Chiu, Lung Chan presented with a 2-year history of Department of Neurology, Taipei Medical University-Shuang Ho Hospital worsening right pulsatile tinnitus that had arisen gradually. Tinnitus was perceived Background According to the louder in a quiet place and disappeared current treatment guidelines for after compression of ipsilateral internal recombinant tissue-plasminogen jugular vein. Physical examination and activator (rt-pa), the presence of an neurological examination revealed intracranial aneurysm is a negative findings. She had normal contraindication to thrombolysis. otoscopic, tympanometric and Non-enhanced computed tomography audiometric evaluations. Auscultation of the brain is the only diagnostic test didn’t revealed any bruit or venous hum required before administration of rt-pa, over head and neck. Brain magnetic but it is difficult to detect cerebral resonance imaging with angiography and aneurysms before receiving venography (MRI with MRA and MRV) thrombolytic treatment. The safety of revealed right sigmoid sinus stenosis. A rt-pa in the setting of intracranial diagnostic digital subtraction aneurysms is unknown still. angiography (DSA) confirmed the stenosis over the right sigmoid sinus and Case Report 2 cases who had excluded dural arterio-venous fistula or incidental finding during followed up other vascular anomaly. Endovascular brain image or angiography after iv procedure with stenting for right sigmoid rt-pa treatment. Both of them had good sinus stenosis was performed smoothly, outcome without aneurysm rupture. A and the pulsatile tinnitus was relieved 53 years old man, suffered from immediately after this stenting. sudden onset of slurred speech with left side weakness and his condition Conclusion We report this case here improved after intravenous (iv) rt-pa and suggest that pulsatile tinnitus may be (NIHSS from 9 to 2). A 3mm caused by sigmoid sinus stenosis, and un-rupture saccular aneurysm over left endovascular procedure may relieve this A-com noted in his follow-up MRI. symptom successfully. Another 58 years old female, admitted with sudden onset of consciousness 20 change admitted to ER with NIHSS 16. 顱內血管瘤是否仍然是急性梗塞性腦I.V. r-tPA was prescribed but no 中風病患接受r-TPA治療的絕對禁忌?

18 improvement. MRI showed right internal candidate for thrombolytic therapy at carotid artery total occlusion, then the triage and thus the stroke code can intra-arterial recananlization was not be initiated properly. The aim of performed successfully and a 3mm this study is to evaluate the sensitivity saccular aneurysm was found over left of “code stroke” protocol in National ICA. Taiwan University Hospital. We especially focus on the patients who Conclusion In the general population, were misclassified as non-stroke or the prevalence of intracranial aneurysms stroke more than 3 hours but actually is 3 to 6 %. One report showed that 4 % fulfilled the criteria for the patients with intracranial aneurysms had thrombolytic therapy. symptomatic intracranial hemorrhage. The concern that these patients are at Methods A “code stroke” rapid increased risk of hemorrhage after access protocol was implemented at thrombolysis is still unwarranted. Should the National Taiwan University since CT or MR angiography is needed before July 2010. We analyzed patients who thrombolysis? Or rt-pa is safe in these were discharged from August 2010 to patients? We will need more evidence to August 2012 with the diagnosis of guide the treatment. ischemic stroke and arrived at our emergency department within 3 hrs 21 after symptoms onset. Data including 符合靜脈血栓溶解劑病患未即時啟動 demographics, initial presenting 急性腦中風流程的原因分析 symptoms, initial NIHSS, neuroimage 柯孋衿 1 湯頌君 2 鄭建興 2 1 亞東醫院神經內科 2 台大醫院神經部 findings and clinical outcomes (modified Rankin Scale) at 3 months Causes of Failed Identification for follow up was collected. possible candidate of IV-tPA in acute ischemic stroke patients at the initiation of stroke code Results During the study period, a 1 2 2 Li-Chin Ko , Sung-Chun Tang , Jiann-Shing Jeng total of 203 ischemic stroke patients 1 Division of Neurology, Department of Internal Medicine, Far Eastern Memorial Hospital, New had the final diagnoses as within 3 Taipei, Taiwan hours symptom onset and 161 (79.3 %) 2 Department of Neurology, National Taiwan University Hospital, Taipei, Taiwan subjects were initiated as “stroke code”. Of the 42 subjects not initiated as Background “Code stroke” is a stroke code at the triage, 4 still protocol to achieve rapid stroke received tPA treatment with the assessment and treatment. However, average of door to needle time of certain percentage of acute ischemic 93±24 minutes. Of the other 38 stroke patients may not be identified as a subjects, 8 subjects fulfilled the

19 guideline and recommended criteria of and 10%.The pathophysiology of IV-tPA therapy. 3 of them who had transient global amnesia is still un-determinated and venous ﬂow middle cerebral artery territory infarcts abnormalities have been suggested to with the presentation of conscious be associated with the pathophysiology disturbance or confusion, another 3 with of TGA. posterior circulation (brainstem, cerebellum) infarcts initially presenting Case Report A 73-year-old woman as dizziness, and vertigo, and the last 2 was admitted due to acute onset of subjects were partial middle cerebral quadriparesis, severe dysarthria and artery territory infarct. respiratory failure after arguing with

her family. Rapidly improvement of Conclusion Although the code stroke the neurological deficits evolved system may shorten in hospital within 48 hours. Her daily activities therapeutic delays, certain percentage of became independent, but obvious stroke patients suitable for thrombolytic antegrade amnesia was noted. This therapy may not be identified in triage amnesia persisted but got slowly based on our screening strategy, improved in 2 weeks. During this especially for patients presenting with attack, her brain magnetic resonance atypical stroke symptoms, such as images, biochemical, consciousness disturbance, dizziness, or electroencephalography and drugs vertigo examinations were all within normal

range except left internal jugular 22 venous reflux after valsalva maneuver. 左側內頸靜脈逆流病患所表現的反覆且長時間全腦性失憶發作 One year later, the similar prolonged 紀心怡 1 徐敏獻 1 黃華思 2 amnesia developed again and there was 彰化秀傳醫院神經科 no any clinical laboratory abnormality.

Case report : repeated prolonged global amnesia in a patient with left Conclusion internal jugular venous reflux Venous outflow resistance has been Hsin-I Chi, Min-Hsien Hsu, Hua- tzu Huang 1Department of Neurology, Show Chwan documented with several neurological Memorial Hospital diseases such as transient monocular 2Department of Radiology, Show Chwan Memorial Hospital blindness, normal pressure hydrocephalus, and chronic cerebral Background Transient global spinal venous insufficiency. In this amnesia(TGA) is characterized by a case report, we offered a possible sudden onset of an anterograde and connection between venous reflux retrograde amnesia that lasts up to 24 hours. The rate of annual recurrence of a disorders and transient global amnesia second or a third episode is between 6% with focal neurological deficits.

20 23 AIS patients potentially eligible for 如何減少急性缺血性腦中風病患接受 thrombolytic therapy, the ER physician 血栓溶解劑的等待時間：一個適合在 should complete the brain computed 社區醫院執行的處置流程 tomography as well as other laboratory 胡孟綺 1 謝鎮陽 1,2 陳滄山 1 1 台南新樓醫院腦中風中心暨神經內科 tests at the soonest, check the 2 國立成功大學臨床藥學與藥物科技研究所 contraindications of thrombolytic

therapy according to the patients’ past How to Reduce the Door-to-Needle Time for Acute Ischemic Stroke history, find the key person of the Patients: a New Treatment Algorithm patient who could sign the informed Suitable for Community Hospital consent, and let the patients and Monica Hu1, Cheng-Yang Hsieh1,2, Tsang-San Chen1 families watch a video (either 1Stroke Center and Department of Neurology, Tainan Sin Lau Hospital mandarin or Taiwanese version) 2Institute of Clinical Pharmacy and dictating the risk and benefit of Pharmaceutical Sciences, National Cheng Kung University, Tainan, Taiwan thrombolytic therapy for AIS. Those jobs should be done before the arrival Objective To present our new treatment of neurologists from home. In the part algorithm for acute ischemic stroke (AIS) 2, neurologist should confirm the patients who arrived within 3 hours of diagnosis of AIS, make the final onset and want to share our experience decision, and get the informed consent. with our neurologic colleagues in other community hospitals suffering from the Results There were four and three same difficult situation. AIS patients received thrombolytic therapy before and after the Background Compared with medical implementation of the new treatment centers with in-house neurologist algorithm, respectively. The stand-by, it’s a difficult job for door-to-needle time was 84.3±19.8 vs. community hospitals with 2-3 63.3±5.0 minutes (Mann-Whitney U neurologists to perform thrombolytic test, p=0.229). Of note is that 2 of the 3 therapy for patients with AIS. It’s even thrombolyzed AIS patients after harder to reduce the door-to-needle time implementation of the new algorithms below 60 minutes as the guideline came to our ER at night or holiday, i.e. suggested. neurologists should took about 10-20 minutes to arrive ER from their home. Methods We separate the preparation of thrombolytic therapy as two parts: the Conclusion Our new treatment part 1 is what emergency room (ER) algorithms reduced the door-to-needle physician can do and the part 2 is what time to nearly 60 minutes, though the neurologist can do. In the part 1, for there’s no statistical significance due to

21 small sample size. Our experience may (NIHSS score: 6) 30 days after the be shared with other neurologists who stroke. On 2012/04/17, we were asked to do thrombolytic therapy implemented a large tapered nitinol and reduce door-to-needle time in stent in his left proximal internal community hospitals. carotid artery. Hours after the procedure, hypotension (85/50 mmHg) 24 and sinus bradycardia (38/min) were 經頸動脈支架置入術後產生持續性低 found. Treatment with isotonic solution 血壓和心律過緩 and atropine injection were provided 1 胡智銘 1 洪國華 2 楊明祥馬偕紀念醫院台東分院 1 神經內科 2 放射科 initially. For persistent hypotension and bradycardia, dopamine infusion (5~12 Prolonged hypotension and ug/kg/min) and temporary pacemaker bradycardia after carotid artery were administered in subsequent 9 stenting Chih-ming Hu, Kuo-Hua Hung, Ming-Chiang days. His hemodynamic status was Yang Department of Neurology, Radiologist, Mackay stabilized after that. Memorial Hospital Taitung Branch Conclusion Reviewing the literatures, Background Hypotension and several studies indicated that there are bradycardia are well-recognized several features which predicted the complication after carotid artery stenting occurrence of hypotension or (CAS) and took the median time of 12 bradycardia after CAS. In this case, we hours (1-96 hours). We presented a case postulated that the calcified plaque and who suffered from prolonged a large stent may be the major factors hypotension and bradycardia after CAS which caused the prolonged that subsided in 9 days. hypotension and bradycardia after

CAS. Case report A 72-year-old male who is a heavy smoker with untreated 25 hypertension for years was admitted to 退化性關節炎為中風之風險因子 this hospital on 2012/03/15. He had right 徐敏獻葉宗勳 hemiplegia and mixed transcortical 彰化秀傳紀念醫院神經內科 aphasia (NIHSS score:21). Magnetic Whether Osteoarthritis Is A Stroke resonance imaging disclosed infarction Risk Factor ? A Population Based of the left superficial middle cerebral Cohort Study Min-Hsien Hsu MD, Chung-Hsin Yeh MD PhD artery territory. Severe stenosis with Department of Neurology, Show-Chwan calcified plaque in his left proximal Memorial Hospital, Chang-Hua City internal carotid artery was showed by Background Osteoarthritis (OA) computed tomography angiography. His features a chronic inflammatory state neurologic deficits improved gradually

22 that may have systemic impact on Conclusions Approximately 40% of atherosclerosis. In a population based stroke patients may have poorly retrospective cohort study, we aimed to defined stroke risk factors. Results examine whether osteoarthritis is a stroke from the present study suggest that risk factor. osteoarthritis (OA) is one of the modest but significant stroke risk Methods From a randomly sampled factors. cohort of one million health insurance subscribers clamed from the Taiwan 26 National Health Insurance, which 缺血性中風病患住院期間死亡之相 identified 18,453 patients with 關因素翁埈德宋昇峰吳啟順許永居蘇裕翔 osteoarthritis (ICD-9-CM code 715) 洪菱謙李建欣 between 1999 and 2007. The control 嘉義基督教醫院神經科 group was a randomly selected Factors related to in-hospital population of 36,906 matched with age, mortality in ischemic stroke patients sex. Subsequent occurrence of stroke was Cheung-Ter Ong, Sheng-Feng Sung, Chi-Shun Wu, Yung-Chu Hsu, Yu-Hsiang Su, Chen-Hsien measured from their index ambulatory Li, Ling-Chien Hung visit at least 5 years, and the association Department of Neurology, Ditmanson Medical Foundation, Chia-Yi Christian Hospital, between OA and the hazard of Chia-Yi Taiwan developing stroke was estimated using Cox proportional hazard models. Object To evaluate the prevalence of in-hospital mortality and to investigate Results Of the osteoarthritis cohort, the causes and risk factors of 1,331 (13.7/1,000 person-years) in-hospital mortality. experienced stroke during the follow-up period as compared to 2,219 (10.2/1,000 Background At present, in-hospital person-years) in the control cohort. The mortality in stroke patients still adjusted hazard ratios for all stroke was occurred in clinical practice. We need 1.18 (95% CI, 1.10 - 1.28), ischemic to investigate the risk factors of stroke 1.24 (95% CI, 1.15 - 1.34) and in-hospital mortality and to reduce hemorrhagic stroke 1.29 (95% CI, 1.08 - in-hospital mortality rate. 1.55) respectively by the Cox proportional hazard model with Methods The stroke included all of adjustment for age, gender and the patients who were admitted to comorbidities including hypertension, Chia-Yi diabetes, coronary arterial diseases, atrial Christian hospital between 2007-1-1 fibrillation and dyslipidemia. and 2011-12-31 and them was diagnosed as ischemic stroke. We excluded the patients whom were

23 diagnosed as transient ischemic attack or 27 vertebrobasilar artery insufficiency. 急性脊髓梗塞無法由核磁共振擴散影像顯示病灶：個案報告高伊慧湯頌君鄭建興 Results Between 2007-1-1 and 台大醫院神經部 2011-12-31 there were 2557 patients admitted to Chia-Yi Christian hospital Acute Spinal Cord Infarction Without Visible MR Diffusion and were diagnosed as ischemic stroke. Imaging Lesion: A Case Report There were 78 patients die in hospital. Yi-Hui Kao, Sung-Chun Tang, Jiann-Shing Jeng The in-hospital mortality rate is 3.07% Department of Neurology, National Taiwan (78/2557). After logistic regression University Hospital, Taipei, Taiwan. analysis, heart disease and stroke severity Background Spinal cord infarction was significant related to the risk of (SCI) is an uncommon disease in-hospital mortality. In comparison to characterized by acute motor and the patients without heart disease, the sensory deficits below the affected odds ratio of in-hospital mortality is 6. cord level. Diagnosis of acute SCI is The causes of in-hospital mortality were often difficult and heavily relied on the stroke in 48 patients (40 anterior spine MRI study. Here, we presented a circulations, 8 posterior circulations), case of SCI with initial negative spine hemorrhagic transformation in 4 patients, MRI, including diffusion-weighted respiratory failure in 8 patients, infection imaging (DWI), at hyperacute stage. with septic shock in 7 patients, acute myocardial infarction in 3 patients, Case report A case of 60-year-old multiple organ failure in 3 patients, other man with history of hypertension causes in 4 patients. visited our emergency room for acute

onset of chest pain and weakness of Discussion The in-hospital mortality bilateral hands. Initially, aortic rate (3.07%) is lower than previous dissection was suspected, but chest CT report in Taiwan, which the fatality rate showed diffuse arthrosclerosis of the of acute ischemic stroke is 6.7% (1984 aorta without evidence of dissection. -1986). Heart disease is a risk factor of Neurological examination showed in-hospital mortality in ischemic stroke weakness of bilateral wrists and hands, patients. The main cause of mortality is and thus acute cervical radiculopathies stroke itself. Whether the reduction in were impressed. However, spine MRI in-hospital mortality is related to performed at 4 hours after symptom thrombolytic therapy, need further onset showed no abnormality on both investigation. T2 weighted image and DWI. He had

bilateral leg weakness and numbness

over the legs and trunk below the T2

24 dermatome a few hours later. Lumbar complication of myocardial infarction puncture showed normal cell count, (MI). We presented a case of acute glucose and total protein. Autoimmune ischemic stroke (AIS) following acute and tumor marker screenings were all myocardial infarction (AMI) treated negative. Follow-up cervical cord MRI 4 with intravenous tissue plasminogen days later showed intramedullary activator (tPA). hyperintensity on T2WI with slight contrast enhancement at the C6-T1 level. Case report A 68-year-old single Increased DWI with matched decreased man was admitted due to AMI and AIS ADC was noted in the aforementioned (left sided weakness and slurred speech) regions, suggestive of SCI. He was treated with intravenous tPA injection treated with aspirin since the 2nd day, in a local hospital. Tracing his history, and his neurological recovery was well he was sent to the emergency with only mild four limbs weakness and department (ED) of a local hospital due numbness. He could ambulate without to dyspnea, chest tightness and assistance 2 months after discharge. near-syncope. Left sided weakness and slurred speech attacked at the ED No 註解 [CM1]: NIHSS?, lesion Conclusion Spine MRI, including DWI, intracranial hemorrhage (ICH) was vessel? could be negative in SCI patients at the found by computed tomography (CT) (Right pontine infarction hyperacute stage. Careful clinical of the brain and his National Institutes noted by MRI later, MRA: no evaluation especially repeated MRI exam of Health Stroke Scale (NIHSS) was 9. significant stenosis of VA / should be performed in patients with Then he received intravenous tPA BA). acute myelopathy and initially negative therapy (dosage for AIS, total 50mg, 註解 [CM2]: ? 50 mg image results. body weight 64 kg) under the impression of AIS. Left sided 28 weakness and chest tightness improved 靜脈注射血栓溶解劑治療急性心肌梗 partially after tPA injection. However, 塞伴隨急性缺血性腦中風: 一病歷報 high troponin-I was found and AMI 告 was confirmed then. Under the request 張偉倫洪翠虹葉宗勳 of the patient, he was transferred to our 彰化秀傳紀念醫院神經內科 ICU (intensive care unit). Acute Ischemic Stroke Following Acute Left sided weakness progressed on Myocardial Infarction Treated With the next day and brain CT showed right Intravenous Tissue Plasminogen Activator – A Case Report pontine infarction. Then we started Wei-Lun Chang, Tsui-Hung Hung, Chung-Hsin Yeh dual antiplatelet and enoxaparin Department of Neurology, Show-Chwan Memorial Hospital, Changhua, Taiwan therapy. Magnetic resonance imaging (MRI) of the brain revealed right Background Stroke is a severe pontine infarction without significant

25 stenosis of basilar or vertebral artery on pulmonary complications such as the third day after admission. Dyspnea aspiration pneumonia (AP) and choking each year in the United States. and pulmonary edema persisted under This study aimed to determine the medical treatment so he received frequency of reporting of AP or percutaneous coronary intervention (PCI) choking as a cause of death (COD) on 註解 [CM3]: How long after 10 days after admission. Stenting for death certificates with mention of AIS? Which vessels was right coronary artery (RCA) and plain stroke in the US. occluded (RCA and LCX)? old balloon angioplasty (POBA) for left Heparin use after PCI (No)? circumflex artery (LCX) was done. His Methods We used the multiple COD Brain CT after AIS (right symptoms improved after PCI; hence he database of CDC WONDER for the pontine infarction) and before discharged under a stable condition later. years 2001–2009 to identify death PCI (no)? certificates with mention of stroke Discussion MI in recent three months (ICD-10 code I60-I69), AP (ICD-10 is a contraindication for tPA code J69) and choking (ICD-10 code administration in AIS. Similarly, history W78-W80) for those aged 65 years or of ischemic stroke within the preceding above for analysis. three months is a contraindication for tPA administration in AMI but an important Results Of 1 916 882 death exception is AIS seen within three hours certificates for those aged 65 years or which may be treated with thrombolytic above with mention of stroke in the US therapy. The dosage of tPA is different between 2001 and 2009, 5.7 % (n=109 between AMI and AIS. Further guideline 038) reported AP as a COD and 1.7 % is needed for the usage of tPA in the (n=31 605) reported choking as a COD. setting of two diseases attack The frequency of reporting of AP as a concurrently. COD was highest in Hawaii (9.6%) and lowest in New York (3.5%). The 29 frequency of reporting of choking as a 老年中風病人以吸入性肺炎或嗆入為 COD was highest in Vermont (3.5%) 死因的報告(美國) and lowest in Maryland (0.6%). The 張嘉祐 1 鄭天浚 1 呂宗學 2 1 奇美醫學中心神經內科 odds ratio of reporting AP or choking 2 國立成功大學醫學院公衛所 as a COD decreased from 2001 to 2009,

was higher among male deceased, and Reporting of Aspiration Pneumonia or Choking as a Cause of Death in increased with age of the deceased. Elderly Patients Who Died with Stroke Chia-Yu Chang, MD, MS; Tain-Junn Cheng, MD, PhD; Tsung-Hsueh Lu, MD, MPH Conclusions On average, around 12000 elderly patients with stroke died Background and purpose Little is from AP and 3500 elderly patients with known regarding how many patients who stroke died from choking per year in have had a stroke die from fatal

26 the US during the past decade. Efforts are ultrasonography. Cardiac autonomic needed to reduce the number of deaths activities were represented by the from these two preventable COD. measurement of baroreflex sensitivity (BRS). All parameters were followed 30 at baseline, 1, 3, 6 and 12 months after 頸動脈支架置放術前後之腦血流動力 CAS. 學及感壓反射的變化許立奇 1,3 張豐基 2,3 郭博昭 4 翁文章 1,3 胡漢華 1,3 Results The MBFV and PI of 1 台北榮民總醫院神經醫學中心 ipsilateral MCA were significantly 2 放射線科 lower than those of the contralateral 3 國立陽明大學醫學系 4 國立陽明大學腦科所 side preoperatively. Postoperatively, there was a significant increase of Cerebral Hemodynamic and MBFV in the ipsilateral MCA till 2 Baroreflex Sensitivity after Carotid Artery Stenting weeks after stenting and then gradually Li-Chi Hsu1, 3, Feng-Chi Chang2, 3, Terry BJ Kuo4, decreased but remained stable 1 year Wen-Jang Wong1, 3, Han- Hwa Hu1, 3, 1 Department of Neurology after CAS. A non-significant increase 2 Department of Radiology, Taipei Veterans of MBFV in the contralateral MCA General Hospital 3 National Yang-Ming University School of was also noted after CAS. In contrast, Medicine the values of BRS decreased 4 Institute of Brain Science, National Yang-Ming University, Taipei, Taiwan significantly 1 month after stenting and returned to baseline levels six months Objectives The long-term after CAS. hemodynamic effects of carotid angioplasty and stenting (CAS) remain Conclusions CAS resulted in unclear. Therefore, we sought to improved global cerebral investigate longitudinally the alterations hemodynamic status. In contrast, BRS in cerebral hemodynamics in patients did not normalize but only returned to undergoing CAS. its baseline value 6 months after

stenting. Materials and Methods A total of sixty-three symptomatic patients (all 31 male, 19 had transient ischemic attack 同時存在於顱內硬膜之多發性動靜 and 44, minor stroke; mean age: 77.3±6.3 脈廔管及脊髓之動靜脈血管瘤：案例 years, range: 51-86) were studied 報導 prospectively. The mean blood flow 連家儀，許哲維，陳尚德高雄長庚紀念醫院神經內科 velocities (MBFV) and pulsatility index (PI) of bilateral middle cerebral arteries Concomitant intracranial dural (MCA) were evaluated by transcranial arteriorvenous fistulas and spinal arteriorvenous malformation: report color-coded Doppler (TCCD)

27 of one case revealed diffuse interventricular Chia-Yi Lien, Wei-Che Hsu, Shang-Te Chen Department of Neurology, Chang Gung Memorial hemorrhage. Heparin was discontinued Hospital, Kaohsiung Medical Center and emergent surgery with bilateral external ventricular drain placement Background Arteriorvenous fistulas for hydrocephalus was done at the (AVFs) are characterized by abnormal same day. Comatose status persisted shunting of blood between the arterial and patient died 5 days after surgery. system and the venous system, without the presence of a normal intervening Conclusion Concomitant existence capillary bed. However, concomitant of cranial dural arteriovenous fistulas intracranial dural arteriorvenous fistulas (DAVFs) and spinal AVM is (DAVF) and spinal arteriorvenous uncommon, and DAVF may malformation (AVM) is rarely reported in masquerade as cerebral venous the literature. thrombosis. Thus, the angiography should be performed for the precise Case Report A 57-year-old man was diagnosis for the vascular admitted due to progressive cognitive abnormalities and for the early deterioration for one month. He has past multidisciplinary management of the history of intracerebral hemorrhage and DAVF. seizure, diagnosed in 2004. After admission, initial brain MRI revealed 32 multiple small hemorrhage over bilateral 慢性腎疾對中風病患接受血栓溶解 thalami and basal ganglia. However, 治療預後的影響 during hospitalization, he was found to 陳志昊湯頌君鄭建興國立台灣大學醫學院附設醫院神經部 be disoriented, and had urine incontinence. Because brain MRV Impact of Chronic Kidney Disease revealed left carotico-cavernous sinus on Outcome of Ischemic Stroke Patients Receiving Intravenous fistula, and deep vein thrombosis over Thrombolysis distal straight sinus, heparin was given. Chih-Hao Chen, Sung-Chun Tang, Jiann-Shing Jeng Conventional angiography showed (1) Department of Neurology, National Taiwan dural arterio-venous fistula over midline University Hospital, Taipei, Taiwan suboccipital area (2) left Background and Purpose Patients carotico-cavernous sinus fistula and (3) with chronic kidney disease (CKD) are high cervical arterio-venous known to have abnormalities in malformation; thus, Onyx embolization coagulation and platelet function. It to feeding artery was performed. Three remains uncertain whether impaired days after embolization, acute renal function may refer to poor deterioration of consciousness was noted outcome in acute ischemic stroke (E1-2VeM2), and emergent brain CT

28 patients receiving thrombolytic therapy. than those with eGFR ≥45. The Previous study showed that estimated proportion of unfavorable outcome was glomerular filtration rate (eGFR) <60 significantly higher in patients with ml/min/1.73m2 was not a poor outcome eGFR <45 (78.3% vs. 60.8%, P = indicator in recombinant tissue 0.015). After adjustment of age and sex, plasminogen activator (rt-PA) treated eGFR <45 showed a trend toward stroke patients. As demonstrated by a unfavorable functional outcome (odds large population-based study, the graded ratio 2.13, 95% confidence intervals risk of cardiovascular events and 0.95–4.74, P = 0.065). mortality rose sharply for subjects with an eGFR <45 ml/min/m2. This study Conclusion Although not statistically aimed to evaluate if moderate renal significant, acute stroke patients with function impairment (eGFR <45) is a moderately impaired renal function significant outcome predictor in rt-PA may tend to have unfavorable outcome treated patients. after intravenous thrombolysis. Further study with larger sample size is Methods We reviewed consecutive potentially indicated to verify our patients who received intravenous rt-PA preliminary result. for acute ischemic stroke during the period of January, 2006 and September, 33 2012 from the National Taiwan 頸脊髓合併雙側小腦梗塞：病例報告 University Hospital Stroke Registry data. 陳泓儒黃志善 An unfavorable outcome was defined as 臺北醫學大學市立萬芳醫院神經內科 modified Rankin scale >1 at 3 months after stroke onset. The association Combined cervical cord and between renal function and outcome at 3 bilateral cerebellar infarction: A months after stroke was evaluated, as Case Report well as any difference of hemorrhagic Hung-Ju Chen, Chih-Shan Huang Department of Neurology, Wan Fang Hospital, transformation after rt-PA administration Taipei Medical University between CKD and non-CKD patients. Background Spinal cord infarction Results Of 265 included patients, 159 (SCI) is uncommon, accounting for (60%) were male, the mean age was 68.4 1.2% of all strokes, but the outcome ± 12.4 years, and 47 (17.4%) patients had may be more disabling. Combined eGFR <45. Patients with eGFR <45 were cerebellar and spinal ischemic stroke is older, higher frequencies of hypertension, even rarer. Diffusion-weighted diabetes mellitus, hyperlipidemia, magnetic resonance imaging coronary artery disease, and old stroke (DW-MRI) is potentially a useful and

29 feasible technique for the early detection abnormalities including aortic of spinal cord infarction. We report a dissection. Autonomic dysfunction patient with combined cervical spinal associated with spinal cord infarction cord and bilateral cerebellar infarction. was impressed.

Case Report A 49-year-old man Conclusion The lesions of spinal presented with sudden onset of cord in our case could be confirmed as quadriparesis and urinary retention with acute infarction based on the preceding back pain after he lifted heavy simultaneous involvement of bilateral objects. Hypoesthesia below the C8 level, cerebellum on DW-MRI. In spinal cord impaired proprioception and hyporeflexia infarctions, brain involvement might were also found. Cervical spine MRI be considered especially the territory showed longitudinal high signal intensity of posterior circulation. The at C3-C7 and ‘owl’s eyes’ sign on relationship and etiology need further T2-weighted image, suggesting spinal investigations. cord infarction. DWI showed hyperintense signals at C3-C7 and 34 bilateral cerebellar hemisphere which 以單側週邊型顏面神經麻痺為表徵 were compatible with recent infarction. 之橋腦梗塞─個案報告陳建志劉崇祥蔡崇豪 Extracranial carotid duplex sonography 中國醫藥大學附設醫院神經科部 revealed no abnormal findings and the 24-hour Holter electrocardiogram (ECG) A Case Report of Pontine Infarction reported no significant arrhythmia such Presenting with Unilateral Peripheral-type Facial Palsy as atrial fibrillation. Echocardiography Jiann-Jy Chen, Chung-Hsiang Liu, Chon-Haw showed no evidence of intramural Tsai Department of Neurology, China Medical thrombus. He had normal level of protein University Hospital, Taichung, C, protein S, anti-thrombin III, homocysteine and erythrocyte Background 造成週邊型顏面神經 sedimentation rate. He had negative 麻痺最常見的原因為貝氏麻痺 results for rheumatoid factor, anti-nuclear (54%)，其次為耳性帶狀疱疹 antibody, anti-cardiolipin antibody, (Ramsay-Hunt 症候群)(20%)、外傷性 anti-phospholipid antibody, and venereal 麻痹(12%)或其他(4%)。以腦幹中風 disease research laboratory test. The 為表徵者實屬罕見，特提出報告。 patient received aspirin for secondary stroke prevention. During hospitalization, Case Report 71 歲老年男性，因突發 hypotension with excessive sweating had 左側顏面神經麻痺至本院急診，合併 been noted. Computed tomography 左側突發性耳聾、眩暈及寬大步伐。 angiography (CTA) showed no vascular 理學檢查顯示左側額頭抬頭紋消

30 失，眼皮無法閉攏，鼻唇溝淺化，嘴角 spontaneous. We present a case of 無法上揚，臉頰更無法呈現笑紋，應為 delayed trauma-related internal carotid dissection with special pattern of 左側週邊型顏面神經麻痺(House- language recovery. Brackmann 分級第 4 級)。腦部磁振造影顯示左側下橋腦及小腦下半月葉之急 Case Report A healthy 38-year-old 性梗塞。美國國家衛生研究院中風評量 right handed single man, has 18 years 表(NIHSS)評為 4 分，由於本院「症狀 of education, was brought to ED after a 輕微」之定義為 NIHSS < 6，本個案暫 traffic accident. He majored in English 不須接受靜脈注射組織胞漿素原活化 in college and has been worked as a 劑，僅須接受點滴輸液及抗血小板劑 manager in standard foods company. aspirin 治療，並同時保護眼睛，給予局 No loss of consciousness or focal 部復健及針灸治療。爾後 1 週，左側聽 neurological signs were noted. He was 力障礙及顏面神經麻痺並無恢復，但眩 discharged four hours later. He was 暈緩解，遂於狀況穩定下出院。往後追 admitted to our hospital the next day 蹤 4 個月，左側聽力障礙及顏面神經麻 for management of left facial bone 痺依舊。 fracture. He developed acute global aphasia and weakness of right side Conclusion 橋腦梗塞僅以週邊型顏 limbs 17 hours after trauma. NIHSS 面神經麻痺為表徵者很罕見，目前僅有 score was eighteen and left MCA 個案報告而已。本個案之單側週邊型顏 infarct was impressed. Brain CT 面神經麻痺同時合併了同側突發性耳 showed left dense middle cerebral 聾、眩暈及小腦徵兆，不應視為常見之 artery (MCA) sign and loss of 貝氏麻痺，應考慮腦幹病變。 lentiform nucleus sign 3.5 hours after stroke. Brain CTA revealed left internal 35 carotid artery (ICA) total occlusion at 延遲外傷性內頸動脈剝離導致中左大 proximal portion 8 hrs after stroke. 腦動脈中風臨床失語症恢復之特殊變 Absent flow of left ICA, decreased 化：病例報告 mean velocity with low pulsatility 陳桂銓葉旭霖吳亞縈葉建宏邱浩彰新光吳火獅紀念醫院神經科 index of left MCA and focal stenotic flow of left MCA were found on Delayed traumatic internal carotid carotid duplexes twenty-two hours artery dissection leads to middle cerebral artery infarct with special after stroke. B-mode of extracranial pattern of language recovery: A case duplex revealed intimal flap and report hypoechogenic vessel wall in left ICA. Guei-Chiuan Chen, Hsu-Ling Yeh, Ya-Ying Wu, Jiann-Horng Yeh, Hou-Chang Chiu ICA dissection was highly suspected. Departments of Neurology, Shin Kong Wu Ho-Su Brain MRA revealed dissection of left Memorial Hospital, Taipei proximal ICA two days after stroke. He Background The most possible could pronounce single sounds three etiology of carotid artery dissection was days after stroke. Gradual

31 improvement of muscle power in right Background Aneurysm of the side limbs was also found. He started to extracranial internal artery is rare, with say single Chinese words five days after incidence of 0.8-1% of all aneurysms. stroke. He was able to walk Embolic stroke is one of the independently around ten to fourteen complications. We present a case with days after stroke. Three weeks after severe ischemic stroke combined with stroke, he could repeat a whole sentence a giant aneurysm of extracranial and comprehend partially, but still poor internal carotid artery. There was good in using English. Two months after recovery after angioplastic surgery. stroke, he still have some difficulty in simple communication, though he was Case Report A 73 year-old married able to give longer sentences. Follow-up man was admitted due to sudden onset brain MRA revealed mild recanalization of left hemiplegia. In the morning, he twenty days after stroke. Brain duplexes didn’t get up as usual. His wife found revealed no intimal flap and recovery of him on the bed. He was then sent to left ICA dissection forty-seven days after hospital. There were flaccid weakness stroke. of left upper and lower limbs and forced right deviation of both eyes. Conclusion We present this relatively Initial brain computed tomography (CT) delayed onset of traumatic ICA dissection with special pattern of showed normal findings. One day later, language recovery. The ICA dissection magnetic resonance imaging (MRI) of was conformed by brain MRI and carotid brain showed acute brain infarction duplex. Discrepancies existed in mother language and second language. over right middle cerebral artery territory (MCA). Echocardiography 36 and electrocardiography showed 成功地以手術治療巨大的顱外內頸動 normal results. He had hypertension 脈動脈瘤併發大範圍的缺血性腦中風 without regular medication. He didn’t 1 2 1 1 1 陳崇文吳清文陳淑儀楊自強許弘毅 have diabetes mellitus, hyperlipidemia, 1 童綜合醫院神經內科 2 童綜合醫院心臟血管外科 or smoke habit. Carotid color duplex (CCD) showed a large thrombotic Giant aneurysm of the extracranial internal carotid artery presenting with plaque-like lesion of right internal ischemic hemispheric stroke carotid artery (ICA), causing severe successfully treated by surgery stenosis. 256-sliced CT angiography Chung-Wen Chen1, Wu Cing-wen2, Shu-Yi Chen1 , Chi-Chiang Yang1, Hung-Yi Hsu1 (CTA) revealed a giant aneurysm of 1 Section of Neurology, Department of Internal right ICA and multiple hemorrhagic Medicine, Tung’s Taichung MetroHarbor Hospital infarcts of right MCA with mass effect. 2 Department of cardiac surgery, Tung’s Taichung On the day of CTA, he became MetroHarbor Hospital drowsier. One week later, after high

32 intracranial pressure was under control, This study prospectively assessed the excision of aneurysm and endarterectomy efficacy and feasibility of applying 10 were performed. After surgery, his repetitions of HBOT at 2.0 ATA for one condition got better. He could sit in the hour in patients with mild acute wheelchair, with left hemiplegia, and talk ischemic stroke within 3-5 days after as before, continuing physical therapy 2 stroke onset to reinvestigate the role of months later. Repeated CCD showed HBOT in treating acute ischemic patent and smooth lumen of right ICA. stroke.

Conclusion Aneurysm of internal Methods The HBOT group (n = 16) carotid artery related embolic stroke is a received conventional medical rare condition. This case received surgery treatment with 10 sessions of in the subacute stage of stroke. The adjunctive HBOT within 3–5 days after prognosis was good. CTA is important in stroke onset, while the control group (n patient with stroke to make correct = 30) received the same treatment but diagnosis and early treatment. To get rid without HBOT. Early (around two of cause of stroke, such as surgery for weeks after onset) and late (one month aneurysm, is mandatory. after onset) outcomes (National Institutes of Health Stroke Scale, 37 NIHSS scores) and efficacy (changes 重覆性高壓氧治療對急性腦中風的前 of NIHSS scores) of HBOT were 驅研究 evaluated. 陳紹原王文奇王國慶陳昭境陳志豪劉議謙葉炳強財團法人天主教耕莘醫院神經內科高壓氧科; Results The baseline clinical 天主教輔仁大學醫學系 characteristics were similar in both

Effects of Repetitive Hyperbaric groups. Both early and late outcomes Oxygen Treatment in Patients with of the HBOT group showed significant Acute Cerebral Infarction: A Pilot difference (P ≤ 0.001). In the control Study Shao-Yuan Chen1, 2, 3 ,Vinchi Wang1, 3 , group, there was only significant 1 1, 3 Kaw-Chen Wang ,Chao-Ching Chen difference in early outcome (P = 0.004). Chih-Hao Chen1 ,Yi-Chien Liu1 , Ping-Keung Yip1,3 For early efficacy, there was no 1 Department of Neurology, Cardinal Tein Hospital, New Taipei City, Taiwan difference when comparing changes of 2 Department of Hyperbaric Medicine, Cardinal NIHSS Tein Hospital, New Taipei City, Taiwan 3 School of Medicine, Fu Jen Catholic University, scores between the two groups (P = New Taipei City, Taiwan 0.140) but there was statistically significant difference when comparing Objective The role of hyperbaric changes of NIHSS scores at one month oxygen therapy (HBOT) in the treatment (P ≤ 0.001). of acute ischemic stroke is controversial.

33 Conclusion The HBO therapy used in cortex of left parietal lobe. On this study may be effective for patients admission, we checked laboratory data with acute ischemic stroke and is a safe including lipid profile, thyroid and harmless adjunctive treatment. function , HbA1c, homocysteine, autoimmune profile, function of 38 coagulation and antiphospholipid 案例報導:蛋白 S 缺乏症與中風之懷孕 antibody. All above finding are normal. 婦女 24hrs Holtor’s scan showed sinus 陳惠萱 1 蔡銘駿 1 中國醫藥大學附設醫院神經部 1 rhythm with isolated atrial ectopy. Transcranial Doppler showed Case Report : Protein S deficiency and increased flow velocity of left middle pregnant woman with ischemic stroke Hui Hsuan Chen1 ,Ming-Jun Tsai1 cerebral artery. Although low protein S 1 Department of Neurology, China Medical (52.2%) was detected, anticoagulation University Hospital, Taichung, Taiwan was not used because of first trimester Background Pregnancy women are at of pregnancy. We prescribed increased risk of thromboembolic disease. clopidogrel 75mg once daily for stroke We reported a case of pregnant woman prevention. Two weeks after discharge, with ischemic stroke that is associated the patient received artificial abortion with protein S deficiency. due to severe hyperemesis. Two weeks after abortion, protein S was back to Case Report This 33-year-old previous normal range (92.2 %). No recurrent healthy pregnant (G3 P1 AA1) woman stroke or other complication was suffered from sudden onset of disturbed detected three months after stroke. consciousness and right side weakness at the gestational age of 9 weeks. Discussion Stroke occurring during Severe hyperemesis was noted during pregnancy is a rare condition. Pregnant first trimester of this pregnancy. On the women have increased risk of strok. day of admission, she suffered from Previous case series studies showed sudden onset of consciousness protein S deficiency resulted in disturbance in the afternoon which hypercoagulability which primarily persisted for minutes. Mild right side affects veins and rarely causes arterial weakness and little spontaneous speech thrombosis. Whereas, many were found. On examination, neurologic meta-analysis still showed protein S examination showed decreased muscle deficiency appears to be associated power on right extremities (4 point) and with arterial thrombosis in the brain. motor aphasia. Brain MRI (DWI) We suggested clopidogrel may be safe showed lesions with increased intensity in the first trimester of pregnancy and in left insular cortex and small area of protein S deficiency during pregnancy

34 can be corrected by abortion, whereas the her TCD findings. The TCD showed role of abortion in protein S deficiency increasing mean velocity with during pregnancy is still undetermined turbulent flow over multiple segmental and further investigation may be needed. vessels including bilateral ACAs, MCAs, Left PCA and basilar artery. 39 After Nimodipine treatment, her 顱內血管超音波系列追蹤在可逆性腦 headache was gradually subsided. We 血管收縮症候群的應用 followed her TCD 4weeks and 8 weeks 陳貴津吳毓嘉劉雅婷林秀芬高雄醫學大學附設中和紀念醫院神經內科 after disease attack. The serial TCD revealed gradually normalized flow in Serial Transcranial Color Doppler all cerebral arteries. Evaluation in Reversible Cerebral Vasoconstriction Syndrome Kuei-Chin Chen, Yu-Chia Wu, Ya-Ting Liou, Conclusion TCD serial changes of Hsiu-Fen Lin Department of Neurology, Kaohsiung Medical the present patient highlighted the University Chung-Ho Memorial Hospital non-invasive follow up method would

enable us to monitor the degree of Background Reversible cerebral vasoconstriction in RCVS. vasoconstriction syndrome (RCVS) is a rare disorder characterized by 40 thunderclap headaches with reversible 可逆性腦血管收縮症候群合併頭痛 vasoconstriction of cerebral arteries. 及聽力損失-病例報告 1 2 3 1 Follow up of reversible segmental 陳嘉偉莊俊義沈昭諭蔡世傑呂聰明 1 1 1 1 周希諴陳秋媚孔勝琳 cerebral vasoconstriction is usually 中山醫學大學附設醫院神經科 1 耳鼻喉科 2 performed by cerebral angiography, but 醫學影像科 3 using non-invasive transcranial Doppler Reversible cerebral vasoconstriction (TCD) to monitor vasoconstriction could syndrome presents with thunderclap be a better tool. headache and sudden hearing loss: A Case Report Chia-Wei Chen1, Chun-Yi Chuang2, Chao-Yu 3 1 Case Report A 58-year-old female was Shen , Shih-Jei Tsai , Tsong-ming 1 1 1 Leu ,Hsi-hsien Chou ,Chiu-mei Chen , admitted due to acute onset episodic 1 Sheng-Ling Kung thunderclap headaches without other Department of Neurology1, Otolaryngology2, 3 accompanying clinical features. Diagnostic Radiology , Chung Shan Medical University Hospital Computed tomography (CT) revealed subarachnoid blood along bilateral Introduction Reversible cerebral cerebral sulci. Computed tomography vasoconstriction syndrome is angiography (CTA) work-up for characterized by recurrent episodes of aneurysm was negative but cerebral thunderclap headache and by reversible angiography had evidence of multiple vasoconstriction which typically segmental stenosis which matched with affects young women leaving minimal

35 or no neurologic deficits. It is nimodipine (a calcium channel blocker, characterized by multifocal narrowing of used as a vasodilator) administration the cerebral arteries that resolves over and she was discharged with oral days to weeks. Diagnosis can be nimodipine 60mg 4 times per day. The hampered by the dynamic nature of following contrast-enhanced brain clinico-radiological features. Stroke can MRI scan in 1 month and 5 months occur a few days after initial normal later demonstrated remission of the imaging, and cerebral vasoconstriction is vascular lesions. at a maximum on angiograms 2-3 weeks after clinical onset. Conclusion Acute hearing impairment is seldom reported in RCV Case presentation This is a healthy cases. In our case, the clinical 45-year-old female without the history of presentation of dramatic remission of systemic disease or headache. She both hearing impairment and presented with the complaint about thunderclap headache resulting from abruot onset of severe headache in the RCVS after nimodipine therapy may right frontal region associated with suggest these two diseases shared tinnitus in the right ear for 5 days. Pure common pathogenesis mechanism. tone audiometry disclosed right ear low tone sensorio-neural hearing loss. Except 41 hearing loss, patient presented no other 2012 年台灣世界中風日活動後民眾 neurologic deficits on examination. 的腦中風知能探討報告陳龍 1 施淑芳 2 Serial image studies included brain CT 1 台北醫學大學署立雙和醫院神經內科 and MRI with contrast enhancement 2 國立台灣師範大學健康促進與衛生教育學 indicated suspect focal stenosis at the 系

M1-M2 junction of left MCA and the V4 Stroke literacy after the World segment of the left vertebral artery. Stroke Day 2012 Lung Chan1, Shu-Fang Shih2 Laboratory workups including blood cell 1Department of Neurology, Taipei Medical count, chemistry, and CSF studies University-Shuang Ho Hospital 2Department of Health Promotion and Health yielded no evidence of CNS infection, Education, National Taiwan Normal hemorrhage or inflammatory course. Six University. days after the onset of symptoms, Background Stroke is a preventable intracranial angiography was performed and treatable disease, by controlling and it disclosed multiple segmental the risk factors we can reduce the stenosis of the intracranial arteries incidence of stroke. The World involving bilateral anterior and posterior Stroke Day (WSD) proclamation was circulation. The hearing impairment first initiated in the 4th World Stroke dramatically improved after intravenous Congress in Vancouver by the World

36 Stroke Organization (WSO), and was have the questionnaire. Only 57.89% announced in 2006 in the 5th World attendances could successfully told that Stroke Congress in Cape Town in 29 the brain was the target-damaged organ October. The objective of the WSD is to in stroke and better (64%) in the motivate people, health workers, policy post-test. Ischemic stroke (85.94%) makers, governments and aid agencies to was the most common stroke type and take action against stroke. Health hypertension was the most common literacy is the degree to which risk factor recognized by the individuals have the capacity to obtain, attendances, followed by process, and understand basic health hyperlipidemia, over-weight, diabetes, information and services needed to make and smoking. Three most common appropriate health decisions. A good recognized stroke symptoms are limb stroke literacy including awareness of weakness, slurred speech, facial stroke risk factors, symptoms and weakness, and followed by unsteady treatment, can correctly identified that gait and numbness. Most of the brain is the organ damaged in stroke, and attendances knew that they should seek would have a promptly response (call for medical help and call 119 if stroke emergency service) during stroke attack. happened. For the main themes, With adequate stroke literacy, “Golden hours 123” and “FAST”, only pre-hospital delayed may be reduced, and 56.16% and 19.76% respectively had increased the opportunity to receive heard the slogan before the WSD thrombolytic therapy. carnival. Only half of the attendances Aim (50.44%) knew that the thrombolytic We would like to analysis the basic therapy is the treatment choice of knowledge of stroke in people attended stroke. 58.06% and 40.32% the WSD Taiwan carnival, to evaluate attendances felt partially and their level of stroke literacy, and their completely matched their expectation expectation of the carnival. respectively. Almost 72% of the attendances would like to join the Method A short questionnaire was WSD carnival in 2013. Good stroke conduction during the WSD Taiwan literacy if people can correctly: 1. carnival in October 27, 2012 in the New Recognize brain is the target damage Taipei County Citizen Plaza by our organ; 2. Recognize at least 5 stroke trained interviewers. Both stroke risk factors; 3. Recognize more than 5 specialists and public health professions stroke symptoms, 4. An appropriate were invited to review the questionnaire. response when stroke happened, call Results 119 immediately11. Around 350 people, Total 350 attendances were invited to only 20 of them had a good stroke

37 literacy that are mostly women, younger Background Delayed inflammatory than 65 years old, and received at least mechanisms intrinsic to the damaged high school education. brain tissue as well as those from the circulating blood are the critical Conclusion The overall performance of mediators causing cell death, the WSD carnival is barely satisfaction. angiogenesis or cell regeneration after The main themes of WSD carnival stroke. Activation of hyaluronic acid setting were failed to meet the need of (HA) and associated enzyme synthesis the attendance. In the future carnival had been shown in experimental planning, need assessment should be studies. In our study, we investigated conducted before the plan. The design the plasma levels of HA in acute stroke of the booths should be based on the patients at two different time points as result of the survey. Advertisements <48 hours (h) and 48-72 h after stroke should be conducted in a more efficient onset and their associations with way before the carnival, by the mass functional outcome. medium and Internet. Post-carnival survey should be carried out as a routine Subject and method This is a process to evaluate the performance of multi-centre study. Patients with acute the WSD carnival. stroke and age and sex matched controlled subjects were recruited. 42 Clinical information including stroke 急性中風病患的血中玻尿酸濃度與功 subtypes, and 3 months functional 能性預後的相關性 outcome, defined as modified Rankin 湯頌君 1 彭家勛 2 胡朝榮 3 邱弘毅 4 鄭建興 1 1 台大醫院腦中風中心暨神經部 scale (mRS), was collected. Plasma 2 三軍總醫院神經部 levels of HA were determined via 3 台北醫學大學雙和醫院附設神經部 standard ELISA method. 4 台北醫學大學公共衛生學院

Association of Plasma Levels of Results There were 159 non-stroke Hyaluronic Acid and Functional controls and 206 stroke patients Outcome in Acute Stroke Patients Sung-Chun Tang1, Giia-Sheun Peng2, Chaur-Jong including 43 intracerebral hemorrhage Hu3, Hung-Yi Chiou4, Jiann-Shing Jeng1 (ICH) and 163 ischemic stroke patients 1Stroke Center and Department of Neurology, National Taiwan University Hospital, Taipei (IS). The plasma levels of HA (log 2Department of Neurology, Tri-Service General transformed) were 4.84±0.75, Hospital. 3 5.10±0.72, and 5.17±0.97 ng/ml in Department of Neurology, Taipei Medical University-Shuang Ho Hospital, Taipei controls, <48h and 48-72h after stroke, 4 School of Public Health, Taipei Medical respectively (p<0.001 and 0.01 University, Taipei, Taiwan between controls and strokes). In ICH patients, multivariate analysis

38 suggested that HA levels (<48h) emergency medical service (EMS) exceeded 500 ng/ml was independent could increase use and expedite poor outcome predictor (odds delivery of the thrombolytic therapy in ratio=44.151, 95% CI: 3.6 to 1444.346; acute ischemic stroke patients. p=0.01). In IS patients, although levels of HA failed to be an independent outcome Background Thrombolytic therapy parameter, further analysis using a can only be administered in patients generalized additive model identified a whose stroke onset within 3-4.5 hours. diverse associations between the plasma In addition, it has been shown that the levels of log HA (48-72h) and outcome sooner that thrombolytic therapy is after adjustment of independent clinical given to stroke patients, the better the parameters, that IS patients with high functional outcome. (>6.12) or low (<4.55) all tend to have poor functional outcome. Methods We analyzed consecutive patients presenting to the emergency Conclusion Plasma levels of HA may department (ED) with an ischemic increase after the onset of stroke and stroke within 72 hours of symptom levels of HA within 48h after stroke onset from a prospective stroke onset may help in predicting functional registry in a tertiary medical center. outcome in ICH patients. Variables associated with early ED arrival (within 3 hours of stroke onset), 43 and administration of intravenous 利用緊急醫療系統可以有效增加急性 thrombolytic therapy were analyzed 缺血性腦中風病患接受血栓溶解治療 with multivariate logistic regression 的比例 and linear regression models. 黃光雨1 謝明儒2 湯頌君1 蔡力凱1 馬惠明2 鄭建興1 1臺大醫院神經部 2臺大醫院急診醫學部 Results During the study period from

Utilization of Emergency Medical January 1, 2010 to July 31, 2011, a Service Increases Chance of total of 1764 patients were collected in Thrombolytic Therapy in Patients the registry. Patients were excluded if with Acute Ischemic Stroke 1Kuang-Yu Huang, 2Ming-Ju Hsieh, 1Sung-Chun they were diagnosed as intracerebral Tang, 1Li-Kai Tsai, 2Matthew Huei-Ming Ma, hemorrhage, subarachnoid hemorrhage, 1Jiann-Shing Jeng 1Department of Neurology, National Taiwan transient ischemic attack or cerebral University Hospital venous thrombosis (n=428), 2Department of Emergency Medicine, National Taiwan University Hospital onset-to-ED time＞72 h (n=247), and in-hospital stroke (n=8). Finally, 1081 Object The aim of this study was to patients (62.3% men, age 69.6 ± 13 determine whether utilization of years) were included in this study.

39 Among them, 289 (26.7%) arrived in the Kung University College of Medicine and Hospital, Tainan, Taiwan ED within 3 hours, and 88 (8.1%) received thrombolytic therapy. Patients Background and Purpose Lower who arrived to the ED by EMS (n=279, high-density lipoprotein cholesterol 25.8 %) were independently associated (HDL-C) level has been reported to be with earlier ED arrival (adjusted odds associated with atherosclerotic stroke. ratio [OR] = 3.68, 95% confidence In acute stage of stroke, HDL-C has interval [CI] = 2.54 to 5.33), and higher been shown to be a neuroprotective chance of receiving thrombolytic therapy agent and decreases stroke severity in (adjusted OR = 3.89, 95% CI= 1.86 to animal experiments. We performed a 8.17). Furthermore, utilization of EMS prospective cohort study to evaluate significantly decreased onset-to-needle the influence of baseline HDL-C on time by 26 minutes in patients receiving initial stroke severity and 6-month thrombolytic therapy. prognosis.

Conclusions Utilization of EMS can Methods From August 2006 through not only help acute ischemic stroke December 2011, acute stroke patients patients in early presentation to ED, but fulfilling the following criteria were also effectively facilitate thrombolytic recruited for the present study: (1) age therapy and shorten the onset-to-needle 18 years or older (2) no lipid-lowering time. Our data highly strengthen the drugs use prior to admission, and (3) importance of EMS activation in acute ischemic stroke or transient ischemic stroke management. attack (TIA) due to large artery atherosclerosis and small vessel 44 occlusion. Total cholesterol, 高密度膽固醇影響粥狀動脈硬化性中 triglyceride, low-density lipoprotein 風的預後葉珀秀 1 楊浚銘 1 林聖翔 2 王偉銘 2 林慧娟 1 cholesterol (LDL-C) and HDL-C were 林高章 1 張嘉祐 1 鄭天浚 1 李貽恆 3 checked and National Institutes of 1 奇美醫院神經內科 Health Stroke Scale (NIHSS) score 2 成大醫院臨床醫學研究所 3 成大醫院內科部 were obtained at admission. The primary outcomes were a composite Low level of high-density lipoprotein end point of all-cause mortality, cholesterol in patients with atherosclerotic stroke recurrent stroke, or occurrence of Poh-Shiow Yeha1, Chun-Ming Yang1, ischemic heart disease at 6-month Sheng-Hsiang Lin2, Wei-Ming Wang2, Huey-Juan Lin1, Kao-Chang Lin1, Chia-Yu Chang1, follow up. Results: During the study 1 3 Tain-Junn Cheng , Yi-Heng Li period, 3,093 subjects (mean age 67 1 Department of Neurology, Chi-Mei Medical Center, Tainan, Taiwan yrs; male 62%) with ischemic stroke or 2 Department of Internal Medicine TIA were qualified for this study. 3 Institute of Clinical Medicine, National Cheng

40 Among them, 675 patients (22%) had Conclusion Baseline low HDL-C (≤ HDL-C ≤ 35 mg/dL at admission. At 35 mg/dL) at admission is associated admission, the patients with low HDL-C with higher initial stroke severity and had higher NIHSS score (low vs normal is an independent prognostic indicator HDL-C: 6.4±7.1 vs 5.3±6.1, p < 0.001). at 6-month follow up. Although After adjusting for all clinical factors in isolated low HDL-C is associated with multivariate logistic analysis, we found the risk of composite end point as the low HDL-C at admission (OR, 1.79, 95% non-isolated low HDL, this lipid CI, 1.40-2.29) was significantly phenotype was significantly less associated with higher stroke severity treated. (NIHSS score > 6). During the follow-up, 280 patients (9%) had developed one of 45 the components of the composite end 以臺灣腦中風登錄資料分析慢性腎 point, including 76 (11.3%) in patients 疾病對急性大血管阻塞性腦中風預後之影響 with low HDL-C and 204 (8.4%) in 葉馨喬 1 湯頌君 1 蔡力凱 1 鄭建興 1 patients with normal HDL-C at 臺灣腦中風登錄團隊 admission (p<0.001). The Kaplan-Meier 1 臺大醫院神經部暨腦中風中心 analysis showed a significantly worse The Impact of Chronic Kidney clinical outcome in patients with low Disease on the Outcome of Large HDL-C (Log-rank test, p = 0.017). In Artery Atherosclerotic Stroke: Results from Taiwan Stroke Registry multivariate Cox regression analysis, Shin-Joe Yeh1, MD; Sung-Chun Tang1, MD; after adjusting for all clinical factors, low Li-Kai Tsai1, MD, PhD; Jiann-Shing Jeng1, MD, PhD; and Taiwan Stroke Registry HDL-C at admission (HR, 1.41, 95% CI, Investigators 1.02-1.95) was a significant independent 1 Stroke Center and Department of Neurology, National Taiwan University Hospital and predictor of the composite end point at National Taiwan University College of 6-month follow up. The patients with low Medicine, Taipei, Taiwan

HDL-C were divided into 2 groups: Objective To investigate the impact isolated low HDL-C (HDL ≤ 35 mg/dL of chronic kidney disease (CKD) on and LDL < 160 mg/dL and triglyceride < the outcome of acute ischemic stroke 200mg/dL), and non-isolated low HDL-C with large artery atherosclerosis (LAA) (HDL ≤ 35 mg/dL combined with LDL ≧ 160 mg/dL and/or triglyceride > Background and Purpose CKD has 200mg/dL). During follow-up, composite been shown a risk factor for the end point occurred in 53 (12%) patients development of LAA and stroke. with isolated low HDL-C group and 23 However, the impact of CKD on the patients (9.8%) in non-isolated low outcome of acute stroke patients with HDL-C group (p = 0.392). LAA has not been well documented.

41 Methods Patients who were mortality at 6 months after stroke. prospectively registered in Taiwan Stroke Patients with mild renal impairment Registry (TSR) and had LAA subtype of had significantly higher risk of acute ischemic stroke were included. The recurrent stroke within 6 months after severity of renal function impairment stroke (adjusted hazard ratio, 1.68; was evaluated by estimated glomerular 95% confidence interval, 1.02-2.78). filtration rate (eGFR) as normal (>90 mL/min/1.73 m2), mild (60-89 Conclusions In patients with LAA subtype of acute ischemic stroke, renal mL/min/1.73 m2), moderate (30-59 function at admission is crucial for 2 mL/min/1.73 m ), and severe (<30 prediction of long-term functional mL/min/1.73 m2) renal function outcome, mortality, and recurrent impairment. CKD was defined as stroke. eGFR<60 mL/min/1.73 m2. The 46 association of renal function with acute 胰臟癌與腦中風之風險：以台灣人口 stroke severity was analyzed. Logistic 為基礎的世代研究 regression analysis, Kaplan-Meier 詹博棋紀心怡徐敏獻張偉倫葉宗勳 method and Cox proportional hazard 秀傳醫療社團法人秀傳紀念醫院神經內科 model were applied to assess factors Pancreatic Cancer and Risk of determining the outcome, recurrent Stroke: A Population-Based Cohort stroke and death at 6 months after stroke. Study Po-Chi Chan, Shin-Yi Jih, Min-Hsien Hsu, Wei-Lun Chang, Chung-Hsin Yeh Results Between May, 2006 and April, Department of Neurology, Show Chwan Memorial Hospital 2009, there were 7728 patients (male,

61.8%; average age, 68.6 years) with Background Cerebrovascular disease LAA subtype of ischemic stroke. Acute occurs commonly in cancer patients. stroke severity (represented as initial However, literature on risk of stroke in NIHSS scores) was correlated patients with pancreatic cancer is significantly with moderate and severe sparse. This study aims to investigate CKD (P=0.026 and <0.0001, the risk of stroke in pancreatic cancer respectively). Severe renal impairment patients using a nationwide, was a major determinant for worse population-based database in Taiwan. functional outcome (adjusted odds ratio, 1.39; 95% confidence intervals, 1.06 to Methods For the study cohort, we 1.83). Patients with moderate (adjusted identified 7,116 patients newly hazard ratio, 2.62; 95% confidence diagnosed with pancreatic cancer in interval, 1.18-5.83) and severe (adjusted 2000-2007 from the Taiwan National hazard ratio, 5.48; 95% confidence Health Insurance Research Database. interval, 2.39-12.56) CKD had higher For the comparison cohort, 28,464

42 subjects without pancreatic cancer Review Chi-Li Liao, Jung-Lung Hsu, Jiann-Horng Yeh, matched in terms of sex, age, and the Hou-Chang Chiu index year were randomly extracted from The Department of Neurology, Shin-Kong Wu Ho-Su Memorial Hospital, Taipei the same dataset. Events of stroke from 2000-2009 were ascertained from Background Mirror movement is an medical claims (International involuntary, synkinetic mirror reversals Classification of Disease, Ninth Revision, of an intended movement of opposite Clinical Modification, ICD-9-CM, codes side. Erlenmeyer first used this term in 430-438). Stratified Cox proportional 1879. We reported a 74 year-old hazard regression was performed on the woman who had an embolic infarct in two cohorts to compare the risk of stroke the right MCA territory with the mirror during follow-up period. movement

Results The incidence of stroke was Case Report This 74 year-old woman higher in patients with pancreatic cancer, had a history of HTN and SLE. She as compared to the noncancer group was just admitted on 03/19 due to the (32.43 vs. 16.83 per 10,000 person-years) generalized weakness and poor verbal during the follow-up period. After the output for 5 minutes at home. stratified Cox proportional analysis for Pneumonia, pancytopenia, SLE patients’ demographic characteristics and encephalopathy was initial diagnosed. comorbidities, overall risk of ischemic She was discharged on 04/03 with stroke and hemorrhagic stroke during the prednisolone 4# BID for SLE. Two follow-up period after diagnosis of days later, her daughter found that she pancreatic cancer was 2.07 (95% became easily choking, slurred speech, confidence interval (CI) = 1.77-2.42) and and slow response at dinner. She still 1.88 ( CI = 1.22-2.90), respectively. could walk by herself. However, she started to have left side weakness with Conclusion Pancreatic cancer is dysphagia and dysarthria since on associated with an increased risk of 04/06 afternoon. No dizziness, no subsequent ischemic and hemorrhagic diplopia, no drop attack, no lost of stroke. consciousness. She was sent to our ER where E4V5M6, T/P/R: 36.2/ 92bpm/ 47 14rpm, Bp: 174/98mmHg. 中風病人表現之 Mirror movement- Dysphagia(+), Dysarthria(+), left 個案報告與文獻回顧 hemiparesis(MP:4+/4+). Brain CT 廖岐禮徐榮隆葉建宏邱浩彰新光醫院神經內科 revealed no hemorrhage but right parieto-occipital hypodense and left Mirror movement in a patient with basal ganglion lacunar infarct noted. stroke - A Case Report and Literature

43 ECD reported no obvious stenosis, but Predicting Mortality in Malignant Middle Cerebral Artery Infarction TCD disclosed bilateral MCA occlusion. patients by using Under the impression of MCA-PCA Susceptibility–Weighted MR stenosis, she was admitted for treatment. imaging Shu P. Chao1, Chia Y. Chen2, Fong Y. Tsai3, During admission, mirror movement was Wing P. Chan2, Chin- I Chen1 noted (synchronous movement of right 1 Department of Neurology, 2Radiology , Wan Fang Hospital , 3Taipei Medical University hand and mouth when patient squeezed Medical Imaging Research Center, Taipei her left hand). Brain MRI showed right Medical University

MCA territory multifocal infarct, and Objectives Malignant middle cerebral embolic stroke was suspected. Both artery (MCA) infarctions are defined cardiac echo and TEE (due to no finding as hypodensity in more than 50-75% of in heart echo) reported no emboli noted. the MCA territory on the computer After discussed with family, we kept tomography and have been associated antiplatelet medication usage. We with high mortality rate. arranged rehabilitation for her when no Susceptibility-weighted imaging (SWI) stroke in evolution noted, and now can depict deoxyhemoglobin dysarthria and dysphagia improved. (deoxy-Hb) and has been shown to MP(4+/4+). She could stand without predict stroke evolution. We use SWI titubation, and walk without assistance. and DWI to evaluate malignant MCA Mirror movement also improved (not infarction patients and correlate the totally subsided). She discharged on images to the clinical outcome. April 18 with OPD follow-up.

Methods We retrospectively Conclusion Mirror movements are the analyzed large MCA infarction patients soft neurological signs that are rarely who were admitted to Wanfang encountered in routine clinical practice hospital during past three years. MRIs and are bound to be missed if not were reviewed by one neurologist and specifically sought for. This patient one radiologist respectively. We used represented mirror movement in the the Alberta Stroke Program Early CT beginning of stroke, and improved Score (ASPECTS) to evaluate patients’ gradually. diffusion - weighted imaging (DWI)

and SWI lesions obtained within 7 48 days after stroke. 利用磁敏感加權成像預測惡性中大腦動脈梗塞病患的死亡率趙書屏 1 陳家媛 2 蔡芳洋 3 陳榮邦 2 陳晉誼 1 Results Sixteen malignant MCA 1 臺北醫學大學 infarction patients are enrolled into this 2 萬芳醫院神經科放射科 3 臺北醫學大學影像研究中心 study and divided into mortality (n=6) and survival group (n=10). The

44 patients’ clinical profiles and stroke risk aim of the present study would observe factors are documented (p >0.1). the dynamics changes in cerebral blood Although the DWI-ASPECTS and flow of acute ischemic stroke patients clinical profiles are similar in both treated before and after acupuncture groups, the SWI-ASPECTS in mortality therapy. group are significantly lower than that in survival group (p < 0.001). Methods we collect newly onset of acute ischemic stroke patient without Conclusions The extent of underling systemic major illness, and de-oxygenation area showed by SWI in randomized assign the collected malignant MCA infarction patients can patients into two groups as predict mortality. Lower SWI-ASPETS is acupuncture group and normal control potentially better parameter than group. Both group all treat with routine DWI-ASPECTS to predict poor outcome. western medicine as standard stroke Further prospective study and more cases therapy. The methods of acupuncture are needed to clarify the role of SWI and performed by Jin’s 3- needle technique guiding intervention therapy. (靳三針) with‘The 3-arm Points: Quchi (LI 11), Waiguan (TE 5), Hegu 49 (LI 4)’and ‘The 3-Leg Points: Zusanli 針刺治療對急性缺血性腦中風患者腦 (ST 36), Sanyinjiao (SP 6), Taichong 血流速度的影響 (LR 3)’, ‘Three temporal points’ and 劉競雄曾弘斌林俊良林鴻志羅東博愛醫院神經內科 ‘Four Spirit points’ by ‘uniform reinforcing-reducing method’. The The Effects of Acupuncture Therapy therapeutic course of acupuncture in on the Cerebral Blood Flow Velocities theses patients performed once daily of Acute Ischemic Stroke Patients Ching-Hsiung Liu, Hung-Pin Tseng, Chun-Liang for ten days. The blood flow velocity Lin, Hung-Chin Lin Department of Neurology, Lotung Poh-Ai and other parameters were measured in Hospital, Ilan, Taiwan. bilateral internal carotid artery (ICA) and middle cerebral artery (MCA) Objectives Acupuncture in traditional before and after acupuncture treatment. Chinese medicine has been verified in many disease by thousands yeas. The Results Total 34 patients were acupuncture therapy applied in post included in the study. 17 case for stroke functional recovery has been acupuncture group and 17 case for considered as more benefit than physical control group. The acupuncture group therapy only to post stroke rehabilitation.. did not approach significant difference However, the effect and mechanism of in all parameter of ICA and MCA in acupuncture therapy in acute ischemic sonographic studies. However, after stroke patient still remains uncertain. The

45 sub-grouping the cases, the right ICA ‘s promotes functional recovery after pulsatility index (PI) and resistance index stroke. Valproate (VPA), a histone (RI) decreased significantly in deacetylase (HDAC) inhibitor, is acupuncture group with the infarction known to protect against cerebral area of the left side. ischemia; however, its effects on BBB protection and angiogenesis are still Conclusions Acupuncture therapy may unknown. decreased the PI and RI of ICA contra-lateral to infarction side in acute Methods Male rats underwent middle stroke patients. The results coubld be cerebral artery occlusion (MCAO) for explain the possibly mechanism of 60 minutes followed by reperfusion. acupuncture effect on cerebral blood VPA (200 mg/kg, intraperitoneally) flow which increasing the collateral was administrated once daily for 14 arterial flow in acute stroke stage. days. Assessed parameters included the Rotarod test, T2/perfusion-weighted 50 MRI, Evans blue extravasation Valproate 透過降低 BBB 破壞和增進血 analysis, Western blotting, 管新生而促進腦梗塞大鼠的功能性恢 immunohistochemistry, and gelatin 復 zymography. 蔡力凱 1,2 王志菲 2 冷豔 2 Peter Leeds2 莊德茂 2 1 台大醫院神經部暨腦中風中心 Results Postischemic VPA treatment 2 美國國家衛生院 NIMH improved the Rotarod performance and

Valproate Attenuates BBB Disruption, reduced infarct area in MCAO rats on Enhances Angiogenesis, and Promotes Days 7 and 14 after ischemia. VPA Functional Recovery in a Rat Model of treatment attenuated MCAO-induced Ischemic Stroke Li-Kai Tsai, MD, PhD1, Zhifei Wang, PhD2, Yan BBB disruption and brain edema 24 2 2 Leng, MD , Peter Leads , hours after stroke. Meanwhile, VPA De-Maw Chuang, PhD 2 1 Department of Neurology, NTUH reduced MCAO-induced elevation of 2 NIMH, National Institutes of Health, MD, USA MMP-9 and degradation of tight

junction proteins. In addition, VPA Objective To investigate the therapeutic enhanced microvessel density, mechanisms of valproate (VPA) in facilitated endothelial cell proliferation, cerebral infarct. and increased cerebral blood flow in

the ipsilateral cortex on Day 14. The Background Blood-Brain barrier (BBB) hypoxia-inducible factor-1α (HIF-1α) disruption after stroke results in brain and its downstream proangiogenic edema and deteriorates the neurological factors, VEGF and MMP-2/9, were condition. Enhanced angiogenesis upregulated after MCAO and facilitates neurovascular remodeling and

46 potentiated by VPA. The beneficial hemodynamic compromise in patients effects of VPA were abolished by HIF-1α with unilateral severe cervical carotid inhibition. stenosis.

Conclusion VPA treatment attenuates Methods A total of 200 patients with BBB disruption, enhances angiogenesis, unilateral high-grade cervical carotid and promotes functional recovery after stenosis/occlusion were diagnosed by brain ischemia. These effects may color-coded duplex ultrasonography. involve suppression of MMP-9 (early) Of the 152 patients with unilateral and upregulation of HIF-1α and its high-grade cervical carotid stenosis downstream proangiogenic factors (late). were divided into 2 groups, according to the presence or absence of ROAF, to 51 compare their hemodynamic 眼動脈逆流於內頸動脈狹窄患者之臨 parameters. Out of 200 patients, 159 床運用 underwent brain magnetic resonance 蔡佳霖 1 李俊泰 1 陳俊安 1 劉孟達 2 陳震宇 3 胡漢華 4 彭家勛 1 imaging and were analyzed for risk 1 三軍總醫院神經內科 factors impacting functional outcomes 2 國軍花蓮總醫院內科 including ROAF. 3 三軍總醫院放射科 4 台北榮民總醫院神經內科 Results Of the initial 200 patients, Clinical implications of reversal of 152 (76.0%) were diagnosed with ophthalmic artery flow in patients with high-grade carotid stenosis and 48 unilateral severe carotid stenosis (24.0%) with occlusion; ROAF was 1 1 1 Chia-Lin Tsai , Jiunn-Tay Lee , Chun-An Cheng , observed in 68 (34.0%) patients. Meng-Ta Liu2, Cheng-Yu Chen3, Han-Hwa Hu4, Giia-Sheun Peng1 Patients with ICA occlusion had 1 3 Departments of Neurology and Radiology, significantly higher percentage of Tri-Service General Hospital, National Defense Medical Center, Taipei; 2Department of ROAF compared to patients with Internal Medicine, Hualien Armed Forces high-grade stenosis (44.1% vs. 13.6%, General Hospital, Hualien, and 4Department of Neurology, Taipei Veterans General Hospital, p < 0.001). In the ROAF group, there Taipei, Taiwan were significant differences in all the

hemodynamics parameters of Objective High-grade cervical carotid retrobulbar arteries between stenotic stenosis (70–99%) or occlusion often and non-stenotic vessels. The patients accompanies the occurrence of reversed (n = 159) with history of stroke (P = ophthalmic artery flow (ROAF), but its 0.035), ROAF (P = 0.023) and clinical implications in patients remain intracranial stenosis (P < 0.001) uncertain. The purpose of the present exhibited significantly higher study was to assess ROAF and the incidence of poor functional outcome related variables caused by carotid

47 compared with the corresponding control Methods We retrospectively groups. Multivariate analysis revealed reviewed the medical records of all 3 that patients with cervical and patients discharged from Far Eastern intracranial stenosis in combination had a Memorial Hospital with a diagnosis of seven-fold (OR: 7.60, 95% CI: isolated cortical vein thrombosis from 3.44–16.81) higher risk for the presence January 2010 to December 2012. Brain of ROAF. magnetic resonance imaging (MRI) and angiography were performed in all Conclusion ROAF may result from patients. We evaluated their treatment intracranial hemodynamic compromise. and outcomes. Patients with unilateral high-grade Results Three female patients (mean cervical carotid stenosis/occlusion in age 35 years; range, 30-40 years) were combination with intracranial stenosis included. All presented with focal appear to be a significant risk factor for neurologic deficits. Headache was poor functional outcome and increased noted in two of three patients. None of incidence of ROAF. the patients have seizure attacks. Brain MRI demonstrates parenchymal 52 hyperintensities involving the 台灣的孤立性皮質靜脈血栓研究：3 例 cortical-subcortical regions around the 臨床報告 ICVT on T2-weighted/FLAIR images. 鄭又禎 1 李少白 1 賴資賢 1,2 1 亞東紀念醫院神經內科 Angiography showed isolated cortical 2 國立陽明大學醫學院 vein thrombosis without sinus involvement. Two of the patients Isolated cortical vein thrombosis in Taiwan: a 3 cases series received anticoagulant treatment while Yu-Chen Cheng1, Siupak Lee1, Tzu-Hsien Lai1,2 the other received antiplatelet 1Department of Neurology, Far Eastern Memorial Hospital treatement. Clinical outcomes were 2National Yang-Ming University School of uniformly good, despite variable Medicine treatment strategies. Background Isolated cortical vein thrombosis (ICVT) without sinus Conclusion The results of our study involvement is uncommon and difficult show that there are distinct clinical to diagnose. Our aim is to investigate the characteristics and neuroradiologic clinical characteristics and findings in isolated cortical vein neuroradiologic findings in patients with thrombosis from those findings in isolated cortical vein thrombosis in venous sinus thrombosis . The outcome Taiwan. of the patients was good and the treatment options are discussed.

48 53 patients. The results of this study were 以中風醫療品質突破系列活動提升急 compared to those of the previous 性缺血性中風醫療品質 Taiwan Stroke Registry (TSR, 22642 鄭建興 1 連立明 2 邱弘毅 3 邱浩彰 2 中風醫療品質突破系列研究群 AIS patients from 39 hospitals, 1 臺大醫院神經部 2006-08). 2 新光醫院神經科台北醫學大學公衛學院

Quality Improvement in Acute Results Data from 24 hospitals with Ischemic Stroke Care Through the 13181 AIS patients during a 1-year Breakthrough Series - Stroke period were analyzed. The BTS-Stroke Jiann-Shing Jeng1, Li-Ming Lien2, Hung-Yi Chiou3, Hou-Chang Chiu2, on behalf of the (2010-11) had better performance as BTS-Stroke Investigators compared to the TSR (2006-08): 1 Department of Neurology, National Taiwan University Hospital, intravenous thrombolysis frequency for 2 Department of Neurology, Shin Kong WHS Memorial Hospital, all AIS patients (4.1% vs 1.5%), 3 School of Public Health, Taipei Medical symptomatic hemorrhage after University, Taipei, Taiwan intravenous thrombolysis (6.0% vs Background and Purpose Guideline 8.2%), early antithrombotics (96.6% vs adherence for acute ischemic stroke (AIS) 94.1%), anticoagulation for atrial management is often suboptimal, fibrillation (57.1% vs 28.3%), lipid particularly in thrombolytic therapy and lowering drugs for low-density anticoagulants for atrial fibrillation. We lipoprotein >100 mg/dL (63.4% vs sought to achieve quality improvement of 38.7%), antithrombotics at discharge AIS patients via a collaborative learning (94.0% vs 85.5%), and one-month model, the Breakthrough Series mortality (3.5% vs 4.0%). Temporal (BTS)-Stroke, in a nationwide, improvement was noted in 7 of 14 multi-center activity in Taiwan. performance measures when the fourth BTS-Stroke quarter compared with the Methods A BTS Collaborative, a first quarter: intravenous thrombolysis short-term learning system for a large frequency for all AIS patients (4.1% vs number of multidisciplinary teams from 3.7%), symptomatic hemorrhage after hospitals, was applied to enhance AIS intravenous thrombolysis (3.4% vs care quality. There were 24 teaching and 5.5%), lipid lowering drugs for community hospitals participating in and low-density lipoprotein >100 mg/dL submitting data for this stroke quality (67.3% vs 60.5%), antithrombotics at improvement campaign from August discharge (95.5% vs 91.4%), dysphagia 2010 to June 2011. The Get With The screening (81.9% vs 63.4%), early Guideline (GWTG)-Stroke measures rehabilitation (71.7% vs 63.6%), stroke were adopted to evaluate the education before discharge (95.6% vs performance and outcome of the AIS 83.4%).

49 Conclusions A BTS collaborative in the above area. Fundus exam and learning and campaign model can fluoroangiogram did not show obvious improve the guideline adherence of arterial occlusion or disc abnormality. stroke. The GWTG-Stroke can be The flash visual evoked potential was successfully applied to other countries absent in the left eye. After rapid outside the United States. normalization of blood pressure, right visual field defect and limb weakness 54 improved significantly, but a 以併發高血壓性腦幹病變及後部缺血 permanent visual loss persisted after 性視神經病變之高血壓危象：個案報告 three months. 賴星融湯頌君鄭建興台灣大學附設醫院神經部 Conclusion Our case demonstrated a Hypertensive emergency presents with rare presentation of coexistent hypertensive brainstem hypertensive brainstem encephalopathy encephalopathy and posterior ischemic optic neuropathy: a case report and ischemic optic neuropathy in HE. Hsing-Jung Lai, Sung-Chun Tang, Jiann-Shing Jeng Department of Neurology, National Taiwan 55 University Hospital, Taipei, Taiwan 探討急診進行靜脈血栓溶解治療病患的美國國家衛生研究院中風量表 Introduction Hypertensive emergency 各項分數分佈 (HE) represents acute impairment of one 謝函潔 1 宋昇峯 2 林慧娟 3 陳右緯 4 曾美君 5 6 or more organ systems related to 陳志弘 1 國立成功大學附設醫院斗六分院內科部 uncontrolled hypertension, but 神經內科 concurrent involvement of central and 2 戴德森醫療財團法人嘉義基督教醫院內科部 peripheral nervous systems has not been 神經內科 3 奇美醫院神經內科 reported. Here we reported a case with 4 壢新醫院神經內科 HE, suffering from reversible brainstem 5 壢新醫院資深研究員 6 國立成功大學附設醫院神經部 encephalopathy and unilateral posterior ischemic optic neuropathy (PION). The Distribution of the Items of National Institutes of Health Stroke Case report A 32-year-old man visited Scale (NIHSS) in Patients Receiving Intravenous Thrombolytic Therapy our emergency room with high blood at Emergency Department pressure (226/160mmgHg), left 1Han-Chieh Hsieh, 2Sheng-Feng Sung, 3Huey-Juan Lin, 4Yu-Wei Chen, 5Mei-Chiun monocular visual loss, right temporal Tseng, 6Chih-Hung Chen visual loss and mild left upper limb 1 Division of Neurology, Department of Internal Medicine, Dou-Liou Branch, weakness. Brain CT showed unilateral National Cheng Kung University Hospital 2 right midbrain and bilateral pontine Division of Neurology, Department of Internal Medicine, Chia-Yi Christian hypodensity. Brain MRI showed Hospital 3 increased apparent diffusion coefficient Department of Neurology, Chi-Mei Medical Center

50 4 Department of Neurology, Landseed Hospital weakness into consideration, the 5 Senior researcher, Landseed Hospital 6 Department of Neurology, National Cheng Kung percentage rose to 97.8%. University Hospital

Conclusions Motor weakness of any Background and purpose Stroke code extremity is the most common sign in facilitates early recognition of stroke and patients receiving intravenous speeds up the assessment. To accelerate thrombolytic therapy at ED. The high the evaluation currently performed by sensitivity is sufficient to identify most triage personnel, a rapid and simple candidates for thrombolytic therapy for protocol for detecting stroke the first step of triage. The present symptoms/signs is valuable. We aimed to triage protocol for acute stroke determine the most sensitive sign(s) identification at ED may be simplified among the items of NIHSS that could be accordingly. used to identify patients most likely to be eligible for thrombolysis. 56 醫院與緊急醫療系統的合作計畫提 Methods Data were retrospectively 升急性中風照護 collected from January 2007 to June 謝明儒1 湯頌君2 蔡力凱2 黃光雨2 馬惠明1 2 2012 in four Stroke Centers (National 鄭建興臺大醫院 1急診醫學部 2神經部 Cheng Kung University Hospital, Chi-Mei Medical Center, Chia-Yi Hospital and Emergency Medical Service Cooperation Program Christian Hospital, and Landseed Enhances Acute Stroke Care Hospital). We explored the most Ming-Ju Hsieh1, Sung-Chun Tang2, Li-Kai 2 2 critically relevant items from NIHSS in Tsai , Kuang-Yu Huang , Matthew Huei-Ming Ma1, Jiann-Shing Jeng2 patients receiving intravenous tissue Departments of 1Emergency Medicine and 2Neurology, National Taiwan University plasminogen activator (tPA) at Hospital emergency department (ED). Background The importance of Results A total of 627 patients received emergency medical service (EMS) on tPA at ED, with a baseline median hyperacute stroke care has been NIHSS score of 13 (interquartile range 8 emphasized by the guidelines of to 19). The most common single American Stroke Association because abnormal NIHSS item is facial weakness utilization of EMS can improve both (88.2%). If we added the items of motor pre-hospital and in-hospital delays. weakness (5aL+5bR+6aL+6bR) together, Nevertheless, a close cooperation the most prominent sign was motor between hospitals and EMS for stroke weakness in at least one extremity patient care had not been well (scored ≥1 point in the subtotal of motor established in Taiwan until recent years. category) (95.1%). After taking facial This study aimed to assess the impact

51 of the cooperation program on acute onset among patients with acute stroke stroke care. and transient ischemic attack was elevated significantly from 22.3% to Methods From December, 2010 to 25.5% (rate ratio, 1.14; 95% February, 2011, emergency medical confidence interval, 1.01 to 1.29; technicians (EMTs) in the catchment area P=0.031); the percentage of patients of NTUH were educated to identify receiving thrombolytic therapy among stroke patients with symptoms onset less acute ischemic stroke patients than 3 hours by using Cincinatti increased significantly from 5.8% to Prehospital Stroke Scale and to perform 8.1% (rate ratio, 1.39; 95% confidence prehospital notification if such patients interval, 1.01 to 1.92; P=0.043). were identified. Since March, 2011 to November, 2012, each prenotified acute Conclusions Through the stroke patient’s characteristics and cooperation program, the EMTs can diagnosis was recorded. We also make correct identification and reviewed acute stroke patients during prenotification of acute stroke. The January, 2010 and February, 2011 for frequency of thrombolysis in acute historical comparison. ischemic stroke improved significantly.

Results Regarding the clinical 57 performance of prenotification by EMTs, 預測急性缺血性腦中風病患接受血栓 the positive predictive value was 78.7% 溶解治療之後腦內出血與院內死亡的因子: 一個為期 8 年的全國性調查 (196/249) and the sensitivity was 59.4% 謝鎮陽 1,2 陳志弘 3 楊高雅慧 2 (196/330). Seizure (35.8%) and onset 1 台南新樓醫院腦中風中心暨神經內科 time >3 hours (26.4%) were the two most 2 國立成功大學臨床藥學與藥物科技研究所 3 國立成功大學附設醫院腦中風中心暨神經部 common conditions leading to incorrect prenotification. Among all prenotified Factors Predicting Intracerebral patients, 21.3% (53/249) received Hemorrhage and In-hospital thrombolytic therapy. Hemorrhagic Mortality after Thrombolytic Therapy for Acute Ischemic Stroke stroke / ischemic stroke with Patients: a 8-year Nationwide hemorrhagic transformation (40.3%) and Survey Cheng-Yang Hsieh1,2, Chih-Hung Chen3, minor symptom (NIHSS<4) / rapid Yea-Huei Kao Yang2 recovery (17.3%) were the two most 1 Stroke Center and Department of Neurology, Tainan Sin Lau Hospital common reasons for no thrombolytic 2 Institute of Clinical Pharmacy and therapy in the prenotified stroke patients Pharmaceutical Sciences, National Cheng Kung University, Tainan, Taiwan arriving within 3 hours. After the 3 Stroke Center and Department of Neurology, cooperation program, the percentage of National Cheng Kung University Hospital, Tainan, Taiwan patients arriving at ED within 3 hours of

52 Objective To analyze factors predicting mortality are 3.96% and 9.18%, intracerebral hemorrhage (ICH) and respectively. After adjusting for age, in-hospital mortality after thrombolytic sex, co-morbidities, weekend therapy for Taiwanese acute ischemic admission, and physician’s specialty, stroke (AIS) patients using a nationwide we found ICH was significantly dataset. associated with an increase of in-hospital mortality (adjusted odds Background Recombinant tissue ratio [OR]: 5.288; 95% confidence plasminogen activator (rtPA) is the only interval [CI]: 3.167-8.831), while rtPA effective treatment for AIS. However, experience of each hospital was debate has been raised when regulators associated with a decrease of ICH used hospital accreditation to implement (adjusted OR: 0.992, 95% CI: rtPA across the nation. The hypothesis we 0.986-0.998) for patients receiving want to test is that the experience of rtPA. thrombolytic therapy of each hospital has an inverse relationship with Conclusion The maxim “practice complications after rtPA. makes perfect” may be also applicable in stroke thrombolysis. Our results Methods We identified all hospitalized provide evidence regarding the urgent AIS patients receiving rtPA in the need to integrate the emergency whole-population National Health medical service and centralize Insurance Research Database, 2003-2010. hospitals providing thrombolytic The outcome of interest included ICH therapy for AIS patients in Taiwan. and in-hospital mortality after rtPA for each AIS admission. In addition to total 58 rtPA experience of each hospital, the 降血壓處置延遲血栓溶解劑治療及 following factors were adjusted in the 其結果簡崇曜宋碧姍 multiple regression models including age, 國立成功大學醫學院附設醫院神經部暨中 sex, co-morbidities of the patients (i.e. 風中心 Charlson co-morbidity index), weekend Antihypertensive Therapy Before admission or not, and physician’s Thrombolysis specialty. Chung- Yao Chien, Pi-Shan Sung Department of Neurology and Stroke Center, National Cheng Kung University Hospital Results We identified a total of 2,742 AIS patients with thrombolytic therapy, Background Pre-thrombolytic accounting for 0.68% of all AIS antihypertensive therapy in ischemic hospitalized patients during 2003-2010. stroke patient , which mean systolic blood pressure >185mmHg or diastolic The rates of ICH and in-hospital blood pressure > 110mmHg, is

53 suggested to avoid risk of symptomatic mRS was 2; the other 24 patients intracranial hemorrhage. It may exceed received pre-thrombolytic golden time of recanalization while antihypertensive therapy and average managing elevated blood pressure. We DNT of 71 minutes and the median of try to examine the possible inferior mRS was 3.5. The median of NIHSS consequence due to delaying intravenous was 11 in former group and 9 in latter thrombolysis, even in short period. group.

Method We reviewed medical records Discussion According to our of ischemic stroke admissions treated experience, receiving pre-thrombolytic with intravenous thrombolysis over a antihypertensive therapy delayed DNT 6-year period at National Cheng Kung 10 minutes in average. The median of University Hospital. Those patients with NIHSS in patients without delayed door-to-needle time (DNT) by pre-thrombolytic antihypertensive any other reasons were excluded, such as therapy was slightly higher than that in cases prior to stroke code system those received blood pressure lowering establishment, cases without initiating agents, which means no existence of stroke code, off-lable usage of tissue significant difference between these plasminogen activator and complicated physical disorder before thrombolysis. two groups. It might be the The included patients were divided into consequence of delaying 10 minutes two groups according to whether that inferior outcome in those needed receiving pre-thrombolytic pre-thrombolytic antihypertensive antihypertensive therapy or not. We therapy, demonstrated by mRS in one analyzed door-to-needle time and month. However, higher blood pressure outcome (modified rankin scale in one after ischemic stroke may indicate month) of these two groups larger area penumbra or severer arterial independently. stenosis, which may not directly reflect in NIHSS. It may also contribute the Result Total 285 patients were enrolled. inferior outcome in patient needed We excluded those patients with delayed pre-thrombolytic antihypertensive DNT due to prior to group-call system therapy. establishment (36 patients), missed group-call, all off-label usage of tissue Conclusion Early detection and plasminogen activator (45 patients) and effective management of elevated complicated physical disorder before pre-thrombolytic blood pressure may thrombolysis (8 patients). Thus 196 be another importance issue for patients were included. There were 172 patients did not receive pre-thrombolytic improving outcome in patient receiving antihypertensive therapy, with average thrombolytic therapy. DNT of 61 minutes in and the median of

54 facilitating system (North-up v.s. 神經行為學 Track-up) and landmarks (with 59 landmarks v.s. without landmarks). 輕度阿茲海默氏症患者之認路功能：比 They then needed to point out the start 較指北地圖/路徑地圖與地標之輔助功 point and were asked to pick out a 能 correct route on the paper. . 方楨文 1 林松農 2 歐陽昆 2 柳永青 2 1 台大醫院雲林分院神經內科 2 雲林科技大學管理學院 Results These groups did not differ in age, gender, education, and VFD Comparison study of north-up and track-up electronic maps with and scores. MMSE, TMT-B and clock without landmarks on the wayfinding drawing test were the 3 tests mostly performance of mild Alzheimer's corrected to error times. (P<0.001) 3 disease patients Fang, Chen-wen1, Sung-Nung Lin2, Yang-Kun participant groups all made fewer Ou2, Yung-Ching Liu2 errors with track-up maps (0.18) than 1 Department of Neurology, National Taiwan University Hospital (Yunlin Branch), Yunlin, with north-up maps (1.83); better Taiwan outcome was also performed in maps 2 Department of Industrial Engineering and Management, National Yunlin University of with landmarks (0.91) than in maps Science and Technology, Yunlin, Taiwan without landmarks (1.10). In 3

participant groups, track-up map had Objective In this study, through four shorter performance (126.85 s) time different exams on a virtual than the north-up map (170.17 s). But, three-dimensional simulated maze, we maps without landmarks had a shorter compared the ability of way finding in performance time (140.49 s) than the three different groups of elderly. (Normal map with landmarks (156.53s). aging/ Minimal cognitive impaired

(MCI)/ Early Alzheimer's patient) with 註解 [YHL4]: Mild cognition Conclusion We found that track-up different navigation systems. impaired? map system could effectively improve

the way-finding ability of people with Methods We prospectively recruited 40 Alzheimer's disease and minimal participants (10 AD. 10 MCI. 20 normal cognitive impairment. We also noticed 註解 [YHL5]: Mild? control) with similar demographic that several surrogate markers in distribution of age and gender. They cognitive tests were much correlated received a series of cognitive battery with the performance of way-finding. exams, including complex figure test-copy version, clock drawing test, 60 visual form discrimination, trail making 職能治療對輕中度失智症患者之療效 test, useful field of view task. They then 王瑾琦劉景寬周美鵑李建勳龔達瑋 tried to reach the destination in 4 許惠敏 different mazes with 2 different 高雄醫學大學醫學系神經學碩士班高雄醫學大學附設中和紀念醫院神經內科高雄市

55 立大同醫院高雄市立小港醫院高雄市失智症 Results In total, 50 subjects (21 male 協會 and 29 female) participated in the Effect of Occupational Therapeutic study with a mean age of 73.94 (SD Program for Patients with Early to 7.35) years old and a mean education Mild Dementia of 9.08 (SD 5.56) years. With Chin-Chi Wang1,2,5, Ching-Kuan Liu1,2, Mei-Chuan Chou1,2,3, Chien-Hsun Li1,2,4, paired-samples T test, the CASI Ta -Wei Kung5, Hui-Min Hsu1,2 1 Department of Master’s Program in Neurology, (t=-3.17, p=.003) and CES-D (t=2.42, Faculty of Medicine, College of Medicine, p=.002) scores of subjects showed Kaohsiung Medical University; 2 Department of Neurology, Kaohsiung Medical significant effect for intervention. University Hospital; There was also a significant difference 3 Department of Neurology, Kaohsiung Municipal Ta-Tung Hospital; of the scores of quality of life of the 4 Department of Neurology, Kaohsiung Municipal family caregivers before and after Hsiao-Kang Hospital; 5 Kaohsiung Dementia Association intervention for the patients (t=-2.12, p=.040). However there was no Objectives We examined the efficacy of significant difference of the CES-D of occupational therapeutic program the family caregivers (t=1.94, p=.059). modified from School of Wisdom of Taiwan Alzheimer’s Disease Association Discussion Qualitatively, in the (TADA) in improving cognitive function beginning, many of the patients may and emotion and prompting quality of have lower activation and motivation life of family caregivers. for the program and have resistant passive behaviors. When it came to the Methods This is a single-blinded middle or late stage of the group, they interventional trial. Subjects with early to showed more spontaneity and less mild dementia received occupational resistance. After the program, the therapeutic program two hours once a intervention the participants showed week. It lasted for 10 weeks each group. increased social interaction in the We also provided educational-support group, enriched verbal expression, groups for the family caregivers. The improved positive mood, and gained primary parameters were Cognitive self-confidence and both the patients Abilities Screening Instrument (CASI) and family caregivers expected a and Center for Epidemiologic continuous group for them. There were Studies-Depression Scale (CES-D). The also individual effects for respective family caregivers were also evaluated group observed. During the program, with CES-D and quality of life subjects shared their suffering and (WHO-QOL). Paired-samples T tests adjustment for dementia, which could were used to examine the intervention help them cope with their illness; effect. family caregivers could gain

56 psychological support to help them and has been widely used for dementia relieve their stress. screening in several epidemiological studies. However, its usefulness in Conclusion The results were consistent evaluating treatment outcome of AD findings of TADA. It implied that has not been examined. intervention of occupational therapeutic program could improve cognitive Methods Consecutive patients function of early to mild dementia fulfilled the diagnostic criteria of AD patients and quality of life of their family from January 2007 to September 2010 caregivers. Occupational therapy could were prospectively registered into our benefit dementia patients and their family, computerized database. For each which deserves further promotion in recruited AD patient, a series of Taiwan. neuropsychological assessments, including Mini-Mental State Exam 61 (MMSE), CASI, and Clinical 阿茲海默氏症病患經乙醯膽鹼酯脢抑 Dementia Rating (CDR), were 制劑治療後於認知功能篩檢量表之變 conducted annually to evaluate the 異 therapeutic responses to AChEIs 何柏陵賴秋蓮周美鵑劉景寬楊淵韓高雄市立大同醫院高雄醫學大學附設中和 therapy. 紀念醫院神經內科

Results A total of 385 AD patients The Change of CASI and Its Subscale after the Treatment of were included in our study; 89 patients Acetyl-Cholinesterase Inhibitor in with global CDR 0.5, 237 with CDR Patients with Alzheimer's disease 1.0, and 59 with CDR 2.0. The mean Bo-Lin Ho, Chiou-Lian Lai, Mei-Chuan Chou, Ching-Kuan Liu, Yuan-Han Yang age was 76.96 ± 7.76 years and there Department of Neurology, Kaohsiung Municipal Ta-Tung Hospital, Kaohsiung Medical University were 113 men (29.35%). In CASI Hospital, Kaohsiung Medical University subscales analysis, statistically significant improvements were Objective To evaluate the cognitive performed in fluency and short-term outcomes in Alzheimer's patients by memory, but declines were Cognitive Abilities Screening Instrument demonstrated in orientation and (CASI) after annual medical treatment in long-term memory. Intergroup Taiwan differences showed statistical

improvement in concentration and Background Acetyl-cholinesterase orientation in patients with CDR 2.0. inhibitors (AChEIs) are widely used for the treatment of Alzheimer's disease Conclusion Based on the (AD). The CASI provides quantitative observational results of our study, the assessment on nine cognitive domains,

57 treatment outcomes of AChEIs for AD at left inferior temporal gyrus and patients were multidimensional and made detail distinction of impaired diverse in view of different cognitive word retrieval from other possible domains. This may provide practical causes. guidance for treatment options and prognostic evaluation in clinical settings. Case Report A 82-year-old right handed woman with hemorrhage of left 62 inferior temporal gyrus demonstrated 病例個案報告: 左側下顳葉腦迴出血 long-lasting word retrieval deficit, in 引起之辭彙提取障礙 the presence of fluent spontaneous 凃敏謙 1 嚴寶勝 2,3 林佩怡 1 1佛教慈濟綜合醫院台中院區神經內科 speech, accurate writing, and a few 2影像醫學部 paraphasic errors. Neuropsychological 3 慈濟大學 assessment suggested a mismatched

Word retrieval deficit after left phonological representation with inferior temporal gyrus hemorrhage: preserved semantics and visuospatial A case report function, typical for genuine word Min-Chien Tu 1, Pao-Sheng Yen2,3, Pai-I, Lin 1 1 Department of Neurology, retrieval failure (pure anomia). 2 Department of Radiology, Buddhist Tzu Chi Confrontation naming tests suggested General Hospital Taichung Branch, Taichung, Taiwan, response favorable for category “tools” 3 School of Medicine, Tzu Chi University, Hualien, than “plants”/”animals” and partial Taiwan benefit from phonological cue. Brain Abstract magnetic resonance images had Background Albeit learning process unveiled the Wallerian degeneration and speech production stream varied from previous old hemorrhagic nidus. across different languages, similarity of neuronal substrate activation and mental Conclusion A distinct role of inferior conceptualization during oral word temporal gyrus in dominant production might exist. Previous studies hemisphere was proposed, as the word had highlighted the role on anterior and being retrieved from the semantic posterior temporal structures, denoting system. Our study implied mechanism semantic and lexical processing, underlying phonological word respectively. It is often the case that production depends on different, but speech disturbance happening among overlapping areas, in line with the subjects with multi-domain cognitive deafferentation processs from impairment consequent to more neuroimages. On the basis of extensive brain pathology. Herein we category-dependent response, it is presented a case with word retrieval possible the result of more perceptual deficits resulting from focal hemorrhage attributes being allocated during

58 naming animals. Benefit from became more clumsy and his wife also phonological but not semantic cue noted that he became more dullness suggested a novel network, independent and forgetful. He was brought to our to semantic access, parallels major hospital for further evaluation. On cascade representation process. Our examination, he was cooperative and study declared the Chinese phonological orientated. He had good attention and naming process was largely consistent his speech was fluent but slow. His with literatures from other languages. comprehension on single command was preserved. On short-term memory, 63 he only could memorize 5 words after 失用症在皮質基底核退化症:個案研究 3 trials of 10 words list learning study. 徐榮隆 The spontaneous recall and recognition 新光吳火獅紀念醫院神經科 test showed severe deficit. He also Apraxia in corticobasal syndrome: a demonstrated severe impairment on case study constructional ability and frontal lobe Jung-Lung, Hsu Department of Neurology, Shin Kong WHS test such as category verbal fluency Memorial Hospital and Luria 3-steps test. He had a mask Background Apraxia is an uncommon face and his left arm showed slow of neurobehavioral symptom. However, it is motion, increased muscle tone. He often a presenting feature making it as could perform voluntary movement in one of the hallmark signs of corticobasal his right hand but he could not perform syndrome, one of the rare parkinsonism. simple movement in his left hand. His Here we present a clinical case study of left hand could not pantomime apraxia with image findings. brush/smoking movement. The imitation test also failure in his left Case Report A74-year-old retired high hand. He also could not perform school chemistry teacher, a simple gesture in his left hand. right-handedness, was admitted due to However, he could understand the the delusion of infidelity and forgetful for gesture meaning by visual inspection. 6 months. His wife first noted that he had No tremor, myoclonus or alien hand the delusion of infidelity, irritable mood sign noted. His stance was flexion and poor sleep 6 months ago. He was posture and no wide base. His gait was visited a psychiatric clinic and slow, with mild propulsive gait. On anti-psychotic medication was prescribed. image study, brain MRI showed right Two months later, his insomnia became frontal and bilateral parietal atrophy. more prominent and a sleep study was HMPAO-SPECT study also arranged in the other hospital. No REM demonstrated right frontal and bilateral behavior was documented. His gait parietal decreased perfusion.

59 TRODAT-SPECT study showed who presented with mania during decreased the bilateral putamen binding his/her disease courses. The age of the ratio with more severe in the right side. patients is between 79 to 89 years old. The diagnoses of the patients include Conclusion Apraxia in corticobasal right hemisphere infarction (1 case), syndrome is a frequent feature. This dementia and parkinsonism (5 cases). patient demonstrates the limb apraxia in The clinical dementia rating scores of corticobasal syndrome is due to the patients are 2–3. The symptoms of production system defect rather than the mania include decreased need of sleep, concept system or sensory/perceptual being hypertalkative and restless, system lesion. singing, and psychotic features. Some patients show persisted mania and 64 some have fluctuation of mood states. 老年人的次發性躁症: 個案研究與文 All 5 patients have been treated with 獻回顧 valproic acid and most of them show 陳軾正奇美醫療財團法人柳營奇美醫院神經內科 responses.

Secondary Mania in the Elderly: Case Discussion Dementia with Lewy Study and Literature Review Shih-Cheng Chen bodies is suspected in my reviewed Division of neurology, department of internal patients, which is proposed an medicine, Chi Mei medical center, Liouying important etiology of secondary mania Background "primary mania" means a in the elderly. Even low-dose state in bipolar disorder, whereas dopaminergic therapy is at risk to "secondary mania" results from trigger a mania episode in such pharmacological, metabolic, or patients. Lesions involving the right neurologic causes. Mania has been hemisphere and specifically the estimated to represent the cause of 4.6% orbito-frontal, basal temporal and basal to 18.5% of all geriatric psychiatric ganglia areas are associated with admissions. Mania in older patients used mania. Dopamine dysregulation with to manifest with more irritability, post-synaptic hypersensitivity has been confusion, psychosis, and mixed features. implied in the pathogenesis of mania. This syndrome is clinically important Valproic acid seems a reasonable because it is associated with relatively choice to treat secondary mania, as poor outcomes in terms of cognition, long as the hepatic function is morbidity and mortality. preserved. In conclusion, mood disorder is an important part of Case Study 5 cases regularly behavior and psychological symptoms followed-up in my clinic were reviewed, of dementia, and mania should be

60 considered in dementic patients with the ALS center of Taipei City Hospital. insomnia, confusion or psychotic features, Subjects were divided into two groups since the treatment responses differ. depending on whether they used (n = 10) or did not use (n = 10) an 65 eye-tracking communication-assistive 眼控電腦溝通輔具介入對改善運 device. We assessed patients’ quality of 動神經元疾病患者生活品質及照 life and severity of depression using 護者生活壓力之探討 the ALS Specific Quality of Life 黃啟訓 1 翁賀修 1 王麗芬 1 張顥騰 2 1 台北市立聯合醫院忠孝院區神經內科 Instrument-Revised and the Taiwanese 2 中國醫藥大學分子系統生物醫學研究所 Depression Questionnaire, respectively.

The Caregiver Burden Scale was used The application of eye-tracking assistive device for improving the life to assess the burden on caregivers. The quality of ALS patients and the burden data were analyzed using GraphPad on caregivers Prism software. For comparisons Chi-Shin Hwang1, Ho-Hsiu Weng 1, Li-Fen Wang 1, Hao-Teng Chang2 between two groups, the two-tailed 1 Department of Neurology, Taipei City Hospital - Zhong Xiao Branch Student’s t-test was used. 2 Graduate Institute of Molecular Systems Biomedicine, College of Medicine, China Medical University Results The eye-tracking assistive device significantly improved patients’ Objective Amyotrophic lateral sclerosis quality of life, as compared with (ALS) is a relentlessly devastating patients in the non-user group. The neurodegenerative disease characterized assistive device also reduced the by selective loss of motor function. As burden on caregivers. This likely such, ALS patients usually retain a resulted from more-effective normal conscious level, but disease communication between patients and progression eventually results into caregivers. quadriplegia and severe handicap of oral communication. This communication Conclusions Our study shows that an barrier can generate a great deal of stress eye-tracking communication for ALS patients and their caregivers. We technology can reliably improve the aimed, therefore, to know whether the life quality of ALS patients and relieve use of a communication-assistive device the burden on their caregivers. could improve the life quality of ALS patients and relieve the burden on their 66 primary caregivers. 改善 β-澱粉樣蛋白(1～40)誘發學習記憶障礙模式的替代療法 1,2 3 2 3 Methods Twenty quadriplegic severe 葉宗勳 , 謝明村 , 葛其梅 ,吳啟瑞 , 廖俊旺 4, 周寬基 5 handicapped ALS patients with their 1 秀傳紀念醫院神經內科 primary caregivers were collected from 2 國立中興大學生命科學系

61 3 中國醫藥大學藥學院中國藥學暨中藥資源學 hippocampus. Oral administration of 系 4 國立中興大學獸醫病理學研究所 medium and high dose of YCTMT 7 days 5 國立中興大學生物醫學研究所 after the Aβ1-40-infusion decreased amyloid

accumulation area and reversed Alternative therapy on amyloid β 1-40 -induced Alzheimer's disease-like acetylcholine decline in the Aβ1-40-injected phenotype hippocampus, suggesting that YCTMT Chung-Hsin Yeh 1, 2, Ming-Tsuen Hsieh3, Chi-Mei Hsueh2, Chi-Rei Wu3, Jiunn-Wang Liao4, might inhibit Aβ plague accumulation and Kuan-Chih Chow5 rescue reduced acetylcholine expression. 1Department of Neurology Show Chwan Memorial Hospital, Changhua, Taiwan, (ROC) This study has provided evidence on the 2 Graduate Institute of Life Sciences, National beneficial role of YCTMT in ameliorating Chung Hsing University, Taichung 40001, Taiwan, (ROC) amyloid-induced AD-like symptom, 3 School of Chinese Pharmaceutical Sciences and indicating that YCTMT may offer an Chinese Medicine Resources, College of Pharmacy, China Medical University, Taichung alternative strategy for treating AD. 40001, Taiwan, (ROC) 4Department of Veterinary Medicine, National Chung-Hsing University, Taichung 40001, Taiwan, 67 (ROC) 5Graduate Institute of Biomedical Sciences, 晚年代謝症候群預防七十五歲以上 National Chung Hsing University, Taichung 高齡男性的認知功能下降 40001, Taiwan, (ROC) 劉建良 1,2 林明憲 1,2 彭莉甯 1,2 陳亮恭 1,2 蘇千田 3,4 劉力幗 1,2 陳亮宇 1,2 1 國立陽明大學高齡與健康研究中心 Abstract Alzheimer's disease (AD) is an 2 台北榮民總醫院高齡醫學中心高齡醫學科 irreversible neurodegenerative disorder 3 臺北醫學大學附設醫院家庭醫學科 4 characterized by amyloid accumulation, 臺北醫學大學公共衛生暨營養學院

neuronal death and cognitive impairments. Late-Life Metabolic Syndrome YCTMT is one of traditional Chinese Prevents Cognitive Decline among decoction, which has never been used to Older Men Aged 75 Years and Over: enhance cognitive function and treat One-Year Prospective Cohort Study Chien-Liang Liu1,2, Ming-Hsien Lin 1,2, Li-Ning neurodegenerative disorders such as senile Peng 1,2, *Liang-Kung Chen 1,2, Chien-Tien Su3,4, Li-Kuo Liu 1,2, Liang-Yu Chen1,2 dementia. Whether YCTMT has a beneficial 1 Aging and Health Research Center, National role in improving learning and memory in Yang Ming University, Taipei, Taiwan 2 Center for Geriatrics and Gerontology, Taipei AD patients remains unclear. The present Veterans General Hospital, Taipei, Taiwan 3 study showed that oral administration of Department of Family Medicine, Taipei Medical University Hospital YCTMT ameliorated amyloid β 4 School of Public Health, College of Public (Aβ )-injection induced learning and Health and Nutrition, Taipei Medical 1-40 University, Taipei, Taiwan memory impairments in rats, examined using passive avoidance and Morris water-maze Background Although metabolic tests. Immunostaining and western blot syndrome (MetS) has been reported to results showed that continuous be associated with cognitive decline

Aβ1-40-infusion caused amyloid accumulation and dementia, little was known about and decreased acetylcholine level in late-life MetS and cognitive decline

62 among older old population. The main III was a protective factor (OR: 0.20, purpose of this study was to evaluate the 95% CI: 0.04-0.94, P=0.041). The role of MetS and cognitive decline protective effect of MetS remained the among men aged 75 and over in Taiwan. same when MetS was defined by the criteria of International Diabetes Methods This is a prospective cohort Federation. study which recruited men aged 75 years and older with intact cognitive function Conclusions Age and central obesity living in the Banciao Veterans Care were significant risk factors of Home, a retirement community for cognitive decline, but late-life MetS, veterans in northern Taiwan. All however defined, had protective effect participants received complete history on cognitive function. Further taking, physical examinations, global investigation is needed to clarify the cognitive tests and laboratory tests. possible mechanism of MetS and Cognitive status was re-evaluated one cognitive function in older adults. year after enrollment to evaluate the role of MetS to cognitive decline in this study 68 population. 阿茲海默症或庫賈氏症？蕭元淳白明奇國立成功大學醫學院附設醫院神經部暨 Results Overall, 338 people 失智症中心 participated in the study and 62 of them were excluded due to low baseline Alzheimer disease or MMSE score, and the remaining 276 Creutzfeldt-Jacob disease? people (mean age: 82.4±4.2 years) were Yuan-Chun Hsiao, Ming-Chyi Pai Department of Neurology & Alzheimer’s enrolled for study. The prevalence of Disease Research Center, National Cheng MetS and annual cognitive decline were Kung University Hospital, Tainan, Taiwan 22.5% and 15.6%, respectively. During the follow-up period, 9 (3.3%) Case Report A 77 year-old retired participants died, 229(83.0%) complete pharmacist, with elementary education, the study. Subjects with cognitive decline first visited our Dementia Clinic on 26 were older and had lower serum levels of Aug 2011. Episodic memory serum total cholesterol. Multivariate impairment, repetitive queries, logistic regression showed that older age misplacement, spatial navigational (OR:1.13, 95% C.I.: 1.01-1.25, P=0.026) impairment, executive dysfunction, and central obesity (OR: 4.19, 95% CI: change in sense of taste, and stealing 1.26-13.91, P=0.019) were independent delusions were noted by his family for risk factors for cognitive decline; and 2 years or longer which deteriorated in MetS defined by Adult Treatment Panel recent few months. His basic activities of daily living were all independent.

63 Apart from the aforementioned restricted to the cortex (“cortical ribbon symptoms, he had no sleep deprivation, sign”), striatum, or both in DWI and REM sleep behavioral disorders, daytime FLAIR were reported 92% and 94% hypersomnolence, visual hallucinations, respectively. The CSF 14-3-3 testing cognitive fluctuation, unexplained falls, for CJD in recent studies also shows startle response or myoclonus. He had no specificity 48% and specificity 65%. family history of dementia or Our patient has both cortical ribbon Creutzfeldt-Jacob disease (CJD), surgery sign and positive14-3-3 protein. The history or experience of blood absence of family history, blood transfusion or donation and no history of transfusion or traveling abroad made traveling abroad in his life. familial CJD or variant CJD unlikely. His CASI was 57/100 and MMSE 17/30 AD, CJD or even a new variant disease and EEG with diffused intermittent theta is a diagnostic challenge. activities in August 2011. Brain MRI showed cortical ribbon hyperintensities 69 on DWI and FLAIR in bilateral 代謝症候群與 apolipoprotein E 基因 fronto-temporo-parietal lobes and a 多型性於行為監控的角色賴秋蓮 1, 2 劉立民 2 徐崇堯 1, 2 王貞婷 2 diagnosis of CJD or encephalitis was 劉景寬 1, 2 made by our radiologist. 1 高雄醫學大學醫學系神經學科暨碩士班 2 In January 2012, his cognitive function 高雄醫學大學附設醫院神經科 deteriorated with CASI 52/100 and The role of metabolic syndrome and MMSE 17/30, and he was admitted to apolipoprotein E genotypes in action our hospital in March 2012. CSF studies monitoring Chiou-Lian Lai1,2, Li-Min Liou2, Chung-Yao showed no evidence of infection, but Hsu1,2, Chen-Ting Wang2, Ching-Kuan Liu1,2 1 14-3-3 protein was positive. His Department of and Master’s Program in Neurology, Faculty of Medicine, College of mentality got worse after discharge with Medicine, Kaohsiung Medical University 2 Department of Neurology, Kaohsiung CASI 38/100 and MMSE 11/30 in Medical University Hospital August 2012. Brain MRI and EEG were done in June and August 2012 Objective The aims of the present respectively and showed no progression study are to investigate the effect of compared with previous studies. metabolic syndrome and apolipoprotein E (ApoE) genotypes on Conclusion Our patient’s clinical action monitoring in middle-aged and presentation had been in favor of elderly adults without dementia. Alzheimer disease (AD) instead of CJD, Background Action monitoring is an although his cognitive deterioration was important component of executive faster than in typical AD. The sensitivity function. The metabolic syndrome, a and specificity of cortical hyperintensity constellation of cardiovascular and

64 metabolic origin, may be a risk factor for syndrome, ε4 carrier and action cognitive decline. Apolipoprotein E (Apo monitoring in mentally healthy E) is a lipoprotein that transports middle-aged and older adults. cholesterol and other lipids. It is well accepted that ε4 is a risk factor for the 動作障礙學 development of AD, although the ε4 effect is discrepant for cognitive decline 70 in normal elderly. The role of metabolic 高血糖性半側舞蹈症病患運動皮質 syndrome and Apo E genotypes in action 興奮性之改變李介元張秀妘 monitoring needs to be reappraised. 高雄榮民總醫院神經內科

Methods We recruited 89 mentally Changes in motor cortex excitability in patients with hyperglycemic healthy middle-aged and older adults hemichorea-hemiballism (M:F=32:57; age=65.96±5.81 y/o). Jie-Yuan Li, Betty Chang Cognitive assessment (Cognitive Division of Neurology, Kaohsiung Veterans General Hospital Abilities Screening Instrument, CASI and Mini-Mental State Examination, Objective Hemichorea-hemiballism MMSE), flanker task, metabolic (HC-HB) in uncontrolled diabetes syndrome, and Apo E genotypes were mellitus is an uncommon manifestation measured for all participants. of hyperglycemia. The pathophysiology of hyperglycemic Results The percentages of metabolic HC-HB is not well understood. A syndrome and ε4 carrier among all previous report showed increased participants were 34.83 and 17.98, intracortical inhibition in the motor respectively. When the participants were cortex (MC) in a diabetic patient with stratified according to the presence of HC-HB. The objective of this study is metabolic syndrome, there was no to investigate MC excitability in difference in scores of CASI and patients with hyperglycemic HC-HB. MMSE-CE, the percentage of ε4 carriers We hypothesized that intracortical and measurements of flanker task. The ε4 inhibition measured with transcranial carriers did not display significant magnetic stimulation (TMS), which difference in CASI and MMSE scores, likely reflects the excitability of the percentage of metabolic syndrome, cortical GABAergic circuits, would be and measurements of flanker task. impaired in patients with hyperglycemic HC-HB. Conclusion Our findings suggest that there is no association between scores of Methods We studied 15 patients with CASI and MMSE-CE, metabolic mean age 71.5 years (range 48 to 94

65 years) and 12 age-matched healthy Chia-Fang Lee, Li-Kai Tsai Department of Neurology, National Taiwan subjects. The MC contralateral to the University Hospital, Yun-Lin Branch hemichorea was tested. TMS studies Background Huntington’s disease included motor evoked potential, (HD) is characterized by choreiform recruitment curve, GABAA mediated movements, psychiatric problems, and short interval intracortical inhibition dementia. However, chorea is notably (SICI), intracortical facilitation (ICF) and absent but with variable presentations GABAB mediated silent period (SP) including parkinsonism, behavior duration and long interval intracortical problems, and seizure in juvenile HD, inhibition (LICI). also known as Westphal variant HD.

Results There was no significant Case report A 23-year-old woman, difference in motor threshold, without history of systemic diseases, recruitment curve response, SICI, ICF in presented as progressive flattening both rest and active conditions between dysarthric speech and unsteady gait for patients with hyperglycemic HC-HB and 2 years. She also had psychiatric normal subjects. However, LICI was symptoms and depressive mood. She significantly increased during muscle denied usage of illicit drug or activation but not at rest in patients with neuroleptic agents and the family hyperglycemic HC-HB. The SP duration history was unremarkable. is also increased in patients with Neurological examination revealed hyperglycemic HC-HB. prominent extrapyramidal signs, such

as bradyphrenia, rigidity, Conclusion LICI and SP are increased blepharospasm, and torticollis. in the MC contralateral to the hemichorea Monotonous speech with explosive in hyperglycemic HC-HB, but only character, impaired pursuit and during muscle activation. HC-HB may voluntary saccades of eyes, and ataxic be associated with increased GABAB gait were also noted. Brain MRI receptor mediated inhibitory activity in showed diffuse cortical atrophy. the MC. Laboratory data showed a low

ceruloplasmin level of 16.8 mg/dL 71 初始表現類似威爾森氏症的早發型亨 (Norm, 20-60) with normal serum and 丁頓舞蹈症之一病例報告 urine copper level. She further received 李佳舫蔡力凱 serial studies for diagnosis of Wilson’s 台大醫院雲林分院神經部 disease. However, the results of liver

function tests and slip-lamp A Case of Juvenile Huntington’s examination were normal. Liver biopsy disease with Initial Presentations Mimicking Wilson’s disease showed no copper accumulation. The

66 results of autoimmune survey, thyroid electrophysiologically similar to those function, and anti-HIV were all within seen in painful legs and moving toes normal limits. The genetic results for syndrome, but without any associated SCA (Spinocerebellar ataxia) 1, 2, 3, and pain or predisposing factors. 17 were negative. Finally, the genetic study confirmed the diagnosis of HD Case Report A 14-year-old girl with 17/59 CAG repeats in HTT gene. presented with involuntary movements of her toes of the left foot without any Conclusion Juvenile HD is clinically associated pain from 6 months ago. distinct from classic HD and may be The movements were not related to the difficult to be differentiated from other time of day and persisted during sleep. neurodegenerative disorders, such as Besides, the movements could be Wilson’s disease. Genetic testing is suppressed for a moment but would essential in diagnosis. begin again when she focused on other things. She didn’t have urges to move 72 her legs or walk around. She didn’t 不痛的腿及移動腳趾之個案報告 have any past history of trauma or 1 2,3 李杰勳李介元 family history of movement disorder. 1 寶建醫院神經內科 2 高雄榮民總醫院神經內科 She denied taking any psychotropic 3 國立陽明大學醫學院醫學系 agents in the past. Neurological

examination revealed no abnormal A Case Report of Painless Legs and Moving Toes Syndrome finding except the involuntary 1 2,3 Chieh-Hsun Lee , Jie-Yuan Li movements of the toes. The 1 Division of Neurology, Pao-Chien Hospital, Pingtung, Taiwan, movements were low frequency, 2 Division of Neurology, Kaohsiung Veterans semirhythmic and flexion-extension, General Hospital, Kaohsiung, Taiwan, 3Faculty of Medicine, School of Medicine, National with a sinusoidal, wriggling and Yang-Ming University, Taipei, Taiwan quivering appearance. Blood analyses

and brain CT scan were normal. An Background Painful legs and moving electromyography and nerve toes is a rare syndrome consists of conduction studies revealed no continuous or semicontinuous evidence of lumbosacral radiculopathy involuntary movements of toes or peripheral neuropathy. MRI of associated with pain in the affected lumbosacral spine was normal. extremity. With respect to the painless variant, very few patients have been Conclusions Painless legs and reported and most of them were moving toes is a very rare syndrome. idiopathic. We report a patient with Until now, only 10 cases of painless 6-month history of involuntary legs and moving toes syndrome were movements of the toes clinically and

67 reported. To our knowledge, this is the was not performed for hydrocephalus eleventh reported case. because of old age.

73 Conclusion Like previous case 急性硬膜下出血合併雙側肢體舞蹈症 reports, our patient suggests the 林奇模張揚沛施邦英賴秋蓮 paraballism may be a clinical 高雄醫學大學附設中和醫院神經內科 manifestation of acute subdural Acute subdural hemorrhage hemorrhage. presenting as paraballism Chi-Mo Lin, Yang-Pei Chang, Pang-Ying Shih, Chiou-Lian Lai 74 Department of Neurology, Kaohsiung Medical 以反應緩慢及步態異常做為臨床表 University Hospital 現的MELAS粒腺體基因突變個案

林邵臻陳瓊美張國軒 Background Rarely, chorea may be a 林口長庚紀念醫院神經內科 presentation of subdural hemorrhage which is shown on MRI imaging. A patient with slow responsiveness and gait disturbance carrying MELAS mitochondrial gene Case Report A 84-year-old single mutation woman was admitted due to accidental Shao-Cheng Lin, Chiung-Mei Chen, Kuuo-Hsuan Chang falling down and head injury. There were Department of Neurology, Chang-Gung Memorial Hospital and University College of no obvious focal neurological sign at Medicine, LinKou Medical Center emergency department. Brain CT revealed skull laceration and falx Background and Objective subdural hemorrhage. Acute onset of Mitochondrial DNA 3243 A>G intermittent bilateral limb mutation has demonstrated a strong large-amplitude and violent movement association with mitochondrial was noted three days later. In addition, myopathy, encephalopathy, lactic she could not control her movement and acidosis, strokes syndrome (MELAS). multiple bruise was found over four However, a number of patients limbs. Neurological examination was carrying this mutation developed other essentially normal except for paraballism neurological features, such as over four limbs. Brain CT revealed mild bradykinesia, gait disturbance and hydrocephalus and no progression of dyskinesia as reported below. subdural hemorrhage. Limb movement improved gradually 4 days later. Her past Case report A female diabetic patient history included hypertension, hepatitis B, began to develop progressive slow chronic kidney disease. Brain MRI was responsiveness, gait disturbance and not able to perform due to large right hand dyskinesia at the age of 20 amplitude of limb movement. Operation years old. Bilateral hearing impairment

68 was found two years later. Headache, stroke, visual impairment, seizure, Conclusion The m.3243 A>G mental or other developmental MELAS mutation can be found in retardation were absent. She described a young-onset movement disorder family history of young-onset diabetes patients especially with basal ganglion mellitus and hearing impairment, which calcifications and diffuse cortical is noted in her mother and uncle. hypoperfusion. She was in a relatively small-sized body picture with a height of 143.5 cm and a 75 weight of 35 kg. The neurological FBXO7 基因在台灣巴金森症病患之 examinations revealed bilateral 角色林靜嫻陳孟伶戴春暉吳瑞美 bradykinesia, shuffling gait and dystonic 臺大醫院神經部 movements in her right hand. She had elevated HbA1c (14.7%). Her serum Mutational analysis of FBXO7 gene in Parkinson's disease in a levels of calcium, inorganic phosphate, Taiwanese population copper, T3, T4, intact-PTH and Chin-Hsien Lin, Meng-Ling Chen, Chun-Hwei Tai, Ruey-Meei Wu ceruloplasmin were normal. Bilateral Department of Neurology, National Taiwan peripheral sensori-hearing blockages University Hospital were found by brain stem evoked Objective To investigate the mutation potential study. Calcification of bilateral frequency of FBXO7 in patients with caudate heads, lentiform nuclei and Parkinson’s disease (PD) in Taiwan. thalamus, and diffuse cerebral and cerebellar cortical atrophy were shown Background The by head computed tomography and ubiquitin-proteasome pathway plays a magnetic resonance imaging studies. Her major role in the degradation of Tc99m-ethyl cysteinate dimer single mis-folded or regulatory proteins in photon emission computed tomography cells. Mutations in the FBXO7 gene (SPECT) study showed decreased (PARK15), which protein is a perfusion of bilateral cerebral cortex. component of ubiquitin E3 ligase, However, no presynaptic dopaminergic cause an autosomal-recessive lesion was found by Tc99M-TRODAT early-onset parkinsonism with dopamine transporter SPECT study. pyramidal tract signs. The information Results from genetic test found the regarding FBXO7 in risk of PD in m.3243A>G MELAS gene mutation. Her other ethnicity is limited. slow response, gait disturbance and right hand dystonia were mildly improved by the treatment with low dose sinemet (200 Methods We assayed FBXO7 gene in mg per day). a total of 900 participants comprising

69 448 PD patients and 452 age/gender matched controls from Taiwan. The Background Bilateral subthalamic entire FBXO7 coding region and nucleus deep brain stimulation intron-exon boundaries were sequenced. (STN-DBS) is the choice of treatment for motor complications of Parkinson Results We identified two novel disease. In previous reports, some missense substitutions, p.Ile87Thr and cases developed stimulation and p.Asp328Arg, in a single heterozygous medication insensitive dyskinesia soon state in two early-onset PD (EOPD) after implanting the electrodes. The patients individually (1.1% in EOPD). dyskinesia resolved spontaneously in These two variants were not observed in less than 12 weeks. Here we report a controls with total 904 normal alleles. case presents different pattern of Additionally, we also found one dyskinesia after the surgery. non-coding variant, exon 1 IVS-329C>T, modestly associated with PD. The Case report A 69 years old female is frequency of CT/TT genotype was higher a case of Parkinson’s disease for 13 in PD patients compared with controls years with the initial presentation of (OR=1.43, 1.02-2.01, P=0.04). The tremor and rigidity in the left limbs. clinical phenotypes of genetic variant She underwent bilateral STN-DBS due carriers are similar to that seen in to rigidity and intractable neck idiopathic PD. dystonia. Pre-operation UPDRS scores were 43.5 at on phase and 57 at off Conclusion We conclude that FBXO7 phase. Dyskinesia rating scale before gene contributes little to typical PD in operation in medication on and off was our population. Further studies in other 0. There was no complication in the ethnic cohorts will be important to perioperative period, and the address its potential pathophysiologic implantable pulse generator (IPG) was role in PD. installed. However, she developed dyskinesias in the right leg 2 weeks 76 later without turning on the stimulator. 視丘下核深腦刺激術後持續過久之異 Brain MRI disclosed neither 動症 microbleed nor malposition of the 翁儀靜林衛邑陳瓊珠陸清松林口長庚紀念醫院神經內科 electrodes. The stimulation was switched on 2 weeks later. The Prolonged dyskinesia after bilateral dyskinesia was chorea like and STN-DBS Yi-Ching Weng, Wey-Yil Lin, Chiung-Chu Chen, persisted. There was also some Chin-Song Lu interference in walking and daily living Department of Neurology, Chang Gung Memorial Hospital Linkou, Chang Gung University due to chorea. The dyskinesia rating

scale in medication on and off was 3. At Management, Taipei Medical University.

the same time, right side rigidity and Objective To examine the immediate bradykinesia improved as left side and no (within 1 week) and short-term (at 4 tremor. The dyskinesias were not altered weeks) effects of a 12-week treadmill by stimulation on, stimulation off, exercise training program on backward changing stimulation settings, reducing walking (BW) gait in patients with levodopa (from 800 down to 400mg per early stage Parkinson’s disease (PD). day) or stopping all levodopa. The

dyskinesias were not improved by Background Treadmill exercise biperiden, amantadine, clonazepam or training for PD patients improves haloperidol. The dreadful dyskinesia in forward walking (FW) gait; however, the right leg disappeared spontaneously 6 training effects on BW gait are still months later. undetermined.

Conclusion The patient developed Methods Sixteen patients with early prolonged dyskinesia after bilateral PD (8 males, 8 females) received a STN-DBS. The dyskinesia could not be 12-week (36 sessions) exercise training relieved by medication or changing the program by walking forwards on parameters of stimulation. The duration ® treadmill. A GAITRite walkway was of complicated dyskinesias after IPG used to quantify the spatial and implantation was even longer than those temporal parameters of BW and FW reported in the literature described as gait before training, within 1 week and long dyskinesia. The underlying at the 4th week after treadmill training pathogenic mechanisms are warranted for was completed. A repeated measures further exploration design was applied to compare among

pre- and post-training gait in BW and 77 FW. 跑步機運動訓練改善巴金森氏病病患

往後步態之成效袁瑞昱1,2曾櫻枝3 Results After training, PD 1臺北醫學大學醫學院醫學系神經學科 participants walked backwards and 2臺北醫學大學附設醫院神經內科 3 forwards with increased velocity, 臺北醫學大學護理學院老人護理暨管理學系 enlarged stride length, prolonged Effects of treadmill exercise training swing phase, and decreased double on backward gait in early Parkinson's support phase. These BW and FW gait disease Rey-Yue Yuan1,2Ing-Jy Tseng 3 improvements were seen within 1 1 Department of Neurology, College of Medicine, th Taipei Medical University. week and at the 4 week after training 2 Department of Neurology, Taipei Medical was completed. Moreover, University Hospital. 3School of Geriatric Nursing and Care post-training swing time and stride

71 length became more consistent in implicated in serotonin syndrome . We backward and forward walking; the present a case of serotonin syndrome, swing time variability and stride length shortly after combined use of tramadol, variability in BW and FW were reduced levodopa, and dextromethorphan. within 1 week and at the 4th week after training. Post-training gait improvements Case Report A 86-year-old man, with in BW and FW were comparable. history of chronic pulmonary However, cadence did not differ among obstructive disease, was brought to pre and post-training gait in BW and FW. emergent department due to change in consciousness and generalized Conclusion These data extend the myoclonus, one day after discharged current knowledge, indicating that a from chest care ward. 12-week treadmill exercise training Dextromethorphan (120 mg/d) was program in forward walking improves regularly used chronically. Newly both BW and FW gait for up to 4 weeks added tramadol(94.5mg/day) and duration in early PD patients. Treadmill levodopa( 300mg/d) were used for few exercise training can be considered part days. At ED, high fever to 40°C, of a rehabilitation strategy to overcome agitation followed by severe gait disturbances of PD in both walking hypotension(SBP/DBP:62/44 mmHg) directions. were noted. Stimulus sensitive generalized myoclonus occurred in 78 trunk and limbs simultaneously. 合併使用 Tramadol, Initially, midazolam( 5mg) was Dextromethorphan, Levodopa-引起血 administered for agitation and 清素症候群-個案報告 hyperthermia. 許家綸吳亞縈 12 葉建宏新光吳火獅紀念醫院神經科 Laboratory investigation revealed leukocytosis with left shift, elevated Serotonin syndrome resulting from hepatic enzyme (GPT:61U/L) and CPK coadministration of tramadol, levodopa and dextromethorphan - level (856U/L). There was no Case report infectious source. A presumptive Chia-Lin Hsu, MD1, Ya-Ying Wu, MD12, Jiann-Horng Yeh,MD 1 diagnosis of serotonin syndrome was 1 Department of Neurology, Shin Kong Wu Ho-Su made. The offending drugs were memorial Hospital, Taipei, Taiwan 2 Institute of Brain Science, National Yang-Ming discontinued, and oral cyproheptadine University, Taipei, Taiwan was administered (12mg, followed by

4mg, Q6h for one day, then taper to Background Serotonin syndrome is 4mg Q8h for a day). The next day, the induced by several drugs interaction, agitation, myoclonus and such as SSRI, SNRI, antiemetics, and consciousness improved gradually. MAOI. Drug interactions are commonly

72 4 Department of Neurology, Chang Gung Memorial Hospital Discussion This is the first case report of serotonin syndrome caused by Background McLeod syndrome is a combination of tramadol, levodopa, rare subtype of neuroacanthocytosis. It dextromethorphan. Tramadol inhibits is an X-liked multisystem disorder reuptake of serotonin and involving central nervous system, norepinerphrine.Dextromethorphan binds neuromuscular, hematologic, to serotonergic receptors and could cardiovascular and liver function. The induce serotonin syndrome by genetic cause is mutations of the XK conventional dose. Levodopa is proposed gene. to involve displacement of endogenous serotonin into the synaptic cleft. Case report A 67-year-old male first Serotonin syndrome is far more common presented with paranoid persecutive to be caused by a combination of drugs delusion, anxiety and depression at the that act at different 5 HT receptor sites. age of 48. Abnormal liver function was We want to emphasize high risk of noted at the age of 56 but serology of developing serotonin syndrome after hepatitis B and C were negative. combination of these commonly used Arrhythmia and coronary artery drugs, especially in the elderly. Early disease were diagnosed at the age of 58. diagnosis is crucial. Myalgia, fatigue and muscle wasting developed since he was 62 years old. 79 Motor restlessness, choreoathetosis, McLeod syndrome:以神經精神症狀為 grimacing face, shoulder shrugging, 起始表現且經基因證實之台灣第一位 tics, tongue biting and unsteady gait 病例報告 1 陳珮昀 1 甄瑞興 2 楊智超 2 李銘仁 3 邱延慧 4 gradually developed at the age of 63. 賴思佳 4 葉篤學 4 陸清松 Laboratory investigations were 1 台北市立聯合醫院仁愛院區神經內科 2 台灣大學附設醫院神經內科 unremarkable except marked serum 3 台北市立聯合醫院教學研究部 creating kinase elevation (602-3328 4 林口長庚紀念醫院神經內科 U/L; normal, <190 U/L). Nerve

McLeod syndrome: the first conduction studies and genetic-proved Taiwanese case with electromyography revealed sensory initial neuropsychiatric presentation predominant axonal polyneuropathy. 1Pei-Yun Chen, MD, 1Sui-Hing Yan, MD, 2Chih-Chao Yang, 2Ming-Jen Lee, 3Yen-Hui Chiu, Brain computed tomography and 4 4 Szu-Chia Lai, MD, Tu-Hsueh Yeh, MD, PhD, magnetic resonance image were 4Chin-Song Lu, MD 1 Department of Neurology, Taipei City Hospital, unremarkable. The peripheral blood RenAi branch 2 Department of Neurology, National Taiwan smear revealed 36% acanthocytes. University Hospital Genetic testing of the patient showed a 3 Department of Education and Research, Taipei City Hospital novel nonsense mutation (c.154C>T;

73 p.Gln52X) at the exon 1 of KX gene. His Institute of Neural and Cognitive Sciences, China Medical University4, Taiwan elder brother had epilepsy history and a special serology phenotype-McLeod Background Patients with phenotype was found incidentally when Dopa-responsive dystonia (DRD) had he underwent hip joint surgery. been shown to be caused by 5 different mutations, including Ser81Pro, Ser76X, Conclusion To the best of our Gly203Arg, 249delA and IVS5+3insT, knowledge, this is the first in the GCH1 gene in Taiwan. Herein, genetic-proved Taiwanese report of we report a family with DRD with a McLeod syndrome. The collecting mutation of Arg 88Leu at exon 1b. The phenotype of idiopathic movement family extended the DRD genotypic disorder, psychiatric symptom, peripheral spectrum of the country. neuropathy, associated with markedly elevated creating kinase indicates the Subject and Methods The necessity of erythrocyte morphology and 24-year-old woman developed feet serology test for Kell blood group clawing with slow wiggling movement antigen/antibody and genetic test for XK since her 15 years. The symptoms gene mutation. Multidisciplinary would interfere with her running or management including genetic even walking. The foot symptoms counseling, surveillance for would also hinder her from falling cardiovascular, hepatic, neuromuscular, asleep normally and she usually had to epileptogenic symptoms, as well as put her soles against the bed plate so as banked autologous blood and to suppress the involuntary movements Kx-negative blood are important for at bedtime. Sleep benefit for around 1 patient care. hour was traced. Her symptoms were exacerbated a bit in recent 2 years. 80 Neurological examination revealed feet GTP cyclohydrolase 1 (GCH1) Exon 1b dystonic movement. There was no Arg88Leu 突變所致之多巴胺反應型肌 akinesia, rigidity or tremor. Her feet 張力不全症家族 dystonia responded to sinemet in daily 楊程智 1 劉彥良 3 陸清松 2 賴思佳 2 劉瑞貞 1 呂明桂 1 蔡崇豪 1,4 dose of 150 mg well. Her father and 2 1 2 3 中國醫藥大學附設醫院林口長庚醫院 uncles have tremor of the limbs and 署立彰化醫院神經內科 probably her 2 grandaunts also had A Family of Dopa Responsive Dystonia Caused by Arg88Leu at GTP limbs tremor. No neurological cyclohydrolase 1 (GCH1) gene exon 1b symptom could be traced of her 2 elder

CC Yang1, YL Liu3, CS Lu2, SC Lai2, JJ Liu1, MK sisters. Direct sequencing of the Lu1, CH Tsai1,2 GCH1Gene revealed a point mutation Department of Neurology, China Medical University Hospital1, Chang Gung Memorial of Arg 88Leu at exon 1b of the index Hospital2, Chang Hua Hospital3; Graduate

74 case, her father and asymptomatic eldest percent of total body. Tetraplegia and sister. urinary retention were found after Conclusion The current report extends recovery from rhabdomyolysis with the DRD genotypic spectrum in Taiwan. acute renal failure three days later .The In the genetically proved cases, the neurological examination showed phenotypes are variable and range from paraplegia and decreased muscle asymptomatic, tremor to full blown strength with Medical Research features of dystonia. The phenomenon council of Great Britain (MRC) grade raises the caution for the neurologists on 1/5 in both arms and 4/5 in distal part visiting the possible candidates in of upper limbs, general areflexia and practice, especially in young patients. mild reduction of pain sensation to pinprick below fourth thoracic level and loss of vibration sensation over 神經肌病學 legs. The T2 weighted imaging of 81 spinal magnetic resonance study 閃電擊傷造成之脊髓病變併周邊神經 revealed petechial hemorrhage and 病變 edema over bilateral lateral cervical 林君賢郭弘周 cord. The motor and sensory evoked 林口長庚紀念醫院神經內科 potential studies demonstrated cervical Lightning strike with dorsolateral cord myelopathies. The nerve conduction syndrome and peripheral neuropathies studies revealed bilateral sciatic Chun-Hsien Lin, Hung-Chou Kuo Department of Neurology, Chang Gung Memorial neuropathies that worse in right side Hospital, Lin-Kou Medical Center and needle electromyogram showed marked spontaneous activities in all Background Spinal cord injury tested muscles. The nerves of upper following electrical trauma was rarely limbs were relatively spare on the reported. We present an unusual clinical electrophysiological studies. manifestation with myelopathy associated with severe peripheral nerves Conclusion The mechanism of injuries in a patient struck by lightning. neurological damage following electrical injury remains unclear. In the Case report There is a 61 year old literature review, the lesion caused by male got lightning strike during mountain lightning injury was reported along climbing. The lighting caused inlet neuraxis, from cerebral cortex, wound on right occipital scalp and outlet cerebellar cortex, spinal cord and wound over right sacrum, lateral aspect peripheral nerve. In view of our patient, of right distal leg and foot and left the large myelinating fibers of central metatarsal-phalangeal regions and and peripheral nervous system are resulted in burning injury of seven

75 labile to electrical injury. In addition to about the nerve pathohistologic motor neuron syndrome or analysis of CMT1A and HNPP in rhabdomyolysis, the neurological literatures, but no reports focus on their complication is dependent on the entry regenerative myelinated fibers. and direction of lightning electrical transduction that descending from Materials and Methods The study cervical through lumbosacral cord to recruited 5 cases with PMP22 peripheral nerves of lower limbs. duplication, 6 cases with PMP22 deletion and 6 control subjects. 82 PMP22 gene duplication or deletion PMP22 基因數目對再生髓鞘神經纖維 was confirmed by “hot spot” detection 的形態學影響 by polymerase chain reaction (PCR) 林恭平李宜中臺北榮民總醫院神經醫學中心 with allele-specific primers or microsatellites methods. Their bioptic Morphometric Analysis of specimens of sural nerve were Regenerative Myelinated Fibers examined by light microscope, Related to Dosage Effects of PMP22 Gene electromicroscope, teased single fiber Kon-Ping Lin, Yi-Chung Lee and histopathological morphometric Neurological Institute, Taipei-Veterans General Hospital analysis.

ABSTRACT Results The results showed the Objective We will examine the patients with PMP22 duplication, morphological changes of regenerative whose cluster ratio, 9.51, 6.9, 16.7, myelinated fibers(RMF)in sural nerve 12.51 and 6.75, mean axon diameters biopsies from patients with PMP22 of RMF, 1.83±0.65μm, 2.54±1.06μm, duplication (Charcot-Marie-Tooth 3.03±1.11μm, 3.06±1.26μm and disease type 1A, CMT1A), PMP 22 3.07±0.97μm, and g-ratio, 0.38±0.11, deletion (Hereditary Neuropathy with 0.51±0.12, 0.62±0.11, 0.51±0.12 and Liability to Pressure, HNPP) and normal 0.57±0.13 respectively. G-ratio plot subjects. Try to understand the dosage axon diameter scatter gram revealed effects of PMP22 gene on the RMF distributed out of the AIII fibers pathohistologically morphometric range to the AII fibers range; The parameters of their regenerative patients with PMP22 deletion, whose myelinated nerves such as cluster ratio cluster ratio, 0, 0, 0.48, 0, 1.03, 2.24, (cluster number per 1000 myelinated mean axon diameters of RMF, 0, 0, fibers), axon diameter, g-ratio (axon 1.8±0.38μm,0,3.06±0.95μm, and diameter/fiber diameter) and scatter plot 3.09±1, and g-ratio, 0, 0, 0.52±0.08, 0, of g-ratio to axon diameter. 0.65±0.09, 0.71±0.09 and 0.51±0.12 Background There were many reports

76 respectively. G-ratio plot axon diameter neuropathy other than diabetics may scatter gram revealed RMF distributed also superimpose on an existing within the AIII fibers range. The diabetic neuropathy. normal subjects whose cluster ratio, 0, 3.14, 1.75, 7.13, 7.42 and 7.63, mean Case report A 65-year-old male axon diameters of RMF, 0, 2.4±0.93μm, patient with 10 years type II diabetes 2.0±0.85μm,1.64±0.58μm,2.44±1.07μm, mellitus history had suffered from and 1.6±0.28μm, and g-ratio, 0, insidious onset unsteady and feet 0.62±0.09, 0.58±0.1, 0.59±0.1, 0.69±0.08 numbness for 3 years. The numbness and 0.53±0.06 respectively. G-ratio plot was exacerbating, ascending from toes axon diameter scatter gram revealed to ankles with symmetric distribution. RMF distributed within the AIII fibers The neurological examination showed range as well. The cluster ratio, mean generalized reduced reflexes and axon diameters and g-ratio showed no absent ankles jerks. The muscle power, statistically significance among these 3 pin-prick and vibration sensation were groups of subjects. also diminished distally. Nerve conductions and electromyographic Conclusions The pathohistologically study indicated a generalized morphometric anaylsis of RMF in sensorimotor peripheral neuropathy. subjects with different PMP22 gene Under the impression of dosage showed the decompensation of polyneuropathy, a detail neuropathic nerve regeneration in PMP22 duplication risk factors survey, including (CMT1A), but normal in PMP 22 immunological, oncological, and deletion (HNPP) and normal subjects toxins screen, were done. The tumor marker screen showed elevated PSA 83 level. Urology surgical intervention 癌症導致之周邊神經病變發生在糖尿 was performed and pathology proved 病病患 to be a prostate adenocarcinoma. After 林舜文吳嘉倫基隆長庚醫院神經內科 the cancer removal surgery, the neuropathic sign/symptoms, including Paraneoplastic neuropathy in a distal weakness and numbness, all diabetic patient Shun-Wen Lin, Chia-Lun Wu showed dramatically improvement. Department of Neurology, Chang Gung Memorial The final diagnosis was paraneoplastic Hospital, Keelung neuropathy, possible superimposed on Back ground Peripheral neuropathy is a diabetic neuropathy. The pathology a major complication of diabetes mellitus, of paraneoplastic neuropathy was but any other causes of neuropathy could thought to be antibody direct against also happen in diabetic patients. A intra-cellular antigen in the cytoplasm

77 or neucleus in the neurons or and limb weakness. NCS showed extra-cellular antigen such as membrane sensory and motor axonal channel. polyneuropathy, with the motor nerves more severely involved. Hallucination Conclusion Peripheral neuropathy and generalized tonic–clonic convulsion caused by any etiology could be episodes were also noted after admission. happened, or superimposed on an Her abdominal pain score, existing neuropathy, in diabetic patients. neuropsychiatric manifestations, and Wild range screen studies are essential porphyrin biochemical data (2+, ALA: for the definite diagnosis. Even the 70.91 mg/dL [reference values: 1.3–7 pathogenesis of paraneoplastic mg/dL], PBG: 184.87 mg/dL [reference neuropathies are immune mediated, values: 0-2 mg/dL)] obtained from results of conventional immunotherapy 24-hour urine collection and axonal are always disappointed. The most polyneuropathy were compatible with effective treatment of paraneoplastic those for AIP. After hematin use, she neuropathy is removal of the underlying demonstrated rapid clinical and malignancy. biochemical improvement (including decreased urine levels of porphyrin, 84 PBG, and ALA). Acute pain was Case report: CPT test 評估 Gabapentin relieved by analgesics such as morphine 治療紫質症神經痛的成效 and meperidine. Unfortunately, she 林鼎鈞賴曉玲黃彥筑李建德羅榮昇嘉義長庚紀念醫院神經內科 developed tolerance to morphine and meperidine. The chronic neuropathy pain Concurrent perception threshold test was not well controlled. We designed a for the evaluation of neuropathic pain in acute intermittent porphyria study to observe the effect of gabapentin Ting-Chun Lin, Shiao-Lin Lai, Yen-Chu Huang, on pain relief using CPT testing and Jiann-Der Lee, Long-Sun Ro Department of Neurology, Chang Gung visual analogue scales. Gabapentin was Memorial Hospital, Chiayi started at 300 mg/day and was slowly

titrated to 1800 mg/day. After Background To our knowledge, gabapentin use, the necessity of gabapentin treatment and using CPT tests morphine or meperidine for acute pain to monitor neuropathic pain severity in decreased, fewer seizure episodes AIP patients has not been reported. We occurred, and improvement in limb pain used CPT testing to investigate the was observed. There was a probable mechanisms and effects of dose-dependent trend in current gabapentin on AIP neuropathic pain. perception thresholds using a 2000-,

250- and 5-Hz stimulus. (Table1 and Case Report A 28-year-old woman was Figure 1) admitted to a hospital for abdominal pain

78 Conclusion CPT tests could provide 86 information regarding the function of 胃切除手術後之神經學併發症 different sized fibers in peripheral 曹維珈郭弘周林口長庚紀念醫院神經內科 neuropathy associated with gabapentin use. The results obtained in this study Neurological complication after may provide an alternative for the gastric surgery: a report of two cases Wei-Chia Tsao, Hung-Chou Kuo evaluation and management of Department of Neurology, Chang Gung neuropathic pain in AIP patients. Memorial Hospital, Lin-Kou Medical Center

Background The neurological Table 1: Current Perception complication following gastric surgery Threshold (CPT) test data after is rare. We described two patients had gabapentin use severe neurological complications both Dose 0 300 600 120 1800 in central and peripheral nervous systems after gastric surgery. The VAS 9 9 8 8 7 clinical picture, brain MRI and 2000Hz 152 104 228 172 348 electrophysiological studies were also 250Hz 69 46 72 65 174 presented. 5Hz 47 23 53 53 133 Case Report The first patient was a 33-year old woman who received Roux-en-Y surgery for morbid obesity six years before. Cervical myelitis-like syndrome, and an episode of amnesic syndrome were noted about 2 years after operation. This time she suffered from recurrent myelitis. Only relative low normal limitation of serum level in

folate and 25-hydroxyvitamin D were

noted. Her muscle strength had no Fig. 1. The effect of gabapentin on response to pulse therapy, besides, current perception thresholds of the right nausea/vomiting developed after pulse index finger in patients with porphyria therapy. Nerve conduction study and neuropathic pain. There is a electromyography showed an acute dose-dependent trend in current motor axonal neuropathy. Intravenous perception thresholds using a 2000-, 250- vitamin B complex for 2 weeks as an and 5-Hz stimulus. intervention for acute stage and then

oral multiple vitamin B was supplied

for long team management. The

79 neurological symptom was much better 郭芷毓張宏旭呂榮國林口長庚紀念醫院神經內科 during the 6 months follow-up period. The second patient was a 66 year-old Concomitant Involvement of the man who undergone subtotal gastrectomy Central and the Peripheral Nerve Systems in a Female with and Billroth II anastomosis 14 months Undetermined Etiology before for gastric signet ring cell Chih-Yu Kuo, Hung-Hsu Chang, Rong-Kuo Lyu carcinoma. Weight loss of 6 kilograms in Department of Neurology, Chang Gung 2 months related to poor appetite was Memorial Hospital, Lin-Kou Medical Center reported. He got gradual progression of Background We describe a case with weakness over legs for one month and a dominant clinical feature of sensory rapid progression of limbs weakness ataxia. Electrophysiology studies within a week, proceeding to stupor, suggested involvement of the tetraplegia and ophthalmoplegia. peripheral nervous system. A Plasmaphoresis was arranged on the subsequent spinal MRI revealed assumption of demyelinating diseases. non-enhanced lesions in the posterior Brain magnetic resonance imaging (MRI) column of the spinal cord. Extensive revealed non-alcoholic Wernicke’s investigation showed no evidence of encephalopathy, and vitamin B complex infections, neoplasms orautoimmune was given. Nerve conduction study disorders. A transient improvement to showed severe axonal polyneuropathy. intravenous immunoglobulin infusion His neurological condition also got was noted but the symptoms and signs improvement during the 6 months otherwise persisted in spite of follow-up period. immunosuppressive or

immunomodulative treatment. Conclusion We should put suspicion of nutritional deficiency in patients who had Case Report A 32-year-old female acute severe neurological syndromes complained acute onset followed by involving both central and peripheral slowly progressive ascending nervous systems after receiving gastric numbness. This was followed by mild surgery several years later, especially weakness of four limbs. After who had concomitant gastrointestinal admission, her condition deteriorated symptoms and weight loss. Intensive and became dependent in standing or multiple vitamin B and balanced ambulation. nutrition supplement could be benefit for Physiological examinations were them. unremarkable. Neurological

examinations revealed no cranial nerve 87 侷限性中樞暨周邊神經病變：不典型特 or cerebellar dysfunction. There was 殊個案討論 asymmetrical and mild weakness

80 without muscle wasting in the four limbs. Discussion We report an unusual Babinski sign was negative. Sequential case who had a disorder involving the sensory tests revealed progressive peripheral and central proprioceptive deterioration of the proprioceptive systems. No identified causes were perception while the nociception was found in spite of extensive evaluation. relatively spared. There were diffuse Although the response of therapies hyporeflexia, pseudoathetosis, a positive targeting on dysimmune diseases was Romberg test and sensory ataxia. unsatisfactory, we thought autoimmue Routine hemogram and blood chemistry factors, either humoral or cellar, may were unremarkable. There were also underlie the pathogenesis because of negative findings of endocrine studies, the specificity of the affected tissues. selective virus antibodies, autoimmue, infection disease and tumor markers. 88 Serum and CSF protein electrophoresis 僵直性肌陣攣運動失調症-個案報告 were negative for paraproteins or 郭詠怡甄瑞興王毓禛陳珮昀林怡君台北市立聯合醫院仁愛院區神經內科 monoclonal gamma globulins. A routine CSF study showed marked elevation of Opsoclonus myoclonus ataxia – protein concentration. Nutrient and toxin a case report Wing-Yee Kwok, Sui-Hing Yan, Yuh-Jen Wang, survey were negative. No abnormality Pei-Yun Chen, Yi-Chun Lin was detected in a whole body CT. Department of Neurology, Taipei City Hospital Renai Branch Electrophysiological studies showed absence or marked amplitude decrease of Background Opsoclonus myoclonus sensory nerve action potential in the four ataxia (OMA) is a rare neurological limbs and were compatible with sensory disorder. It appears to be the result of dominant polyneuropathy or sensory an autoimmune process involving the neuronopathy. Autoimmune mediated nervous system that includes neuropathy was suspected. Steroid, opsoclonus along with diffuse or focal intravenous immunoglobulin infusion body myoclonus with or without ataxia and plasmapharesis were given with a and other cerebellar signs. OMA in transient partial response but overall, adults may be paraneoplastic , there was no significant clinical parainfectious or idiopathic in orgin. improvement. In spite of clinical stationarity, there was gradual Case Report A 50-year-old female improvement of electrophysiological was admitted due to sudden onset of abnormality of nerve functions. A spinal blurred vision when she tried to fix her MRI revealed long-segmental (C1 to T4) vision on a focal object in the early lesions without enhancement in the morning after she arrived from the U.S. th posterior column. on 27 September, 2012. The patient

81 had an URI attack one week before . On disease with anti-neutrophil neurological examination ,conjugated cytoplasmic antibody dancing eyeballs was detected in (ANCA)-associated vasculitis and different direction , and the condition granulomatous inflammation. We will was enhanced if the patient focused on give an overview of the clinical one object. The patient also had manifestations of the peripheral paroxysmal left leg myoclonus and nervous system. unstable gait one week later . Lumbar puncture showed inflammatory pattern Methods Retrospective analysis of with CSF WBC 15, glucose 87, protein: reviewed the medical records of all 50, LDH: 126. The result of serum HSV, patients discharged from our hospital EBV, HIV, TPHA/ RPR, ANA, C3, C4, with a diagnosis of WG from January Ig A, IgE, IgM, IgG were all within 2000 to December 2011. There were normal range. No malignant tumors were totally 14 patients, including 9 patients detected after brain MRI, tumor markers, who definitely diagnosed by GYN ultrasound, chest CT, whole body pathological study and 5 patients who Ga-67 tumor and PET scan . Opsoclonus , diagnosed by positive serological test myoclonus and ataxia did not improved of C-ANCA and clinical manifestation. after using steroid therapy initially but We reviewed the clinical, gradually showed response to Beclofen. electrophysiological, radiological, and serological data. Conclusion Since no tumor was found after a series of examinations , Result In this study, 5 patients were parainfectious OMA following a flu like identified to be associated with virus infection was considered . peripheral neuropathies out of 14 patients with WG. The nerve 89 conduction studies showed 2 (14%) 韋格納肉芽腫對周邊神經系統的侵犯- patients with axonal polyneuropathy; 1 醫學中心病例分析 patient with demyelination 黃俞華郭弘周林口長庚醫院神經內科 polyneuropathy (7%); 2 patients with mononeuropathy multiplex (14%) Wegener’s granulomatosis with peripheral nervous system Conclusion Nervous system involvement: A medical center based study manifestation in WG is less frequent

Yu-Hua Huang Hung-Chou Kuo than classical manifestations such as Department of Neurology, Chang Gung Memorial Hospital, Lin-Kou Medical Center lung and kidney involvement. However, neurologic involvement is Objective Wegener’s granulomatosis not uncommon in WG which requires (WG) is a rare systemic autoimmune

82 fast diagnostic work-up and early discretion of the investigator. DPNP introduction of highly potent severity was evaluated using CGI-S immunosuppression in order to prevent (Clinician Global Impression-Severity), or reduce potential damage. PGI-S (Patient Global Impression-Severity), and BPI. To 90 evaluate the primary objective, patients 糖尿病周邊神經痛之治療:病患的期望 were asked to rank each of the 7 BPI 與醫師的判斷 interference items for which they most 葉如芬 expect improvement. PATIENT EXPECTATIONS AND PHYSICIANS’ JUDGEMENT IN THE TREATMENT OF DIABETIC Result 2576 patients (mean age: 65.8 PERIPHERAL NEUROPATHIC years) were enrolled by 307 physicians. PAIN Mean (SD) BPI average pain was 1 2 3 Ju-Fen Yeh , Edith Schneider , Dan Ziegler , 5.1(2.04). While the interference score Stefan Wilhelm2, Alexander Schacht4, Frank Birklein5 was 4.8(2.18), items most impaired 1 Presenting on behalf of Eli Lilly & Company, were 'walking ability' 5.5(2.60) and Indianapolis, IN(禮來股份有限公司醫藥學術部) 'general activity' 5.4(2.37). The most 2 Medical Department Neuroscience, Lilly relevant items for which patients Deutschland GmbH, Bad Homburg 3 Deutsche Diabetes Zentrum, Heinrich-Heine expected improvement were 'general Universität, Düsseldorf activity' (29.3%; 95%CI: 27.5%-31.0%) 4 Global Statistical Sciences, Lilly Deutschland GmbH, Bad Homburg and 'walking ability' (24.4%; 95%CI: 5 Klinik und Poliklinik für Neurologie, Johannes Gutenberg Universität, Mainz 22.8%-26.1%), followed by 'sleep' (14.7%), 'enjoyment of life' (13.6%), Purpose Pain reduction is the main 'mood' (8.3%), 'normal work' (7.7%) objective when treating patients with and 'relations with other people' (1.9%). diabetic peripheral neuropathic pain CGI-S assessment showed a moderate (DPNP). However, DPNP is associated correlation with PGI-S (0.587; 95%CI: with further substantial patient burden 0.562- 0.612), but weaker correlations which often is not appropriately with BPI severity (0.365; 95%CI: addressed. Primary objective of this 0.331-0.398) and interference scores study was to evaluate which interference (0.420; 95%CI: 0.388-0.451). category from the Brief Pain Inventory (BPI) is most relevant from patients' Conclusion In addition to pain relief, perspective. this study identified 'general activity' Method: Baseline data of a German and 'walking ability' as most relevant prospective, non-interventional, 6 months BPI interference items in which study in DPNP patients starting or patients expect improvement from switching pain medication at the DPNP treatment.

83 91 sudden movement ignition and sound 侵襲性胸腺瘤引發之僵人症候群 stimuli. Neurologic examination 葉建治林精湛蔡崇豪黃匯淳 revealed rigidity and brisk deep tendon 中國醫藥大學附設醫院神經部暨神經醫學實驗室 reflexes in the lower extremities, and bilateral plantar extensor response. Stiff-person syndrome associated with invasive thymoma: a case report Laboratory data showed anti- glutamic Jian-Jhih Ye, Ching-Chan Lin, Chon-Haw Tsai, acid decarboxylase (anti-GAD) Hui-Chun Huang Neuroscience Laboratory, Department of antibody level was >300 Neurology, China Medical University Hospital, u/mL(normal<1.0). Computer Taichung, Taiwan Department of Oncology and Hematology, China tomography of the chest revealed one Medical University Hospital, Taichung, Taiwan huge anterior mediastinal tumor in

prevascular space. Invasive thymoma, Background The stiff-person syndrome type B2, with bilateral pleural invasion, is a rare neurologic disorder lung metastasis and pericardium characterized by progressive paroxysmal invasion was considered. The surface axial and proximal limb rigidity and electromyography did not detect a painful tonic spasms due to continuous continuous muscle discharge at rest in motor unit activity. It has been reported the lower extremities, but episodic to be associated with autoimmune motor unit potentials during diseases or paraneoplastic diseases such paroxysmal painful muscle spasms as thymoma, adenocarcinoma of breast, precipitated by unexpected auditory adenocarcinoma of colon, Hodgkin’s stimuli were recorded in bilateral lymphoma, small-cell carcinoma of the quadriceps and tibialis anterior muscles. lung. Herein, we report a case of According to the combination of stiff-person syndrome associated with clinical manifestations and the elevated invasive thymoma treated with anti-GAD antibody level, the diagnosis chemotherapy and prednisolone with of stiff-person syndrome was made. good response. The patient was treated with baclofen

(80 mg/day) and rivotril (2 mg/day) Case Report A 47-year-old woman with good response, but the drugs was with a history of invasive thymoma was titrated progressively according to her admitted because of falling backward progressive condition. She was not repeatedly and unexpectedly due to allowed to receive thymectomy due to episodes of sudden stiffness in bilateral the invasive status of her thymoma. lower extremities for two weeks. The Instead, she was treated with regimen episodes of stiffness were accompanied as three courses of chemotherapy, by paroxysmal painful muscle spasms, using 90 mg cisplatin and 150mg involving proximal lower extremities etoposide per day. Her painful tonic chiefly, which was precipitated by

84 spasm was more severe after the first on lounge chair for 8 hours while regimen of chemotheraphy, but was waking up in the morning. At that time, subsided after accompanied with he noticed a severe swelling over dexamethasone treatement. Her lower posterior aspect of right thigh, with legs rigidity, hyper-reflexia and painful ecchymotic and pain. He insisted drank tonic spams were subsided progressively bottles of beer without drunk the last and we had tapered the baclofen, rivotril night and denied any history of and prednisolone slowly. She was systemic diseases, coagulopathies, asymptomatic now during one-year medication, blunt trauma or exercise. follow-up visit. Due to right foot drop and subsequent walking difficulty, he received Conclusion It is important to survey conservative treatment in the and treat the underline disease beneath district-hospital and was transferred to stiff-person syndrome. our hospital after 2 weeks. At interview, he appealed of right anterior tibialis 92 muscle and hip tenderness. Mild 自發性後方腔室症候群造成之急性坐 weakness (4/5) of right hip 骨神經病變 flexion/extension, knee 蔡睿蘋袁瑞昱許昭俊游家銘高克培何俊森 extension/flexion was noted but ankle 台北醫學大學附設醫院神經科 plantarflexion /dorsiflexion and big toe extension were completely paralyzed Acute sciatic neuropathy due to spontaneous posterior compartment (0/5), together with hypesthesia of L5 syndrome dermatone of right lower leg. Pelvic Jui-Ping Tsai, Rey-Yue Yuan, Jau-Jiuan Sheu, Jia-Ming Yu, Ko-Pei Kao, Chun-Sum Ho magnetic resonance imaging showed Department of Neurology, Taipei Medical extensive ischemic muscular necrosis University Hospital with right sciatic nerve compression, Background The common causes of indicated rhabdomyolysis. Symptoms sciatic neuropathy have been reported as as right foot drop and paraesthesia hip surgery, diabetic mononeuropathy, showed poor recovery under acute external compression, trauma or medication and rehabilitation after idiopathic. We presented here an discharge. infrequent cause, about a case after sleeping on lounge chair for 8 hours, Conclusion A clinical diagnosis of secondary to spontaneous posterior spontaneous atraumatic posterior compartment syndrome of the thigh. compartment syndrome was made, base on the patient’s history and Case Report A 45 years old healthy clinical presentation. If an early male had right leg paralysis after sleeping surgical intervention had been

85 performed, the patient might have she visited ER. Because of the different outcomes. difficulty in chewing, she became unable to have a meal smoothly. About 93 two weeks after the onset of diplopia, 侵犯性胸腺瘤相關之僵人症候群併發 intermittent jerk of four extremities 急性呼吸衰竭 developed with increasing frequency 蕭丞宗蔡清標林恭平尤香玉李宜中台北榮總神經內科 and intensity, which interfered her walking and she became bed-bound. At Stiff-person syndrome associated with the same time, she had frequent invasive thymoma and complicated by general painful spasms, which were acute respiratory failure Cheng-Tsung Hsiao, Ching-Piao Tsai, Kon-Ping usually provoked by movements. Lin, Hsiang-Yu Yu, Yi-Chung Lee The Neurological Institute, Taipei -Veterans On the neurological examination, the General Hospital patient had normal consciousness and cognition, no specific cranial nerve Background Stiff-person syndrome is signs, almost normal muscle strength, characterized by progressive rigidity of generalized hyper-reflexia, rigid the axial musculature, superimposed by muscle tone, normal coordination, and painful spasms with an exquisite no sensory deficit. Repeated episodes sensitivity to external stimuli. It can be of four limbs jerks and an opisthotonus paraneoplastic sometimes and the followed by hypercapnia respiratory coexistence of stiff-person syndrome and failure happened during observation, thymoma with or without myasthenia so she received endotracheal intubation gravis has been previously characterized. with mechanical ventilator support. The respiratory function is rarely During the hospitalization, the compromised in stiff-person syndrome. video-EEG with surface EMG Herein, we report a patient with recorded events characterized by malignant thymoma presenting with generalized myoclonic jerk, mainly on progressive general malaise and acute trunk and sometimes caused respiratory failure as the main generalized stiffness. The titer of serum manifestations of the paraneoplastic anti-GAD antibody is 121.50 U/mL stiff-person syndrome. (reference: < 1 U/mL). The diagnosis of stiff-person syndrome is established Case Report A 44-year-old lady with on the base of clinical manifestation recurrent invasive thymoma visited the and a positive serum anti-GAD emergency room due to progressive axial antibody. The initial treatment with stiffness and general malaise for five Methylprednisolone pulse therapy months. She was able to deal with the (1g/day for 5 days) and five course of activities of daily living until double plasmapheresis had only little effect. vision occurred about one month before

86 1 The condition began to obviously Department of Neurology, E-DA Hospital / I-Shou University, Kaohsiung, Taiwan improve after administration of 2 Department of Food Science, National intravenous immunoglobulin (IVIG: Taiwan Ocean University, Keelung, Taiwan

0.4g/kg). The axial stiffness subsided and Background The 45 year-old man, the patient received extubation of with untreated diabetes for 3 years, endotracheal tube smoothly. However, complained of acute dizziness, vertigo, the patient still had general spasm once nausea, and vomiting half an hour after daily, although treated adjuvant with taking over 200 pieces of spiral sea Clonazepam, Baclofen, Levetiracetam, shells captured in southern Taiwan and Pregabalin. The symptoms were Strait. Soon after, he had circumoral totally resolved after she received four numbness and dead skin sensation in courses of chemotherapy with the fingers and toes. The numbness regimen of ascended up to forearms on the next Cyclophosphamide-Vincristine, and plus day when he came to our emergency Prednisolone for the malignant thymoma. department.

Conclusion Generalized muscle Case Report On examination, there spasms could result in acute respiratory was decreased muscle strength (4/5 in failure in stiff-person syndrome. In MRC scale) in distal upper limbs and addition to plasmapheresis, IVIG should decreased pinprick sensation in hands, be considered as an alternative feet, and the middle area of the chest therapeutic choice in stiff-person and abdomen. Impaired joint position syndrome. As a GABA agonist, and vibration sensation developed in Pregabalin also have a temporal effect to the following 1 to 2 days. All blood relieve symptoms for stiff-person tests including biochemistry, VDRL, syndrome. It is still necessary to and HIV were unremarkable. Nerve aggressively manage underlying conduction study showed malignancy for eradicating the length-dependent sensori-motor stiff-person syndrome with polyneuropathy without evidence of paraneoplastic oringin. demyelination. All symptoms gradually subsided in 9 days after intravenous 94 hydration. 河豚毒中毒以格林-巴利徵候群表現 1 2 1 1 戴逸承黃登福陳彥文崔煥文 1 義大醫院神經科 Conclusion The sea shells were 2 國立海洋大學生命科學系 recognized as Niotha clathrata and

Zeuxis scalaris. Tetrodotoxin was Tetrodotoxin Intoxication mimicking Guillian-Barre syndrome found in the remnants of Niotha 1 2 Yi-cheng Tai, M.D., Deng-fwu Hwang, Ph.D., clathrata (82±39 MU) and Zeuxis 1 Yen-wen Chen, M.D., 1Huan-wen Tsui, M.D.

87 scalaris (129±65 MU), the patient’s urine pigmentosa since the age of 5, and (1.28ng/mL), and stool (1.79ng/mL). hearing impairment since the age of 15. Both species are distributed in India This time, he began to develop acute Ocean, Red Sea, and Southeast Asia, bilateral legs weakness and distal limbs including Taiwan. The major reported numbness, which impaired his walking presentations of sea shell TTX in two weeks. Vaccination, infectious intoxication were acute progressive event or diarrhea was not found before circumoral numbness, dizziness, hand the onset of limbs weakness. There was numbness, ataxia, and nausea, as those of no remarkable family history or our patient. These symptoms may mimic abnormal developmental milestones. Guillian-Barre syndrome and thus TTX His physical examinations revealed intoxication should be considered as one impairments of pinprick, thermal, joint of the differential diagnosis of position and vibration senses over all Guillian-Barre syndrome. limbs. Mild weakness (MRC grade 4) was noted over lower limbs. He had 95 diffuse arreflexia and sensory ataxia. 在尤塞氏症候群病人身上表現免疫性 Cerebrospinal fluid revealed 多發神經病變 albuminocytologic dissociation 謝宗達張宏旭羅榮昇張國軒林口長庚紀念醫院神經內科 (protein 302.4 mg/dL, leukocyte count 0 uL). Results of his nerve conduction Immune-mediated polyneuropathy in study were compatible with a a patient with Usher syndrome Tsung-Ta Hsieh, Hong-Shiu Chang, Long-Sun Ro, sensory-predominant axonal Kuo-Hsuan Chang polyneuropathy. His visual evoked Department of Neurology, Chang Gung Memorial Hospital and University College of Medicine, potential demonstrated prolonged P100 Lin-Kou Medical Center on both sides. Treatment with

double-filtrating plasmapheresis Background Usher syndrome is an significantly improved his sensory autosomal recessive genetic disorder impairment and gait disturbance. characterized by progressive sensorineural deafness and retinitis Conclusion This is the first case pigmentosa. A number of report about the presentation of neuropsychiatric presentations have been immune-mediated polyneuropathy in shown in the patients with Usher the patients with Usher syndrome. syndrome. However, polyneuropathy has More clinical studies should be not been seen in those with Usher performed to clarify the association syndrome between acute sensory neuropathy and

Usher syndrome. Case Report A 26-year-old male had progressive visual loss caused by retinitis

88 96 acetazolamid. Their family histories 先天性肌強直 : 關於兩例偶發性病例 were reviews and genetic analyses 報告 were performed. 簡士超簡浴沂林口長庚醫院神經內科

Myotonia congenita: A report of 2 神經基因學 sporadic cases Chien Shih Chao Chien Yu Yi 97 Department of Neurology, Chang Gung Memorial 血漿 FGF21 在小腦萎縮症病患與粒 Hospital, Linkou, Taiwan 線體疾病病患之表現情形巫錫霖 1 黃靜珊 2 鄭文玲 2 劉青山 1,2,3 Background Generally speaking, the 1.財團法人彰化基督教醫院神經醫學部 2. causative gene for classic myotonia 財團法人彰化基督教醫院血管暨基因體研究中心 congenita is CLCN1 gene, and, thus far, 3.中國醫藥大學中西醫學結合研究所 over 60 mutations in this gene have been identified. However, disorders, meet FGF21 in ataxia patients with spinocerebellar atrophy and criteria of myotonia congenita, have mitochondrial disease different kinds of clinical presentations, Shey-Lin Wu1, Ching-San Huang2, Wen-Ling Cheng2, Chin-San Liu1,2,3* for example, the response of myotonia to 1.Department of Neurology, Changhua medications, the age of onset, or the Christian Hospital, Changhua, Taiwan 2.Vascular and Genomic Center, Changhua pattern of attack. We are curious to know Christian Hospital, Changhua, Taiwan 3. the factors causing these differences. Graduate Institute of Integrated Medicine, College of Chinese Medicine, China Medical University, Taichung, Taiwan Case report We have studied 2 sporadic Objective Serum fibroblast growth cases of myotonia congenita with clinical factor 21 (FGF21) was proven to be a features resembling, as well as differing useful biomarker for the presence of from, most cases of autosomal recessive mitochondrial neuromuscular disease. myotonia congenita. Both patients progress slowly during childhood and Methods In the present study, we adolescence, neither presents as muscular used the difference in the serum dystrophy, and both have myotonic FGF21 level to differentiate between discharges on EMG, rather high serum ataxia patients with hereditary CK levels, and type 1 muscle fiber spinocerebellar atrophy (SCA-ataxia) atrophy on muscle biopsy. and those with mitochondrial

syndrome (Mito-ataxia). Patients with Result/Conclusion One has herculean SCA-ataxia (SCA2, SCA3) and appearance whose myotonia is alleviated Mito-ataxia (MELAS, MERRF, LHON, by both mexiletine and acetazolamide, maternal inherited hearing impairment and the other is short but not stocky mtDNA A1555G mutation) were whose myotonia response only to

89 recruited in this study. All SCA-ataxia Objective To identify the causative patients revealed a consistent pattern of gene for a novel type of dominant cerebellar atrophy. On the contrary, some intermediate Charcot-Marie-Tooth of the Mito-ataxia patients exhibited a disease (DI-CMT.) vascular lesion with cerebellar infarction. Background We previously Results An extremely high level of characterized a three-generation serum FGF21 was found in the Chinese family afflicted with a Mito-ataxia patients with MERRF and DI-CMT, and mapped the locus of the MELAS diseases, but not in patients with candidate gene to chromosome SCA-ataxia or LHON/mtDNA A1555G 3q28-q29. mutation. The positive trend correlation between the mtDNA heteroplasmy and Methods Exome sequencing was the level of serum FGF21 was indicated utilized to identify the mutation in either MERRF (P = 0.003, r = 0.923) causing CMT in this family. We further or MELAS (P = 0.070, r = 0.566) screened the index patients of 88 patients. families selected from 251 CMT pedigrees after excluding mutations in Conclusion The serum FGF21 level the common CMT causative genes. can be applied as the first molecular Mutant gene products were surveyed screening in patients with hereditary on human sural nerve samples by ataxia and neuromuscular degeneration immunohistochemistry and in vitro to prior to mass genetic screening. investigate their functional impact.

98 Results We identified a heterozygous GNB4 基因突變會造成顯性中間型遺傳 p.G53D mutation in the Guanine 性運動感覺神經病變 nucleotide-binding protein subunit 李宜中 1 高閬仙 2 宋秉文 1 張鳴宏 3 林恭平 2 1 台北榮民總醫院神經醫學中心 beta-4 gene (GNB4) that co-segregated 2 陽明大學生命科學院 with the CMT phenotype in the 3 台中榮民總醫院神經內科 original family. Further sequence

Mutations in GNB4 cause Dominant analysis identified a de novo GNB4 Intermediate Charcot-Marie-Tooth mutation, p.K89E, in another CMT disease 1 2 1 patient. Guanine nucleotide-binding Yi-Chung Lee , Lung-Sen Kao , Bing-Wen Soong , Ming-Hon Chang3, Kon-Ping Lin1 protein beta-4 subunit (Gβ4) is 1 The Neurological Institute, Taipei Veterans General Hospital abundantly expressed in the axons and 2 School of Life Science, National Yang-Ming cytoplasm of Schwann cells of sural University 3 Division of Neurology, Taichung Veterans nerves. In a functional assay for Gβ4, General Hospital in contrast to the wild-type Gβ4 which

90 facilitates the bradykinin-induced Subjects and Methods We cytosolic inositol triphosphate (IP3) previously characterized a large production and intracellular calcium Chinese family with progressive ataxia concentration elevation, both mutant designated SCA22. The disease locus proteins, Gβ4 G53D and Gβ4 K89E, in a French family and an Ashkenazi inhibit the bradykinin-induced signaling. Jewish American family was also mapped to this region. Members from Conclusion This study identified all three families were enrolled. Whole GNB4 as a novel gene that, when exome sequencing was performed to mutated, causes a CMT phenotype. identify candidate mutations, which Heterozygous GNB4 mutations occurred were narrowed by linkage analysis and in 0.8% of our patients with CMT. GNB4 confirmed by Sanger sequencing and mutations cause CMT by abolishing the co-segregation analyses. Mutational Gβ4-related G-protein couple signaling, analyses were also performed in 105 which may play an important role in the Chinese and 55 Japanese families with pathogenesis of CMT. cerebellar ataxia. Mutant gene products were examined in a heterologous 99 expression system to address the KCND3 基因的序列變異是引起第二十 changes in protein localization and 二型脊髓小腦運動失調症的原因 electrophysiological functions.. 李宜中 1 黃彥華 2 連正章 3 宋秉文 1 1 台北榮民總醫院暨國立陽明大學神經內科, 2 國立陽明大學系統及合成生物學中心, 3 國立 Results We identified heterozygous 陽明大學神經科學研究所 mutations in the voltage-gated

Mutations in KCND3 cause potassium channel Kv4.3-encoding spinocerebellar ataxia type 22 gene KCND3: an in-frame 1 2 Yi-chung Lee, Yen-hua Huang, Cheng-chang three-nucleotide deletion Lien,3 Bing-wen Soong1 1 Department of Neurology, National Yang-Ming c.679_681delTTC p.F227del in both University School of Medicine and Neurological Institute, Taipei -Veterans General the Chinese and French pedigrees, and Hospital a missense mutation c.1034G>T 2 Department of Biochemistry and Center for Systems and Synthetic Biology, National p.G345V in the Ashkenazi Jewish Yang-Ming University, Taipei, Taiwan family. Direct sequencing of KCND3 3 Institute of Neuroscience, National Yang-Ming University, Taipei, Taiwan further identified three mutations, c.1034G>T p.G345V, c.1013T>C Objectives To identify the causative p.V338E and c.1130C>T p.T377M, in gene in SCA22, an autosomal dominant three Japanese kindreds. cerebellar ataxia mapped to chromosome Immunofluorescence analyses revealed 1p21-q23. that the mutant p.F227del Kv4.3 subunits were retained in the

91 cytoplasm, consistent with the lack of affected patients often presented with A-type K+ channel conductance in tremor-predominant whole-cell patch-clamp recordings. levodopa-responsive parkinsonism. Haplotype analyses revealed that there However, clinical variants in different was no common founder between the populations and non-motor symptoms pedigrees from Taiwan and French. in this kind of pathogenicity have not yet been reported. Conclusion Our data identify the cause of SCA22 in patients of diverse ethnic Methods A total of 15383 individuals origins as mutations in KCND3. These (8870 cases and 6513 controls).form findings further emphasize the important 23 sites representing 19 countries in role of ion channels as key regulators of the Genetic Epidemiology of Parkinson neuronal excitability in the pathogenesis disease (GeoPD) Consortium were of cerebellar degeneration. included for genetic analysis for the pathogenicity of VPS35 gene variants 100 in this study. Overall, there were 巴金森氏病合併 VPS35 基因顯現臨床 mutations with p.Asp620Asn in seven 異質表徵 cases, p.Leu774Met in six cases and 林瑞榮樂國柱國際巴金森氏病基因流行病研究協會 one control, p.Gly51Ser in three cases 竹山秀傳醫院神經科 and two controls. One o PA patients had double mutations (p.Asp620Asn Clinical Heterogeneity Associated with and p.Leu774Me). We further analyze VPS35 Gene in Parkinson Disease Juei-Jueng Lin, Kuo-Chu Yueh, on behalf of the the clinical manifestations, including Genetic Epidemiology of Parkinson’s disease motor and non-motor symptoms, in Consortium Departments of Neurology, Chushang those 15 PD patients carried Show-Chwan Hospital, Nantou, Taiwan pathogenic variants and two controls..

Results All PD patients were Objective A large multi-centre study to clinically diagnosed with PD. Two out analyze clinical manifestations in patients of seven patients carrying the with Parkinson’s disease (PD) with p.Asp620Asn variant presented pathogenicity of vacuolar protein sorting without any family history for PD. 35 (VPS35) variants in diverse Affected individuals exhibited classical populations worldwide. symptoms of PD (resting tremor,

bradykinesia, rigidity). The clinical Background Current studies identified diagnosis of PD was made by a mutation (p.Asp620Asn) in the movement disorder specialists who vacuolar VPS35 gene responsible for an used UK brain bank criteria for PD. autosomal dominant Parkinson disease. Non-motor symptoms were present in The clinical manifestations in the

92 the majority of PD patients carrying a express of OPA1 (p < 0.05) and PINK1 (p < 0.05) were noted in leukocyte of pathogenic variant. Interestingly, SCA3 patients compared with healthy hallucinations and dementia were also subjects and corresponding to the observed in one asymptomatic carrier expansion of CAG repeat number and the disease severity of SCA3.

Conclusion Our study represents the Conclusion Mitochondrial fusion first evidence for the clinical and mitophagy are related to the heterogeneity associated with VPS35 in pathogenesis of SCA3 which may PD patients among different populations capitalize the mechanism of stress-induced mitochondrial fusion to worldwide. reserve the availability of mitochondria in recycling as well as quality control. 101 Further investigation is needed for 第三型小腦萎縮症病患之 OPA1 與 ascertaining the OPA1 and PINK1 expression levels as biomarkers for PINK1 基因異常表現 SCA3 disease progression. 劉青山 1,2,3 鄭文玲 2 1 財團法人彰化基督教醫院神經醫學部 2.財團法人彰化基督教醫院血管暨基因體研究中心神經生理及電生理學 3.中國醫藥大學中西醫學結合研究所 102 Over Expression of Opa1 and PINK1 大腦預設模式網路活性之一致性及 Genes in Patients with SCA3 振動性在早期阿茲海默症與輕度知 Chin-San Liu1,2,3*,Wen-Ling Cheng2 1. 能障礙患者的不同: 以腦波研究 Department of Neurology, Changhua Christian 1 2 Hospital, Changhua, Taiwan 王毓禛蕭富榮 1 2 2 .Vascular and Genomic Center, Changhua 台北市立聯合醫院神經科及教研部 Christian Hospital, Changhua, Taiwan 3. Graduate Institute of Integrated Medicine, Altered Oscillation and College of Chinese Medicine, China Medical Synchronization of Default-Mode University, Taichung, Taiwan Network Activity in Mild Objective Mitochondrial fusion, fission Alzheimer’s Disease Compared to and mitophagy may be involved in Mild Cognitive Impairment: An Electrophysiological Study neuropathology of spinocerebellar ataxia 1 2 Yuh-Jen Wang , Fu-Jung Hsiao type 3 (SCA3). 1 Department of Neurology and 2Department of Education and Research,Taipei City Hospital, Methods Ten healthy subjects and Taipei, Taiwan twenty SCA3 patients were recruited. Scale for the Assessment and Rating of Objective To examine whether Ataxia was applied for clinical evaluation. The CAG repeat number of ataxin-3, electroencephalography (EEG) is as expression of mitochondrial fusion genes useful as functional magnetic (OPA1 and MFN2), fission genes resonance imaging (fMRI) in (DNM1L and FIS1) and mitophagy genes (PINK1, PARK2 and RHOT1) were investigating brain default mode evaluated by RT-PCR. network (DMN) activity and differentiating early stage AD from Results Significantly increased of

93 mild cognitive impairment (MCI). the medial temporal lobe correlated significantly with scores obtained on Background Some researchers have the Mini-Mental State Examination suggested that the DMN plays an (MMSE). important role in the pathological mechanisms of Alzheimer’s disease (AD). Conclusion EEG is a useful tool for However, fMRI is not available in every investigating the DMN in the brain and institution. Instead, EEG is more differentiating early stage AD and MCI accessible. patients. This is a promising finding; however, further large-scale studies are Methods EEG was recorded from 21 needed. mild AD and 21 mild cognitive impairment (MCI) patients during an 103 eyes closed, resting-state condition. The 以穿顱直流電刺激右背外側前額葉 spectral power and functional 皮質調節空間工作記憶的干擾作用吳怡真 1 張期富 2 曾祥非 2 阮啟弘 2 白明奇 3 connectivity of the DMN were estimated 林宙晴 3 using a minimum norm estimate (MNE) 1 國立成功大學附設醫院斗六分院神經科 2 combined with fast Fourier transform and 國立中央大學認知神經科學研究所 3 國立成功大學附設醫院神經部 imagery coherence analysis. Modulating the Interference Effect Results Our results indicated that on Spatial Working Memory by Applying source-based EEG maps of resting-state Transcranial Direct Current activity show alterations of cortical Stimulation over the Right spectral power in mild AD when Dorsolateral Prefrontal Cortex Yi-Jen Wu1, Chi-Fu Chang2, Philip Tseng2, compared to MCI. These alterations are Chi-Hung Juan2, Ming-Chyi Pai3,Chou-Ching 3 characteristic of attenuated alpha or beta Lin 1 Division of Neurology, National Cheng Kung activities in the DMN, as are enhanced University Hospital, Dou-Liou Branch 2 Institute of Cognitive Neuroscience, National delta or theta activities in the medial Central University temporal, inferior parietal, posterior 3 Department of Neurology, National Cheng Kung University Hospital cingulate cortex and precuneus. With regard to altered synchronization in AD, Objective Spatial working memory altered functional interconnections were (SWM) is the ability to temporarily observed as specific connectivity patterns store and manipulate spatial of connection hubs in the precuneus, information. It has limited capacity and posterior cingulate cortex, anterior is quite vulnerable to interference. cingulate cortex and medial temporal Dorsolateral prefrontal cortex (DLPFC) regions. Moreover, posterior theta and is one of the brain regions in the SWM alpha power and altered connectivity in network but its role in SWM is still

94 under investigation. This study tDCS shortened the reaction time of investigated the role of the right DLPFC SWM performance and improved in processing the interference effect on SWM capacity in high-difficulty SWM. conditions with concurrent interference. These results suggest the right DLPFC Methods Eleven healthy young adults plays a crucial role dealing with the were enrolled. Transcranial direct current interference effect for spatial working stimulation (tDCS) noninvasively memory. provides polarity-specific electric stimulation to the targeted brain region. 104 Anodal tDCS (1.5mA) was applied on 引發多發性神經病變及認知功能失 the right DLPFC for 15 minutes before 調病例報告林圻域蔡松彥 participants performed a forward- and 彰化基督教醫院雲林分院神經內科 backward-recall computerized Corsi Polyneuropathy and possible Block Tapping task (CBT), both with and cognitive dysfunction induced by exposure to ethylene oxide without a concurrent motor interference Ethylene oxide task (the modified Luria manual sequencing task). The response accuracy Background Ethylene oxide is a for the memorized span and reaction time well-known neurotoxin which cause were recorded. polyneuropathy. The involvement of central nervous system is also reported Results With tDCS, participants’ in the past. In this case report, the reaction time improved in all four patient suffered from antonomic conditions (forward, backward, forward dysfunction, including abdominal with interference, and backward with fullness and impotence as first interference). Although there was a symptoms after exposure to Ethylene consistent decline of memory span in the oxide for 4 years. motor interference conditions, tDCS significantly improved participants’ Case Report A 47 y/o married man SWM span (sham group: 5.1, tDCS visited our outpatient department due group: 5.7, p<0.05) in the to progressive bilateral calf cramp and “backward-recall with motor acroparesthesia for more than one interference” condition, which was the month. He has been a sterilizer worker most difficult condition. exposed to ethylene oxide for 4 years. Initially, he experienced abdominal Conclusion This study demonstrated fullness, poor appetite and impotence that the motor interference degraded in the first week of June and followed SWM performance. Right DLPFC anodal by bilateral calf cramp, which got

95 worse in the night. One month later, he case report and this case. suffered from acroparesthesia and poor attention also. There were no fever and 105 other autonomic dysfunctions including 足底振動改變單腳站立時的姿勢控 nausea, vomiting, diarrhea, urinary or 制林聖皇錢振愷李筱媛黃信祺郭藍遠楊 bowel movement dysfunction. 志鴻 Neurological examination revealed intact 財團法人佛教慈濟綜合醫院神經內科 of cranial nerve, full strength of limbs 慈濟大學醫學院物理治療學系高雄醫學大學醫學院運動醫學系 muscle power, decreased deep tendon reflexes, distal sensory impairment, Foot Plantar Vibration Changes predominantly lower limbs, including all Postural Control With One-Leg Standing modalities which are pinprick, light Sheng-Huang Lin, Chen-Kai Chien, tough, vibration and joint position sense, Hsiao-Yuan Lee, Sin-Chi Huang, Lan-Yuen Guo, Chich-Haung Yang and positive Romberg’s test. The brain Department of Neurology, Buddhist Tzu Chi CT and cervical/lumbosacral MRI did General Hospital; Department of Physical Therapy, College of Medicine, Tzu-Chi not show abnormal findings. The NCV University; Faculty of Sports Medicine, College of Medicine, Kaohsiung Medical study found polyneuropathy. The ABEP University study revealed bilateral brain stem dysfunctions. He was diagnosed as toxic Abstract Postural control is critical in polyneuropathy caused by ethylene oxide. human upright activities (e.g. standing, After using personal protective mask walking and running), which is an during working, his symptoms including integral output of musculoskeletal, bilateral calf cramp, acroparesthesia and motor and sensory nervous system. impotence were improved. Notably, somatosensory input of plantar area is an essential role in Conclusion Neuropsychologic postural control and compromised in impairment and Peripheral neuropathy patients with polyneuropathy. The after long term exposture to EtO has beed purpose of this study was to examine documented in past series report. This whether plantar vibration may alter patient has autonomic dysfunction, postural control in people with plantar including abdominal fullness, nausea, desensitization after ice immersion. A vomiting and impotence. In addition, we single-leg upright posture over a also complete evaluated Eto toxic forceplate was selected to examine the damage to nerve system, including capability of postural control under 3 anatomic and functional study. We also conditions (pre-intervention, plantar known that CNS and PNS involvement desensitization, and with vibration). has reversible effect, but autonomic The preliminary results have shown effect has uncertain damage in the past that significant reduced postural control in both eyes open and eyes

96 close conditions were found in people suffered from PDz for more than 10 with plantar desensitization (after ice years. Also medical Hx of CAD, old immersion), which indicating increased CVAs, depression disorder, DJD of T-L area, root mean square and mean velocity spine with scoliosis and marked of center of pressure (COP) (p < 0.05). kyphosis, T12-L1 compression Fx, Meanwhile, plantar vibration osteoporosis with T-score:-2.5 to –2.7, intervention did not improve the VHD, HCVD, chronic liver Dz, senile parameters (Area, RMS and MV) of COP. anemia, calcified tendinitis of Rt The findings confirm the role of plantar supraspinatus, chronic paranasal sensory input is a key role to mediate the sinusitis……etc. Generalized twitching postural control. In contrast, plantar of all limbs and the trunk occurred vibration over the calcaneus provides during regular OPD visit. Seizure adverse effect on the postural control in a disorder was concerned and was single-leg upright standing posture. admitted in the ward at once. After hospitalization, mentality was clear but poor and dull responses. No definite 癲癇與腦波 cerebral or cerebellar dysfunction. 106 Brain MRI disclosed many old lacunar 巴金森氏病臨床第五期之高齡老婦左 infarcts with moderate cerebral cortical 上牙齦蜂窩性組織炎合併骨髓炎以全 atrophy. EEG told us no epileptic focus. 身不自主抽搐表現 Biochemical studies, including 王崇仁 1 賴達昌 2 彭志鋼 3 振興醫療財團法人振興醫院 1 老年醫學科 2 神 electrolytes, thyroid function test, Vit 經內科 3 牙科 B12 & folate check-up were within normal limits except high sensitivity of An Parkinson’s Disease Clinical Stage C-reactive protein (CRP-HS) was up Five Old Lady Left Upper Gum Cellulitis and Osteomyelitis from 0.37 to 1.51 to 9.53 (normal Manifested as Generalized Involuntary <0.80 mg/dl). The leucocyte was Twitching around 5500 to 7800. The temperature Chung-Jen Wang 1, Ta-Chang Lai 2, Tzi-Kang Peng 3 was never over 38℃. No evidence of Division of Geriatrics 1, Division of Neurology 2, Department of Dentistry 3, Cheng Hsin General UTI, pneumonia or hepato-biliary tract Hostipal inflammation. We tried Depakine and Rivotril, the involuntary movement Background We reported a Parkinson’s little improved. Pt just called help of Dz clinical stage V female patient dentist of Taipei-VGH for periodontal suffered from severe infection of the problem about one month prior to this gingivae, presented as atypical toxic admission. We consulted our dentist symptom and sign. and the diagnoses were left upper gum

cellulitis with pus formation plus Case Report One 85-year–old woman,

97 osteomyelitis on #21-26 gingivae. Patients with Focal Neocortical Epilepsy Debridement and removal of necrotic Yue-Loong Hsin1, Shu-Chun Peng2 bone with 20 stitches suture were 1Department of Neurology, Hualien Tzu Chi Medical Center performed. The pus culture were Staph. 2Department of Electrical Engineering, aureus, Kleb. pneumoniae, Acin. National Central University baumannii & Pseudo. aeruginosa. The pathological Dx showed picture of Objective If a patient has one seizure chronic osteomyelitis, composed of focus, the seizure semiology tends to sequestrum surrounded by granulation be stereotyped. That supports the tissue with aggregated of inflammatory hypothesis: certain nerve fibers are cells & fibrosis. Tapimycine & responsible for the sequential Teicoplanin were prescribed at first and propagation of seizure electric activity shifted to Ampicillin & Sulbactam 13 into the corresponded days later. After next 2 weeks, changed to symptomatogenic areas. We analyzed Augmentin. The total hospital days was 6 tensor brain MRI, functional brain weeks. Dental procedure intervened 10 MRI, ictal intracranial EEG and days after admission, involunraty cortico-cortical evoked potential movement of whole body, low-grade (CCEP) study from patients with fever, anorexia and constipation partial neocortical epilepsy. Our aim is improved day by day. to depict the neural networks, which correlate with seizure electric activity. Conclusion Physical Examination in the elderly, oral condition evaluation is Methods Four patients with partial omitted most of the time. In this case, we neocortical epilepsy and with normal delayed over 1 week. Be careful, brain MRI who underwent epilepsy somewhere is under obvious infection in surgery were studied. The location of the senior person even no leukocytosis, each subcortical electrode extracted by no high fever and even NO PAINFUL rendering the post electrodes SENSATION! implantation brain CT was used to identify region of interest (ROI). We 107 compared the regional differences of 新腦皮質癲癇異常神經纖維與癲癇電 apparent diffusion coefficient (ADC) 生理活動的關聯研究 and fractional anisotropy (FA) of brain 1 2 辛裕隆彭徐鈞 tensor images with age-matched 1 花蓮慈濟醫院神經內科 2 國立中央大學電機工程系 normal subjects. We used resting-state functional MRI to detect high Abnormal Neural Networks connection nodes and edges of neural Responsible for the Spread of Seizure networks over the ROI. The Electric Activity: Studying the Reorganization of Nerve Fibers in temporospatial dynamics of seizure

98 2 electric activity were represented by the 國立成功大學醫學院臨床藥學與藥物科技研究所 individual seizure electrocorticographies. 3 台南新樓醫院神經內科 Then we conducted cortico-cortical Adherence to Antiepileptic Drugs in evoked potentials study by Pediatric Patients with Newly paired-electrode stimulation to detect Diagnosed Epilepsy 1 2 neurophysiological connectivity. Shih-Chieh Shao , Edward Chia-Cheng Lai , Yea-Huei Kao Yang2, Cheng-Yang Hsieh2,3 1 Department of Pharmacy, Keelung Hospital, Department of Health, Executive Yuan, Results Increased diffusivity Keelung, Taiwan (increased ADC and decreased FA were 2 Institute of Clinical Pharmacy and Pharmaceutical Sciences, College of the major finding in tensor imaging study) Medicine, National Cheng Kung University, adjacent to the seizure-onset zone was Tainan, Taiwan 3 Department of Neurology, Sin-Lau Christian found. The high connection nodes and Hospital, Tainan, Taiwan. edges of networks colocated with the seizure-onset zone and the regions with Objective To investigate the early seizure spread. High P1/N2 waves adherence to antiepileptic drug (AED) of CCEPs over the early seizure-spread therapy in pediatric patients with regions were obtained by electrical newly diagnosed epilepsy in Taiwan. stimulation over the seizure-onset zone. Background Adherence to AED for Conclusion Changes in the white at least two years was important for matter integrity of the regions with patients with epilepsy to control their epileptic discharges indicated the seizure. However, the information of microstructural reorganization of nerve AED adherence in pediatric patients fibers even the patients with no visible with epilepsy was insufficient. lesion in brain MRI. The spatial accordance of dynamics of seizure Methods A retrospective cohort electric activity with specific functional study was conducted by using National network suggested a fundamental system Health Insurance Research Database underlying the direction of seizure (NHIRD) containing all pediatric propagation. Our findings support that patients who received AED for there are designated routes in for epileptic purpose. We included patients conducting the electric current in the aged eighteen or less who were newly brain with focal epileptogenicity. prescribed with AED from 1 Jan, 2005 to 31 Dec, 2008 and diagnosed with 108 epilepsy within six months prior to or 初診斷小兒癲癇病患用藥順從性之研 following the initiation of AED. 究 Eligible patients were followed by 邵時傑 1, 2 賴嘉鎮 2 高雅慧 2 謝鎮陽 2, 3 1 each eight quarters of the two years 行政院衛生署基隆醫院藥劑科

99 after AED initiation. We measured stimulation of the hippocampus in a patients’ adherence to AED therapy by medically refractory, mesial temporal calculating medication possession ratio lobe epilepsy with multi-epileptic foci (MPR) and defined as MPR less than 0.8 patient. was non-adherent. Case report: A 30 year-old male who is Results A total of 9,897 patients with a chronic epilepsy patient had 55.9% male gender and age of 8.9±5.1 generalized tonic clonic seizure at first years were identified. The mean MPR onset age of 9 year-old. At age of 21, was 0.73 in the first quarter, and then the seizure pattern changed to 0.61, 0.58, 0.56, 0.55, 0.54, 0.53, 0.52 in automotor seizure with impairment of following seven quarters. About 58.5% consciousness. He had aura of bitter of the patients were non-adherent and the taste, post-ictal confusion with déjà vu, overall mean first two-year MPR was and frequency of 1-2 attacks per month, 0.59. despite under valproate 1000mg/day and lamotrigine 300mg/day. On Conclusion We found that the 2012/08/15, he was admitted for long adherence to AED therapy in pediatric term video EEG monitoring and found patients significantly decreased with time, phase reversal at F8T2 with and more than half of the patients are synchronized Fp2 > Fp1 sharp wave, non-adherent in the first two year after sphenoid EEG revealed F8 = T2 = Sp2 initiation of AED. Our findings implicate but sometimes Fp2 > F8 > T2 > Sp2. In considerably poor AED adherence in addition, neuroimaging MRI disclosed pediatric patients with epilepsy and right hippocampal atrophy and PET warrant attention and further action of the showed decreased glucose metabolism health care providers. at right mesial temporal lobe. He was diagnosed of right mesial temporal 109 lobe epilepsy with bilateral frontal 頑固型多發性顳葉癲癇治療:海馬迴刺 involvement. After fully discussed the 激術 pros and cons to the patient and family, 曾偉恩吳禹利林口長庚紀念醫院神經內科 bilateral hippocampal depth electrode was implanted on 2012/11/19. Depth Hippocampal stimulation in treatment electrode recorded three times of of medically refractory, multi-epileptic foci of mesial temporal lobe epilepsy recruiting pattern of increasing Wei-En Johnny Tseng, Tony Wu amplitude and frequency of RT0-3 Department of Neurology, Chang Gung Memorial Hospital, Lin-Kou Medical Center rhythmic spike-wave complex with phase reversal at RT1, suspect seizure Objective: Our aim was to evaluate the attack without clinically ictal safety and efficacy of electrical

100 presentation. Under test stimulation pathogeneses are not fully understood. parameters (90usec, 1V, 145 Hz), the Here we analyze clinical features and amount of inter-ictal epileptiform seizure pattern in Wilson's disease and discharge has reduced > 50% compared correlated with the brain MRI. to non-stim situation. He was discharged under previous AEDs dosage and OPD Subjects and Methods We follow up for stimulation initiation and retrospectively reviewed the medical seizure frequency monitoring. records of all patients from CGMH with the diagnosis of WD from January Conclusion: Treatment challenge arises 2001 to October 2012 and charted the in mesial temporal lobe epilepsy with clinical features, EEG findings and multi-epileptic foci patient. Our case MRI findings. presented a 50% reduction in inter-ictal Results Six out of 91 patients (6.6%) epileptiform discharge after bil. with WD have seizures. The types of hippocampal stimulation. Long-term seizures included generalized monitor for ictal frequency is warrant for tonic-clonic (2 patients), complex efficacy evaluation. partial (1 patient), and partial seizures with secondary generalization (3 110 patients). One patient had status 癲癇和威爾森氏症: 腦波,神經影像和 epilepticus. EEG abnormalities were 臨床相關性 noted in 5 out of 6 patients consisting 黃啟瑞黃錦章林口長庚紀念醫院神經內科 of background slowing and epileptiform discharges. Brain MRI Epilepsy in Wilson's disease: EEG, revealed varying degrees of atrophy neuroimages and clinical correlation Chi-Jui Huang, Chin-Chang Huang and signal changes involving Department of Neurology, Chang Gung Memorial subcortical white matter of the frontal Hospital, Lin-Kou Medical Center lobe in the patients with partial Objective To investigate clinical seizures with secondary generalization, characteristics of seizures in Wilson's while mild cortical atrophy in another disease (WD) and to correlate with patient with generalized tonic-clonic electroencephalography (EEG) changes seizure. The outcome of seizures was and magnetic resonance imaging(MRI) relatively good with seizure free abnormalities. (33.3%), good control (50%), and poor control (16.7%). Background Seizures may occur in Conclusion Seizures are not WD, with a prevalence of 4.2%-8.3%, uncommon in WD. Patients with which is uncommon and atypical. seizures usually had subcortical white However, the mechanisms and matter changes than those without.

101 Patients with seizures and white matter was treated with PGZ (i.p. at doses of changes tended to have partial seizures 10 mg/kg), and the control group was with secondary generalization and had a treated normal saline injection (i.p.) good drug response. one hours before pilocarpine (60 mg/kg, i.p.)-induced seizures and 111 excitotoxicity. Then the PGZ group 過氧化體增殖活化受體 (γ)在癲癇發 was fed with PGZ (10 mg/kg) for 28 作與癲癇生成之角色 days. We evaluated the acute seizure 黃欽威 1 蔡景仁 1 黃朝慶 2 1 成大醫院神經科 susceptibility, severity, and mortality. 2 小兒科 Both acute neuronal loss and chronic mossy fiber sprouting in the CA3 area The role of peroxisome proliferator-activated receptors-γ in were evaluated. Both groups were epileptic seizures and blood-sampled to monitor the blood epileptogenesis glucose level before and 30 minutes Chin-Wei Huang1, Jing-Jane Tsai1, Chao-Ching Huang2 after seizure challenge. 1 Department of Neurology; 2Department of Pediatrics, National Cheng Kung University Hospital Results The blood glucose level before and after pilocarpine-induced Background Peroxisome seizures was similar in both groups. proliferator-activated receptor (PPAR) is The PGZ group had longer latency to a member of the nuclear hormone severe seizures, compared to the receptor superfamily, which plays an control group The PGZ group showed important role in adipocyte similar neuronal loss in the differentiation, lipid biosynthesis, hippocampal CA3 area with the control glucose homeostasis and group. In contrast, the PGZ group immunomodulation. Among them, the attenuated chronic mossy fiber isoform PPARγ, in addition to the lipid sprouting. and glucose modulating effects, have been found to be actively involved in Conclusion PPARγ agonist neuroprotection. Thus, we conducted an (pioglitazone) appeared to attenuate experimental study to know if the PPARγ pilocarpine acute seizures and agonist-pioglitazone could ameliorate the excitotoxicity. The similar neuron loss in seizures and epileptogenesis in an animal both the control and the PGZ groups model of epilepsy suggested the dissociation between acute

seizures and excitotoxic neuronal death. Materials and methods Adult male B6 Our studies will provide a potential mice were divided into two groups: the therapeutic gateway in epileptogenesis pioglitazone (PGZ) group and the normal and epilepsy prevention. saline (control) group. The PGZ group

102 112 consciousness gradually after 頭胞類抗生素引起之廣泛週期性癲癇 withdrawal of cephalosporin. The rest 樣腦波: 苯二氮平是否有所助益? of one patient who did not receive 劉虹余 1,3 尤香玉 2,3 1 佛教慈濟綜合醫院台北分院神經科 BZDs or any AEDs recovered his 2 台北榮民總醫院神經醫學中心神經內科 consciousness gradually in 1 day after 3 陽明大學醫學院 cephalosporin was stopped.

Cephalosporin related generalized periodic epileptiform discharges on Conclusions BZDs did not EEG: should benzodiazepines be ameliorate the clinical manifestations given? in most of the patients with Hung-Yu Liu1,3, Hsiang-Yu Yu2,3 1 Department of Neurology, Buddhist Tzu Chi cephalosporin related GPEDs. General Hospital, Taipei, Taiwan 2 Department of Neurology, Neurological Institute, Taipei Veterans General Hospital, 113 Taipei, Taiwan 3 School of Medicine, National Yang-Ming 臉臂肌失張性癲癇發作的腦波表現: University, Taipei, Taiwan 橋本氏腦病變蔡孟翰莊曜聰高雄長庚醫院神經內科系腦功能暨癲癇科 Objectives To investigate the response to benzodiazepines (BZDs) of patients Electroecephalography of with cephalosporin related generalized faciobrachial dystonic seizures in a patient with Hashimoto periodic epileptiform discharges (GPEDs) encephalopathy. on electroencephalography (EEG). Meng-Han Tsai, Yao-Chung Chuang Division of Epilepsy, Department of Neurology, Kaohsiung Chung Gung Memorial Methods We collected the cases with Hospital, Taiwan

GPEDs on EEG, and selected those Background Faciobrachial dystonic whose GPEDs were temporally related to seizures (FBDS) have recently been cephalosporin use. The medical records described as an special seizure type of of the patients were reviewed for the limbic encephalitis associated with clinical presentations, treatments and leucine-rich glioma inactivated-1 outcomes. (LGI1) antibodies. Nonetheless, its

EEG correlates have not been clearly Results Nine patients with characterized. cephalosporin related GPEDs on EEG with certain degree of consciousness Case report Here we report this disturbances were included. Eight distinctive seizure semiology in patients were treated as nonconvulsive association with clinical diagnosis of status epilepticus (NCSE) and received Hashimoto encephalopathy. A 58 BZDs. Only one patient improved year-old man presented with cognitive immediately after BZD administration decline and personality change, while the other 7 patients recovered their

103 followed by frequent episodes of Chein-Wei Wang, Ming-Jun Tsai Department of Neurology, China Medical unilateral facial grimacing and upper University Hospital, Taichung, Taiwan limb dystonic posturing. Ictal electroencephalography showed that Introduction Pseudotumor cerebri FBDS were preceded by a contralateral (PTC) is a syndrome characterized by high amplitude direct current (DC) shift elevated intracranial pressure of associated with background rhythm unknown cause. The syndrome attenuation. Brain magnetic resonance classically manifests with headaches, imaging (MRI) revealed increased T2 transient visual obscurations, and signal in both temporal lobes. pulsatile tinnitus in the overweight Conventional anti-epileptic drug women. There is normal cerebrospinal treatment was ineffective but steroid fluid (CSF) composition, and no therapy resulted in significant hydrocephalus or other cause of improvement. intracranial hypertension evident on neuroimage. We report a case of newly Conclusion We described a unique diagnosed adenocarcinoma of the lung EEG pattern in associated with FBDS. metastatic to meninges presenting We emphasized the importance of symptoms and signs consistent with recognition of FBDS because of the pseudotumor cerebri. implication of earlier diagnosis of autoimmune encephalopathies. Prompt Case presentation A previously treatment of steroid may prevent healthy 47-year-old woman came to long-term cognitive sequelae and fatal our emergent department for outcome. Screening of anti-thyroid deteriorated headache since 1 month antibodies in addition to anti-LGI1 ago. About one month ago, gradual antibodies in epilepsy patients presented onset of neck tightness, occipital and with FBDS may be a useful alternative bi-temporal headache developed. The and worth further studies. pain was improved slightly with acetaminophen at the beginning. Two weeks ago, the frequency and the 頭痛及疼痛 severity of headache increased, and 114 episodes of nausea/vomiting, transient 以大腦假性腫瘤為首要表現的轉移性 visual change and left tinnitus were 肺腺癌 associated. Headache aggravated at 王建瑋蔡銘駿 midnight and sometimes awaked her 中國醫藥大學附設醫院神經部 from sleep, and it was also enhanced Pseudotumor cerebri as a first by Valsalva maneuver. One week ago, manifestation of metastatic double vision occurred. On adenocarcinoma of the lung

104 examination, the patient was afebrile Discussion PTC is a diagnosis of without meningismus at admission. exclusion. The modified Dandy criteria Extraocular movement revealed bilateral proposed for diagnosis are (1) abducens palsy. A funduscopic Symptoms and signs of increased examination demonstrated mild bilateral intracranial pressure (2) No other papilledema. On pure tone audiometry, neurologic abnormalities or impaired left-sided buzzing tinnitus with mild level of consciousness (3) CSF sensorineural hearing loss was detected. pressure greater than 200 mmH2O The CSF profiles demonstrated an with normal composition (4) Normal opening pressure of 36 cm H2O, white brain CT scan. The clinical blood cells 4/μl, red blood cells 2/μl, manifestation and examination of our micro protein 21 mg/dl and glucose 41 case correspond with the modified mg/dL. CSF cytology revealed no Dandy criteria. Whereas, PTC has been malignant cell. Initial brain CT showed reported with other potential secondary unremarkable findings (fig 1). The causes, such as vitamin A excessive symptoms were temporarily subsided intake, steroid withdrawal, oral after the lumbar puncture, so contraceptives, hypoparathyroidism, pseudotumor cerebri was impressed. obstructive sleep apnea, obstruction of Whereas, headache got worse, and cerebral venous drainage, infection, complicated with generalized seizure in carcinomatous or lymphomatous the following days. She was treated with meningitis. In our case, brain MRI ventriculoperitoneal shunt. Brain DWI (DWI and flair T2) showed significant and T2 flair MRI(fig 2) showed increased abnormal findings compatible with intensity in anterior cisterna around pons carcinomatous meningitis and further and one small enhanced nodule (about examination proved the origin as 8mm in diameter) in the left parietal lobe. adenocarcinoma in the lung. Friedman CEA was elevated (491ng/mL). and Jacobson updated the criteria and Stereotactic biopsy demonstrated as emphasized the importance of MRI metastatic adenocarcinoma. Malignancy and MR venography. In summary, our survey revealed pathology-proved findings emphasized the significance adenocarcinoma of the left lung(fig 3). of MRI (especially DWI and flair T2) Oral gefitinib (IRESSA) was used in in a case of pseudotumor cerebri, even combination with whole-brain normal CSF profile with negative radiotherapy (WBRT). Her headaches finding of malignant cells. resolved much and no abducens palsy was noted at follow up. 115 內皮前驅細胞於可逆性腦血管收縮症候群王署君 1,2 傅中玲 1,2 陳世彬 1,2

105 1 台北榮民總醫院神經醫學中心神經內科 p=0.032), but not CD34+CD133+ EPCs 2 國立陽明大學醫學系 (0.039±0.019% vs. 0.054±0.034%, Circulating endothelial progenitor cells p=0.126), in comparison with controls. in reversible cerebral vasoconstriction The numbers of CD34+KDR+ (r=-0.64, syndromes + + Shuu-Jiun Wang,1,2 Jong-Ling Fuh,1,2 Shih-Pin p=0.018) and CD34 CD133 (r=-0.56, Chen,1,2 p=0.049) cells were negatively 1 Department of Neurology, Neurological Institute, Taipei Veterans General Hospital, correlated with the LI of the right Taipei, Taiwan. MCA. 2 Faculty of Medicine, National Yang-Ming University School of Medicine Conclusions Patients with RCVS Background The pathophysiology of had reduced circulating EPCs, which reversible cerebral vasoconstriction was correlated with the severity of syndromes (RCVS) remains unknown. vasoconstrictions. Endothelial We hypothesized that patients with dysfunction might be important in the RCVS might have reduced capacity of pathogenesis of RCVS. circulating endothelial progenitor cells (EPCs) to repair the dysfunctional 116 endothelial vasomotor control. 慢性偏頭痛患者氣味恐懼的腦部構造改變: 前驅研究 1,3 1,3 2,3 1,3 Methods We prospectively recruited 王嚴鋒傅中玲凌憬峰王署君 1 台北榮民總醫院神經內科, 2 台北榮總放射 RCVS patients (within 2 weeks of 線部, 3 國立陽明大學醫學院 disease onset) and healthy controls. Flow cytometry was used to quantify the Brain structural changes in chronic numbers of circulating EPCs defined as migraine patients with osmophobia: A + + + + pilot study KDR CD133 , CD34 CD133 , Yen-Feng Wang1,3, Jong-Ling Fuh1,3, CD34+KDR+ double-positive Jiing-Feng Lirng2,3, Shuu-Jiun Wang1,3 1Department of Neurology, and 2Department of mononuclear cells. The mean flow Radiology, Taipei Veterans General Hospital, 3 velocities of middle cerebral arteries National Yang-Ming University School of Medicine (VMCA) and Lindegaard index (LI) in patients with RCVS were recorded via Objective To investigate the brain transcranial color-coded sonography. structural changes in chronic migraine (CM) patients with osmophobia with Results This study recruited 18 patients voxel-based morphometry (VBM). with RCVS and 17 age-and-sex matched controls. Patients with RCVS had Background Osmophobia during + + reduced numbers of CD34 KDR migraine attacks is reported by as (0.010±0.006% vs. 0.017±0.011%, many as 62.2% of migraineurs. The + + p=0.031) and KDR CD133 cells presence of osmophobia was (0.030±0.012% vs. 0.043±0.022%,

106 1 2 associated with higher Hospital Anxiety 佛教慈濟綜合醫院臺中分院神經內科放射診斷科 3 慈濟大學醫學院, and Depression Scale scores. There are significant overlaps in brain structures Bortezomib-induced posterior processing olfaction and emotion. reversible encephalopathy syndrome- A case report and review Whether there is structural alteration in of literature. these brain areas remains to be Chien-Hui Ho1,, Chung-Ping Lo2,3, Min-Chien 1 determined. Tu 1Department of Neurology, 2Department of Radiology, Buddhist Tzu Chi General Hospital Taichung Branch, Taichung, Taiwan, 3School of Methods We prospectively enrolled Medicine, Tzu Chi University, Hualien, Taiwan CM patients. Brain magnetic resonance imagings were obtained using a 3T MR Background Posterior reversible system (Trio, SIEMEMS Medical encephalopathy syndrome (PRES) is a Solutions, Erlangen, Germany). rare and unappreciable complication Structural alterations were assessed by among patients receiving targeted voxel-based morphometry (VBM) using chemotherapy. Herein we presented a Diffeomorphic Anatomical Registration case of PRES after bortezomib Through Exponentiated Lie Algebra treatment and proposed possible (DARTEL) method, and comparisons underlying mechanism on the basis of between patients with and without image findings. osmophobia were made. Case presentation A 51-year-old Results In total, 18 patients (5M/13F, woman who received three cycles of age 35.1±11.4 years) fulfilling ICHD-2 bortezomib (2.5 mg/each) within one criteria were enrolled. Of them, 12 month as multiple myeloma treatment patients (66.7%) had osmophobia. As developed generalized tonic clonic compared to those without, patients with seizure with post-ictal confusion after osmophobia appeared to have increased two hours of blurred vision and volumes in right insula and bilateral headache. Initial brain magnetic temporal areas on VBM analyses. resonance images (MRI) showed cortical-subcortical gyriform Conclusion The presence of enhancement, sulci effacement, and osmophobia in CM patients seemed to be vasogenic edema with diffusion associated with volume increases in right hyperintense periphery at posterior insula and bilateral temporal areas. poles, which were dramatically reversed to faint subcortical 117 hyperintensities 2 weeks later. 由 Bortezomib 誘發之可逆性後腦病變症候群：個案分析與文獻討論 Conclusion With increasing 何建輝 1 羅中平 2,3 凃敏謙 1

107 application of targeted chemotherapy, age-and-sex matched controls from 2010 to 2012 at Taipei Veterans prompt recognition of PRES turned to be General Hospital. The demographics a clinical issue. The vasogenic edema and vascular risk factors of participants with preferential white matter were collected. We measured the involvement was attributed to caliber width of retinal arterioles and venules coursing through a zone overwhelmed cerebral autoregulation, between 0.5 and 1 disc diameter from whilst cortical-subcortical gyriform the optic disc margin. The diameters of enhancement was inferred as the retinal arterioles and venules were corrected with Parr and Hubbard toxin-mediated blood-brain barrier formula. The retinal arteriole to venule damages. Our report may raise the ratio (AVR) was derived by dividing alertness of clinician on this rare the mean arteriolar caliber with the mean venular caliber. syndrome and prudent use of targeted chemotherapy. Result Patients with RCVS (n=27)

had larger retinal arteriolar and venular 118 calibers in the right eye in comparison 可逆性腦血管收縮症候群之視網膜血管研究 with the control group (n=31 ) (retinal 杜怡萱傅中玲王署君陳世彬 arterioles: 118.9±12.3 vs. 109.2±13.1 台北榮民總醫院神經內科國立陽明大學醫學 μm, p=0.008; retinal venules: 院 139.3±17.9 vs. 127.9±13.9 μm, Retinal Microvascular Involvement in p=0.012). The AVR in patients with Reversible Cerebral Vasoconstriction RCVS was significantly smaller on the Syndrome Yi-Hsuang Dou, Jong-Ling Fuh, Shuu-Jiun Wang, left eye (0.83±0.06 vs. 0.88±0.10, Shih-Pin Chen p=0.035) and nominally smaller on the Department of Neurology, Neurological Institute, Taipei Veterans General Hospital, National right eye (0.70±0.06 vs. 0.74±0.08, Yang-Ming University School of Medicine p=0.115) in comparison with controls.

Introduction Reversible cerebral vasoconstriction syndrome (RCVS) is Conclusion Patients with RCVS had characterized by recurrent thunderclap low AVR attributed to headache and reversible vasoconstrictions involving medium to disproportionately dilatation of retinal large intracranial arteries. Whether there arterioles and venules. Longitudinal is microvascular involvement in RCVS is study is required to elucidate whether still unknown. The cerebral and retinal microvasculature shared important the retinal microvascular abnormalities anatomic, physiologic, and embryologic are inherited traits or could evolve with features, which means change in the disease status. retinal microvasculature may mirror changes in the cerebral microvasculature. 119 Method We investigated the 錢幣性頭痛合併高頻率之自體免疫 retinography in patients with RCVS and 障礙

108 1 1 2 2 1 李姿慧李連輝邱文燦陳忠仁陳偉熹 anticoagulant in 1 patient, whereas 高雄長庚紀念醫院神經內科 1 風濕免疫科 2 HLA-B27 seropositivity was detected A High Frequency of Autoimmune in 1 patient. A total of 15 patients (65%) Disorders in Nummular Headache were finally diagnosed as having Tzu-Hui Li1, Lian-Hui Lee1, Wen-Chang Chui2, Chun-Jen Chen2, Wei-Hsi Chen1 Sjogren/sicca syndrome, rheumatoid 1 2 Departments of Neurology and Rheumatology , arthritis or antiphospholipid antibody Kaohsiung Chang Gung Memorial Hospital, and College of Medicine, Chang Gung University, syndrome afterwards. Kaohsiung, Taiwan

Conclusion A high prevalence of Objective The nummular headache abnormal autoimmune indices and (NH) is currently considered a form of disorders is present in primary NH peripheral neuralgia originating from the patients, suggesting a probable terminal branch in epicranial tissue but relationship between autoimmunity its etiopathogenesis is still unknown. aberration and epicranial neuralgia in Since autoimmune disorders often NH. involve the trigeminal sensory nerve to provoke craniofacial pain, we hypothesize that autoimmunity aberration 120 雙局部錢幣性頭痛：臨床分析和世界 may play a role with regards to NH. 文獻報告林宏昇陳昶宏傅睦惠李姿慧李連輝 Methods The antibodies to antinuclear 陳偉熹高雄長庚紀念醫院神經內科 factor, ds-DNA, extracted nuclear antigens, rheumatoid factor, as well as Bifocal Nummular Headache: A antiphospholipid antibodies, were Clinical Analysis and Review of Literature examined in 23 primary NH patients. The Hung-Sheng Lin, Chang-Hung Chen, Mu-Hui diagnostic criteria proposed by the 2nd Fu, Tzu-Hui Li, Lian-Hui Lee, Wei-Hsi Chen Department of Neurology, Kaohsiung Chang edition of International Classification of Gung Memorial Hospital, and College of Headache Disorders were adopted. Medicine, Chang Gung University, Kaohsiung, Taiwan

Results Among them were 16 patients Objective Nummular headache (NH) (69.6%) found as having at least one is a new category of primary headache abnormal autoimmune indices, namely, disorder characterized by consistent antibodies to antinuclear factor in 8 location, size, and shape of painful patients, SSA/La in 6 patients, areas. The pathogenesis is uncertain. rheumatoid factor in 4 patients, SSB/Ro Bifocal painful areas are rare in 2 patients, and ds-DNA in 1 patient, manifestations but may expand the respectively. An abnormal increase of clinical diversity of NH. blood anti-beta2-glycoprotein I antibody was noted in 4 patients and lupus Methods The clinical characteristics

109 of five bifocal NH patients were reported 121 and those of 11 patients in previous 穿蝶竇手術後引起之錢幣性頭痛 1 2 studies were reviewed. The diagnostic 崔智陳偉熹屏東寶建醫院神經外科 1 criteria proposed by the 2nd edition of 高雄長庚紀念醫院神經內科 2 International Classification of Headache Disorders were adopted. We classified Nummular Headache Shortly After Trans-sphenoidal Surgery the bifocal NH into two types. Type I was Chi Chui1, Wei-Hsi Chen2 defined as a simultaneous activation of Department of Neurosurgery1, Paochien Hospital, Pingtung, Taiwan two painful areas while type II was Department of Neurology2, Kaohsiung Chang defined as two painful areas occurring in Gung Memorial Hospital, and College of Medicine, Chang Gung University, Kaohsiung, different times. Taiwan

Results All 16 patients were female, Background Nummular headache with mean age of onset and initial (NH) is a newly-categorized primary presentation of 54.7 years and 58.2 years, headache characterized by a consistent respectively. There were seven type I and location, shape and size of painful area nine type II patients. The parietal area, in each attack. The etiopathogenesis is especially the tuber parietale, was the entirely unknown. Currently, the leading site of involvement in both types peripheral theory of epicranial of patients. The shape and size of painful neuralgia is widely accepted than the areas were also similar between these central theory but it cannot fully two groups. There was an equal explain the clinical picture. frequency of ipsilateral and contralateral painful areas. The pain intensity was Case Report A patient who suffered a similar in both types of patients but was relapsing and remitting course of NH milder in new painful areas than in at the high parietal area and vertex previous painful areas in type II patients. shortly after trans-sphenoidal surgical resection for pituitary prolactinoma Conclusion Bifocal NH suggests a was reported. There was no focal central role of NH but does not debunk trophic change or paresthesia but a the peripheral theory of NH. The mild allodynia in the painful area. accumulated findings in bifocal NH Patient did not exhibit patients do not support a generalization trigeminosensory feature or cranial of pain occurrence or a reproduction of trauma entirely. The pain favorably local process of epicranial neuralgia at responded to gabapentin. multiple sites in NH. Conclusion The patient herein was the second one formally reported in

110 literature. Physicians should be alerted headache types and PPI use were also for postoperative NH which is amenable performed. The conditional logistic to treatment. The findings in our patient regression model was used to estimate support a dual mechanism of NH and the odds ratios (OR) and their 95% central NH a form of referred pain. confidence intervals. The adjusted OR (aOR) was estimated after controlling 122 for confounding medication exposures. 質子幫浦抑制劑增加頭痛的風險: 一全國性病例交叉研究 Results The aOR of all types of 梁仁峰 1,,3 陳永泰 2 傅中玲 1,3 王署君 1,3 台北榮民總醫院 1 神經醫學中心神經內科 headache risk with PPI exposure was 2 內科部腎臟科 1.52 (95% confidence 3 國立陽明大學醫學系 interval=1.24–1.85) using 7-day time

Association of Headache Events with windows, and the ORs were 1.48, 1.37 Proton Pump Inhibitors Use: A and 1.22 separately using 14, 28 and Nationwide Case-Crossover Study 56-day time windows. The risks of Jen-feng Liang1,3, Yung-Tai Chen2, Jong-Ling Fuh1,3, Shuu-Jiun Wang1,3 tension-type headache or other 1 Department of Neurology, Neurological Institute, non-specific headaches were elevated and 2Division of Nephrology, Department of Medicine, Taipei Veterans General Hospital, in 7 or 14- day time windows, but risk 3Faculty of Medicine, National Yang-Ming University of migraine only increased in 28 or 56- day time windows. Compared with Objective The study sought to assess other PPIs, rabeprazole were not the risk of headache events associated associated with developing headache with use of proton pump inhibitors across all time windows. Younger (PPIs). patients who took PPIs had a greater risk to develop tension-type headache Method We conducted a case-crossover and other non-specific headaches. The study of 320,963 new-onset headache sex and age of PPIs users were not patients (ICD-9-CM codes: 784.0, 346.x, associated with an increased risk of 307.81) enrolled in the National Health migraine. Insurance Research Database in Taiwan from 1998 to 2010. The authors Conclusions PPIs usage, except compared the rates of PPIs use during raberprazole, was associated with an case and control time windows of 7, 14, increased risk of headache. The risk 28 and 56 days. Stratified analyses were was decreased over time after PPIs performed for valuing the interaction exposure. Most headaches tended to between the headache risk of PPIs use occur within 2 weeks of PPI exposure, and PPI type, age and sex. The analyses whereas migraine tended to develop of association between different after 2 weeks.

111 123 missing data. In this study the mean 偏頭痛患者的失眠情形 age was 41.85 and standard deviation 1 2 陳怡如王博仁 (SD) was 17.05. The mean insomnia 1 活水神經內科診所 2 新樓醫院神經內科 severity index was 9.68 with standard Insomnia in migraineurs deviation 6.67. There were 15 CDH Yi-Ju Chen1, Po-Jen Wang2 1Living Water Neurological Clinic; patients and 88 non-CDH patients. 2Department of neurology, Sin Lau Christian Twenty five patients had insomnia Hospital problem (insomnia severity index≧

15 ) ; 7 patients belonged to the CDH Purpose Sleep disturbance is group, 18 to non-CDH. In other words, frequently found in migraineurs. Poor in our study 46.67% CDH patients sleep quality often triggers a migraine suffered from insomnia and 20.45% attack; on the other hand, headache non-CDH patients complained about would interfere the sleep. After long term insomnia. However, 10 CDH patients insomnia, the migraineurs would get (66.67%) reported that they had more attacks and the quality of life insomnia more than 15 days in one becomes worse. The aim of this study is month, and 37(42%) reported in the to evaluate whether there is a higher non-CDH patients. incidence rate of insomnia in more severe migraineurs. Conclusions The results showed a

higher rate of insomnia in CDH Materials and methods This is a patients. However, overestimated cross-sectional research design. Totally insomnia was also noted in these 107 patients with diagnosis of migraine patients. Nevertheless, insomnia was and probable migraine were recruited also not infrequent in non-CDH from living water clinics from March, patients. We need to pay more attention 2012 to June 2012. We used insomnia to sleep problem in migraineurs. severity index (ISI) as a tool to evaluate the severity of insomnia. According to 124 the definition, the insomnia severity 椎體骨泥整形術後引起顱內低壓性 index ≧15 indicated clinical insomnia. 頭痛之案例報告 At the same time, estimated insomnia 陳彥文陳蔚邦張振聲許世斌郭弘昌 days in recent one month were recorded. 義大醫療財團法人義大醫院神經內科義守大學 The migraineurs were divided into chronic daily headache (CDH) group and Intracranial Hypotension as a Rare non-CDH group. Complication Post Vertebroplasty: A Case Report Yen-Wen Chen, Wei-Pang Chen, Chen-Sheng Result Finally 103 migraineurs‘ data Chang, Shih-Pin Hsu, Hung-Chang Kuo Department of Neurology, E-DA Hospital / were analyzed; 4 were excluded due to I-Shou University, Kaohsiung, Taiwan

112 Background The vertebral Conclusion Our report highlights the compression fracture was one of the possibility and importance of frequent complications of osteoporosis. intracranial hypotension related to CSF The percutaneous vertebroplasty (PVP), leakage after procedure of PVP. It a minimally invasive therapeutic reminded the clinicians watching for procedure, has been applied in treatment any possibility of occurrence of such of osteoporotic vertebral compression complication during or after procedure fractures with generally considered low of PVP and there was no definite risk of complication. benefit than other conservative treatment. Case report A 51-year-old healthy woman without any history of neurologic 125 or orthopedic illness suffered from severe 可逆性腦血管收縮症候群之高解析 low back pain since about ten days ago. 度 3T 血管壁磁振造影研究傅中玲 1,2 王署君 1,2 陳世彬 1,2 Her pain radiated to bilateral subcostal 1 台北榮民總醫院神經醫學中心神經內科 areas and was aggravated by body 2 國立陽明大學醫學系 bending forwardly. The orthopedic Intracranial arterial wall imaging survey included T-L spine X ray and MRI using high-resolution 3-tesla MRI in revealed T12 compression fracture. Bone reversible cerebral vasoconstriction densitometry (BMD) examination proved syndromes Jong-Ling Fuh,1 2 Shuu-Jiun Wang,1,2 Shih-Pin osteopenia. Those symptoms were not Chen1,2 1 relieved under conservative treatment or Department of Neurology, Neurological Institute, Taipei Veterans General Hospital, medication only. PVP was performed and Taipei, Taiwan. 2 the whole operation course was smooth Faculty of Medicine, National Yang-Ming University, Taipei, Taiwan without obvious intra-operative complication. However, symptoms of Background Limited intracranial hypotension including acute histopathological studies suggested onset of severe orthostatic headache, that there was no vascular wall head fullness sensation, nausea, and inflammation in reversible cerebral vomiting were noted just a few hours vasoconstriction syndromes (RCVS); after PVP. The followed spinal MRI however, the procedure was invasive revealed fluid accumulation at posterior and there could be sampling biases. We epidural space of T11-12-L1 and investigated the vascular wall cerebrospinal fluid (CSF) leakage was pathology in RCVS using a impressed. After adequate hydration and non-invasive high-resolution magnetic complete bed-rest for five days, her resonance arterial wall imaging. symptoms got successful improving.

113 Methods We prospectively recruited Conclusions Half of the RCVS patients with RCVS from the headache patients could have a vascular wall clinic of Taipei Veterans General pathology using a high-resolution Hospital from 2010 to 2012. Age-and-sex arterial wall imaging, but the etiology matched migraine patients were recruited could be heterogeneous. Reversibility for comparison. All participants received of vascular wall enhancement in vascular wall imaging using contrasted selected cases suggested that a certain T1 fluid-attenuated inversion recovery degree of vascular wall inflammation (T1-FLAIR) with a 3-tesla magnetic should still be considered. resonance machine. The vascular wall involvement was rated as marked, mild 126 (patchy) and no vascular wall 臺灣陣發性與慢性偏頭痛患者失能 enhancement. Some patients with 情況、生活品質、及醫療資源利用之比較分析 vascular wall enhancement received 彭冠博，王博仁，傅中玲，王署君 follow-up images to evaluate whether the 台北榮民總醫院; 活水神經內科診所 enhancement was reversible. Comparisons of disability, quality of

life, and resource use between Results Fifty-four patients with RCVS chronic and episodic migraineurs: A and 20 patients with migraine were clinic-based study in Taiwan Kuan-Po Peng, Po-Jen Wang, Jong-Ling Fuh, recruited during the study period. After Shuu-Jiun Wang excluding patients with poor imaging Taipei Veterans General Hospital; Living Water Neurological Clinic quality, 46 RCVS and 16 migraine patients were eligible for final analysis. Background Chronic migraine (CM) In patients with RCVS, 6 (13%) had was associated with a higher disease marked, 17 (37%) has mild, and 23 (50%) burden than episodic migraine (EM) in had no vascular wall enhancement; while a recent international web-based survey. only 2 (13%) of the migraine patients had However, Asians with CM were mild vascular wall enhancement and underrepresented in this study. none had marked vascular wall enhancement (p=0.031). The arterial wall Objectives We investigated if CM enhancement was not associated with any and EM differed in headache-related vascular risk factors, severity of disability, health-related quality of life vasoconstriction or triggering factors. (HRQoL) and health care resource Three patients with marked vascular wall utilization in Taiwan. enhancement received follow-up; one Methods: This study enrolled patients had complete resolution of enhancement, with EM and CM from two headache one improved to only patchy clinics in Taiwan, the Taipei Veterans involvement and one remained the same. General Hospital and Living Water

114 Neurological Clinic. Migraine diagnosis 127 was made by physicians based on the 顳動脈炎頭痛之病例報告及文獻回顧 International Classification of headache 黃子洲蘇慧真柯登耀王博仁活水神經內科診所 nd disorders, 2 edition (ICHD-2) and CM diagnosis based on the Silberstein-Lipton Headache Attributed to Temporal Arteritis–a case report and criteria. All participants completed a literatures review questionnaire including Tzu-Chou Huang, Hui-Chen Su, Teng-Yao Ke, sociodemographics, Migraine Disability Po-Jen Wang Living Water Neurological Clinic Assessment (MIDAS), EuroQol five-dimensions (EQ-5D), Background Headache attributed to Migraine-Specific Quality of Life v2.1 temporal arteritis (TA) is a secondary (MSQ), Patient Health Questionnaire-4 headache caused by granulomatous (PHQ-4), productivity and health care inflammation of temporal artery. It resource utilization. affects individuals over 50 years of age and is quite rare in Taiwan. We report a Results A total of 331 patients patient who had pathology-proved TA completed the study, including 164 and discuss about the cases which had (49.5%) EM patients and 167 (50.5%) been reported in Taiwan. CM patients. CM patients, compared to EM patients, reported a higher MIDAS Case report A 73-year-old woman score, lower generic (EQ-5D visual has past history of coronary artery analogue scale score and EQ-5D index disease. She complained of bilateral score) and migraine-specific (all three temporal headache 2 weeks prior to the domains of MSQ) HRQoL, higher levels visit. It was relatively acute onset with of anxiety and depression (PHQ-4 ≥6), throbbing character and was moderate and greater health care resource in severity. The pain persisted all the utilization and productivity loss. Positive day and would extend to frontal and correlations were found between these occipital areas sometimes. No nausea instruments and levels of anxiety and or tearing was combined. She also depression. complained blurred vision and bilateral jaws soreness especially when chewing. Conclusion Compared to EM, CM was Physical examination showed nothing significantly associated with higher special except for visible, engorged disability, lower HRQoL, and greater tender vessels over bilateral temporal health care resource utilization and areas. Lab result revealed elevated productivity loss. ESR level. We soon prescribed prednisolone and did temporal artery biopsy (TAB). The pathology showed

115 positive for temporal arteritis. We then complication of blindness, one had kept on prednisolone treatment. This tongue necrosis and one had patient got obviously improved after basilar-vertebral artery involvement treatment. and eventually expired. Severe outcome is not uncommon in Taiwan. Discussion TA is rare in eastern countries. Although pathological proof is 128 important, it is not essential for the 鼻腔內麻醉時的雷擊性頭痛 diagnosis. According to the criteria of 黃敬棠陳盈助佛教慈濟綜合醫院台中分院神經科 American Academy of Rheumatology, new headache, onset age more than 50, Thunderclap Headache During physical finding on the affected temporal Intranasal Anesthesia Ching-Tang Huang, Ying-Chu Chen artery and elevated ESR are sufficient to Neurological Institute, Buddhist Tzu Chi make diagnosis. International General Hospital Taichung Branch

Classification of Headache Disorders–II Background Thunderclap headache criteria require new headache, tender refers to an abrupt and very severe scalp vessel and elevated ESR (or CRP) headache reaching peak intensity or positive TAB, plus an effectiveness of within 1 minute. In addition to steroid treatment. Headache is the most aneurysmal subarachnoid frequent symptom, but the other systemic hemorrhage, there are many secondary or specific symptoms should also be causes which need thorough noted. These include fever, malaise, jaw investigations. We present a case of claudication, polymyalgia rheumatica recurrent thunderclap headache with and, most notable, visual complaints. onset during intranasal anesthesia. Temporal artery is not the only artery which would be affected. Clinicians Case Report A 39-year-old female should pay attention to other arteries without systemic disease or recent such as coronary artery when treating trauma history presented to our these patients. There are 8 TA patients emergency room complaining of being reported in literatures in Taiwan. sudden onset bilateral occipital Five of them are male. Their age ranged thunderclap headache during intranasal from 68 to 93. Six of them are anesthesia with lidocaine-epinephrine pathology-proved. All of them had soaked cottage, and two recurrences in elevated ESR, 7 had engorged or tender the following 5 days: one attack temporal artery, 4 had jaw claudication, 3 provoked by defecation and another had diplopia, and 3 had systemic spontaneous attack during sleep. Initial complaint such as malaise or body brain CT was normal. However, weight loss. Four of them had minimal cortical subarachnoid

116 5 hemorrhage at bilateral occipital lobe Department of Radiology, National Yang-Ming University, was found in brain MRI (T2-FLAIR). 6 Department of Biomedical Imaging and Further cerebral angiography study Radiological Sciences, National Yang-Ming University disclosed multifocal vasospasm involving the bilateral anterior and Objective Previous functional middle cerebral arteries. This patient was imaging studies in episodic cluster given oral nimodipine, and got satisfied headache (CH) patients revealed outcome. The clinical presentation and altered brain metabolism concentrated imaging findings are consistent with on the central descending pain control reversible cerebral vasoconstriction system. However, it remains unclear syndrome (RCVS). whether changes in brain metabolism during the “in bout” period are due to Conclusion RCVS can be primary or structural changes and whether these secondary. Vasoactive substances are structural changes vary between the “in important secondary factors, such as bout” and “out of bout” periods. intranasal administration of lidocaine-bosmin in our case. Clinicians Methods To quantify brain structural should be aware of this neurological changes in CH patients, the regional emergency in order to provide early gray matter volume (GMV) was diagnosis and treatment. compared among 49 episodic CH patients during the “in bout” period 129 and 49 age- and sex-matched controls 叢集性頭痛患者在額部痛覺調控網絡 using voxel based morphometry 中灰質體積之改變 approach (VBM). Twelve patients 楊富吉 1,2, 周坤賢 3, 傅中玲 4, 凌憬峰 5, 林永煬 1,4, 林慶波 3,6*, 王署君 1,4* were rescanned during the “out of 1 國立陽明大學腦科學研究所 2 三軍總醫院神 bout” period to evaluate the changes, if 3 4 經內科國立陽明大學神經科學研究所台北 any, between these two periods. 榮民總醫院神經內科 5 台北榮民總醫院放射科 6 國立陽明大學生物醫學影像暨放射科學系 Results Compared with healthy Altered Gray Matter Volume in the controls, CH patients showed Frontal Pain Modulation Network in significant “in bout” GMV reductions Patients with Cluster Headache Fu-Chi Yang 1,2, Kun-Hsien Chou 3, Jong-Ling in the bilateral middle frontal, left Fuh 4, Jiing-Feng Lirng 5, Yung-Yang Lin 1,4, 3,6* 1,4* superior and medial frontal gyri. Ching-Po Lin , Shuu-Jiun Wang 1 Institute of Brain Science, National Yang-Ming Compared to “out of bout” scans, the University, 2 Departments of Neurology, Tri-Service General “in bout” scans revealed significant Hospital, National Defense Medical Center, GMV increases in the left anterior 3 Institute of Neuroscience, National Yang-Ming University, cingulate, insula, and fusiform gyrus. 4 Department of Neurology, National Yang-Ming Additionally, compared to healthy University,

117 controls, the “out of bout” scans revealed Case Report The first case is a a trend of GMV reduction in the left 47-year-old woman who suffered from middle frontal gyrus. No area of GMV intermittent severe throbbing headache increase was found in patients during the at posterior neck to vertex, associated “in bout” or “out of bout” periods. with visual disturbance, nausea, and vomiting for about one week. Physical Conclusion These affected regions and neurologic examination showed primarily belong to frontal pain elevated blood pressure (168/113 modulation areas. Therefore, these GMV mmHg), poor attention, and visual changes may reflect insufficient field defect. Diffusion-weighted brain pain-modulating capacity in the frontal MRI revealed bilateral parieto-occipital areas of CH patients and may be high signal intensity and MR important to the pathophysiology of CH. angiography showed multifocal stenosis at bilateral MCAs. She was 130 treated with nimodipine and aspirin, 可逆性後腦病變症候群併腦血管收縮 with gradual improvement. The second 症候群-個案報告 case is a 33-year-old pregnant woman 蔡志宏黃文柱國立成功大學醫學院附設醫院神經部 (GA 32+1wks) who suffered from headache at posterior neck, elevated Posterior reversible encephalopathy blood pressure (191/118 mmHg), and syndrome associated with reversible HELLP syndrome (hemolysis, elevated cerebral vasoconstriction syndrome- Case report liver enzymes, and low platelet count). Chih-Hung Tsai, Wen-Juh Hwang She developed two episodes of Department of Neurology, National Cheng Kung University Hospital generalized convulsions, with postictal right hemiparesis. Brain CT showed Background Posterior reversible hypodense lesions at bilateral encephalopathy syndrome (PRES) is a frontoparietal lobes and left occipital clinico-radiological diagnosis and the lobe. Brain MRI FLAIR sequence 註解 [SC6]: And MRA? patient typically presents with headache, revealed multifocal stenosis in bilateral visual disturbance, elevated blood ACAs, MCAs, and PCAs in addition to pressure, confusion, or seizures. the high signal change at bilateral Reversible cerebral vasoconstriction frontoparietal and left occipital lobes. syndrome (RCVS) usually presents as a After emergent cesarean section and thunderclap headache in middle-aged treatment with phenytoin, women. We presented 2 cases who levetiracetam, and nimodipine, her suffered from both PRES and RCVS at general condition gradually returned to the same time. normal.

118 Conclusion We reported two headache state-dependent manner in middle-aged women with headache patients with migraine. On the other whose image studies showed the findings hand, EEG gamma-band activity has not yet been adequately evaluated in of both PRES and RCVS. We suspect patients with migraine. that the vasogenic edema seen in the

PRES and the mutifocal stenosis of Objective To investigate the resting cerebral vessels in the RCVS may share EEG power spectrum and its relation to some common pathophysiology. Brain migraine state. MRI and MRA may be indicated in patients whose CT scan sowed the Methods Resting EEG data (30 findings of PRES. Prompt diagnosis and seconds for 3 times) was recorded with 256 Hz sampling rate, and 19 adequate treatment are crucial for electrodes positioned according to the preventing brain damage and irreversible international 10-20 system, while the neurological deficits. subjects kept awake and alert without attention demand or cognitive load. 131 After manual and automatic rejection 偏頭痛患者腦波頻譜於近頭痛期之正 of artifacts, independent component 常化現象 analysis (ICA) was applied to remove 賴冠霖 1,2,3,4 蕭富榮 5 廖光淦 2,3 傅中玲 2,3 the noises. Minimum norm estimate 王署君 2,3,5 (MNE) analysis was then used to 1 台北市立關渡醫院神經內科, 2 台北榮民總醫 transform the original electrode-based 院神經內科, 國立陽明大學醫學院 3 醫學系, 4 EEG signal into specific brain 臨床醫學研究所, 5 腦科學研究所 region-of-interest (ROI)-based EEG Normalization of EEG Power activity. After fast Fourier Spectrum in Patients with Migraine transformation (FFT), individual alpha During Peri-ictal Periods frequency (IAF) peak was firstly Kuan-Lin Lai1,2,3,4, Fu-Jung Hsiao5, Kwong-Kum determined. With reference to the IAF, Liao2,3, Jong-Ling Fuh2,3, Shuu-Jiun Wang2,3,5 the frequency bands of interest were 1Department of Neurology, Taipei Municipal classified into delta (IAF-8 to IAF-6 Gandau Hospital; 2Neurological Institute, Taipei Hz), theta (IAF-6 to IAF-2 Hz), alpha Veterans General Hospital; National Yang-Ming (IAF-2 to IAF +2), beta (13-30 Hz), University School of Medicine, 3Faculty of and gamma (30-40 Hz). Furthermore, a Medicine, 4Institute of Clinical Medicine, normalization of spectral power was 5Institute of Brain Science done by normalizing the power at each frequency band with total power from Background Previous resting EEG delta to gamma to reduce the spectral analyses in patients with inter-individual variability. We then migraine showed inconsistent results. compared the relative power of each The inconsistency might be related to the band across all ROIs in patients with spontaneous fluctuation of migraine and control subjects. electrophysiological activity in a Statistical significance was defined as a

119 p-value of < 0.05. All tests were Migraine Disability Assessment two-tailed. Scale and cortical thickness in patients with migraine

1-3Tzu-Hsien Lai, MD; 1Yi-Chia Kung, MA; 2, Results One hundred and twenty-seven 4Jong-Ling Fuh, MD; 1Kung-Hsien Chou, episodic migraine patients were recruited PhD; 1Ching-Po Lin, PhD; 2, 4Shuu-Jiun Wang, MD (M/F: 29/98, age: 35.1 ± 10.3, headache 1Section of Neurology, Department of Internal frequency: 4.3 ± 2.6 (days/m). Compared Medicine, Far Eastern Memorial; 2Institute of 3 to age- and gender-matched controls Neuroscience and Department of Medicine, National Yang-Ming University; 4Department (n=20, M/F: 8/12 [p=0.163], age: 36.4 ± of Neurology, Neurological Institute, Taipei 6.8 [p=0.454]), patients during inter-ictal Veterans General Hospital period (n=75) showed decreased alpha and theta power over the whole brain and Objective To discover the main increased delta power over bilateral causes of MIDAS whether related with frontal regions. These spectral emotion or not from the clinical and abnormalities were less obvious or neuroimaging researches. normalized during pre-ictal (n=21), ictal

(n=19) or post-ictal (n=12) periods. Of Method First, we examined the note, compared with controls, patients with migraine had a consistent increment relationship between MIDAS score and of beta- and gamma-band powers clinical variables by the multiple regardless of their clinical headache state. -regression analyses. We then examined the relationship between Conclusions Resting EEG spectral cortical thickness change and Migraine analysis in patients with migraine Disability Assessment (MIDAS) score showed a state-dependent fluctuation at within 94 migraine patients, including delta-, theta- and alpha-band range, 34 episodic migraine (EM) patients which tended to be normalized during peri-ictal periods. Besides, we found that and 60 chronic migraine (CM) patients. gamma-band power, and beta-band In the end, the relationship between power to a lesser extent, were emotional variables, depression and consistently increased in patients with anxiety score of HADS, and migraine, which deserves further studies MIDAS-associated brain areas were to evaluate its potential as a non-invasive analyzed. biomarker.

Results The migraine frequency 132 explained the highest variance of the 偏頭痛失能量表與偏頭痛病人皮質厚度關聯性之分析 MIDAS score together with migraine 2 賴資賢 1-3 龔怡嘉 2 傅中玲 3,4 周坤賢 2 林慶波 2 patients (R =0.244), followed by 3,4 王署君 depression score, photophobia, and 1 亞東醫院神經內科 2 陽明大學神經科學研究所 3 醫學系 4 台北榮總神經內科 migraine intensity with a total variance of 43.6% (F-value=9.406, p<0.001). Investigating the correlation between Cortical thickness was negatively

120 correlated with MIDAS score in patients cervicogenic headache clinically. with migraine over left superior temporal, left precentral, left supramarginal, left Report of Cases A 49 year-old superior frontal, left temporal pole, right worker without headache history insula, and right inferior temporal areas. visited the outpatient clinic due to abrupt thunderclap headache while Conclusions From both the behavioral walking in the previous night. He and biological evidences, the disability complained persistent, extremely assessed by MIDAS was not only related severe headache over left with migraine per se, but also the temporal-occipital area which was comorbid depression though the design exacerbated with neck movement, of this questionnaire has no mention of cough and swallowing and was not the emotion questions. relieved by any medicine at all. And, his motion of neck was restricted 133 because of his severe headache and 頸因性頭痛-一案例報告 neck pain. The initial results of 蘇慧真林典佑許永居王博仁 neurologic examination were 活水神經內科診所國立成功大學附設醫院神經內科 unremarkable except presentation of 戴德森醫療財團法人嘉義基督教醫院神經內科 bilateral Babinski signs. On the other

Cervicogenic headache- A case report day, progressive urinary difficulty was Hui-Chen Su1 Tien-Yu Lin2 Yung-Chu Hsu3 reported; besides, hypethesia over right Po-Jen Wang1 1 Living Water neurological clinic, Tainan, extremities and left upper limb minor Taiwan weakness were noticed. Brain 2Department of neurology, National Cheng-Kung University Hospital, Tainan, Taiwan computed tomography revealed 3 Division of Neurology, Department of Internal segmental hyperdense lesion from left medicine, Ditmanson Medical Foundation Chia-Yi Christian Hospital, Taiwan side cercivomedullary junction to C4 cervical cord. Cervical MRI with Background Cervicogenic headache is gadolinium enhancement showed known as a secondary headache segmental lesion with hypointense originating from neck including cervical signal on T1WI and hyperintense spine, nerve and muscle. The criteria of signal on T2WI, with a small cervicogenic headache in ICDH-II were enhancing nodule within the left C1 proposed. However, the differential spinal cord. The patient received C1 diagnosis with cervicogenic headache is laminectomy with grossly total quite important and the clinical excision of intramedullary lesion and presentation sometimes bothered the duraplasty. Pathological findings neurologist. We presented a patient with commented that there are aggregates of symptomatic headache compatible with small-caliber vessels with thin wall

121 without presentation of elastic fiber after Background Trigeminal neuropathy Verhoeff-van Gieson stain, suggesting with motor involvement are relative vascular malformation. The headache uncommon and the etiology is diverse. relieved after his operation. Trigeminal Schwannomas are the second common intracranial Discussion The cervicogenic headache Schwanommas next to the vestibular is a secondary headache with prevalence Schwannomas. The most common of 1-4%, and much higher to 53% in symptom is facial hypesthesia and pain; patients after neck trauma. It is less masseter muscle atrophy is relatively common than migraine and tension-type rare. headache and is underdiagnosed easily. Although the pathophysiology is still Case Report A 58-year-old male debated, several disorders share similar suffered from progressive facial feature as cervicogenic headache paresthesia and pain located at the including dissecting aneurysm of dermatome of left mandibular nerve vertebral/carotid artery, posterior fossa (V3) for 4 months. Two months lesion, neck-tongue syndrome, C2 following the onset of paresthesia, the neuralgia and cervical migraine, etc. The patient noted atrophy of his masseter diagnosis of cervicogenic headache muscle. Blink reflex study revealed indeed is a challenge to the physicians. normal result while needle Our patient presented as severe headache electromyography showed active and neck pain which correspond with the denervation change over masseter clinical criteria of cervicogenic headache muscle which was compatible with and it resulted from rupture of cervical trigeminal motor neuropathy. The sella vascular malformation. computed tomography did not show any abnormality. A posterior fossa MRI scan was arranged thereafter but still 神經放射線學 revealed no intracranial lesion. Due to 134 symptom progression, we arranged 三叉神經神經鞘許旺氏細胞瘤引起之 thin-section MRI (1.5mm focused on 三叉神經感覺及運動病變：案例報告 sella) 3 weeks after the previous MRI 周政達梁仁峯 study and disclosed a 1.6* 1.5* 0.3 cm 台北榮民總醫院神經醫學中心神經內科 tumor at cavernous sinus and temporal Trigeminal Schwannoma presented as floor with extracranial extension via trigeminal sensorimotor neuropathy: A foramen ovale adhered to mandibular case report Chen-Ta Chou, Jeng-feng Liang nerve. After the tumor removal, the Department of Neurology, Neurological Institute, pathologic report revealed Taipei Veterans General Hospital Schwannoma. This patient got

122 improvement in paresthesia after the Background Chiari malformation operation. (CM) is a congenital disorder in which the cerebellar tonsils protrude through Discussion The common causes of the foramen magnum into the spinal trigeminal neuropathy include trauma, cord. There are four types of CM, and neoplasm, inflammation/autoimmune CM type I (CM-I) can occur in diseases or idiopathic. Most trigeminal children and adults and may or may neuropathy caused sensory symptoms not be symptomatic. Syringomyelia is such as pain, numbness or loss of sensory, present in 25% of patients with Chiari but sometimes motor symptom was noted. Malformation. However, motor involvement with muscle wasting may rarely be seen in Case report A 14 years old girl has autoimmune/ inflammatory related or experienced slowly progressive idiopathic trigeminal neuropathy. The inability to feel hot sensation when existence of trigeminal nerve-innervated taking shower and feel tingling pain by muscle atrophy raises the concern of mosqueto sting over neck, upper arm mass lesion and warrants a detailed and upper back on the left side for 2 image study. years. Initially, Brain CT and EP study showed normal finding 2 years ago . Conclusion Trigeminal neuropathy Recently , expasion of the location of with motor involvement is rarely sensory impairment was noted. She idiopathic. A small tumor, such as denied previous headache, neck or trigeminal Schwannoma is a possible back pain ,tinnitus, vertigo, limb etiology. Thin-section MRI study is weakness, numbness , unsteadiness of required for these cases if preliminary gait , urine or stool disturbance. No image study is fail to demonstrate the previous history of traumatic head, lesion. neck or cervical injury. The neurological examination revealed loss 135 of pin prick and temperature sensation Ｃhiari 氏畸形第一型併脊髓空洞症– over left neck,upper arm and left upper 以單側背心狀感覺缺損表現 trunk above T9 level( unilateral 林鴻志 1 馮偉雄 2 劉競雄 3 羅東博愛醫院神經內科 vest-like pattern) with preservation of soft touch and proprioception. Brain Type I Chiari malformation with and spinal MRI showed a elongated, severe syringomyelia presented with peg-shaped cerebellar tonsiles unilateral “vest-like”sensory impairment extending below the foramen magnum Hung-Chih Lin, Vai-Hong Fong, Ching-Hsiung and a syringohydromyelia at whole Liu Department of Neurology, Lo-Tong Pohai spines. The final diagnosis was Chiari Hospital, I-Lan, Taiwan

123 4 malformation with syringomyelia. No Department of Nuclear Medicine, National Taiwan University Hospital medical management was advised because of no headache or any painful Background Logopenic aphasia sensation. We referred this patient to (LPA) variant of primary progressive aphasia (PPA) is featured with word medical center for further evaluation of retrieval difficulty without memory pediatric neurosurgeon. No clinical impairment. The underlying pathology improvement of sensory deficit after of LPA is mostly linked to amyloid A deposition, as in Alzheimer’s disease. drainage of CSF was mentioned by As compared to another variant of PPA, patient about 1year later. progressive nonfluent aphasia, LPA is rarely reported to associate with Conclusion We report a case of late parkinsonism and the underlying mechanism is unclear. Using childhood onset CM-I with conventional and nuclear imaging syringomyelia which clinically presented studies, we measured whole-brain with painless unilateral“vest-like”sensory amyloid deposition, glucose metabolism, and dopa decarboxylase loss . The presence of syrinx is known to activity in a patient with LPA and give specific signs and symptoms that asymmetric parkinsonism. vary from dysesthetic sensations to Case Report A 74-year-old healthy algothermal dissociation to spasticity and man began to have word-finding paresis , and is one of absolute difficulties and decreased verbal output indications for surgical intervention. since two years ago with preserved comprehension and grammar abilities. PPA was clinically diagnosed 136 according to Mesulam’s criteria. 腦中類澱粉蛋白沈積、多巴脫羧酶活性 Memory and other cognitive 及葡萄糖代謝在一位Logopenic 失語症 impairment developed about one-year 合併巴金森症候群患者的表現 later. Parkinsonism feature with right 范齡勻 1 王樹聲 2 林靜嫻 3 陳達夫 3 曾凱元 4 limbs clumsiness, right jaw tremor and 顏若芳 4 邱銘章 3 facial myokymia gradually developed 1 恩主公醫院神經科 later. Neuropsychological test revealed 2 國立台灣大學醫學院附設醫院家庭醫學科 deficits in language, visuospatial and 3 國立台灣大學醫學院附設醫院神經科 both verbal/non-verbal memory 4 國立台灣大學醫學院附設醫院核子醫學科 functions with MMSE was 6 (education: 6 years). Brain magnetic Amyloid Retention, DOPA resonance image (MRI) demonstrated Decarboxylase Activity and Glucose asymmetrical atrophy in the left Metabolism in A Patient with perisylvian, temporal and parietal areas. Logopenic Aphasia and Parkinsonism The [18F]FDG scan showed 1 2 Ling-Yun Fan , Shu-Sheng Wang , Chin-Hsien hypometabolism in the areas Lin3, Ta-Fu Chen3, Kai-Yuan Tzen4, Ruoh-Fan 4 3 corresponding to MRI atrophy with Yen , Ming-Jang Chiu 1Department of Neurology, En Chu Kong preserved and symmetrical metabolism Hospital of basal ganglia. Consistent with 2Department of Family Medicine, National previous report, the amyloid retention Taiwan University Hospital was diffuse throughout cortexes and 3Department of Neurology, National Taiwan basal ganglia without asymmetry. University Hospital

124 Unexpectedly, the [18F]FDOPA scan Sciences, Kaohsiung Medical University, revealed symmetrical and normal uptake Kaohsiung in bilateral basal ganglia, suggesting no obvious pre-synaptic dopaminergic Background Voxel-based neuronal loss with intact dopa morphometry (VBM) is an automatic decarboxylase activity. The genetic analysis for C9Orf72 gene was normal unbiased method to detect group without increased number of differences in the gray matter (GM). hexanucleotide repeats. Neuroimaging meta-analysis methods

Conclusion Our case demonstrated the are available recently. patterns of preserved and symmetrical glucose metabolism and dopa Objective To identify consistent and decarboxylase activity in bilateral basal specific structure changes in patient ganglia, which are different from the findings of corticobasal degeneration with narcolepsy by reviewing the (CBD), and Parkinson’s disease with literature on the results of VBM of MR dementia. Our findings support a imaging studies. disorganized sensorimotor model that disintegration of circuits between left temporal-parietal cortex and basal Methods We searched PUBMED for ganglion due to cortical amyloid the relevant articles published from deposition and cortical hypofunction causing the asymmetrical Parkinsonian January 2000 to November 2011. This features in our patient rather than review included all VBM research presynaptic dopaminergic dysfunction or papers using the signed differential neuronal loss. mapping (SDM) procedure to compare

either GM concentration or GM 137 以逐體素為基礎的型態計量學之整合 volume to index GM differences 分析來評估猝睡症患者的腦結構變化 between narcolepsy patients and 翁旭惠 1 蔡元雄 1 陳志峰 1 楊子賢 1 李建 normal controls. 2 3 4 德許家瑜楊俊毓 1 嘉義長庚紀念醫院放射診斷科 2 嘉義長庚紀念醫院神經內科 Results People with narcolepsy had 3 雲林長庚紀念醫院神經內科 regional GM reduction in right 4 高雄醫學大學公共衛生學系所 hypothalamus, left anterior cingulate Meta-analytical Comparison of gyrus (BA 32), both sides of inferior Voxel-Based Morphometry Studies in frontal gyrus (BA 47), left medial Narcolepsy Hsu-Huei Weng1, Yuan-Hsiung Tsai1, Chih-Feng frontal gyrus (BA 10) and left middle Chen1, Tzu-Hsien Yang1, Jiann-Der Lee2, temporal gyrus (BA 21), which may be Chia-Yu Hsu3, Chun-Yuh Yang4 1Department of Diagnostic Radiology, Chang related to the daytime sleepiness, Gung Memorial Hospital at Chia-Yi, Taiwan. attention and memory abnormalities 2Department of Neurology, Chang Gung Memorial Hospital at Chia-Yi, Taiwan. seen in this patient population. 3 Department of Neurology, Chang Gung Memorial Hospital at Yun-Lin, Taiwan. 4Faculty of Public Health, College of Health

125 138 Conclusion Tractography in the 以磁振神經纖維追蹤技術評估顳葉梗 present case demonstrated a 塞造成之偏癱合併同側小腦運動失調 cerebellothalamoparietal tract 陳兆煒陳晉誼鄧浩文台北醫學大學-萬芳醫院神經內科 connecting right cerebellum to the portion of left posterior parietal lobe. Ataxic hemiparesis syndrome caused Ataxia of right limbs might be related by parietal infarction: a case evaluated by MR tractography to the left posterior parietal lobe Jowy Tani, Chin-I Chen, Hao-Wen Teng infarction, and the underlying Department of Neurology, Wan Fang Hospital, Taipei Medical University mechanism might be related to the involvement of the Background Ataxic hemiparesis, one cerebellothalamoparietal tract. The of the classical lacunar stroke syndromes, weakness of right limbs in our case is most commonly caused by lesions in might be due to infarction of left pons, the internal capsule, or the corona paramedian precentral cortex. radiata. Although rare, that had been reports that infarction involving posterior 139 parietal lobe can cause ataxic 紅斑性狼瘡併疑似栓塞性血小板減 hemiparesis. In the present report, we 少紫斑症及可逆性腦炎:病例報告及 utilized diffusion tensor imaging (DTI) to 文獻回顧郭曄嶸 1 劉濟弘 1* 秦嗣騏 2 李宗海 1 study the pathway involved in an ataxic 1 2 林口長庚紀念醫院神經內科放射診斷科 hemiparetic patient with frontoparietal cortical infarction. Reversible Encephalopathy in Systemic Lupus Erythematosus and

Thrombocytopenia Mimic Case report A 61-year-old man Thrombotic Thrombocytopenic presented to us with right limbs Purpura: Case Report and Literature Review weakness and ataxia. Sensory 1 1* Yeh-Jung Kuo , Chi-Hung Liu , Shy-Chyi examinations were normal for light touch, Chin2, Tsong-Hai Lee1 1 2 pinprick, temperature, priopioception, Department of Neurology, Department of Medical Imaging and Intervention, Chang and vibratory sensation. A brain magnetic Gung Memorial Hospital, Linkou Medical resonance imaging (MRI) demonstrated Center acute infarction of left parietal cortical Background Thrombotic area and frontal cortex. DTI tractography thrombocytopenic purpura (TTP) and revealed that the acute flare up of systemic lupus cerebellothalamoparietal fibers erythematosus (SLE) share several connecting the left posterior parietal lobe common features including to the right cerebellum passed through thrombocytopenia, acute renal failure, the infarcted region. and central nervous system (CNS) involvements.

126 Case report A 36-year-old male with presence of TTP. underlying SLE experienced progressive bilateral lower leg swelling related to 140 acute renal failure (creatine: 4mg/dL). 無病灶之顳葉癲癇之發作間期 His hemogram showed SPECT :個案系列研究蔡青芳黃欽威 thrombocytopenia (70000/uL), anemia 國立成功大學醫學院附設醫院神經部 (Hb7.4 g/dL) with normal fibrinogen (316 mg/dL) and prothrombin time. Interictal SPECT in nonlesional temporal lobe epilepsy: A case series Biochemistry revealed normal LDH (361 study U/L), direct/total bilirubin(0.2/0.5mg/dL) Ching-Fang Tsai, Chin-Wei Huang Department of Neurology, and haptoglobin (26.2 mg/dL). Serology National Cheng Kung University Hospital studies disclosed decreased C3 (36.7mg/dL), C4 (6.31mg/dL) and Objective Lesional temporal lobe elevated anti-ds DNA (333.9WHO epilepsy (TLE) with mesial temporal U/mL). Intravenous methylprednisolone sclerosis usually shows consistent was given. However, headache and five temporal hypoperfusion in interictal times of grand mal seizure occurred SPECT, clinical intractability and poor without full recovery of consciousness cognitive performance. Nevertheless, and muscle strength. Brain magnetic the clinical indicators associated with resonance imaging showed diffusion intractability and cognitive restriction in the bilateral thalamus with performance in nonlesional TLE extensive hyper-intensities in the bilateral remains incompletely parieto-occipital lobes, cerebellum and understood. We aim to study the role of basal ganglion in T2-weighted sequence. interictal SPECT on nonlesional TLE. Both the arterial and venous system was patent. Plasma exchange was initiated Methods We identified patients with and his conscious and neurologic clear TLE semiology but without a dysfunction recovered. However, the definite lesion in brain magnetic follow-up images remained unchanged. resonance image (MRI) in the Neurological Clinic in our hospital. Conclusion Both CNS lupus and TTP Seizure-related demographics were could occur simultaneously, and present studied. Functional evaluation seizure, reversible encephalopathy, including thrombocytopenia, acute renal failure, electroencephalography (EEG) and and responsiveness to plasma exchange. interictal SPECT were done. Cognitive However, the mortality rate was different. evaluation was done using cognitive Microangiopathic hemolytic anemia or assessment screening instrument renal biopsy may help to define the (CASI).

127 Results A total of 9 people with an Initial Manifestation of Syphilis Chi-Hsin Ting1,2, Chih-Wei Wang3, Fu-Chi Yang nonlesional TLE were recruited and 1 analyzed. There were six male and three 1 Department of Neurology, Tri-Service General females. The age of seizure onset ranged Hospital, National Defense Medical Center, 2 Department of Internal Medicine, Taichung from 11 to 37 years. More than half (5/9, Armed Forces General Hospital, 3 55.6%) had intractability and three of Department of Radiology, Tri-Service General Hospital, National Defense Medical Center them (33.3%) had bilateral temporal spikes in EEG. Four of them (44.4%) had Abstract normal interictal SPECT. Among these Background Bilateral facial paralysis four patients, two (50%) had is a rare condition and represents a intractability and three (75%) had normal diagnostic challenge. Herein, we report cognitive performance. Bilateral the case of a 22-year-old man with temporal hypoperfusion in interictal bilateral facial paralysis as the sole SPECT correlated well with bilateral initial manifestation of syphilis. temporal spikes in EEG. Among the six patients with either uni- or bi-temporal Case Report The patient initially hypoperfusion, four of them (66.7%) had presented with isolated bilateral facial correspondent EEG findings. nerve palsy without any other symptom . At that time, all serum and Conclusion Normal interictal SPECT cerebrospinal fluid examinations may indicate normal cognitive [including the rapid plasma regain performance, but still may be associated (RPR) titer] yielded were normal. with intractability in nonlesional TLE. Within 2 weeks, he developed rashes Most of the abnormal interictal SPECT on the palms and soles, suggesting the correlated well with EEG, esp. those with possibility of syphilis. At this time, we bilaterality. A larger case-control study were informed that he had a history of on nonlesional versus lesional TLE unprotected sexual exposure, and his would be warranted. serum was positive for the RPR and Treponema pallidum hemagglutination 神經感染疾病學 tests. Treatment with high dose penicillin G was effective, and he was 141 discharged with improvement of the 單獨以雙側顏面神經麻痺作為梅毒初 rashes and with only mild peripheral 期表現之症狀 1,2 3 1 type facial palsy on the right side. 丁吉新王志偉楊富吉 1 三軍總醫院神經內科部 2 國軍台中總醫院內科 Conclusion This is a rare case in 3 三軍總醫院放射科部 which a young man with syphilis Isolated Bilateral Facial Nerve Palsy as initially presented with bilateral

128 peripheral facial palsy. Bilateral facial lumbosacral polyradiculitis. palsies usually reflect many etiology . The differential diagnosis includes MG, Case Reports a 26-year-old man, skull base metastasis, Hansen’s disease, who had fever lasting for 1 week, and etc. Syphilis be considered during admitted due to acute urinary retention, the differential diagnosis of bilateral disoriented consciousness, and bilateral facial nerve palsy in young men with lower limbs weakness. Neurologic sexual exposure, even if the initial skin exam revealed positive meningeal sign manifestation of syphilis is lacking. and CSF study showed leukocytosis WBC: 125 (lymphocyte: 78%), RBC: 142 820, xanthochromic:+ with elevated 人類疱疹病毒引致胼胝體壓部回復性 CSF-protein (233mg/dL). CSF HSV 損傷併脊髓發炎與腰薦椎多神經根炎: PCR was positive. There are no other 個案報告 autoimmune or infectious profiles 王威仁陳凱翔台大醫院新竹分院神經內科 identified. Brain MRI showed isolated diffusion restriction at splenium Human Simplex Virus Induced without contrast enhancement. Reversible Splenic Lesion, Myelitis Progressive bilateral lower limbs and Lumbo-Sacral Polyradiculitis : A (MRC:3/5) and hypesthesia below T6 Case Report sensory level with decreased tendon Wei-Jen Wang, Kai-Hsiang Chen reflex over bilateral knee and ankle Department of Neurology, National Taiwan jerks during admission. T-spine MRI University Hospital Hsin-Chu Branch, Hsin-Chu, revealed myelitis from upper T3 to L5 Taiwan level with meningeal enhancement around conus medullaris. Nerve Background Mild conduction study revealed absence of encephalitis/encephalopathy with F-wave and H-reflex implicated reversible splenial lesion(MERS) is a lumbo-sacral polyradiculitis. We added MRI finding induced by variable on steroid pulse therapy condition. Most reported etiologies were (methylprednisone 1g/day) under the CNS infection, epilepsy, and full course of Acyclovir treatment. anti-epileptic drug usage or withdrawal. Clinical symptoms improved gradually Others, like metabolic problems, after steroid usage. Followed-up brain high-altitude illness, and autoimmune MRI on day 14 showed absence of the disease were also reported. Here we splenial lesions. The patient can walk report a 26-year-old man presented with by himself without assistance after human simplex virus (HSV) induced tapering steroid. MERS and accompanied with meningoencephalomyelitis and

129 Conclusion MERS induced by viral TB meningitis is suspected via image infection presented with cytotoxic edema finding and clinical course, though usually had a benign natural course. The objective CSF results for TB survey is pathophysiology is still undetermined but lack. probably due to outburst of immune reaction with transient neuron oxidative Case Report This 73 y/o male farmer damage. Our patient with MERS has with type II diabetes mellitus suffered more extensive nervous system from insidious onset of intermittent involvement but with a similar prognosis. fever and headache for 2 months The steroid pulse therapy could be the following by bilateral abducens nerve key treatment for his prognosis. palsy. Initially, he received standard ceftriaxone and vancomycin for 143 bacteria meningitis diagnosed by CSF 案例報導 :疑似結核菌腦膜炎合併蜘 findings (neutrophilic predominant 蛛膜下腔出血之案例 pleocytosis, low sugar and elevated 呂潔旼許怡婷 protein content. There was no data for 中國醫藥大學附設醫院 1 神經部 2 暨神經精神醫 TB studies because the initial result 學中心 Case report: Subarachnoid and suggested bacterial meningitis. subdural hemorrhage as a However, deteriorated headache and complication of Suspected tuberculous right hypoglossal nerve palsy meningitis Chieh-Min Lu , Yi-Ting Hsu developed. Brainstem MRI revealed Department of Neurology1 and Center for osteomyelitis in the skull base and 2 Neuropsychiatry , China Medical University upper cervical region with exudate Hospital, Taichung, Taiwan within the ventricle and external

ventricular drain was then performed. Background Meningitis are sometimes Nevertheless, there is limited associated with intracranial hemorrhage. improvement of infection picture. Among the pathogen, some lack sensitive Reviewing the pathogen survey, both detective parameter and hardly provide fungus and bacterial pathogen failed to positive yields in culture, including yield in culture. Although tuberculosis fungus and atypical pathogen as profile including culture and PCR were legionella or TB. Although TB negative, anti-TB meningitis therapy meningitis is confirmed via CSF study was used owing to the clinical course, results, there could be false negative of image finding and refractory to empiric pathogen survey. Here we report a patient treatment. After combined usage of presented with chronic meningitis anti-TB formula and previous complicated with skull base osteomyeltis antibiotics, there was a transient and ventriculitis following by clinical downhill suspected therapeutic subarachnoid and subdural hemorrhage.

130 paradox, though this course could not About 20~30% of HIV infected confirm or refute the diagnosis of TB patients developed HIV-associated meningitis. However, there was dramatic dementia. No data existed on conscious change on further admission percentages of HIV infection in day. Emergency brain image showed dementia patients. subarachnoid and subdural hemorrhage. Further surgical intervention was denied Case Report A 41-year-old man, by family. The patient expired as an end previously an English teacher, was result. admitted because of progressive memory decline, mild unsteady gait Discussion Tuberculosis meningitis is a and urine incontinence for 4 months. devastating disease of the central nervous His family also noted the patient system that usually presents with became more irritable and slow in prodromal phase of chronic headache and response. The neurological low grade fever, followed by acute onset examination revealed poor short-term of cranial nerve neuropathy and further memory and bilateral lower limbs sign of parenchyma involved. Infectious weakness with present Babinski sign. intracranial aneurysms and subsequent The score of mini-mental status subarachnoid hemorrhage are rarely examination was 23. CASI revealed encountered complication. Due to the severe memory impairment under rarity of these lesions and variability in intact attention and concentration. Mild their evolution and clinical presentations to moderate impairment of visuospatial, there is no widely accepted management. constructional ability and higher Awareness of the clinical and radiologic function were also noted. Blood test findings along with awareness of the revealed positive HIV screen with possible complications can help guide ELISA method and Western blot. early treatment or surgical intervention. Absolute CD4 count was low (35 cells/uL). CSF studies were normal. 144 Brain MRI showed mild diffuse brain 感染人類後天免疫不全症候群病毒相 atrophy. Brain perfusion scan with 關的年輕型失智症 Tc-99m ECD revealed hypoperfusion 宋兆家吳亞縈新光吳火獅紀念醫院神經科 in bilateral temporal lobes. HIV-associated dementia was HIV-associated dementia diagnosed and HARRT regimen started Jau-Jia Sung, Ya-Ying Wu Department of Neurology, Shin Kong Wu Ho-Su after infection doctor consultant. Memorial Hospital, Taipei

Conclusion The population of HIV Background The prevalence of HIV infected patient is increasing in recent infection is increasing in recent years.

131 year, and the age of HIV infection Methods 18 serum samples from 12 become much more younger. While NMOSD and 6 healthy patient as a managing dementia of young age, HIV comparison set from a neurology clinic infection should be tested. Treatment of were included. Home-made ELISA kit HIV infection should start as soon as and cell-based indirect possible for delaying the progression of immunofluorescence assay (CIIFA) HIV-associated dementia. were performed.

145 Results The quantitative NMOSD 病患 AQP4 抗體之定量研究 measurement of AQP4 antibodies by 及其臨床應用 home-made ELISA kit correlated well 李照琳陳紹原王凱震蔡清標國立陽明大學附設醫院神經內科耕莘醫院神 with the results derived from CIIFA. 經內科振興醫院神經內科台北榮總神經內科 The AQP4 antibodies were significantly higher in patients with Quantitative Measurement of AQP4 Antibodies of NMOSD Patients in long-cord lesion of NMOSD than in Taiwan and its Clinical Implication those with short-cord lesion of Chao-Lin Lee, Shao-Yuan Chen, NMOSD. There is a significant Kai-Chen Wang, Ching-Biao Tsaif Department of Neurology, National Yang-Ming difference of AQP4 antibody titers University Hospital, I-Lan, Taiwan between relapse and remission groups Section of Hyperbaric Oxygen Medicine, Cardinal Tien Hospital, Taipei, Taiwan of NMOSD. Department of Neurology, Cheng Hsin General Hospital, Taipei, Taiwan The Neurological Institute, Taipei Veterans Conclusion Quantitative General Hospital, Taipei, Taiwan measurement of AQP4 antibodies by Background The presence of our Home-made ELISA kit was well antibodies to aquaporin-4 (AQP4) has correlated with CIIFA and provides the been identified as a key characteristic of possibilities of predication of the neuromyelitis optica spectrum disorder extent of spinal cord lesions and (NMOSD), an autoimmune inflammatory monitoring of disease progression or demyelinating central nervous system treatment response in NMOSD. (CNS) disorder. We determined the However, the relevance of its clinical presence of AQP4 antibodies by use needs to be actively investigated in cell-based indirect immunofluorescence a prospective study on a large scale. assay (CIIFA) and Home-made ELISA kit respectively, and evaluated the 146 diagnostic and prognostic relevance of 免疫缺陷病毒感染病患使用高效抗 AQP4 antibodies of patients with 逆轉錄病毒治療後表現進行性多發性腦白質病變 NMOSD in Taiwan. 李翰道宋崇元張國軒林口長庚紀念醫院神經內科

132 Progressive multifocal encephalopathy with PML. His neurological deficits unmasked by HAART in a became stable following a short-term HIV-infected patient Han-Tao Li, Chung-Yang Sung, Kuo-Hsuan treatment with dexamethasone and Chang mannitol. And then his HAART with Department of Neurology, Chang-Gung Memorial Hospital and University College of Combivir and Efavirenz was restarted. Medicine, Lin-Kou Medical Center

Conclusion PML could occur in Abstract HIV-infected patients following Background. For patients with HARRT. The mechanism for the HIV/AIDS, highly active antiretroviral reactivation of JC virus by immune therapy (HAART) is currently the only reconstitution remains elusive. effective therapy. Here we report a case of progressive multifocal 147 leukoencephalopathy (PML), a subacute 以全程口服類固醇 Prednisolone 合併 demyelinating disease caused by 抗結核藥物成功治療結核性腦血管 infection of oligodendrocytes by the 炎:一病例報告 polyomavirus JC, occurs after HAART. 周美鵑 1 余錫婷 2 楊淵韓 1 高雄市立大同醫院（委託財團法人私立高雄 1 醫學大學附設中和紀念醫院經營) 神經內科 Case Report A 52-year-old 2 護理部 HAART-treated HIV-1-infected male patient began to develop progressive Successful treatment of tuberculous cerebral vasculitis with full-course cognitive impairment, disorientation, and oral predinsolone combined with right limb weakness for about two antituberculous drugs : a case report 1 2 months before admission. His Mei-Chuan Chou , His-Ting Yu , Yang-Yuan Han1 neurological examinations showed motor 1Department of Neurology 2 aphasia, acalculia, and ideomotor apraxia. Department of Nursing Kaohsiung Municipal Ta-Tung Hospital Hemiplegia, hyperreflexia and present Babinski sign were noted on his right Introduction Tuberculous cerebral limbs. His brain magnetic resonance vasculitis is a neurological imaging (MRI) showed T2-weighted complication of tuberculous meningitis. hyperintense lesions in left frontal white We reported the imaging of matter and callososeptal area. Laboratory tuberculous cerebral vasculitis and tests found low CD4 T-cell counts of 433 successful treatment with oral cells/μL and high HIV-RNA viral load of predinsolone throughout the course of 43,292 copies/mL. The stereotatic brain antituberculous treatment. biopsy showed inflammatory infiltrations, gliosis with focal demyelination, and Case report This 19 year-old woman, intranuclear inclusion positive for anti-JC a college student with no remarkable virus antibody, which was compatible medical history, was diagnosed with

133 tuberculous meningitis by clinical Follow-up CSF study showed nearly response to antituberculous treatment. normal CSF profile except mild The initial presentation was a 2-month elevation of protein ( CSF protein 67 history of headache, a 3-day history of mg/dl). A seven-day course of fever and first generalized tonic/clonic dexamethasone was administrated 5mg seizure. Blood human immunodeficiency q 8hours intravenously with tapering virus (HIV) test was negative. The dosage. Oral predinsolone at daily symptoms improved dramatically after dosage 60 mg was prescribed later with initiation of antituberculous medication tapering 10mg per week then with on four-drug regimen. However, due to maintenance dosage at 10mg/day severe increased intracranial throughout the course of hypertension, dexamethasone was antituberculous treatment. Follow-up administrated 5mg q 8hours brain MRI after 2 months revealed intravenously with tapering dosage mild improvement of multiple followed by oral predinsolone tapered hyperintensity lesions and further gradually over 6 weeks after discharge. improvement was noticed at After treatment with antituberculous full-course antituberculous treatment. medication for 2 months, the course was complicated with tuberculous myelitis Discussion Corticosteroid has been involving spinal cord at C3-5 level and used for tuberculous meningitis since T7-9 level. Pulse methylprednisolone the 1950s. While some believe therapy was given 1000 mg daily for 3 corticosteroids improve outcome due days with following oral predinsolone at to reduce inflammation of small blood daily dosage 60 mg with tapering 10mg vessels and therefore reduce damage per week. from blood flow slowing to the In the 5th month, she presented with underlying brain tissue, it is possible 2-week history of episodic dystonia that steroids may harm tuberculous involving left hand and left foot and meningitis by suppressing the immune forgetfulness. Brain FLAIR MRI mechanism. While prescribing steroid, revealed extensive irregular hyperintense benefits to risks ratio should be lesions with peripheral enhancement considered. Steroid use in tuberculous mainly involving corpus callosum, cerebral vasculitis should not be bilateral cerebral deep white matter, tapered too rapidly to avoid recurrence subcortical white matter of right parietal of vasculitis. In this case, we and left temporal lobes, bilateral basal maintained the full course of ganglia, midbrain, pons, and medulla predisolone after discharing the patient. which were compatible with tuberculous No recurrence of vasculitis was cerebral vasculitis with vasogenic edema. noticed .

134 Conclusion Corticosteroids should be showed normal cranial nerves. The routinely used in HIV-negative people neurological examination, including with tuberculous meningitis to reduce deep tendon reflexes, was otherwise death and disabling residual neurological normal. Pinprick sensation was deficit amongst survivors. Full-course reduced over the palmar aspect of both oral prednisolone of low dose may be fourth and fifth digits. Nerve helpful for patients with tuberculous conduction studies of bilateral upper cerebral vasculitis. limbs revealed normal distal latency and F wave, but slow conductive 148 velocity, and reduced amplitude of 黴漿菌肺炎感染後引起的臂神經叢病變 CMAPs. Sensory nerve conduction 林冠宇彭家勛徐昌鴻 studies demonstrated low amplitude of 三軍總醫院神經內科國防醫學院 ulnar sensory nerve action and slowing Brachial plexopathy complicating of ulnar sensory nerve conduction Mycoplasma pneumoniae infection in velocity. Needle electromyography a Chinese adult Guan-Yu Lin, Giia-Sheun Peng and Chang-Hung examination pointed out increased Hsu amplitudes, prolonged durations and Department of Neurology, Tri-Service General Hospital, National Defense Medical Center, polyphasic morphology. No muscles Taipei, ROC demonstrated any fasciculations or

Background Mycoplasma pneumoniae complex repetitive discharges. Specific infections are often asymptomatic but upper limb muscles tested included the can involve multiple organ systems. A following: deltoid, triceps, biceps immune-mediated pathological brachii, extensor indicis proprius, first mechanism has been suspected to be the dorsal interossei, abductor digiti main cause of neurological minimi, and abductor pollicis brevis. manifestations due to the presence of Cervical paraspinal muscles were cross-reactive antibodies to the brain and normal. These studies indicated the other neurologic structures presence of bilateral brachial plexus neuropathies. An MRI of brachial Case Report A previously healthy, plexus was normal without evidence of 21-year-old man presented with slowly organic compression. The neural progressive weakness and numbness of foramina of the cervical spine were his bilateral upper limbs for one week. patent. Serology was positive for One month prior to the occurrence of Mycoplasma pneumoniae IgM. Other weakness, he had flu-like symptoms lab tests were unremarkable. lasting for fourteen days with full recovery. He did not have shoulder, Conclusion Onset of his neurological scapular or neck pain. Examination symptoms one month after

135 Mycoplasma pneumoniae infection acid fast stain, TB PCR and cytology implied a causal relationship. The case is study were negative in the CSF. being reported for its rarity and to Although weakly positive in serum, the highlight the spectrum of cryptococcal antigen was negative in extra-pulmonary complications. the initial two CSF studies. There was no evidence of pulmonary or other 149 extrapulmonary TB except for a 病例報告: 隱球菌與結核菌共同感染 positive adenosine deaminase reaction 導致的腦膜炎 in serum. 林奕辰 1 鄧浩文 1 萬芳醫院神經內科台北醫學大學 After empirical anti-TB treatment Meningitis caused by Co-infection of including systemic corticosteroid for Cryptococcus and Tuberculosis one month, there was substantial Yi-Chen Lin1, Hao-Wen Teng1 1Department of Neurology, Wan Fang Hospital, improvement in headache and gait Taipei Medical University ataxia. Follow-up brain MRI scan and

CSF analysis showed almost complete Background Tuberculous (TB) and regression of the aforementioned cryptococcal meningitis are the two most abnormal findings. However, the CSF common differential diagnoses among all profiles started to deteriorate and cases of subacute to chronic meningitis. cryptococcal antigen in the CSF began Here we report a case of combined TB to rise gradually since the 4th week and cryptococcal meningitis in a after treatment. The fungal culture non-HIV patient. obtained from the CSF 4 weeks after

anti-TB treatment, but not the initial Case Report A 60-year-old male orchid CSF, yielded Cryptococcus farmer presented with persistent neoformans. After antifungal treatment, headache followed by gait ataxia for there was further improvement in CSF totally 6 weeks. There was no fever, data and fever. meningismus, blurred vision, or impaired mental status. He had no history of Conclusion It has been proposed that immunodeficiency and the serum reactivation of latent infection may anti-HIV titer was negative. Brain MRI contribute significantly to the revealed diffuse leptomeningeal development of cryptococcosis. enhancement and multiple nodular Systemic corticosteroid, which has enhancing lesions with perifocal edema been included in the standard treatment at bilateral cerebellar and cerebral protocol of TB meningitis in the last hemispheres. CSF study showed normal decade, can lead to pressure, lymphocyte-predominant immunocompromised state and pleocytosis, increased total protein and theoretically reactivate a latent low glucose levels. The Indian ink and

136 cryptococcal infection. From our case it including vomiting and diarrhea. is emphasized that follow-up of CSF Acyclovir was used after knowing the studies cannot be omitted in the result of CSF studies. treatment of subacute to chronic In ICU, continuous EEG monitoring meningitis despite an initial response to showed active epileptogenic foci over treatment. bilateral temporo-occipital regions, more on right side. We have added 151 multiple antiepileptic medications, Bocavirus 腦炎在年輕男性引起重積癲 including phenytoin, midazolam, 癇發作之個案報告 propofol, and oral levetiracetam. 洪煒斌黄欽威陳志弘國立成功大學醫學院附設醫院神經部 However, refractory clinical and EEG epileptic activity persisted and multiple Bocavirus related aseptic encephalitis organ failure developed in such high induces status epilepticus in a young male: a case report. dose antiepileptic agents. The patient Wei-Pin Hong, Chin-Wei Huang, Chih-Hung passed away four days after status Chen Department of Neurology, National Cheng Kung epilepticus. The CSF specimen sent to University Hospital. center for disease control for real time

PCR showed bocavirus. Background Status epilepticus related to primary CNS infection in young male Conclusion Bocavirus, a DNA virus, is often fulminant with high mortality is a new respiratory tract infectious rate. Especially in aseptic encephalitis, antigen, discovered in 2005. To our the pathogen is usually not able to be knowledge, this is the first case report cultured in general hospital except for about bocavirus related aseptic special methods or sending CSF encephalitis inducing status epilepticus specimen to centers for disease control in young male. for PCR.

152 Case Report A 21-year-old, otherwise 急性腦幹中風為 HIV 陽性者罹患神經 healthy man suffered from new onset 性梅毒的起始表徵 complex partial seizure with secondary 陳明華 1,2 李俊泰 2 徐昌鴻 2 宋岳峰 2* 2 generalization, and then progressed to 1 國軍桃園總醫院國防醫學院三軍總醫院神經科部 status epilepticus. CSF studies showed pleocytosis, lymphocyte predominant, Acute brainstem infarction as the without glucose decrease and only very initial presentation of neurosyphilis in a HIV-positive patient mildly elevated protein, which suggested Ming-hua Chen1,2, Jiunn-Tay Lee2, 2 2 aseptic encephalitis. 1 week before Chang-Hung Hsu , Yueh-Feng Sung * 1Department of neurology, Taoyuan Armed symptoms onset, he suffered from mild Forces General Hospital, 2 gastroenteric symptoms of viral infection, Departments of Neurology, Tri-Service General Hospital, National Defense Medical Center

137 Background A worldwide resurgence deficits improved gradually after a of neurosyphilis has occurred since the two-week therapy of intravenous advent of the HIV epidemic. Comparing penicillin. with typical course of syphilis, patients with concurrent HIV-infection may show Conclusion High variety of clinical a more rapid progression of manifestations in patients with neurosyphilis. neurosyphilis and HIV co-infection should be delivered. We highlight the Case Report A 22-year-old man, importance of considering the having a history of hypertension with diagnosis among the young patients poor medical compliance for one year, presenting with atypical clinical course presented with numbness on left side of and symptoms of stroke. face and body for three days. He had a history of edema in the lower legs and 153 skin rashes on the palms and soles one HIV 陰性病人感染隱球菌腦膜腦炎 month before the event, with 引起顫抖之不自主運動陳致霖莊介森 spontaneous remission. Headache 彰化基督教醫院神經醫學部 without neck stiffness and intermittent Tremor associated with cryptococcal fever were noticed for one week prior to meningoencephalitis in an HIV seronegative patient: a case report the admission. He denied having sexually Chih-Lin Chen, Chieh-Sen Chuang transmitted diseases. The neurological Neurological Institute, Changhua-Christian Hospital examinations showed paresthesia on left side of face and body. Some faded rashes Background Common clinical on the soles were noticed. The brain CT presentations of cryptococcal showed no hemorrhage. The brain MRI meningoencephalitis in HIV revealed acute ischemic infarction with seronegative patients are variable. water diffusion restriction over right pons. Movement disorders were infrequently Studies for young stroke showed observed. Several cases of positive RPR (1:32), positive TPPA hemichorea-hemiballism in AIDS (>1:1280), and positive anti-HIV patients had been reported, but tremor antibody and western blot. Cerebrospinal was rarely observed. fluid analysis revealed lymphocytic pleocytosis, increased protein content Case Report A 67-year-old woman and positive VDRL test (1:1). was admitted because of insidious Neurosyphilis and HIV infection were onset of bilateral hands tremor for 3 diagnosed. The patient’s CD4 count was weeks prior to admission. Before 598 and HIV 1 RNA viral load was admission, she had sore throat, 51300 copies/ml. The neurological productive cough, fever on-and-off and

138 poor appetite for about 6 weeks. In involvement of basal ganglion by addition, insidious onset of bilateral cryptococcal infection. hands tremor and unsteady gait were noticed. She denied weakness or 154 numbness of bilateral limbs. The 慢性多處腦膜炎之切片診斷:中樞神 condition persisted despite taking some 經結核一案例報告黃立楷李薰華胡朝榮 drugs from clinics. There was no history 台北醫學大學-行政院衛生署雙和醫院神經科 of exposure to chicken or pigeon. Neurologic examination showed postural Biopsy diagnosis in chronic and action tremor of bilateral hands, multifocal leptomeningitis: a case report of central nervous system increased muscle tone in right upper limb, tuberculosis mild right hemiparesis, and mild Li-Kai Huang M.D., Hsun-Hua Lee M.D., Chaur-Jong Hu M.D. unsteadiness. Laboratory data revealed Department of Neurology, Shuang Ho Hospital, elevated ESR (42 mm/hr). Thyroid Taipei Medical University, New Taipei City, Taiwan hormones were within normal limits.

Brain MRI demonstrated hyperintensities Background Central nervous system over the right internal capsule, bilateral tuberculosis (TB) is the most severe external capsules, basal ganglia and form of extrapulmonary TB that even bilateral deep cortical white matter on current treatment does not prevent T2WI and FLAIR sequence without death and disability in half of the cases. abnormal signal on T1WI, DWI, ADC or Rapid diagnosis and initiation of SWI sequence. CSF analysis showed effective antituberculous therapy are pleocytosis, elevated protein and low fundamental to a better outcome. glucose level, with positive finding for

Indian ink stain and positive Case Report A 69-year-old man Cryptococcus antigen. Cryptocuccal presented initially of episodic right arm meningoencephalitis was highly numbness for 9 months. The sensory suspected. Symptoms improved after symptoms were responsive to administration of Amphotericin B and treatment with Valpoaic acid 500mg 註解 [7]: Flucytosine. HS. The brain magnetic resonance Your User Name 13/1/21 下

imaging (MRI) post Gd-DTPA 午 1:58 Conclusion The clinical course, enhancement showed multiple 哪些? laboratory studies, brain MRI, and contrast-enhanced leptomeningeal clinical improvement after treatment lesions over the left frontal-parietal establish the relation between tremor and region. Digital subtraction angiography cryptococcal infection in this case. The revealed no dural AVF but focal patient demonstrated the infrequent luminal narrowing at one of left manifestation of tremor caused by superficial cortical veins. Progressively,

139 he became demented with difficulty in Background Syphilic infection of the walking. Three cerebrospinal fluid (CSF) central nervous system mostly results studies revealed elevated protein and in chronic meningeal inflammatory mild pleocytosis with dominant process and parenchymal involvement. lymphocytes. All the TB polymerase The common neuroimaging findings of chain reaction (TB-PCR) and acid-fast neurosyphilis include diffuse stain (AFB) tests showed negative results. inflammation of pia and arachnoid, as Finally, leptomeningeal TB infection was well as ischemic lesions, typically proven by tissue culture from biopsy. The lacunar or middle cerebral artery clinical condition improved gradually territroy involved, in meningovascular after anti-TB therapy. syphilis; intracerebral gummata; mesial temporal lobe or disseminated frontal Conclusion Our patient was diagnosed high-signal lesions on T2-weighted as definite TB meningitis by focal images; and ventriculomegaly. meningeal biopsy with positive TB culture. The significant improvement Case report A 63-year-old, married after anti-TB medication supports that man has HTN and Af with MVR under the original problems have been treated. regular treatment. He had neuralgia However, our attempt to confirm or over bilateral lower extremities and exclude the diagnosis were frustrating, sensory ataxia since 2008, the for negative CSF AFB, TB-PCR and TB neurological examinations revealed culture for a few times until the more diffuse hyporeflexia. The NCV/EMG invasive operation was performed. This studies were unremarkable but case raises the relevant implication that somatosensory evoked potential study meningeal biopsy might be necessary for showed spinal cord lesion between the chronic meningitis patients without cervicolumbar region. However, definite diagnosis by non-invasive insidious onset of cognitive decline examinations. with incoherent speech and wording difficulty developed since January 155 2011. He also had two episodes of 神經性梅毒之特殊影像學病灶：一病例 conscious loss in January and March 報告 2011 and seizure was diagnosed. In 馮博裕章寶倫林口長庚紀念醫院神經內科 November 2011, subacute onset of mentality change, disorientation and Juxtacortical lesions in Neurosyphilis : mild delirium were noticed. The CSF A Case Report study showed mild lymphocytic Po-Yu Fong, Bao-Luen Chang Department of Neurology, Chang Gung Memorial pleocytosis with mild elevation of total Hospital, Lin-Kou Medical Center protein and normal glucose level. The

140 RPR was 1:16 in CSF and 1:128 in Department of Neurology, Kaohsiung Medical University Hospital, Kaohsiung, Taiwan serum. The serological TPHA was >1:1280 and HIV screening test was Background Vasculitis and infarcts negative. Brain magnetic resonance are well-established sequelae of imaging showed high signal lesions in bacterial meningitis. However, early juxtacortical areas over bilateral insular, large-vessel involvement is rare, frontal, temporal and parietal regions on particularly within the brainstem. We FLAIR and T2-weighted images without present one case of brainstem T1-weighted gadolinium enhancement. infarction associated with bacterial Therefore, neurosyphilis was diagnosed meningitis. then he received intravenous penicillin G treatment. Under one year follow up, his Case Report This 31-year-old male symptoms and signs had gradually had viral meningitis 3 years ago. The improved and a follow-up brain MRI patient had been to Tailand about one revealed totally improvement of the week before admission. He suffered juxtacortical lesions. from headache, dizziness and binocular diplopia at first. Then, progressive Conclusion The juxtacortical lesions in double vision, lethargy, right side neurosyphilis were rarely reported and it numbness(both face, trunk and face), can mimic a demyelinating lesion (such slurred speech developed while he as multiple sclerosis), Creutzfeldt-Jakob woke up in the morning on the next disease, HIV encephalitis, Binswanger day. Brain CT showed old infarction disease, cerebral autosomal dominant over left basal ganglion when he arteriopathy with subcortical infarcts and arrived the emergency room(ER). leukoencephalopathy(CADASIL), and Neurological examination revealed progressive multifocal bilateral abducen nerve palsy, right leukoencephalopathy in both clinical trigeminal, facial nerve lesions, right manifestations and neuroradiologic hemiparesis and paraesthesia. Besides, studies . fever was noted (39.1C) on arrival to ER. Cerebrospinal fluid (CSF) analysis 156 revealed glucose 59 mg/dl (blood 143 以腦幹梗塞為早期臨床表現之細菌性 mg/dl), protein 210 mg/dl, total 腦膜炎 nucleated cells of 740 (98% 鄞秀靜陳俊鴻林瑞泰賴秋蓮陳建甫高雄醫學大學附設中和紀念醫院神經科 neutrophils, 2% monocytes). The patient was treated initially with Brainstem Infarction as An Early intravenous ceftriaxone, vancomycin, Manifestation of Bacterial Meningitis and intravenous dexamethasone before Hsiu-Ching Yin, Chun-Hung Chen, Ruey-Tay Lin, culture and sensitivity results. A Chiou-Lian Lai, Chien-Fu Chen

141 magnetic resonance imaging (MRI) scan accompanied with severe headache, of the head 2 days after admission vomiting and diarrhea 2 days before revealed subacute infarction with admission. CSF study showed hemorrhagic transformation in the pons. lymphocytic leukopleocytosis (9), Young stroke survey showed mild elevated protein (120mg/dL) and elevating Clotting factor VIII and ESR. normal sugar (42mg/dL). PCR for There were no positive results including HSV-1/2, TB, and influenza antigen viral, aerobic or anaerobic bacterial were negative. He was diagnosed as culture, fugal, TB-PCR tests of CSF viral meningoencephalitis and analysis. receiving Acyclovir. Decreased verbal output, myoclonus and genralized Conclusion Vasculopathy of the basilar tremor were noted on 3 days after artery and its perforating arteries may admission. Follow-up CSF study lead to brainstem ischemia. It is showed more elevated total protein important to aware that clinical signs of (367mg/dL) and lyphocytic ischemia may be an early clinical leukocytosis (Lymphocyte/Neutropil : manifestation of meningitis. 40/3) but both negative in HSV-PCR and TB-PCR. Head magnetic 157 resonance image (MRI) showed one 以可逆性似急性腦中風損傷之胼胝體 2.4cm focal area of limited diffusion 病灶為表現的病毒性腦炎:個案報告 coefficiences and focal swelling near 劉翁銘林靜嫻台大醫院神經部 the splenium of the corpus callosum, with hyperintensity in T2-weighted A Reversible Stroke-Like Splenial imaging (T2WI) and diffusion Lesion in Viral Encephalopathy: Case weighted image (DWI), hypointensity Report Weng-Ming Liu, Chin-Hsien Lin in apparent diffusion coefficient (ADC) Department of Neurology, National Taiwan University Hospital and faint hypointensity on T1-weighted imaging (T1WI), mimicking acute Background An ovoid reversible lesion infarction. MRI with contrast showed in the central portion of the splenium of obvious leptomeningeal enhancement. the corpus callosum (SCC) without any His consciousness remained drowsy accompanying lesions in MRI was and had agitation and occasional uncommon but had been reported in mild myoclonus. Intravenous Acyclovir and encephalopathy/encephalitis patients. anti-tuberculosis agent (Rifampicin, Isoniazid, Pyrazinamide, Ethambutol) Case Report were used. His conscious gradually A 32 years old healthy man suffered from recovered without any neurologic intermittent fever up to 39℃ sequelae. Follow-up CSF study 2

142 weeks later showed decreased total lesions over left hemisphere with protein (162.5mg/dL) but still perifocal edema and compression of lymphocytic leukocytosis left lateral ventricle. Left MCA (Lymphocyte/Neutropil : 99/0). territory infarction was the first Follow-up MRI 2 months later was impression according to clinical normal without residual lesions. features and brain imaging. Rapid progression of behavioral changes, Conclusion Our case confirmed with dementia, grimacing, myoclonia, previous findings that a reversible seizures, and mutism were noticed stroke-like splenial lesion could be seen within one month. Then, the brain MRI in virus related encephalopathy and revealed hyperintensity over left regarded as a good prognosis marker. caudate in DWI and hypointensity in Transient intramyelinic edema or ADC, and asymmetric frontal cortical inflammatory infiltrate is the possible lesions with left side dominance. mechanism and further studies enrolling Repeated electroencephalograms more related cases will be needed to showed periodic sharp wave in left confirm our finding. frontal region with inter-spike interval of 1 second. The cerebrospinal fluid 158 protein 14-3-3 analysis was positive. 以中風症狀表現之庫賈氏病:個案報告鄭美雲江星逸 Conclusion The unilateral onset 林口長庚紀念醫院神經內科 sCJD is not common and easily Sporadic Creutzfeldt-Jakob disease misdiagnosed as stroke initially. Rapid mimics a left hemisphere stroke: A progressive dementia and appropriate case report Mei-Yun Cheng, Hsing-I Chiang tests, including brain MRI and Department of Neurology, Chang Gung Memorial electroencephalogram, may improve Hospital, Lin-Kou Medical Center the diagnosis of sCJD. Background Unilateral onset pyramidal signs and language 159 dysfunction mimicking left hemisphere 散發性庫賈氏症病人腦波及腦部核 ischemic stroke was found in a patient 磁共症影像之不對稱表現 1 1 with sporadic Creutzfeldt-Jakob disease 謝孟倉黃涵薇 1 (sCJD). 國立成功大學附設醫院神經部

Case Report A 58-year-old man The asymmetric presentation of presented with acute onset right EEG and brain MRI in sporadic hemiparesis, slurred speech, and anomia. Creutzfeldt-Jakob disease patients 1 Initial brain CT showed hypodense Meng-Tsang Hsieh1,Han-Wei Huang

143 Department of Neurology, National Cheng Kung cortical dysfunction in all thirteen University Hospital patients, seven of them were more severe in either side. There were seven Background Creutzfeldt-Jakob disease patients has lateralized positive sharp (CJD) is a rare disease with grave wave complex (PSWC) in the initial prognosis. Death usually occurs within EEG study, and three of the other six one year of symptom onset. CJD used to patients had PSWC in EEG eventually. be described as diffuse neurodegenerative disease. However, Conclusion This result indicates that lateralization findings in magnetic characteristic abnormalities on MRI resonance imaging (MRI) and and EEG findings with asymmetric electroencephalography (EEG) were distribution are not uncommon reported in some patients fulfills the presentation, and may be helpful for WHO criteria of sporadic CJD. early diagnosis of sCJD. Objective: To ascertain the presentation of EEG and brain MRI in patients with 160 sporadic Creutzfeldt-Jakob disease Ramsay Hunt Syndrome 合併三叉 (sCJD). 神經及頸部病灶：一案例報告李薰華黃立楷胡朝榮陳致中行政院衛生署雙和醫院神經內科 Method The subjects were those who were diagnosed as probable or possible Ramsay Hunt Syndrome with Ipsilateral Cranial Nerve V and sCJD in National Cheng-Kung Upper Cervical Nerves Involvement: University Hospital during the period A Case Report from 2006 to 2012. We reviewed the Hsun-Hua Lee M.D, Li-Kai Huang M.D, Chaur-Jong Hu M.D, Chih-Chung Chen M.D chart record, brain MRI, and the EEG Department of Neurology, Shuang-Ho Hospital, reports to evaluate correlations among Taipei Medical University, New Taipei City, Taiwan them. Background Ramsay Hunt syndrome Result In this retrospective research, (RHS) refers to the association of thirteen probable or possible sCJD unilateral peripheral facial nerve palsy patients were identified. There were eight and herpes zoster infection of the ear patients had bilateral bright signal (herpes zoster oticus), with or without intensity on MRI of diffusion-weighted hearing loss. The majority of cases imaging (DWI) of the cortex and/or basal have manifestations limited to the ganglia. (Four of them were more involvement of cranial nerve (CN) VII predominant in one side). The other five and VIII. Atypical presentations of patients had unilateral signal changes in RHS with other CN or cervical nerve MRI DWI series. The EEG showed involvement are uncommon. Here we

144 report an unusual case of RHS with include CN 5,7,8,9,10,11 and 12 and concurrent ipsilateral CN V, C2, and C3 cervical spinal nerve in C2,3 and 4. nerves involvement. Possible explanations of varicella-zoster virus reactivation from Case Report A 69-year-old multiple ganglia are anatomical immunocompetent woman was proximity, selective vulnerability of hospitalized to neurologic ward due to blood vessels and transaxonal spread. acute left facial paralysis, painful skin Such extensive involvements can occur eruption in left earlobe, severe dizziness in both immunocompetent and and hearing impairment for 4 days. One immunocompromised subjects and month before hospitalization, she began carry worse prognosis. to have the symptoms of intermittent fever up to 39℃,sore throat, general 神經免疫學 weakness, changes of taste and persistent tingling pain in left low face and neck. 161 Ten days before hospitalization, she 多發性硬化症與視神經脊髓炎細胞 noticed painful skin redness in left lower 激素分析的差異王凱震李照琳陳紹源楊智雯陳錫洲 face and neck with grouped vesicles 蔡清標 around left upper and lower lips. On 振興醫院神經內科 admission, we noticed vesicular skin Distinct Serum Cytokine Profiles in eruptions in left earlobe, maxillary and Neuromyelitis Optica and Multiple mandibular branches of left trigeminal Sclerosis nerve and left C2 and C3 dermatomes. Kai Chen, Wang, Chao-Lin, Lee, Shao-Yuan, Chen, Chih-Wen, Yang, Shyi-Jou, Chen, and Neurologic examinations revealed Ching-Piao, Tsai Department of Neurology, Cheng Hsin General peripheral type facial palsy (FNGS 2.0 Hospital, Taipei, Taiwan Grade V) and sensorineuronal hearing loss of left side. Brain and cervical spine Objective Multiple sclerosis (MS) is MRI findings are insignificant. the most common prototypic Cerebrospinal fluid analysis showed inflammatory demyelinating disease. normal cell count, sugar and protein. Neuromyelitis optica (NMO) is another Despite intravenous acyclovir and steroid inflammatory demyelinating disease of therapy, the recovery of facial paralysis the central nervous system that exhibits and hearing loss is slow. clinical symptoms mainly associated with optic neuritis and myelopathy. Conclusion Multiple cranial and The inflammatory reaction in MS is cervical spinal nerves involvements are associated with an up-regulation of a uncommon in Ramsay Hunt syndrome. variety of Th1 or Th17-mediated Reported cranial nerve involvement cytokines. However, NMO and MS are intertwined both clinically and

145 pathologically, which complicates their We report a 35-year-old patient with diagnosis and treatment. The aim of this stroke symptom caused by PCNSV. study was to evaluate the differences in Case Report A 35-year-old man serum cytokine levels in NMO and MS without prior history of systemic patients. disease presented to our neurologic clinic because of acute onset of Methods We collected peripheral isolated left hemiparesis lasting for 4 serum from patients with these central hours, and recalled that there was nervous system demyelinating diseases another episode with the same pattern for the study. A cytometric bead array 5 days ago before admission. He was used to assess the cytokine levels denied smoking history, fever or recent using flow cytometry. trauma history. However, another two episodes of recurrent left hemiparesis Results We found more inflammatory developed in the following months. (IL-2, and IFN-γ), and anti-inflammatory Neurological examination was normal (IL-4, and IL-10) cytokines in NMO than except mild left hemiparesis. General in MS. The differences in the optimal laboratory workup was unremarkable. cutoff points of serum cytokines, Series of work-up for young stroke was including IL-2 ≥5 pg/ml, can differentiate conducted including anti-phospholipid NMO from MS. antibody, lupus anticoagulant, anti-nuclear antibodies, anti-ENA Conclusion NMO patients had an antibodies, and antineutrophil increased Th1-mediated inflammatory cytoplasmic antibodies, and no definite response, but similar Th17-mediated abnormality was noted. The levels of inflammation changes compared to MS homocysteine and ESR were normal. patients. Serum cytokine studies can No evidence of intracaridac thrombus differentiate NMO cases from MS. or shunt was found after trans-esophageal cardiac echography. 162 CSF studies showed no signs of 原發性中樞神經系統血管炎：病例報告 infection or cytoalbumin disassociation. 王寧李靜娥 EEG study showed no definite 台北醫學大學市立萬芳醫院神經內科 Primary Central Nervous System evidence of cortical dysfunction. The Vasculitis : A Case Report brain MRI on August 30, 2012 Ning Wang, Jing-Er Lee Department of Neurology, Taipei Municipal revealed one infarction at right internal Wanfang Hospital, Taipei Medical University capsule in DWI (figure 1a), and in the third episode, showed new lesions at Background Primary central nervous right internal capsule, globus pallidus vasculitis (PCNSV) is a rare form of and left corpus callosum (figure 1b and vasculitis limited to brain and spinal cord.

146 1d). MR angiogram on September 16, Intermittent Severe Pain as Initial Presentation of Guillain-Barre 2012 showed no definite vascular lesion. syndrome- A Case Report Cerebral angiography revealed abnormal Meng-ling Wu, Hsu-Ling Yeh, Li-Ming Lien, arterial appearances including alternating Chien-hung Yeh, Hou-Chang Chiu stenosis with dilatation of multiple Departments of Neurology, Shin Kong Wu Ho-Su Memorial Hospital, Taipei intracranial arteries (figure 2); CNS vasculitis was highly suspected(1,2). Background The typical initial Steroid pulse therapy presentation of Guillain-Barre (Methyprednisolone 500 mg daily) was syndrome (GBS) is ascending administrated for five days and followed numbness, proximal weakness, and by prednisolone 60mg daily and there areflexia. We reported a young man was gradual improvement in left who visited emergency department(ED) hemiparesis. No neurological deficit repeatedly for non-specific pain developed thereafter. We tapered the syndrome finally diagnosed as GBS amount of prednisolone with 10 mg per that need ventilation support. week for 2 weeks, and 5 mg per week for the rest. Case report A 44-year-old man, who had chronic headache since adolescent, Conclusion It is important to was in his usual healthy status until 4 intensively search for the possible days before admission when bilateral etiologies in young stroke, especially temporal swelling pain attacked. The with relapses of sign and symptom character of the pain was just the same despite of adequate and proper treatment. as he used to have. In the following PCNSV, although rare, should be days, he experienced malaise and suspected in the context of multiple areas general soreness and pain. The pain of infarction in different vascular went on and off, aching in nature, territories and of different ages revealed visual analogue scale 10/10, migrating by brain MRI. Once clinically suspected, among limbs joints and muscles, and cerebral angiography is a key diagnostic he also had chest, abdomen, and back modality when biopsy is not undertaken. discomfort. He visited ED for 3 times We presented a case of PCNSV whose because of the intractable pain. All neurological deficit improved after routine blood tests including steroid treatment. electrolyte, muscle enzyme, inflammatory profile, and autoimmune 163 profile were normal. No definite lesion 以陣發性嚴重疼痛為起始表現之 could be found through contrast Guillain-Barre 症候群：病例報告吳孟玲葉旭霖連立明葉建宏邱浩彰 enhanced computed tomography from 新光吳火獅紀念醫院神經科 chest to abdomen. We suspected viral

147 infection, hyperventilation syndrome, compared with 12.5% of whom conversion disorder, unknown without the characteristic pain. The autoimmune disease, Dengue fever, and mechanism of the pain is multifactorial aortic dissection but there was no which is possibly related to evidence to support any of these inflammation of nerve roots, irritation diagnoses. The pain responded poorly to of the nervi nervorum, spontaneous or ketorolac and morphine. He had akathisia abnormal activity from large with anxiety. On the 4th day after myelinated sensory afferents, affected symptoms onset, facial asymmetry small nerve fibers, mechanical factors, developed. Neurological examination and immobilization. showed left peripheral type facial palsy and hyporeflexia in knees and ankles. He Conclusion Pain is not rare in GBS. also reported distal limbs paresthesia but We should take GBS into consideration we couldn’t reveal hypoesthesia or in approaching patient with clinical decrease in sense of vibration. Follow up and laboratory unexplainable pain. blood test showed WBC 10300 /µL and CK 821 U/L. GBS was suspected. CSF 164 data and nerve conduction examination Anti-Yo 抗體合併附腫瘤性小腦變性: showed typical change of acute 案例報告李卓育陳律安 inflammatory demyelinating 馬偕紀念醫院神經內科 polyradiculoneuropathy. He was admitted for pain control. Proximal muscle Anti-Yo Associated Paraneoplastic th Cerebellar Degeneration : A Case weakness developed on the 5 day and Report we started plasmapheresis. However, he Chuo-Yu Lee, Lu-An Chen th Department of Neurology, Mackay Memorial became dyspnic on the 6 day. We Hospital th intubated him on the 8 day and the nadir of weakness (MRC 3 in proximal and 0 Background Paraneoplastic th in distal limbs) was on the 10 day. After cerebellar degeneration is a rare 6 sessions of plasmapheresis, his muscle disorder caused by the th power improved after the 16 day but he immune-mediated remote effects of still suffered from neuropathic pain in different types of malignancy. distal limbs when he was discharge on Subacute progressive cerebellar th the 75 day. symptoms usually precede the diagnosis of the cancer. Discussion Ropper et al (1984) first reported that 55% of GBS patient had Case Report A 44-year-old female characteristic pain early in the illness. In was admitted to neurology department these patients, serum CK elevated in 77% with a progressive ataxic gait disorder.

148 She had been well until two months antigen(CA) 153=132.6 U/ml, and CA earlier, when she noticed slurred speech. 125=2126.4 U/ml (normal range Two weeks later, she suffered from <30U/ml, <35 U/ml, respectively). light-headedness, unsteady gait, and Whole abdomen computed bilateral hands clumsiness. She tended to tomography (CT) scan revealed a large reach toward the wall for support as she cystic tumor in pelvic cavity, several walked. These symptoms progressed, and peritoneal masses, and three nodules she gradually could not sit, stand or walk over right subphrenic area. Two weeks without assistance. There was no history after admission, she underwent optimal of cognitive decline, headache, fever, debulking operation with abdominal nausea, vomiting, diplopia, paraesthesia, total hysterectomy, bilateral use of tobacco, alcohol, or illicit drugs. salpingo-oophorectomy, omectomy, There was also no family history of partial hepatectomy, diaphragm tumor movement or gait disorders. On excision, and colon tumor excision. neurological examination, she was alert, Final pathologic diagnosis was ovarian oriented, and had normal cognitive serous carcinoma, International function. The extraocular movements Federation of Gynecology and were full, and no nystagmus was noted. Obstetrics (FIGO) stage IIIC, with Severe dysarthri was found. The metastasis over peritoneal, omentum, remaining cranial nerves were intact. liver surface, diaphragm, and hepatic Sensory examinations were normal. flexure colon. Serum anti-Yo Reflexes were 3+ throughout. Dysmetria antibodies test by Western blotting was and dysdiadochokinesia were positive in this patient. Definite demonstrated in bilateral arms, more paraneoplastic cerebellar degeneration severe on the left side. She also had wide (PCD) was diagnosed according to the base gait, truncal ataxia and bilateral criteria of Graus et al. We arranged a dysmetria while performing the course of plasmapheresis consisting of heel-knee-shin test. five exchanges over six days, and Magnetic resonance imagings (MRI) of chemotherapy with carboplatin and the brain and cervical were normal. paclitaxel. However, she did not make Cerebrospinal fluid (CSF) studies any significant improvement. showed normal cell count but mild elevated protein (64mg/dL). Based on the Conclusion Previous literature presentation of subacute progressive showed most patients had limited cerebellar dysfunction, investigations for oncologic disease at the time of onset paraneoplastic syndrome were of paraneoplastic syndrome. It has undertaken. Elevated serum tumor been postulated that the antitumor markers were found, including cancer immune response may contribute to the

149 small size of the tumor. However, we noted. Papular erythematous skin presented a case of advanced stage rashes appeared progressively over ovarian cancer with anti-Yo associated bilateral proximal limbs, abdomen, and PCD and poor response to operation, inguinal area. She also had arthralgia at chemotherapy, and plasmapheresis. bilateral elbows, wrists and knees. These were followed by weakness of 165 four limbs. After hospitalization, 成人型史迪爾氏症併發腦幹腦炎：一病 intermittent spiking fever persisted. On 例報告 neurological examination, she had 邱劭文張宏旭林口長庚紀念醫院神經內科 bilateral ophthalmoplegia with downbeat nystagmus. Muscle Brainstem Encephalitis in Adult Onset weakness predominantly affected the Still’s Disease: Report of A Case Shao-Wen Chiu, Hong-Shiu Chang proximal parts of the limbs. There were Department of Neurology, Chang Gung Memorial also cerebellar dysfunctions including Hospital, Lin-Kou Medical Center dysmetria and ataxic gait. Objectives We report a case of adult Laboratory tests revealed leukocytosis onset Still’s disease with brainstem (WBC 19000/uL), anemia (Hb encephalitis. 10.1g/dL), elevated ESR (61 mm/hr), high Ferritin level (5284ng/mL), Background Adult onset Still’s disease normal ANA (1:80) and (AOSD) is a systemic inflammatory RF(<10.10IU/mL). A CSF study disorder of unknown etiology, showed normal protein concentration characterized with spiking fevers, without pleocytosis. Brain MRI evanescent rash, arthralgia, and revealed a midline lesion at the multi-organ involvement. Neurological midbrain mainly involving the complications have seldom been reported. periaqueduct area. The lesion was We described a 29-year-old Philippine hypointense on T1WI, hyperintense on woman of AOSD associated with brain T2WI/FLAIR and faintly hyperintense stem encephalitis. on DWI. There was no contrast enhancement. The findings were Case report A 29-year-old Philippine compatible with brainstem encephalitis. woman was admitted to our hospital due AOSD was diagnosed according to progressive bilateral ptosis and toYamaguchi’s criteria. diplopia since 2012/07/06. She initially The patient was treated with pulse experienced acute onset of vertigo with therapy with methylprednisolone 1 gm unsteady gait on 2012/07/01. Later, high per day and her fever, diplopia, fever up to 39’C, cough, sore throat and arthralgia and skin rashes gradually headache over bilateral frontal area were subsided.

150 Conclusion Our case demonstrates that Initially there was no focal weakness, AOSD should be included as one of the but progressive right side clumsiness etiologies for unexplained brain stem was noted within 2-3 days. On encephalitis neurologic examination, direct fundoscopy showed only mild pale on 166 optic disc of left eye, and dysmetria on 案例報告: 神經貝賽特氏病症─影像 right extremities. On the magnetic 及病理發現 resonance imaging (MRI) study, 唐奇峯蔡銘駿中國醫藥大學附設醫院神經部 T2-weighted images and fluid attenuated inversion recovery (FLAIR) Diffuse MRI: Apparent Diffusion images showed multiple Coefficient and Pathologic Findings in a Case of Neuro-Behçet's Disease hyperintensitivity ovoid spots in Chi-Feng Tang, Ming-Jun Tsai bilateral periventriuclar white matter of Department of Neurology, China Medical University Hospital, Taichung, Taiwan cerebrum and cerebellum without obvious enhancement, and apparent Background Behçet's disease (BD) is diffusion coefficient (ADC) showed known with inflammatory perivasculitis round lesions with increased intensity which may involve multiple organs, and on right cerebellum. rarely invade central nervous system. Brain biopsy from the lesion on left Here, we reported a case with frontal lobe was performed and the neuro-Beçhet’s disease with acute onset histologic examination of right cerebellar symptoms. found mild gliosis with abundant foamy histiocytes and no accumulation Case Report A 45-year-old man with a of inflammatory cells within vessels on history of Behçet's disease (BD) came to H-E stain, favored demyelinating our emergency department because of diseases. Pulse therapy with deteriorated ataxia for one week. BD was methylprednisolone 1000mg QD for diagnosed one year ago with the initial five days was given and immune presentation of repeated oral, genital modulation therapy with Cyclosporin ulcers and skin rashes. Uvietis with left 100mg 1# BID; Levamisole 50mg eye total blindness were noted half a year 1#QD; Prednisolone 5mg 8# QD were ago. He regularly took immune maintained. The neurological deficits modulation medication (cyclosporine got gradually improved. He followed 200mg, cyclophosphomide 50mg, up at our OPD for two months, no levamisole 50mg, prednisolone 7.5mg, further deterioration of neurologic colchicine 1mg per day) for disease deficits were noted. Then he got lost of control. follow up. One week prior to admission, he suffered from ataxia with right side deviation.

151 Discussion We reported a case of diagnostic dilemma in differential neuro-BD with initial manifestation as diagnosis of neuro-BD from MS by acute onset of right cerebellar symptoms. McDonald Criteria. The diagnosis of BD is based on clinical manifestation. In our case, brain MRI 167 detected multiple hyperintensitivity 抗磷脂抗體與小腦失調症：臨床分析 ovoid lesions on bilateral cerebrum and 和文獻回顧陳昶宏 1 呂鎮中 2 邱文燦 3 陳忠仁 3 cerebellum on FLAIR and T2-weighted 陳偉熹 1 image, whereas, only one lesion on right 高雄長庚紀念醫院神經內科 1 神經放射科 2 3 cerebellum showed increased intensity 風濕免疫科 on ADC. This lesion detected on ADC Antiphospholipid Antibody and was compatible with clinical Cerebellar Ataxia: A Clinical manifestations. Increased intensity in Analysis and Literature Review Chang-Hung Chen1, Chun-Chung Lui2, ADC suggested vasogenic edema in this Wen-Chang Chui3, Chung-Jen Chen3, Wei-Hsi Chen1 brain lesion. Vasogenic edema in this Departments of Neurology1, Neuroradiology2, brain lesion implied acute change of and Rheumatology3, Kaohsiung Chang Gung Memorial Hospital, and College of Medicine, permeability of involved vessels which Chang Gung University, Kaohsiung, Taiwan may be secondary to perivasculitis, an important pathologic marker of BD. In Objectives Antiphospholipid summary, our case showed clinical antibodies (APAbs) have been found to manifestation of BD with brain bind with and modulate signaling in involvement confirmed by clinical cerebellar neurons in in vitro studies. symptoms and brain image findings, so We review our database and patients in neuro-BD was impressed. According to literature to clarify the relation between the McDonald Criteria 2010, at least one increased APAbs and cerebellar ataxia. episode of attack and multiple cerebral and cerebellar ovoid lesions which Methods A total of 13 patients, disseminated in at least two different collected from the Kaohsiung Chang times in this case suggested probable MS. Gung Memorial Hospital and However, the location of biopsy was not Kaohsiung Medical University active lesion in this case, and the Hospital, who presented with differential diagnosis of neuro-BD from cerebellar ataxia with increased blood MS in this case still needed more clinical APAbs, and three 3 APAb-associated data including longer follow-up period. cerebellar ataxia patients in literature, We suggested ADC sequence may were reviewed. Ataxia was classified contribute to detection of acute brain into acute, subacute and chronic based lesion with vasogenic edema in on the clinical course. neuro-BD. And there still have

152 Results Acute ataxia was present in 3 effective treatment for patients and was exclusively due to APAb-associated cerebellar cerebellar stroke. In the 4 subacute ataxia ataxia. patients, there was no significant responsible structural change in brain. 168 Chronic ataxia was present in 6 patients 高 E 型免疫球蛋白症相關之慢性脫 who exhibited similar findings, including 髓鞘多發性神經炎：案例報告郭書帆陳律安 symmetrically confluent and 馬偕紀念醫院神經內科 non-confluent lesions at bilateral hemispheres, periventricular lucency, Chronic Inflammatory Demyelinating central atrophy, and temporal atrophy, in Polyradiculoneuropathy Associated variable severity. Cerebellum was spared. with HyperIgEaemia: A Case Report The preponderant APAb for acute, Shu-Fan Kuo, Lu-An Chen Department of Neurology, Mackay Memorial subacute, and chronic ataxia was lupus Hospital anticoagulant, anti-beta2- Background Chronic inflammatory glycoprotein I antibody, and demyelinating polyradiculoneuropathy anticardiolipin antibody, respectively. (CIDP) is thought to be a Systemic cancer was found in one out of heterogeneous autoimmune disease four subacute ataxia patients and in four involving either cellular or humoral out of six chronic ataxia patients. immune reaction. CIDP has several Removal of the cancer corrected the variant in clinical presentation and it ataxia and increased APAb in two also associated with many systemic patients. Plasmapheresis successfully diseases. Gammopathy, mostly IgM abolished ataxia in two subacute ataxia and IgG, is one of the common patients whereas immunosuppressive underlying diseases associated with treatment reversed ataxia in three CIDP. In rare condition, previously reported cases in literature. hyperIgEaemia has also been

associated with CIDP. Conclusion Cerebellar ataxia is not an exceptional condition associated with Case Report A 74 -year-old male had APAbs. A specific APAb subtype past history of hyperlipidemia and mechanism mediates ataxia through hypertension. He suffered from acute vascular and neuronal damage, with distal limbs numbness, double vision either structural destructive impairment and ataxic gait. Nerve conduction or functional neurotoxicity. APAbs study (NCS) revealed demyelinating should be examined in cerebellar ataxia change and cerebrospinal fluid (CSF) patients without determined cause. analysis showed albuminocytologic Plasmapheresis is a reasonable and dissociation. Acute inflammatory

153 demyelinating polyradiculoneuropathy therapy. (AIDP) with Miller-Fisher variant was impressed. Both symptoms and NCS Conclusions CIDP can be associated improved after 3 weeks of supportive with hyperIgEaemia but the care but numbness in glove and stocking pathogenesis still remains obscure. It distribution persisted. Three months later, has been speculated that heighted IgE he had another episode of acute response contribute to the peripheral symmetric proximal four limbs weakness nerve inflammation through the action and impending respiratory failure. NCS of mast cells. and CSF showed similar findings as previous episode. Under the impression 169 of AIDP, he received 5 sessions of 以廣泛橫貫性脊髓炎為臨床症狀表 plasmapheresis and both weakness and 現之休格蘭氏症候群：案例報告黃勇評陳培豪 respiratory failure recovered. Concerning 馬偕紀念醫院神經內科 about relapsing-remitting course and A Longitudinally Extensive possibility of CIDP, serial survey of Transverse Myelitis As The Presentation Of Primary Sjögren underlying diseases was done including Syndrome: A Case Report serum protein electrophoresis. No Yung-Pin Hwang1, Pei-Hao Chen1,2 1 Department of Neurology, Mackay Memorial monoclonal band presented but IgE was Hospital as significantly high as 1073 IU/mL. No 2 Graduate Institute of Mechanical and Electrical Engineering, National Taipei other autoimmune, endocrine, infectious University of Technology or malignancy disease were found. 7 months after the first episode, he had Background Sjögren syndrome (SS) another exacerbation of four limbs is a systemic autoimmune disease, weakness and more prominent glove and usually classified as stocking numbness. The symptoms again either primary or secondary. The main improved by 5 sessions of clinical features are xerostomia and plasmaphoresis. The IgE level was 1449 xerophthalmia. Central nervous system IU/mL, suggested the possible involvements in primary SS are not association between IgE titer and the common but include psychiatric clinical deterioration. Skin allergy test disturbances, late-onset migrainous showed strong reaction to mite although episodes, aseptic meningitis, no clinical history of allergy , parasite meningoencephalitis, focal infection or asthma was identified . Oral neurological deficits, and an acute or prednisolone was applied for CIDP. The chronic myelopathy. We herein serial followed IgE level also decreased describe one patient who suffered from from 1449 to 500 IU/mL. No clinical longitudinally extensive transverse exacerbation was noted since steroid myelitis (LETM) as the primary presentation of primary SS.

154 Case Report A 41-year-old female symptoms of dry eyes and difficult patient suffered from acute onset of left swallowing during previous 3 months. side face, trunk and upper limb Sialoscintigraphy showed mildly hypesthesia with tingling sensation and decreased uptake in the bilateral pain since September 21, 2012. salivary glands and Schirmer's test was Accompanying symptoms included abnormal with less than 5 mm, horizontal diplopia, spastic dysarthria, corresponding to the criteria of primary dysphagia and hyperreflexia. She denied SS. We started to treat with high-dose previous systemic disease. Family history methylprednisolone (1000 mg) daily was contributed to her mother’s for 3 days and symptoms showed rheumatic arthritis. No obvious lesion gradual improvement thereafter. She was found on her brain magnetic was able to walk without assistance resonance imaging (MRI). However, after hospital discharge. Visual evoked potential (EP) showed equivocal delayed P100 response Conclusions LETM can result from bilaterally. Somatosensory EP study a wide spectrum of different etiologies showed central somatosensory and NMO is one of the most common conduction delay between the cauda causes. Acute myelitis and optic equina to the cervical cord. MRI of the neuritis are absolute diagnostic criteria spine revealed a longitudinally extensive of NMO. Our patient has negative spinal cord lesion enhanced with NMO-IgG, providing important gadolinium from the level of the second information to exclude NMO and to cervical to the fifth thoracic vertebra. fulfill the criteria for primary SS. Cerebrospinal fluid study demonstrated a normal white cell count of 2 cells/cmm, 170 and an elevation of protein level of 126 Balo 同心圓性硬化症 mg/dL. The IgG index was normal 楊依倩呂明桂蔡崇豪中國醫藥大學附設醫院神經內科 without oligoclonal bands. She was diagnosed to have a longitudinally Balo’s concentric sclerosis Yi-Chien Yang1, Ming-Kuei Lu1, Chon-Haw extensive transverse myelitis, a Tsai 1,2 characteristic feature of neuromyelitis 1 Department of Neurology, China Medical University Hospital, Taiwan optica (NMO), but also occurring in 2 Graduate Institute of Neural and Cognitive various other autoimmune and Sciences, China Medical University inflammatory diseases that involve the Background Balo’s concentric CNS. Her NMO-IgG was negative. sclerosis is a rare variant of multiple Further laboratory examinations showed sclerosis. Histopathologically, it was high titers of anti-SSA antibodies. characterized by alternating layers of Tracing back the history, she had mild myelinated and demyelinated tissue.

155 The current diagnostic method is based Case 2 A 24-year-old woman on brain MRI which demonstrates developed sudden onset of slurred concentric bands of low- and iso-signal speech, swallowing disturbance and lamellar lesions in T1-weighted sequence. right side weakness. Neurological The correct diagnosis is important for examination revealed right visual both the appropriate treatment and blurring, mild dysarthria, trivial right avoiding unnecessary surgical hand weakness and relative intervention. hyperreflexia of right limbs with intact sensation. Brain MRI disclosed one Case 1 A 23-year-old man presented concentric nodule with alternating with progressive left side numbness layers of different signal intensities in descending from face to lower limb the left corona radiata. She received within one week in November 2011. He only supportive treatment and the had a history of lymphoma ten years ago symptoms subsided gradually with and had received chemotherapy for that. only mild sequelae of difficulty in Brain MRI (Fig. 1) revealed two cystic writing. mass lesions in right parietal and frontal lobe, respectively. After brain biopsy, he Discussion The clinical courses of was treated with intravenous Balo’s sclerosis are variable and some dexamethasone under the impression of may lead to a lethal outcome. The two recurrent lymphoma. The symptoms patients in our series seemed behaving resolved gradually during follow-up in in a relatively benign trail. The the outpatient clinic. Eight months later, characteristic MRI features usually another episode of left side numbness lead to a correct diagnosis without was developed in an ascending fashion difficulty for experienced neurologists from left lower limb to the left shoulder, and this is crucial to avoid unnecessary followed by left hemiparesis. Repeated surgical intervention as performed in MRI scan (Fig. 2) showed a novel cystic Case 1. Although the pathophysiology change in right parietal lobe with is not yet known, Balo’s sclerosis concentrically alternating bands of higher usually responds to steroid well and and lower signal intensity; Balo’s some may even remit without concentric sclerosis was diagnosed. We medications. administrated methylprednisolone pulse therapy for a complete course and his 171 symptoms were ameliorated with only 口服 Fingolimod 用於台灣多發性 mild numbness of the left leg 4 weeks 硬化症患者之初步臨床觀察楊博丞 1 楊智超 2 later. 恩主公醫院神經科 1; 國立台灣大學醫學院附屬醫院神經部 2

156 Oral Fingolimod in Taiwanese Patients was observed when measured at with Multiple Sclerosis. A 4 month around 3 weeks, confirming drug assessment. Pao-Chen Yang1, Chih-Chao Yang2 efficacy. Department of Neurology, En Chu Kong Hospital1; Department of Neurology , National Taiwan University Hospital2, Taipei, Taiwan Conclusions This study demonstrated the clinical safety of Background Oral fingolimod (FTY720) fingolimod for the first time in has previously shown clinical efficacy in Taiwanese patients with MS, safety phase II/III studies of predominantly signals are so far consistent with the Caucasian populations with multiple established effects of fingolimod in sclerosis (MS). This is the first time Caucasian patients. fingolimod has been used in Taiwanese patients. 172 Objectives: To report 4-month 肌無力症病人的血漿分離及類固醇 physiological outcomes in Taiwanese 脈衝合併療法 patients with relapsing MS treated with 葉建宏陳威宏邱浩彰新光吳火獅紀念醫院神經科 fingolimod with the context of routine medical practice. Combined plasmapheresis (PP) and intravenous methylprednisolone Methods The purpose of this (IVMP) pulse therapy in myasthenia Jiann-Horng Yeh, Wei-Hung Chen, Hou-Chang observational report is to analyze Chiu Department of Neurology, Shin Kong Wu short-term data on safety and Ho-Su Memorial Hospital, Taipei effectiveness of fingolimod, in Taiwanese patients who are using fingolimod for the Background & purpose IVMP pulse first time. This report explores the therapy is efficacious in quick incidence of selected physiological amelioration of muscle weakness in related outcomes of fingolimod treatment myasthenia gravis (MG), however, during follow-up visits. transient worsening of weakness on corticosteroids is a major concern. Results This is the report of 16 Plasmapheresis has been shown to be a Taiwanese patients currently on successful therapy for patients with fingolimod. The mean duration of MG in crisis or fulminant progression. exposure to fingolimod is 4 month. We applied a combination therapy with During first-dose monitoring, a decrease PP and IVMP pulse for moderate to in mean pulse rate of 5bpm was observed severe MG patients. 1 hour after the first dose of fingolimod, and reached a maximal decrease from Method From January 2011 to pre-dose values of 12 bpm 5 hours after October 2012, 79 generalized MG dosing. Average WBC reduction of 41% patients, 53 women and 26 men, aged

157 13-77 years, refractory to immune in patients with moderate to severe MG therapy were treated with plasmapheresis. in terms of quicker, higher, and more Four out of 79 patients were sustained clearance of pathogenic concomitantly infused with antibodies after treatment as compared methylprednisolone (1 g/day) for 3 days to PP treatment alone. after the second session of plasmapheresis. We prospectively 173 recorded the MG score and measured 視神經脊髓炎病人腦部核磁共振之 acetylcholine-receptor antibody 影像特徵廖洺鋒張國軒呂榮國黃錦章張宏旭 (AchRAb) concentration at baseline and 吳逸如陳瓊美朱俊哲郭弘周徐文俊 after each session of plasmapheresis, and 羅榮昇 monthly after plasmapheresis for up to 3 林口長庚紀念醫院神經內科多發性硬化症中心 months. We compared all these parameters between PP-IVMP group and Magnetic Resonance Imaging PP alone group. Characteristics amongst Patients with Neuromyeitis Optica Spectrum Disorder Results The MG score decreased from Ming-Feng Liao MD, Kuo-Hsuan Chang MD PhD, Rong-Kuo Lyu MD, Ching-Chang Huang a mean of 9.5 at baseline to 4.8 after MD, Hong-Hsiu Chang MD PhD, Yih-Ru Wu PP-IVMP treatment and from 10.6 to 7.0 MD PhD, Chiung-Mei Chen MD PhD, Chun-Che Chu MD, Hung-Chou Kuo MD, after PP treatment alone. The mean Wen-Chiun Hsu MD MSc MPhil, Long-Sun Ro, clearance of AchRAb was 74.3% of the MD PhD Multiple Sclerosis Center, Department of baseline level after PP-IVMP treatment Neurology, Chang Gung Memorial Hospital-Linkou Medical Center and Chang in two seropositive patients. In the PP Gung University College of Medicine Taipei, alone group, the clearance of AchRAb Taiwan seemed to slightly lower after PP and 3 months after discharge as compared to Background Neuromyelitis optica combination therapy (p=0.0753 and (NMO) is an inflammatory disease p=0.0567, respectively). Transient mainly characterized with optic worsening of weakness did occur in one neuritis (ON) and longitudinal out of 4 patients and was noted at the 3rd extended spinal cord lesions (LESCLs). day of IVMP infusion with limb Recently, many studies have reported involvement predominantly. This that there are also brain lesions in the worsening subsided 4 days later. No NMO patients; and those findings infection or gastrointestinal bleeding was cause the challenge to differentiate reported during IVMP treatment. NMO patients from multiple sclerosis (MS). Conclusion The combination therapy with PP and IVMP is safe and effective Objective Characterization of the specific brain lesion features of

158 neuromyelitis optica spectrum disorder 盧彥廷曾昱龍高雄長庚紀念醫院神經內科 (NMOSD) by comparing the cranial magnetic resonance imaging (MRI) of Rapid Life-Threatening Global patients with NMOSD and MS. Cerebral Edema As The First and Methods: Clinical and cranial MRI data Only Sign of The Central Nervous System Manifestation in Patient with of patients who were diagnosed as Systemic Lupus Erythematosus NMOSD (with the presence of Yan-Ting Lu, Yu-Lung Tseng Department of Neurology, Chang Gung anti-aquaporin 4 antibodies) or MS were Memorial Hospital, Kaohsiung Medical Center retrospectively reviewed in a referral medical center in Taiwan. Background The major Results: 44 and 39 cranial MRIs were manifestations of central nervous obtained from 28 patients with NMOSD system of systemic lupus and 18 patients with MS, respectively. erythematosus are boarded. The Ependymal lining (27.3% versus 5.1%, neuropsychiatric manifestations P=0.007) and hypothalamus lesions present a diagnostic and treatment (15.9% versus 2.6%, P=0.04) were seen challenge, but there is rare case more frequently in NMOSD patients than presenting acute and life threatening MS patients. On the other hand, brain global cerebral edema. lesions of MS patients meet the Barkhof Case Report A 31-year-old woman criteria more frequently (13.6% verusus with lupus erythematosus for 6 years 46.2%, P=0.001). Juxtacortical, had no history of neurological disease subcortical, basal ganglion, and was admitted to local hospital with infra-tentorium, corpus callosum and frequent generalized convulsion. A ovoid lesions were also seen in MS more head computerized tomographic scan frequently. In contrast to multiple showed no hemorrhage or infarct. She sclerosis, some brain lesions of NMOSD was intubated with ventilator support patients (28.6%) disappeared during due to status epilepticus On the next follow up. day, she was referred to our emergency

department due to shock. The head Conclusions NMOSD demonstrates computerized tomographic scan ependymal lining and hypothalamic showed diffused cerebral edema with lesion more frequently than MS. These small intracerebral hemorrhage. findings could be helpful to identify Magnetic resonance imaging of the NMOSD before the onset of spinal cord brain showed diffuse cerebral edema, lesions. transtentorial and tonsillar herniation.

C3 complement dropped to 65 mg/dl 174 紅斑狼瘡患者以快速危及生命的腦水 and C4 was within low normal limit of 腫為中樞神經系統的最初及唯一表現 10.2. ANA(1:320 titer) and anti-ENA

159 screen were positive. The patient had which was considered to have progressive herniation and expired within restricted involvement of limbic hours. system.

Conclusion The life-threatening global Objectives To study a patient with cerebral edema and coma are rare CNS anti-AMPA receptor encephalitis complication of lupus. Early survey and during pregnancy. recognition of the neuropsychiatric presentation of SLE and aggressive Case Report A 30-year-old treatment is required to avoid such as primiparous pregnant woman at 11th fetal and rare complication. week gestation was admitted due to headache, behavior change and recent 175 memory impairment for 1 week. She 懷孕期發生之抗 AMPA 受體腦炎: developed fever, dysmetria, ataxia, 一病例報告魏怡嘉 1 劉濟弘 2 林建志 3 林昆儒 4 黃國倫 2 multi-directional nystagmus, 李宗海 2 彭宗義 1 林光麟 3 黃錦章 2 quadriparesis, conscious disturbance, 1 2 3 基隆長庚神經內科林口長庚神經內科小 myoclonic seizure, opisthotonus, and 兒神經科 4 核子醫學科 status epilepticus in the following 5 Rapid Progression of Anti-AMPA days. Status epilepticus was rapidly Receptor Encephalitis during controlled with midazolam infusion, Pregnancy Yi-Chia Wei1, Chi-Hung Liu2*, Jainn-Jim Lin3, propofol infusion, levetiracetam, and 4 2 2 Kun-Ju Lin , Ko-Lun Huang , Tsong-Hai Lee , lamotrigine. Tsung-I Peng1, Kuang-Lin Lin3, Chin-Chang Huang2 The first brain MRI preformed 1 week

after onset showed hyperintensity on 1 Department of Neurology, Chang Gung Memorial Hospital, Keelung Medical Center ﬂuid-attenuated inversion recovery 2 3 Department of Neurology, Division of Pediatric （FLAIR）sequence in the bilateral neurology, 4 Department of Nuclear Medicine and Molecular Imaging Center, Linkou Medical insula, mesial temporal lobes, and Center caudate nucleus. CSF study revealed

Background The α-Amino- 3- eosinophilic pleocytosis with elevated hydroxy-5-methyl-4-isoxazolepropionic protein. Infectious studies showed acid (AMPA) receptor is an iontropic negative results of cryptococcus, glutamate receptor located in CNS. tuberculosis, herpes simplex virus, AMPA receptors major in fast excitatory Angiostrongylus cantonensis, herpes transmission and play a key role in CNS zoster virus, Japanese encephalitis plasticity by mediating long-term virus, and West Nile virus. The potentiation and long-term depression. immunoreactivity study detected Anti-AMPA receptor encephalitis is a anti-AMPA receptor antibodies against rarely reported autoimmune encephalitis, GluR2 in this patient. Underlying

160 malignancy was not found until the latest analogous to amphetamine and related follow up. psychostimulants. However, the neurobehavioral effect is only studied Conclusion Anti-AMPA receptor in experimental animals and has barely encephalitis could be fulminant with been mentioned in human yet. rapid brain atrophy and extensive CNS involvement including diffuse cerebral Methods We reported the clinical and cortex, caudate nucleus, and cerebellum. judiciary investigation of the The reversibility of the atrophic brain and antemortem neurobehavioral the association between anti-AMPA manifestations in 8 patients who died encephalitis and pregnancy need further of PMMA usage. investigation. Results There were 2 different antemortem presentations. The first 其他 group of patients showed delirium, 176 hypertalkativity, and incoherent speech 致命性副甲氧基甲基安非他命濫用死 and then turned into convulsions and 亡前神經行為表現 death. They did not exhibit the typical 尹莘玲 1,2 黄宗揚 3 陳偉熹 4 hyperdopaminergic movement disorder. 高雄醫學大學附設醫院臨床法醫科 1 法務部法醫研究所 2 The second group of patients gradually 3 高雄地方法院 fell asleep and then suffered respiratory 高雄長庚紀念醫院神經內科 4 or cardiovascular collapse. The cardiac The Antemortem Neurobehavior in blood PMMA level was higher in the Fatal Paramethoxymethamphetamine second group than that in the first Usage Hsin-Ling Yin1,2, Chung-Yang Huang3, Wei-Hsi group of patients. Forensic autopsy 4 Chen showed variable findings, ranging from Department of Clinical Forensic Medicine1, Kaohsiung Medical University Hospital and no remarkable change to significant College of Medicine, Kaohsiung Medical pathological damage similar to University, Kaohsiung, Taiwan Institute of Forensic Medicine1, Ministry of serotonin syndrome in both groups of Justice, Taipei, Taiwan Kaohsiung District Local Court2. Justice Yuan, patients. Kaohsiung, Taiwan Department of Neurology3, Kaohsiung Chang Gung Memorial Hospital, and College of Conclusion PMMA seems to Medicine, Chang Gung University, Kaohsiung, enhance serotoninergism than Taiwan dopaminergism, and exerts a Objective concentration-related dual effect on Paramethoxymethamphetamine (PMMA) human. is an emerging and prevalent psychoactive drug with a structure

161 177 told her family that she felt paresthesia 案例報告: 抗 NMDA 受體腦炎 over bilateral lower limbs. Furthermore, 王馨霈楊玉婉 her parents noticed that she became 中國醫藥大學附設醫院神經部暨神經醫學實驗室 agitated and had loosening of association between speech and Case report: Anti-NMDA receptor encephalitis thoughts. Hallucinations developed Hsin-Pei Wang, Yu-Wan Yang later. Physical examinations revealed Neuroscience Laboratory, Department of Neurology, China Medical University Hospital, no prominent abnormality. Laboratory Taichung, Taiwan data showed hyperglycemia (HbA1c:8.8%), elevated liver enzymes Background (GOT/GPT:44/50IU/L), and abnormal Anti-NMDA(N-methyl-D-aspartate) tumor marker (CA-125: 35.5U/mL). receptor encephalitis was first described There was some slimming drugs used in 2007 in a cohort study of 12 women recently. The 24-hours-EEG indicated presenting severe neuropsychiatric generalized paroxysmal sharp wave syndrome with autoantibodies targeting with phase reversal at C4 and T4. No glutamate receptors. After first patient abnormality was disclosed in the brain was diagnosed, there were increasingly MRI and the lumbar puncture. The documented cases. The manifestations autoimmune profile was normal, were usually psychotic problems (bizarre except weakly positive NMDA behavior, hallucination, memory deficits), receptor antibodies. So we applied seizure, and dyskinesia. At the beginning plasma exchange five times every two of diagnosis, large proportion of patients days. Her consciousness was recovery was regarded as drug-induced psychosis. dramatically (from coma to clear). Here we report a patient with suspected anti-NMDA receptor encephalitis. Conclusion Anti-NMDA receptor encephalitis predominantly affects Case Report A 20-year-old female young women and evolves through student with a history of type 2 diabetes several stages: prodromal symptoms, mellitus for 5 years was admitted due to psychotic stage, unresponsiveness with acute disturbing behavior. No hypoventilation, dyskinesia, and prominently developmental delay was autonomic instability. This disease was noted in this patient in the past. Her rare, but it became more recognized in personality was submissive and recent years. The treatment is introverted. However, three months ago, significantly effective. So we should she had transient depression due to keep it in mind that young people arguments with her coworker. Poor presenting with acute psychosis in the attention was noted by her coworker 10 absence of other organic problems days prior to this admission. She also

162 might have anti-NMDA receptor liver segment S4, a hepatectomy was done encephalitis. and tissue was sent for biopsy, which revealed a gross appearance of grayish 178 white tumor. Immunohistochemically, the 肝轉移性顱內腦膜瘤 liver tumor showed diffuse strong 吳鈺慈孫明輝黃志仁 expression for vimentin and focal 光田綜合醫院神經內科 positivity for epithelial membrane antigen, Intracranial Meningioma with Hepatic an immunophenotype typical for Metastasis: A Case report meningioma . Yu-Tzu Wu, Ming-Hui Sun, Chih-Jen Huang Department of Neurology, Kuang-Tien General Hospital Conclusion This case demonstrate the

Background Meningiomas are possibility of Liver metastasis from VP slow-growing intracranial/intraspinal shunt drainage, although the more tumours, with a wide range of commonly postulated venous route of histopathological variants. The more spread could not be entirely excluded. aggressive atypical and malignant types can disseminate via the venous system, 179 老人跌倒整合預防保健介入模式 lymphatics and CSF with the lungs and 李雪楨 1 張谷州 2 曹昭懿 3 洪禎雯 4 黃裕淨 pleura being the most common sites of 2 林桑伊 5 台灣防跌研究團隊 1 extracranial metastasis. 國立陽明大學物理治療暨輔助科技學系 2 高雄長庚紀念醫院神經內科腦血管科 3 台灣大學物理治療系 Case report A 71 year-old female patient 4 高雄長庚紀念醫院復健科 5 who had a history of recurrent meningioma. 成功大學物理治療系

She underwent craniotomy and tumor Effects of a Multifactorial Fall resection 15 year ago. Further resection was Prevention Program on Fall done due to tumor recurrence with invasion Incidence and Physical Function in Community-dwelling Elderly with to skull and scalp that occur on separate Risk of Fall. occasions. Three weeks after her last Lee HC1, Chang KC2, Tsauo JY3, Hung JW4, Huang YC2, Lin SI5, Taiwan fall prevention craniotomy and tumor resection, she was initiatives investigators re-admitted due to communicating 1 Department of Physical Therapy and Assistive Technology, National Yang-Ming hydrocephalus , a ventriculo-peritoneal (VP) University, Taipei, Taiwan 2 shunt was inserted, which was revised 6 Division of Cerebrovascular Diseases, Department of Neurology, Kaohsiung Chang months after due to VP shunt malfunction. Gung Memorial Hospital, Kaohsiung, Taiwan 3 This time, she was admitted due to acute Department of physical therapy, National Taiwan University pancreatitis with concomitant calculous 4 Department of Rehabilitation Medicine, Kaohsiung Chang Gung Memorial Hospital, cholecystitis and cholangitis. Kaohsiung, Taiwan Cholecystectomy was performed. 5 Department of physical therapy, National Cheng Kung University Incidentally, a nodular lesion was found over

163 Objective To evaluate effects of a balance and proprioception), timed multifactorial fall prevention program on up-and-go (TUG), fall incidence and physical function in Taiwanese-International Physical community-dwelling older people. Activity Questionnaire, EuroQoL-5D, Geriatric Depression Scale (GDS), and Desing Multi-center randomized Fall Efficacy Scale at 3 months after controlled clinical trial randomization.

Setting Three medical centers and Results There were 616 participants, adjacent community health centers in aged 76±7 years, including low risk Taiwan. 25.6%, moderate risk 25.6% and marked risk 48.7%. The cumulative Participants Community-dwelling 1-year fall incidence was 25.2% in IG elderly who had fallen in the previous and 27.6% in CG (HR=0.90, 95% CI year or at risk for falls 0.66-1.23). IG improved more favorably than CG on overall PPA Interventions After baseline fall-risk index, reaction time, postural assessment, eligible subjects were sway with eyes open, TUG, and GDS, randomly allocated into the intervention especially for those with marked fall group (IG) or control group (CG) risk. stratified by Physiological Profile Assessment (PPA) fall-risk level. IG Conclusion The multifaceted fall received a 3-month multifactorial prevention program with exercise intervention program including 8-week intervention improved functional exercise training, health education, home performance at 3-months for hazards evaluation/ modification, along community-dwelling elders with a risk for with medication review and falls, but did not reduce falls over 1-year ophthalmology/other specialty follow-up. Fall incidence might have been consultations. CG got health education decreased simultaneously in both groups brochures, referrals and by heightened awareness engendered recommendations without direct exercise during assessments, education, referrals, intervention. and recommendations.

Main outcome measures Primary 180 outcome was the incidence for falls 血栓溶解治療在中央健康保險給付 within one year. Secondary outcomes 規定中之不確定概念林綉紅趙千淑陳廷耀張谷州劉嘉為 were PPA battery (overall fall-risk index, 陳偉熹 vision, muscular strength, reaction time, 高雄長庚紀念醫院神經內科系暨腦中風中心

164 The Uncertain Concept in Payment Using Flow Cytometry to assay Regulation for Thrombolysis by the hematologic disorders Central Health Insurance Bureau, Yi-Pin Chiu 1, Wei-Hsiang Wang1, Yi-Cyun Yang2, Taiwan Ian Liau2, Bak-Sau Yip1 1 Hsiu-Hung Lin, Chien-Shu Chao, Teng-Yeow Tan, Neurological Institute, National Taiwan Ku-Chou Chang, Chia-Wei Liou, Wei-Hsi Chen University Hospital Hsin-Chu Branch, Department of Neurology and Stroke Center, 2 The Department of Applied Chemistry, Kaohsiung Chang Gung Memorial Hospital, and National Chiao Tung University College of Medicine, Chang Gung University, Kaohsiung, Taiwan Background Hematologic disorders 目的由於急性缺血性腦中風並沒有 include erythrocyte disorders, platelet 特別治療之方法，故血栓溶解目前被廣 disorders, blood cell dyscrasias, 泛接受。為了安全起見，中央健康保險 coagulation disorders, etc. These 局設定必須先通過收錄條件和排除條 diseases play an important role in 件才能使用。但是嚴格的收錄條件和排除條件相對地剝奪醫療消費者接受醫 many neurologic disorders. There are 療的權利，也因此在世界各地發生多起 several clinical methods to assay 對收錄條件和排除條件之認知有差異 hematologic disorders, including blood 的司法訴訟。 cell analyzer, bone marrow aspiration and bone marrow biopsy to detect the 方法回顧我國血栓溶解劑藥品給付 blood cell count and morphology. 規定中之收錄條件和排除條件，並與其他國家之規定比較。 Objective The traditional method to 結果在中央健康保險局所設定的收 assay hematologic disorders focus on 錄條件和排除條件中，有三項條文的內 blood cell numbers and cell 容可能對民眾和醫療提供者產生認知 morphology. Our study tried to 差異，分別為臨床嚴重程度、臨床上恢 establish a new method to assay the 復速度和程度、及其他狀況之項目。其差異主要原因是文字解釋和規範不確 functions of blood cell. 定，與疾病狀況裁量範圍過於擴大。 Method Flow cytometry is a laser 結論藥品給付規定之條文內容必須 based, biophysical technology 清楚和合理，讓醫療消費者、醫療提供 employed in cell counting, sorting, 者、行政管理者、法律工作者互相之間的差異減少，認知趨向一致。因此，建 biomarker detection and protein 議部份修改這三條條文之內容，讓各方 engineering, by suspending cells in a 面認知更接近，減少歧見，醫療才能正 stream of fluid and passing them by an 常發展。 electronic detection apparatus. It allows simultaneous multiparametric 181 analysis of the physical and/or 利用流式細胞技術來分析血液病變 chemical characteristics of up to 邱奕賓 1 王韋翔 1 楊逸群 2 廖奕翰 2 葉伯壽 1 1 台大新竹分院神經內科 thousands of particles per second. We 2 國立交通大學應用化學系 use the flow cytometry to assay the function of blood cell.

165 Result By using the new method, we paraphasia, loss of motivation, auditory can assay not only the counts and and visual hallucinations, and sleep morphology of blood cells but also detail disturbance. During the second functions of blood cells. hospital course, CSF cytology revealed increased cellularity and some mildly 182 pleomorphic cells, otherwise 原發性惡性腦膜黑色素瘤: 案例報告 unremarkable. Whole spine MRI 柯子翔邱瑋婷胡朝榮 showed leptomeningeal enhancement 台北醫學大學-行政院衛生署雙和醫院神經科 in the whole spine, brainstem, Primary Malignant Meningeal cerebellum, and the visualized part of Melanoma: A Case Report brain. Open biopsy of the Tzu-Hsiang Ko M.D, Wei-Ting Chiu M.D, Chaur-Jong Hu M.D leptomeninges was performed and it Department of Neurology, Shuang-Ho Hospital, Taipei Medical University, New Taipei City, showed malignant melanoma. There Taiwan was no evidence of intraocular melanoma and skin biopsy of a nevus Background A 41-year-old man, who at right lateral upper arm showed is diagnosed as primary malignant benign intradermal nevus. There was meningeal melanoma, presented with no other lesion detected by PET symptoms and signs mimicking examination. Therefore, primary meningitis. malignant meningeal melanoma was Case Report A 41-year-old man diagnosed. without underlying diseases was initially admitted due to headache, dizziness, and Conclusion Primary malignant vomiting for two weeks. He had been meningeal melanoma is a rare disease. hospitalized in another hospital under the It may mimic the clinical presentations diagnosis of aseptic meningitis, but the of subacute or chronic meningitis. symptoms persisted after anti-viral Meningeal biopsy should be therapy. During the first hospital course, considered if the diagnosis is uncertain. brain MRI revealed diffuse abnormal leptomeningeal enhancement. CSF 183 showed mild pleocytosis only. We gave 中風病患非正式照顧者之負荷程度 him an osmotic agent and the headache 及相關因子 1 2 2 improved gradually. Left facial numbness 洪禎雯黃裕淨張谷州 1高雄長庚紀念醫院神經內科腦血管科 was observed and was considered as a 2高雄長庚紀念醫院復健科 seizure disorder, which subsided with Factors Associated with Strain in anti-epileptic therapy. After discharge, Informal Caregivers of Stroke intermittent headache and vomiting Patients 1 2 continued. The patient subsequently Jen-Wen Hung ; Yu-Ching Huang , Ku-Chou Chang2. developed memory impairment, 1 Department of Rehabilitation Medicine,

166 Kaohsiung Chang Gung Memorial Hospital, caregiver strain. Caregiver factors, Kaohsiung, Taiwan, 2 Department of Neurology, Kaohsiung Chang such as changed employment status, Gung Memorial Hospital and Chang Gung help from formal caregivers, and University College of Medicine, Kaohsiung, Taiwan; depression (BDI 10) were also associated with considerable caregiver Background Stroke is one of the most strain. prevalent causes of adult disability and handicap. Informal caregivers play an Conclusions Nearly 50% of important role in poststroke care. caregivers experienced considerable However, informal caregivers may strain. Interventions aimed at reducing experience strain, which threatens the the caregivers’ strain should focus on recovery of stroke subjects. This study enhancing the functional and emotional aimed to describe changes in strain status of stroke subjects, prevention of experienced by informal caregivers from recurrent stroke, and efficient 3 to 6 months after the stroke, and management of depression symptoms identify the predicting factors. in caregivers.

Methods We recruited pairs of 184 inpatients with ischemic stroke and 全民健康保險腦中風治療效益分析 1 2 1 3 1.4 informal caregivers from a tertiary 張谷州李雪楨黃裕淨洪禎雯邱顯學陳俊忠 2 李宗海 5 referral hospital and interviewed them at 1 高雄長庚紀念醫院神經內科腦血管科 3 and 6 months after the stroke. 2 國立陽明大學物理治療暨輔助科技學系 3 Caregiver strain was evaluated using the 高雄長庚紀念醫院復健科 4 高雄長庚紀念醫院中醫針傷科 Caregiver Strain Index (CSI), with a CSI 5 林口長庚紀念醫院神經內科腦血管科 7 indicating considerable caregiver Cost-effectiveness analysis of stroke strain. Various factors associated with management under a universal caregiver strain were analyzed using health 1 2 generalized estimating equations. Ku-Chou Chang , Hsuei-Chen Lee , Yu- Ching Huang1, Jen-Wen Hung3, Hsienhsueh Elley Chiu1.4, Jin-Jong Chen2, Tsong-Hai Lee5 1 Results Eighty-nine stroke patients and Division of Cerebrovascular Diseases, Department of Neurology, Kaohsiung Chang caregivers completed the study. Gung Memorial Hospital, Kaohsiung, Taiwan 2 Department of Physical Therapy and Considerable strain was reported in 46% Assistive Technology, National Yang-Ming and 43% of the caregivers at the 3rd and University, Taipei, Taiwan 3 th Department of Rehabilitation Medicine, 6 month, respectively. Patient factors Kaohsiung Chang Gung Memorial Hospital, such as severe disabilities (Barthel Kaohsiung, Taiwan 4 Division of Acupuncture & Traumatology, Index?60), poor cognition (Mini-Mental Department of Traditional Chinese Medicine, State Examination 23), depression Kaohsiung Chang Gung Memorial Hospital, Kaohsiung, Taiwan (Beck Depression Inventory [BDI] 10), 5 Stroke Center, Department of Neurology, Linkou and recurrent stroke were predictors for Chang Gung Memorial Hospital, Taoyuan, Taiwan

167 Objective Cost-effectiveness analysis severe IS. NS was the dominant care (CEA) of stroke management was model in mild-moderate HS, while NW evaluated in three care models: appeared to be a cost-minimization Neurology/Rehabilitation wards (NW), model for severe HS. Neurosurgery wards (NS), and General/miscellaneous wards (GW), Conclusions TIA/unspecified stroke under a universal health insurance carried substantial risk of AE. NS system. performed better in serving mild- moderate HS, whereas NW was the Methods From 1997 to 2002, subjects optimal care model in management of IS. with first-ever acute stroke were sampled from NHI claims data of a nationally 185 representative cohort in Taiwan, 貝爾氏麻痺增加癌症之風險：五年 categorized as hemorrhage stroke (HS), 追蹤研究許昭俊 1 林恆慶 2 including subarachnoid hemorrhage 1 台北醫學大學附設醫院神經科 (SAH), and intracerebral hemorrhage 2 台北醫學大學醫務管理學系 (ICH); or ischemic stroke (IS), including Increased Risk of Cancer Following cerebral infarction (CI), and transient Bell's Palsy: A Five-year Follow-up ischemic attack/ unspecified stroke Study (TIA/unspecified); with mild-moderate Jau-Jiuan Sheu1, Herng-Ching Lin2 1 Department of Neurology, Taipei Medical and severe severity. All-cause University Hospital 2 readmissions or mortality (AE) and direct School of Health Care Administration, Taipei Medical University medical costs during first-year (FYMC) after stroke were explored. CEA was performed by incremental Background and Purpose The cost-effectiveness ratios. etiology of Bell’s palsy is usually unclear. The most commonly proposed Results 2368 first-ever stroke subjects pathomechanism involves the including SAH 3.3%, ICH 17.9%, CI reactivation of latent herpes simplex 49.8%, and TIA/unspecified 29.0%, were virus type I and varicella-zoster virus, identified with AE 59.0%, 63.0%, 48.6%, which is associated with and 46.8%, respectively. There were immunosuppressed states. The risk of 50.8%, 13.5%, and 35.6% of stroke cancer is also elevated in patients served by NW, NS and GW, with immunosuppressed patients. The AE 44.9%, 60.6%, and 56.0%, and purpose of this study was to investigate medical costs of US$ 5,031, US$ 8,235, the risk for cancer during a 5-year and US $ 4,350, respectively. NW was follow-up period after diagnosis of cost-effective for both mild-moderate and Bell’s palsy by using a population- based dataset in Taiwan.

168 Methods This study drew a study Because the increased cancer risk in cohort and a comparison cohort from the the follow-up years may be indicative “Longitudinal Health Insurance Database of the presence of an occult 2000” released by the Taiwan National malignancy at the time of Bell’s palsy Health Research Institute. A total of diagnosis, our findings may warrant 2,618 patients with Bell’s palsy were increased screening for occult included as the study cohort and 13,090 malignancies among patients with patients without Bell’s palsy were Bell’s palsy. included as comparison cohort. Each patient was individually tracked for 5 186 years from their index ambulatory visit to 使用動態模型及最適化方法尋找萊 identify those in whom cancer diagnosed. 希尼亨症候群的治療目標酵素許凱程 Cox proportional hazards regression was 許凱程腦神經內科診所 performed to compare the 5-year risk of subsequent cancer between the study and Detection of enzyme targets by comparison cohorts. dynamic modeling and optimization: Application to Lesch–Nyhan syndrome Results We found that the incidence of Hsu Kai-Cheng Hsu Kai-Cheng Neurology Clinic cancer was 1.55 (95 % CI 1.35–1.78) per

100 person-years for patients with Bell’s Motivation Mathematical modeling palsy and 1.09 (95 % CI 1.02–1.18) per and optimization have been used in 100 person-years for comparison patients. detection of target enzyme in human The hazard ratio (HR) for cancer during metabolic disorders. This method the 5-year follow-up period for patients integrates available biomedical data with Bell’s palsy was 1.43 times that for and builds a dynamic model of human comparison patients (95 % CI 1.22–1.73), metabolic system. Target enzymes for adjusting for urbanization, monthly treatment are discovered through income, geographic region, and diabetes. optimization method. Lesh-Nyhan In analysis by cancer location, we syndrome is an inherited disorder with detected increased risk of oral cancer, several neurological symptoms. It is gastrointestinal cancer, and cancer at caused by a deficiency of the enzyme other sites between patients with Bell’s hypoxanthine-guanine palsy and comparison patients. phosphoribosyltransferase (HGPRT)

involving in uric acid metabolism. We Conclusion Our study found that Bell’s introduce the fuzzy optimization palsy was significantly associated with method to find the potential enzymes an increased risk of subsequent cancer for drugs design in this metabolic during a 5-year follow-up period. disorder.

169 Results An existing GMA-system Background National Health model of human uric acid metabolism Insurance Research Database, derived was used. Lesh-Nyhan syndrome was from claims data of NHI, might defined in the model. The fuzzy provide an efficient source for optimization method was used to outcomes research in patients with normalize the concentration of uric acid. CKD. However, in the absence of In addition, the normalization of other laboratory data, one would need to metabolites in the model was also identify patients with CKD from considered with multiple objectives diagnosis codes associated with health optimization. The fuzzy optimization care claims. The validity of this method detected several potential approach to identify patients with CKD enzymes for treatment. The purpose of has not been sufficiently studied in this work is to provide a method to save Taiwan. time and cost of drugs development. Methods From stroke registry 187 database of a single medical center, we 在台灣健保申報資料中找出慢性腎臟 obtained the first serum creatinine 病人: 一個驗證研究 measurement of 1,317 elderly NHI 陳志弘 1, 楊高雅慧 2, 謝鎮陽 2,3 1 成大醫院腦中風中心暨神經部 beneficiaries upon hospitalization for 2 成大醫學院臨藥所 acute ischemic stroke and calculated 3 台南新樓醫院腦中風中心暨神經內科 each patient’s estimated glomerular

Identification of Patients with Chronic filtration rate (eGFR). We then Kidney Disease from National Health searched all other discharge diagnosis Insurance Claims Data in Taiwan: a in the claims data for the presence of a Validation Study Chih-Hung Chen1, Yea-Huei Kao Yang2, diagnosis code for diabetic Cheng-Yang Hsieh2,3 nephropathy, hypertensive nephropathy, 1Stroke Center and Department of Neurology, National Cheng Kung University Hospital chronic renal insufficiency, and 2 Institute of Clinical Pharmacy and miscellaneous other renal diseases. Pharmaceutical Sciences, National Cheng Kung University Using the gold standard of an eGFR 3 Stroke Center and Department of Neurology, Sin less than 60 mL/min/1.73 m2 for Lau Hospital, Tainan, Taiwan definition of CKD, we calculated the Objective To validate several sensitivity, specificity, and positive and claims-based approaches for the negative predictive values for each of identification of patients with chronic these diagnoses and combinations of kidney disease (CKD) by using diagnosis these diagnoses. codes from National Health Insurance (NHI) claims data in Taiwan. Results The sensitivity of individual diagnosis algorithms ranged from 1.6%

170 for diabetic nephropathy to 5.3% for 知義務和違反注意義務。 miscellaneous causes. Besides, the “miscellaneous” group had a comparable 方法以癲通為例，藉由最高法院刑 specificity (97.4%) with all other 事判決 101 年度台上字第 2637 號和 individual diagnosis algorithms (all 先前相關判決之結果，探討我國司法 >99%). Using combinations of these 對衛生署核准適應症外使用之法律 algorithms failed to improve the 演變，和建議醫界因應方向。 sensitivity. Positive predictive values generally were high (88.5% to 100.0%), 結果在本判決中，關於衛生署核准 but negative predictive values were low 適應症外使用，對醫療行為違反告知 (46.6% to 47.8%). 義務和違反注意義務之認知，已經產生重要的影響，預期將改變往後相關 Conclusion High positive predictive 之判決。本案件由 2002 年開始偵查， values indicate that NHI claims data can 至 2004 正式起訴，在各級法院中歷 be used to accurately identify patients 經多次上訴和重審。本案件被認為有 with CKD for study. However, the utility 過失是基於醫師並沒有對病人清楚 of such databases for comparison of 告知癲通之副作用，而不認為有過失 patients with CKD versus lesser degrees 是基於癲通之使用被認為符合合理 of CKD is limited. 使用且副作用無法注意。在本判決中，法官將告知義務和注意義務完全 188 切割，認為縱然違反告知義務，病人衛生署核准適應症外使用司法判決-由之死亡為是否違反注意義務，故過失癲通案件看法律演變和醫界因應必須基於往後是否有違反注意義務。陳偉熹 1 陳志銘 2 高雄長庚紀念醫院 1 神經內科 2高雄地方法院結論由本案件之判決結果，改變以 Jucidiary Decrees in Off-label Use - 往司法只重告知義務，卻忽略病人傷 Legal Change and Professional Response from Carbamazepine Cases 害之事實應為注意義務是否有違反 Wei-Hsi Chen1, Jhih-Ming Chen2 所致。醫界在往後之訴訟中，必須將 Department of Neurology1, Kaohsiung Chang Gung Memorial Hospital, and College of 重點放在違反注意義務之上，而非只 Medicine, Chang Gung University, Kaohsiung, 爭論是否有違反告知義務。 Taiwan Kaohsiung District Local Court2. Justice Yuan, Kaohsiung, Taiwan 189

以胸壁疼痛為初始表現之胸椎原發目的雖然在我國之法律條文中並沒性惡性黑細胞癌有將衛生署核准適應症外使用定義或陳瑋芬 1 周志和 2 列舉，然而衛生署核准適應症外使用產新樓醫院神經內科 1 奇美醫院神經內科生之傷害，被認為是侵害他人之人格 Chest wall pain as the initial 權，故可以循刑法或民法請求賠償。其 manifestation in a patient with primary 侵害他人之權利，主要可以分為違反告 melanoma in the thoracic spine

171 1 2 Wei fen Chen , Chih-Ho Chou improvement of motor weakness. 1 Department of neurology, SinLau Hospital The Presbyterian Church in Taiwan Subsequent radiotherapy was 2 Department of neurology, Chi Mei Medical administered. Center

Background Primary spinal Conclusion Image studies are melanomas, which predominately affect indicated for all cases with back pain the middle or lower thoracic spine, are and chest wall pain that is extremely rare. Melanoma occurs most unresponsive to a few weeks of commonly in white populations and is conservative treatment. Malignancy rare in Asian populations. must always be considered in the differential diagnosis. Case Report A 44 year-old male presented with left lower chest wall pain 190 for several months. He considered that 原發部位不明的轉移癌以右外展神 was contributed to a minor muscle strain 經麻痺為臨床表現:一病例報告 injury. Acute onset of lower abdominal 郭蔭庭亞東紀念醫院內科部神經內科 numbness and bilateral leg weakness has been noted for 10 days and intermittent Metastasis of unknown origin numbness over bilateral inguinal regions presented as right abducens nerve palsy: A Case Report on cough or exertion. The sphincter was Yam-Ting Kwok intact. Spinal magnetic resonance Division of Neurology, Department of Internal Medicine, Far Eastern Memorial Hospital imaging (MRI) revealed a space-occupying lesion at the T8-9 level. Background Abducens nerve palsy A standard posterior midline approach may be a presentation of metastasis or was used under the impression that the even paraneoplastic syndrome but is subacute subdural hematoma was caused rarely caused by unknown origin with by a hidden vascular anomaly or a rare negative neuroimaging findings and pigmented tumor. A dark black-colored CSF studies. and hard intradural mass was found, and gross removal was performed. This was Case Report A 56-year-old woman confirmed as a melanoma by with a past history of chronic headache immunohistochemistry. Dermatologic under control for more than 5 years has and ophthalmologic examinations did not suffered from progressive right reveal any other focus of primary temporal headache for two months and melanoma. Histopathological toothache and swelling on the right investigation confirmed malignant face for two weeks. Sudden onset of melanoma. No hypermetabolic lesions double vision was present at any time were noted on whole-body FDG-PET. as she was gazing to the right in recent The patient was discharged with

172 one week. There was no fever, recent was also arranged, Needle liver biopsy common cold or body weight change. revealed metastatic adenocarcinoma Neurological examination was essentially from lower gastrointestinal tract. normal with isocoric reactive pupils Therefore, under the impression of except impaired abduction of right eye in metastasis of unknown origin (MUO) favor of right abducens nerve palsy. CSF with multiple livers and spines studies including cell counts and involvement, the patient received cytology were normal. Laboratory data linear accelerator teletherapy. showed no evidence of diabetes mellitus or thyroid dysfunction but anemia, Conclusion Malignancy, especially thrombocytosis, elevated C-reactive associated with metastasis, should be protein, CEA, CA-199, and CA-125. always kept in mind in patient with CTA of head revealed no hyperdense sudden onset of focal neurological intracranial hemorrhagic events, but mild deficit. brain cortical atrophy with suspicious osteolytic lesion causing bony 191 destruction at C5 left pedicle and lamina 罕見肝癌轉移至海綿竇 in favor of bony metastases. Mild 曾為世宋岳峰國軍左營總醫院潛水醫學部, 三軍總醫院神 dilatation of distal BA but no aneuysmal 經內科 sac was detected in Willis circle. MRI of brain showed mild brain atrophy. No A rare case of cavernous sinus metatstasis from hepatocellular definite evidence of space-occupying carcinoma lesion or abnormal enhancement within Wei-Shih Tseng and Yueh-Feng Sung Department of Diving Medicine, Zuoying brain parenchyma. Whole body bone Armed Forces General Hospital, Kaohsiung, scan revealed degenerative change or Department of Neurology, Tri-Service General Hospital, Taipei. suspicious bone metastases in cervical spines and thoracolumbar spines with Background Metastasis to the right mastoiditis or otitis. Abdominal cavernous sinus (CS) from echogram revealed multiple hepatic hepatocellular carcinoma (HCC) is tumors, probably metastatic tumor. CT extremely rare. To our knowledge, there scan of chest and liver triphase CT scan are only four such cases reported in the showed multiple metastases at both lobes literature. We report a case of acute of liver and left aspect of lower C spines painful ophthalmoplegia (PO) of right and suspected bilateral lung metastases. abducens nerve secondary to cavernous PES showed gastritis. Colonoscopy sinus metastasis in a 48-year-old revealed colonic ulcers but no evidence Taiwanese male with HCC, which of malignancy. Mammographic finding progressed to total ophthalmoplegia of was negative. The consultation of dentist right eyeball movement rapidly.

173 Case Report A 48-year-old Taiwanese Metastatic Neuroendocrine tumor with initial presentation of Orbital man with hepatitis B virus (HBV)- Apex Syndrome related HCC presented to the Emergency Yen-Yu Huang, Wei-Chih Hsu Department (ED) with a 5-day history of Department of Neurology, Shin-Kong WHS Memorial Hospital gradual onset right-sided inner-orbital pain and double vision. After Background Neuroendocrine tumors hospitalization, steroid was administered are relative slow growing tumor, for suspected Tolosa-Hunt syndrome usually arise from gastrointestinal tract (THS). However, there was no or bronchopulmonary sources. We improvement of his PO after a two-day described a rare case of high grade course of steroid therapy. Magnetic neuroendocrine tumor of unknown resonance imaging (MRI) of his brain primary site with initial presentation of with contrast was performed and detected progressive ptosis and vision loss as a mass in the right CS. Metastasis to the orbital apex syndrome. CS from HCC was highly suspected. After discussion with the patient, Case Report A 64-year-old man was conservative and opioid therapy was previously healthy and denied any started for PO. Ten days after admission, regular medication. He was admitted the patient developed right total for progressive ptosis and insidious ophthalmoplegia with ptosis. He declined dull pain over left eye and temporal palliative radiotherapy treatment and region for 1 month. He also felt general received hospice care. malaise and poor appetite recently, and the body weight loss was 5 kilograms Conclusion Painful ophthalmoplegia within 1 month. There was no fever, involves various etiologic factors in a rhinorrhea, nose stuffy, tinnitus, wide spectrum, from inflammation to diplopia or diurnal change of ptosis. malignancies. Clinicians may wish to Neurologic examination showed left consider the potential in other ptosis, mild swelling palpebrae without malignancies except for the most erythematous change, sluggish light common primary sites of metastasis to reflex, and no EOM limitation or the CS in a patient without any history of visual acuity change on the day of cancer who presents with painful admission. The symptom progressed ophthalmoplegia. rapidly within 1 week that his left eye could only perceive light, and had 192 limitations in vertical gaze. Orbital 以眼眶尖端症候群為初始表現的轉移 apex syndrome was suspected by 性神經內分泌腫瘤 above neurological deficits. Brain MRI 黃彥瑜許維志台北新光吳火獅紀念醫院 showed a soft tissue lesion in

174 3 cavernous sinus with extension to orbital 高雄長庚紀念醫院出院準備服務中心 4 育英醫護管理專科學校老人服務事業管理科 apex, and enlarged left optic nerve. 5 國立臺南大學測驗統計研究所 Theoretically, high dose steroids may be 6 高雄長庚紀念醫院復健科 7 tried in this patient, who had no 國立陽明大學物理治療暨輔助科技學系 malignancy history. After discussion with Rasch Analysis on the Mandarin patient and family members, transnasal Version of Stroke Impact Scale: cavernous sinus biopsy showed Exploration of Prognosis of Stroke Patients Categorized by Initial high-grade malignant tumor with Severity neuroendocrine differentiation. PET Yu-Ching Huang1, Ku-Chou Chang1.2.3.4, Pi- Hsia Hung5, Jen-Wen Hung6, Hsuei-Chen Lee7. showed increased uptake at left orbital 1 Division of Cerebrovascular Diseases, area, spine, rib cage and liver. Department of Neurology, Kaohsiung Chang Gung Memorial Hospital, Kaohsiung, Echo-guided liver biopsy revealed the Taiwan; 2 same pathological characteristics. The Department of Neurology, College of Medicine, Chang Gung University, Taoyuan, Taiwan; primary site of tumor was unclear. 3 Discharge Planning Service Center, Kaohsiung Chang Gung Memorial Hospital, Kaohsiung, Taiwan; Conclusion Nonfunctional 4 Department of Senior Citizen Service Management, Yuh-Ing Junior College, neuroendocrine tumors with orbital Kaohsiung, Taiwan; 5 metastasis are rare, and the diagnosis was Program of Measurement and Statistics, Department of Education, National difficult in the absence of a previous University of Tainan, Taiwan; 6 cancer history. The pathologic evidence Department of Rehabilitation, College of Medicine, Chang Gung University, Taoyuan, is crucial for the diagnosis. Taiwan; 7 Department of Physical Therapy and Immunohistochemistry study may help in Assistive Technology, National Yang-Ming indentifying a neuroendocrine origin. University, Taipei, Taiwan

Therapeutic steroid trial has been Introduction Stroke is the leading regarded as the first line management in cause of adult disability and impacts patients with orbital apex mass of the quality of life due to changes in life unknown nature. However, this strategy style. This study developed a mandarin may delay the diagnosis of our patient. version of Stroke Impact Scale We recommend that tissue proof may be (M-SIS). Validation was done on the considered firstly if the risk is not high reliability, validity, and hierarchical and technique support is sufficient. order of items for the strength measure

and item map. M-SIS was applied in 193 stroke patients categorized by stroke 等距量尺中文腦中風衝擊量表之發展與應用：不同嚴重度腦中風預後復原趨 severity to explore the flux of quality 勢之比較 of life (QoL) at six months after stroke. 黃裕淨 1 張谷州 1.2.3.4 洪碧霞 5 洪禎雯 6 李雪楨 7 1 高雄長庚紀念醫院神經內科系腦血管科 Methods SIS 3.0 with 59 items in 8 2 長庚大學醫學系 domains was used as the prototype to

175 develop the M-SIS by pretests in 25 of QoL after stroke. The hierarchical stroke patients, including 10 mild stroke order of items for the strength measure patients, 10 moderate, and 5 severe and item map of M-SIS from this study stroke patients. M-SIS was applied in might be referenced as one of the 218 stroke patients, including 94 mild outcome measures after stroke. stroke patients, 64 moderate and 60 severe stroke patients. Rasch analysis 194 was performed to evaluate psychometric 單純的非創傷性外展神經麻痺 characteristics of the M-SIS with fit 楊自強童偉輝王馨範陳崇文連啟勳陳淑儀許弘毅 statistic and person or item separation 童綜合醫院神經內科 index. Descriptive analysis was used to evaluate the flux of QoL. Hierarchical Isolated nontraumatic abducens nerve palsy linear modeling was used to explore the Chi-Chiang Yang, Wai-Fai Tung, Hsin-Fan change of the slope of three evaluations: Wang, Chung-Wen Chen, Chi-Hsun Lien, Shu-Yi Chen, Hung-Yi Hsu 2 weeks within admission, 3 months and Department of Neurology Tungs’ Taichung 6 months after stroke. MetroHarbor Hospital

Background Abducens nerve palsy Results First measurement was done at (ANP) is the most common isolated 13.4 days after admission for with the palsy due to the long peripheral course following demographics: mean age 68 of the nerve. The abducens nucleus is years, 57.8% female, 88.1% education located in the caudal pons at the level level less than 6 years, 35.8% illiterate of the facial colliculus. In the vicinity with average Barthel index of 44.3. The of the abducens nerve nucleus are reliability and validity were fairly facial nerve loop and pontine gaze acceptable with internal consistency of center, so nuclear lesion of the coefficient 0.89. The physical contents of abducens nerve is extremely rare. M-SIS correlated with NIHSS by Abducens nerve exits the brainstem at coefficient 0.80 (p<0.05), while the the border of the pons and medullary correlation of psychological contents was pyramids. Upon exiting the brainstem lower than expected. As the it climbs superiorly along the ventral unidimensionality remained, no surface of the pons. On its way to the reduction from the 59 was done. Six cavernous sinus it passes at the apex of months after stroke, the improvement of the petrous portion of the temporal QoL of mild and moderate stroke patients bone. In the cavernous sinus it passes was better than severe stroke patients. medially to the internal carotid artery,

and laterally to oculomotor, trochlear Discussion Generally, M-SIS was a and 1st and 2nd branches of the valid and reliable tool for measurement trigeminal nerve to enter the orbit

176 through the superior orbital fissure. The was undetermined (26%), followed by injury of the abducens nerve can occur hypertension alone (19%), coexistent anywhere along this long course, so hypertension and diabetes (12%), differential diagnosis of ANP trauma (12%), multiple sclerosis (7%), occasionally demands thorough neoplasm (5%), diabetes alone (4%), investigation to find the proper cause. stroke (4%), post-neurosurgery (3%), aneurysm (2%) and others 8%. The Case Report Patient is a 69-year-old role of ESS for prolonged abducens man who complained of intermittent palsy resulting from isolated sphenoid headache without vomiting but with sinus disease (ISSD) is still unclear. double vision since a couple months ago. However, some published report The frequency and intensity of headache pointed out that ESS is a safe and increased gradually. There was no effective treatment for ISSD with apparent fever noted throughout the abducens palsy. course. Patient has a history of hypertension with treatment at a local 195 clinic. He has no history of diabetes 缺血性腦中風病人之家屬拒絕或接 mellitus. After admission, the 受血栓溶解治療:倫理和法律探討趙千淑林綉紅陳廷耀張谷州劉嘉為 neurological examination showed left 陳偉熹 abducens nerve palsy only. The magnetic 高雄長庚紀念醫院神經內科系暨腦中風中心 resonance imaging (MRI) with Accept of Refusal of Thrombolysis gadolinium showed abnormal signal by Family of Ischemic Stroke enhancement at the left sphenoid sinus in Patients: Ethical and Legal Issue Chien-Shu Chao, Hsiu-Hung Lin, Teng-Yeow T1- weighted and T2- weighted images. Tan, Ku-Chou Chang, Chia-Wei Liou, Wei-Hsi Endoscopic sinus surgery (ESS) was Chen Department of Neurology and Stroke Center, done by the Otolaryngologist. The Kaohsiung Chang Gung Memorial Hospital, pathologic diagnosis was chronic and College of Medicine, Chang Gung University, Kaohsiung, Taiwan paranasal sinusitis. The patient was discharged with improvement and stable Objective Thrombolysis is currently condition although the left abducens the golden standard for acute ischemic palsy improved only partially. stroke. In clinical practice, patients may not be able to express their own Conclusion The incidence of ANP is will due to neurological disability. 11.3/10000, with a peak in the seventh Family may take the responsibility for decade of life. Broadly, the causes of making decision. However, ethic and sixth nerve palsy have been studied legal issue arise in this situation as the before, namely in the population based autonomy of patient is neglected. study by Patel et al. The leading cause

177 2 Methods To review the ethical and 台北馬偕醫院精神科 3 國立台灣師範大學教育心理與輔導學系 legal issue for the autonomy of patient when family makes the decision for The Effects of Short Palliative accept or refusal of thrombolysis instead Course in Prevention of the Burnout among Medical Students. of patient oneself. Ming-Liang Lai1, Chun-Kai Fan2, Pei-Yi Lee3 1 Department of Neurology, National Cheng Kung University, Tainan Results Dispute has been encountered 2 Department of Psychiatry, Ma Kay General in both accept and refusal of Hospital, Taipei 3 Department of Educational Psychology, thrombolysis by family instead of patient. National Normal University, Taipei In cases who received thrombolysis, family might challenge the physician Background Burnout is frequently with an underestimation of risk evaluated encountered in the medical personnels, when adverse event occurred. On the especially those in hospice service. other hand, family argued for a failure of Previous studies revealed that 4% to administration of thrombolysis when 18% medical students admitted that neurological deterioration ensued they had difficulties in adjustment afterwards. Both patient and family during their learning period. Among would plead for malpractice in these them, after individual interview, 26% situation. need psychiatric supports. (Saslow 1956) At Taiwan, one study revealed Conclusion In Taiwan, even competent that suicide idea developed in 5.9% of elderly frequently hesitates for making medical students. (Lue et al 2006) The decision that is finally made by adult son purpose of this study is to evaluate the or daughter who is in scenario. The present status of burnout in medical principle of autonomy and the principle students and to find out whether a short of beneficence factually conflicts with term palliative course during the each other or custom in the real word. neurological rotation might helpful in Since the time window is narrow in the prevention of burnout in medical thrombolysis, it is impossible to follow students. the rule as in the Hospice and Palliative Care Act. Therefore, it is necessary for Methods The burnout status was engaging the inform-consent practice in evaluated by the MBI-HSS Score our society. proposed by the Maslach et al. The test has three subtests: exhaustion, 196 depersonalization and diminished 短期安寧教學於預防醫學生耗竭之 personal accomplishment. The 效果 intervention was a three-time course of 1 2 3 賴明亮方俊凱李佩怡 lecture and discussion given in four 1 成功大學附設醫院神經內科 weeks among the 6th grade medical

178 students: one hour with the topic of 197 introduction, one hour of legal aspects, 台灣的猝睡症盛行率以及發生率 and one hour of ethic consideration in 謝正芳徐崇堯高雄醫學大學附設中和紀念醫院神經內科 palliative medicine. Narcolepsy Prevalence and The sixth grade medical students in Incidence in Taiwan Cheng-Fang Hsieh, Chung-Yao Hsu academic year 2010 and 2011 at Medical Department of Neurology, Kaohsiung Medical School, National Cheng Kung University University Hospital, Kaohsiung, Taiwan joined this study. The project was Background There are no nationwide approved by medical center IRB and surveys of narcolepsy in prevalence every student signed the consent after and incidence in Taiwan. detail explanation.

Objective To investigate the Results The medical students in two prevalence and incidence of narcolepsy academic years did not differ in their in Taiwan score before the neurological rotation either in the total score or in the Methods We used National Health individual score of MBI. The results of Research Institutes (NHRI) database to all students were pooled to be compared investigate the prevalence and with the scores before intervention. There incidence of narcolepsy in Taiwan. We were no difference in individual scores defined patients to have probable and total score. However, when narcolepsy if they had a diagnosis of compared with the second year ( after narcolepsy (ICD-9CM code 347). We neurological rotation plus hospice defined patients to have definitive course), there was a significant narcolepsy if they had a diagnosis of improvement in individual score of narcolepsy and also applied for exhaustion (23±2.4 vs 25±2.3 p=0.030). Modafinil (Provigil®). Yet the other two individual scores and total score of MBI did not differ. Results Probable narcolepsy

prevalence and incidence increased Conclusion Short term course of from 2007 to 2009. The prevalence of hospice introduction during neurological probable narcolepsy was 0.18, 0.23 and rotation did improve the threshold of 0.38 per 10,000 persons per year in medical students to the prevention of 2007, 2008 and 2009 respectively. The burnout in the aspect of exhaustion. incidence of probable narcolepsy was Whether this result is related to many 1.21, 1.52, and 2.54 per 100,000 life-threatening disease in neurological persons per year in 2007, 2008 and clinics should wait for further evaluation. 2009 respectively. Definitive

narcolepsy prevalence increased from

179 2007 to 2009 but the incidence was 之參與程度，均值思考。茲以台北地區 nearly stationary. The prevalence of 醫糾刑事案件為素材，研究醫審會鑑定 definitive narcolepsy was 0.06, 0.09 and 報告與判決結果之相關性。 0.11 per 10,000 persons per year in 2007, 2008 and 2009 respectively. The 方法進入司法院網站法學資料檢 incidence of definitive narcolepsy was 索系統(網址：http://jirs.judicial.gov. 0.3, 0.3, and 0.2 per 100,000 persons per tw/Index.htm)，點選「查詢裁判書」， year in 2007, 2008 and 2009 respectively. 勾選「刑事」類別，時間限定為民國 88 年 1 月 1 日至 101 年 12 月 10 日， Conclusion Our survey showed that 以「醫事審議」為檢索詞，搜尋士林、 the prevalence of narcolepsy in Taiwan 台北及板橋等三地方法院之判決 was not as high as that in Hong-Kong 書，並排除以下條件之判決書：不受 Chinese. Underdiagnoses or geographical 理判決、判決理由無交代醫審會鑑定 difference may be the possible reasons. 報告內容者、案由欄非「業務過失傷害」或「業務過失致死」。其次，若 198 一判決中有兩名以上被告獲得不同醫療鑑定與醫療糾紛案件判決結果的判決結果，則當兩件計算，並分別檢相關性研究─台北經驗視醫審會分別鑑定各被告之報告結羅揚清 1 羅雪舫 2 林蓓珍 3 張景翔 4 1 台北榮民總醫院神經醫學中心神經內科專科果。最末，將有罪及無罪判決分別與醫師醫審會鑑定報告(分為「有疏失」、「無 2 法務部司法官訓練所學習司法官疏失或無因果關係」及「無法判斷或 3 立暘律師事務所執業律師 4 臺灣板橋地方法院法官僅提供醫療知識」三種)，以統計軟體SPSS 19 版作卡方檢定(Chi-square A Correlation Study on Medicine test)以檢視兩者是否相關。 Authentication and Medical Controversy - Taipei Experience Lo Yang-Ching, Lo Hsueh-Fang, Lin Pei-Chen, 結果整理得 94 件判決，列表如下： Chang Ching-Hsiang Neurological Institute, Taipei -Veterans General 醫審會鑑判決有罪判決無罪 Hospital; Judges and Prosecutors Training 定報告 (%) (%) Institute, Ministry of Justice; Li-Yang Law Office, Taiwan Banciao District Court 認有疏失 18 (58%) 14 (22%) 認無疏失 7 (23%) 47 (75%) 目標近年醫療糾紛事件頻傳，在媒體 /無因果渲染報導下，導致醫病關係緊張，亦使關係醫事人員對於法院所為醫糾案件判決結無法判斷 6 (19%) 2 (3%) 果有所質疑；然而，是否違反醫療常規 /僅提供及注意義務，法院係委託由醫事審議委醫療知識員會(以下簡稱醫審會)鑑定；醫審會之總件數 31 63 鑑定報告與法院判決結果是否相關，及醫審會審議及決定過程中，醫、病雙方 P<0.05，醫審會鑑定報告與法院判決

180 結果有相關。其中醫審會鑑定報告認無背景視覺障礙影響兒童多方面的疏失或無因果關係的部分，共有 4 件被發展，因此早期的視覺訓練是兒童視挑剔處置過程有瑕疵。覺復健領域重要的議題。然而目前對於視障兒童之視覺訓練仍少有具體結論醫審會鑑定報告為台北、士林、和有效的治療策略，使得這類兒童無板橋等三地院審理醫療糾紛之重要參法於視覺發展關鍵期獲得適當刺激。考。因此，在鑑定過程中，應考慮讓醫事人員作充分陳述，包含當下環境、人方法使用電腦化視覺訓練軟體，依力調度、資源供給、病患狀況或特殊體據視障兒童的視覺功能，調整棋盤狀質問題；又，經醫審會鑑定後，認醫方圖案的大小、閃爍頻率和對比，進行有疏失，法院非必然以刑責相繩，仍須 8 次、每週 2 次、每次 1 小時的視知由法院判斷該疏失與病患之損害，是否覺訓練。兒童需持續注視黑白棋盤，具法律上之相當因果關係。希冀本研究當其他有趣的視覺目標物出現時，需能促進醫療鑑定過程更加細緻化，建立按鍵做反應。同時會有聽覺回饋，以醫、法、病三方之溝通平台，並開創三增加兒童之注意力。療效評估工具包贏之未來。括 Lea Symbols Visual Acuity Test、 Tellar Visual Acuity Cards、功能性視 199 覺問卷和 256 高頻道腦波儀。以棋盤狀圖形於視覺障礙兒童進行知覺訓練的知覺可塑性之探討結果 6名(平均年齡 3.7 歲)視覺路徑蘇裕欽 1 蔡麗婷 2 徐榮隆 345 吳建德 2 1 慈濟綜合醫院台北院區神經內科前段或後段受損之視障兒童參與本 2 台灣大學職能治療所研究，其中 4 位完成完整腦波檢查。 3 新光醫院神經內科視障兒童在視覺訓練 4 週後的平均視 4 台灣大學醫學工程學研究所 5 臺北醫學大學醫學資訊研究所力，從 0.22 進步到 0.38 (p-value <0.05)。功能性視覺問卷顯示，兒童 Perceptual Plasticity of Checkered 訓練前後有功能上顯著的差異 Pattern Perceptual Learning Program in Children with Visual Impairment (p-value <0.05)。腦波檢查顯示，P100 1 2 345 Yuh-Chin Su , LI-Ting Tsai , Jung-Lung Hsu , 波形及延遲時間亦有明顯改善。根據 Chien-Te Wu2 1Department of Neurology, Buddhist Tzu Chi 此研究結果，以棋盤狀圖案為主的電 Hospital, Taipei. 腦化視覺訓練軟體，不論在行為及電 2School of Occupational Therapy, College of Medicine, National Taiwan University. 生理檢查上，均顯示可顯著改善視障 3Department of Neurology, Shin Kong Wu Ho-Su Memorial Hospital. 兒童的視覺功能。 4Institute of Biomedical Engineering, National Taiwan University. 5Graduate Institute of Biomedical Informatics, 200 Taipei Medical University, Taipei, Taiwan Wernicke 氏腦病變併中樞神經性過度換氣：一病例報告研究目的本研究以棋盤狀圖形為主蘇裕翔許永居要視覺刺激，探討視障兒童視覺知覺訓嘉義基督教醫院神經科練之療效。

181 Central neurogenic hyperventilation in showed neither pleocystosis nor Wernicke’s encephalopathy: A case increased protein. Brain MRI with report Yu-Hsiang Su, Yung-Chu Hsu gadolinium enhancement showed Division of Neurology, Department of Internal symmetric bilateral hyperintense Medicine, Chia-Yi Christian Hospital lesions at periaqueductal areas of Background Most reported cases of midbrain, dorsal pons, medulla and central neurogenic hyperventilation result mammillary bodies on T2WI and from pontine astrocytoma or lymphoma. FLAIR, which highly suggested We reported a case of Wernicke’s Wernicke’s encephalopathy. Five days encephalopathy presenting as central later, he received extubation with alert neurogenic hyperventilation. consciousness, and all his symptoms including dyspnea, diplopia, and ataxia Case Report A 54-year-old man came got completely recovered. to our emergent department due to acute onset of tachypnea, double vision, and Conclusion Our patient emphasizes generalized numbness after waking up. that Wernicke’s encephalopathy He used to drink daily for many years involving periaqueductal brainstem and his body mass index was only 18. He could also cause central neurogenic also had cirrhosis without any treatment. hyperventiation. He was clear, oriented, and afebrile. Neurological examination demonstrated 201 bilateral partial ptosis, symmetric pupil 數學學習能力障礙以 SPECT 檢測雙 size and intact light reflex, and there was 側顳葉灌流不足來表現林祐薇 1 蔡景仁 2 no obvious dysconjugation and 1 楊錦標神經科診所 nystagmus, but he subjectively 2 成功大學附設醫院神經科 complained of persistent bi-ocular Mathematical learning disability diplopia. There was no facial palsy, with mild bi-temporal hypoperfusion tongue deviation, or limbs weakness. His in SPECT study Yu-Wei Lin 1 Jin-Jen Tsai2 deep tendon reflexes were symmetrically 1Dr.Yang's Neurology Clinic increased. He also had bilateral dysmetria 2Department of Neurology, National Chen-Kung University Hospital and symmetric distal limbs paresthesia on pinprick. The initial blood gas showed Background Mathematical learning severe respiratory alkalosis. Because of disability is one of the learning persistent hyperventilation and disabilities of human beings. Previous respiratory alkalosis, he underwent studies showed that the causes are intubation. Nutritional supplement variable, from inherited to acquired including intravenous thiamine was soon type (ex. Gerstmann syndrome). We administered. Cerebrospinal fluid study here describe a patient with solitary

182 mathematical learning disability had mild other cognitive impairment. Deficits in bi-temporal hypoperfusion by SPECT “Auditory immediate recall”, study. “Working memory”, and “Speed of processing” contribute to math Case Report A 27-year old cognition disorders in this patient. postgraduate male student was suggested Excellent visual memory and drawing to visit neurology OPD by his teacher ability may be the compensation because of "poor performance in consequence mathematics". According to his mother and his teacher, the patient had "calculation disability" since childhood. He failed in almost all mathematic examination. His daily activities were normal, and he had no problems in learning other subjects. Actually, he got high score in biology, and was able to major in Immunology in NCKU. He was also good in learning drawing, and his teacher said he's excellent in using colors. We arranged series of PE, NE, serum biochemistry profiles, thyroid profiles, autoimmune profiles, CSF studies, and brain MRI studies, while all findings were normal. The neuropsychological assessment (including CASI, WAIS, WMS tests) suggested impairment in "processing speed", "auditory immediate recall", "auditory reception delayed score", and "working memory"; but excellent in "visual immediate recall" and "visual delayed memory". The HMPAO SPECT study revealed "relatively hypoperfusion over bilateral frontal and temporal areas".

Conclusion Bi-temporal lobes dysfunction can present as solitary mathematical learning disability without

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