Neurological Board Examination （I） 2015 09 19
A type: Select the one best answer to each question.
( ) 1. Which of the following description about neurological complications of lithium intoxication is FALSE?
A. Intoxication is not uncommon adverse effect in chronic lithium use, especially in young adult. B. Acute clinical symptoms in the intoxication are often diarrhea, vomiting, arrhythmia, hypotension and /or confusion. C. Tremor is the most common movement disorder associated with lithium intoxication. D. Orolingual dyskinesia could be one of uncommon presentation in lithium intoxication. E. The prognosis of lithium intoxication is benign after normalization of serum lithium level.
解答：A 題目之出處：Acta Neurologica Taiwanica 2013;22:32-35
題目屬性：Acta Neurologica Taiwanica
( ) 2. Which of the following PARK Locus genes will commonly present as juvenile-onset parkinsonism with atypical features (ex: dementia, pyramidal signs, dystonia or oculomotor palsy)?
A. Phospholipase A2, group VI (PLA2G6, PARK14) B. Leucine rich repeat kinase (LRRK2, PARK8) C. F-box only protein 7 (FBXO7, PARK15) D. Parkin (PARK2)
解答： B 題目之出處：Acta Neurol Taiwan. 2012 Sep;21(3):99-107. Review.
題目屬性： Acta Neurologica Taiwanica
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( ) 3. Which of the following effect may result from antiepileptic drugs interaction between valproic acid and lamotrigine?
A. Decreased lamotrigine efficacy due to induction of cytochrome (CYP) 2C9 B. Increased risk of weight gain C. Decreased valproate efficacy due to induction of CYP2C9 D. Valproate toxicity due to inhibition of its β-oxidation E. Lamotrigine toxicity due to inhibition of its glucuronidation
解答： E 題目之出處：2015 癲癇診療指引
題目屬性： Epilepsy, Acta Neurologica Taiwanica
( ) 4. Which of the following is NOT a risk factor of SUDEP (Sudden Unexpected or Unexplained Death in Epilepsy)?
A. Poor antiepileptic drugs adherence B. Male C. Drug resistant epilepsy D. Nocturnal seizure E. Late onset epilepsy
解答： E 題目之出處： 2015 癲癇診療指引
題目屬性：Epilepsy, Acta Neurologica Taiwanica
( ) 5. A healthy 67-year-old male suffers from severe pain in the head, consists of a severe and persistent unilateral sub-occipital headache with diagnosis of occipital condyle syndrome. Which cranial nerve will be involved?
A. Ipsilateral trigeminal nerve
第 2 頁，共 51 頁 B. Ipsilateral hypoglossal nerve C. Ipsilateral vagus nerve D. Contralateral accessory nerve E. Contralateral glossopharyngeal nerve
解答： (B) 題目之出處：Acta Neurologica Taiwanica, 2015;24:11-14
題目屬性： Acta Neurologica Taiwanica, Headache
( ) 6. Comparing with Caucasian populations, the CADASIL in Taiwan has several distinct features, EXCEPT:
A. Less frequent anterior temporal involvement B. Older age at symptom onset C. Higher incidence of intracerebral hemorrhage D. Rarer occurrence of migraine E. Higher prevalence of dementia
解答： (E) 題目之出處：Acta Neurologica Taiwanica, 2014;23:64-74
題目屬性： Acta Neurologica Taiwanica, Headache
( ) 7. In terms of spinal dural arteriovenous fistula, which of the followings is FALSE?
A. Commonly arise from the lower thoracic or upper lumbar levels
B. Female predominant
C. Peak age of presenation in the 5th or 6th decade of life
D. Transarterial angiography is the gold standard of diagnosis
E. Misdiagnosis or delayed diagnosis is not uncommon
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解答： B
題目之出處： Acta Neurol Taiwan. 2014 Mar;23(1):29-35
題目屬性： Pathology &Tumor & spinal disorder
( ) 8.What of the following about hemanigoblastoma (HGB) is FALSE?
A. HGB represents 1 to 2.5% of all brain tumors
B. HGB could be associated with Von Hippel Lindau Syndrome disease
C. HGB is benign, rapidly growing vascular tumors
D. The most common locations of HGB are the cerebellum and spinal cord
E. HGB occurs commonly in adults
解答： C
題目之出處： Acta Neurol Taiwan. 2012 Dec;21(4):176-9.
題目屬性： Pathology &Tumor & spinal disorder
( ) 9. What is the grading of glioma if pathological finding reveals well-differeniated fibrillary or
neoplastic astrocytes?
A. Pilocytic astrocytoma
B. Grade II Astrocytoma
C. Anaplastic astrocytoma
D. Glioblastoma multiforme
E. Ganglioglioma
第 4 頁，共 51 頁 解答： B
題目之出處： Merritt’s Textbook of Neurology 12th Edition , Chapter 61. Page 393.
題目屬性： Pathology &Tumor & spinal disorder
( ) 10. Which is the MOST common site of intracranial ependymoma?
A. Foramen of Monro
B. Trigone of lateral ventricle
C. Third Ventricle
D. 4th Ventricle
E. Septum pellucidum
解答： D
題目之出處： Merritt’s Textbook of Neurology 12th Edition , Chapter 61. Page 401
題目屬性： Pathology &Tumor & spinal disorder
( ) 11. What is the likely minimum level of prolactin for patients with prolactin-secreting
macroadenomas?
A. 50 ng/mL
B. 100 ng/mL
C. 200 ng/mL
D. 500 ng/mL
E. 1000 ng/mL
解答：C
第 5 頁，共 51 頁 題目之出處： Merritt’s Textbook of Neurology 12th Edition , Chapter 64. Page 4
題目屬性： Pathology &Tumor & spinal disorder
( ) 12. Which of the following is the MOST common embryonal CNS tumor?
A. Teratoma
B. Craniopharyngioma
C. Epidermoid
D. Chordoma
E. Ganglioglioma
解答： C
題目之出處： Merritt’s Textbook of Neurology 12th Edition , Chapter 65. Page 432-434.
題目屬性： Pathology &Tumor & spinal disorder
( ) 13. Which statement about “Susac syndrome” is FALSE?
A. It is thought to be an immune-mediated endotheliopathy of unknown etiology
B. It comprises a triad of encephalopathy, branch retinal artery occlusion (BRAO), and hearing
loss.
C. It typically begins in the third or fourth decade, and is male dominant.
D. Callosal lesions that occur with acute encephalopathy are centrally located and have a
“snowball” appearance.
E. Prednisone monotherapy, pulses of methylprednisolone, or a combination of corticosteroids
and IV immunoglobulin appear to be beneficial in some patients.
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解答： C
題目之出處： Merritt neurology 12th, Page 338-339
( ) 14. Which of the followings is NOT considered as a contraindication for r-TPA thrombolysis
in acute ischemic stroke?
A. Onset time > 3 hours
B. Platelet < 100,000 / mm3
C. Blood pressure: 184/90 mmHg
D. Receiving oral anticoagulation with INR:1.7
E. Acute myocardial infarction one week ago
解答： C
題目之出處：台灣腦中風學會 靜脈血栓溶解劑治療急性缺血性腦中風之一般準則
( ) 15. Which of the following antithromotic therapy has NOT been proved of value in cerebral ischemia?
A. Aspirin B. Clopidogrel C. Ticlopidine D. Warfarin in patient with nonvalvular heart disease E. Immediate-release dipyridamole and aspirin
解答： E 題目之出處：Bradley’s Neurology in Clinical Practice 6th p.1042
第 7 頁，共 51 頁 ( ) 16. A 65-year-old woman with HTN history suffered from acute speech impairment without
weakness, which totally recovered 2 hours later. Her BP was 156 /98 mmHg at that time.
According to the “ABCD2” score to assess her risk of stroke after TIA, which of the
followings is TRUE?
A. The 2-day stroke risk for this woman is 1 % B. The “ABCD2” score is 3 C. The “ABCD2” score is 4 D. The “ABCD2” score is 5 E. The “ABCD2” score is 6
解答： D 題目之出處：Merritt neurology 12th, Page 266-7
( ) 17. Which of the following about Fabry disease is FALSE?
A. Angiokeratomas in a predominantly bathing suit distribution
B. Limb pain with febrile illnesses in adult age
C. Polyneuropathy general develops in late childhood or early adolescence.
D. Hypohidrosis
E. Progressive and sudden hearing loss
解答：(B)
題目之出處：Merritt 12th, p.285
( ) 18. Which of the following subarachonoid hemorrhage is NOT caused by aneurysmal
rupture?
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A. Pituitary apoplexy
B. Cocaine and amphetamine use
C. Moyamoya disease
D. Sickle cell disease.
E. Antiphospholipid syndrome
解答：(E)
題目之出處：Merritt 12th, p309.
( ) 19. Which of the following statements is TRUE?
A. Mutation in COL4A1 gene is related with large vessel disease, but not related with small vessel disease. B. Patients with NOTCH3 gene mutation have multiple cortical infarction, migraine with aura, gait disturbance and psychotic symptoms. C. Young male patient with multiple microinfarcts in brain, retina, inner ear and kidney indicate Susac syndrome. D. Fabry disease is AD hereditary disease with α-galactosidase gene mutation. E. Marfan syndrome is AD hereditary diseases and relates with intracranial aneurysm and cervical artery dissection.
解答： (E) 題目之出處： Merritt`s Neurology. 12th Edition, p.281
( ) 20. Which of the following could be used for treatment of MELAS (Mitochondrial Encephalomyopathy, Lactic Acidosis, and Stroke-like Episodes)?
A. L-Arginine B. Carnitine
第 9 頁，共 51 頁 C. Folate D. Peritoneal dialysis E. Stem cell transplantation
解答： (A) 題目之出處：Neurology In Clinical Practice, 6th Edition, p.1486-1487
( ) 21. Which of the following about subarachnoid hemorrhage (SAH) is FALSE?
A. Severe SAH is typically associated with a surge in catecholamine level and sympathetic tone. B. Hyponatremia frequently occurs after SAH. C. Delayed hydrocephalus may develop 3 to 21 days after SAH. D. Vasospasm becomes maximal at 5 to 14 days, and gradually resolves over 2 to 4 weeks. E. Seizure attack at the onset of bleeding increased risk of late seizure.
解答： ( E ) 題目之出處：Merritt’s neurology, 12th edition, p308-315
( ) 22. Which statement about the spinal cord infarction is FALSE?
A. The most common location of spinal cord infarction is the mid- to low thoracic spine. B. Local mechanical vascular compression being the most common cause. C. Sensory loss, back pain at onset, urinary retention without weakness were the most common symptoms D. Systemic hypotension after resuscitation also could produce cord ischemia. E. Myelopathy associated with decompression sickness (also known as Caisson disease) results from circulating nitrogen bubbles that block small spinal arteries.
解答： ( C )
題目之出處：Neurology in Clinical Practice, 6th Edition, p.1096-1099
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( ) 23. Which of the followings is NOT one of the criteria of patient selection for post-acute care
(PAC) initiated by National Health Insurance since 2014?
A. Neurologic condition of acute stroke patient stabilized for more than 72 hours
B. Modified Rankin scale 3-5 C. Complications (Infection, stress ulcer bleeding) were under control D. Patient can sit for at least more than one hour E. ≦30 days after stroke onset
解答： B
題目之出處： National Health Insurance program 2014
( ) 24. Which of the followings is NOT accepted as criteria for Takayasu arteritis?
A. Age under 30 B. Diminished brachial artery pulse C. Subclavian artery bruit D. Blood pressure differences in the arms E. Claudication of arms or legs
解答： A
題目之出處： Merritt’s Neurology, 12th edition, p. 328
( ) 25. Which of the following AEDs has the longest half-life?
A. Topiramate B. Levetiracetam C. Zonisamide D. Pregabalin
第 11 頁，共 51 頁 E. Valproic acid
解答： C
題目之出處： 癲癇診療指引 2013 ,p51
( ) 26. Which of the following AEDs does NOT affect hormonal contraceptive agents?
A. Topiramate B. Levetiracetam C. Oxcarbazepine D. Phenytoin E. Carbamazepine
解答： B
題目之出處： 癲癇診療指引 2013 ,p28
( ) 27. Which of the following anti-coagulant agents will reduce its concentration when combined with carbamazepine?
A. Warfarin B. Apixaban C. Rivaroxaban D. Dabigatran E. All of above
解答： E
題目之出處： 中風後癲癇診療指引 2015 ,p28
( ) 28. Which of the following statement about the AED and its adverse effect is FALSE?
A. Phenytoin: Pathological fracture
第 12 頁，共 51 頁 B. Oxcarbazepine: Hyponatremia C. Valproate: polycystic ovary syndrome (PCOS) D. Gabapentin: Steven-Johnson syndrome E. Topiramate: Renal stone
解答： D
題目之出處： Merritt’s Neurology 12th ,p937
( ) 29. Which of the following AEDs does NOT aggravate myoclonic seizure?
A. Lamotrigine B. Pregabalin C. Oxcarbazepine D. Topiramate E. Tiagabine
解答： D
題目之出處： 癲癇診療指引 2015 ,p17; Handbook of epilepsy treatment, 3rd ; 2010
( ) 30. The following combinations of antiepileptic drugs have been recommended for refractory epilepsy EXCEPT?
A. Valproate plus lamotrigine or levetiracetam for partial and generalized seizure. B. Carbamazepine plus valproate for complex partial seizure. C. Carbamazepine plus lamotrigine for generalized epilepsy D. Levetiracetam plus lamotrigine or tiagabine for partial seizure. E. Topiramate plus lamotrigine or levitiracetam for numerous seizure types
解答：C
題目之出處：Principle of neurology, 9th edition, chapter 16, Table 16-8
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( ) 31. Which of the following monogenic epileptic disorder is NOT caused by ligand-gated channels mutation?
A. Juvenile myoclonic epilepsy. B. Autosomal dominant nocturnal frontal seizure. C. Episodic ataxia type 2 with spike-wave seizures D. Familial generalized and febrile seizures E. Glucose transporter-1 deficiency
解答：C
題目之出處：Principle of neurology, 10th edition, chapter 16, Table 16-3
( ) 32. Which of the following about status epilepticus (SE) is FALSE?
A. Status epilepticus is an emergent condition. B. Continuous clinical seizures for more than 5 minutes should be regarded as SE. C. Treatment of SE includes initial benzodiazepine followed by intravenous antiepileptic drugs and general anesthesia if failed to control seizure activities. D. Refractory status epilepticus means seizure activities persist after 24 hours of general anesthesia therapy. E. Epilepsy surgery or ketogenic diet can be considered for super-refractory status epilepticus.
解答： D
題目之出處：台灣癲癇醫學會癲癇治療指引 2015
( ) 33. Which of the following about pregnancy in epilepsy is FASLE?
A. The seizure control remains unchanged in more than 80% of women with epilepsy B. Breast-feeding is not recommended for woman who takes antiepileptic drugs due to their secretion in the breast milk.
第 14 頁，共 51 頁 C. Valproate has teratogenicity and may cause low intelligence of the newborn. D. Folic acid supplementation is recommended before conception. E. Monotherapy could reduce the risk of teratogenicity compared to polytherapy.
解答： B
題目之出處： Merritt’s 12th edition, p.941 &台灣癲癇醫學會癲癇治療指引 2015
( ) 34. Which of the following is NOT the feature of simple febrile seizure?
A. Generalized convulsions B. Only once within 24 hours C. Last more than 10-15 min. D. Without Todd paresis E. Without focal sign
解答： C
題目之出處：Merritt’s Neurology, 12th edition, p. 949
( ) 35. Benign childhood epilepsy with central-midtemporal spikes accounts for 15% of all pediatric seizure disorders. About EEG finding, which one is FALSE?
A. Stereotyped diphasic or triphasic sharp waves B. Over rolandic regions C. Never generalized spike-wave activity occurs D. Increase in abundance during sleep E. Unilateral or bilateral spikes
解答： C
題目之出處：Merritt’s Neurology, 12th edition, p. 93
第 15 頁，共 51 頁 ( ) 36. Which of the following about epilepsia partialis continua is TRUE?
A. A special type of focal motor epilepsy characterized by persistent rhythmic tonic-clonic movements of part or all of one side of the body B. Metabolic problem could be one of the etiologies, especially hyperglycemia C. The underlying cause is always symptomatic D. A common etiology in adult is Rasmussen encephalitis E. EEG may not reveal epileptiform discharges
解答： B
題目之出處：Merritt’s Neurology, 12th edition, p. 930
( ) 37. Median sensory nerve conduction study showed deceased nerve action potential amplitude and prolonged distal latency. Which disease is NOT consistent with above data?
A. Carpal tunnel syndrome B. Lower trunk of brachial plexus lesion C. Hereditary neuropathy with liability to pressure palsy D. DM polyneuropathy E. Charcot Marie Tooth Disease
解答： B
題目之出處：Kimura J. Electrodiagnosis in diseases of nerve and muscle 2001: New York: Oxford
University 3rd edition
( ) 38. Needle EMG revealed a few of fibrillation during examination of biceps muscle. Which disease is NOT consistent with above EMG finding?
A. Polymyositis B. Musculocutaneous nerve lesion C. C5/C6 radiculopathy
第 16 頁，共 51 頁 D. Motor neuron disease E. Posterior cord of brachial plexus lesion
解答： E
題目之出處：Kimura J. Electrodiagnosis in diseases of nerve and muscle 2001: New York: Oxford
University 3rd edition
( ) 39. Which of the following differentiates acquired demyelinating neuropathy from hereditary motor and sensory neuropathy (Charcot-Marie-Tooth disease)?
A. Prolonged ulnar distal motor latency B. Symmetric slowing of conduction velocity C. Prolonged tibital F wave minimal latency D. Partial median nerve conduction block in the forearm E. Low amplitude fibular (peroneal) compound muscle action potential
解答： D
題目之出處：Kimura J. Electrodiagnosis in diseases of nerve and muscle 2001: New York: Oxford
University 3rd edition
( ) 40. A 76-year-old man comes to the emergency department because of an acute change in speech, binocular double vision, and left-sided extremity weakness. On examination, the patient has weakness of the right facial weakness and a right abductor pasly on extraocular muscle testing. He has a left-sided hemiparesis and Babinski sign. The rest of the neurologic examination is unremarkable. Which of the following is MOST likely?
A. Millard-Gubler syndrome B. Foville’s syndrome C. Claude’s syndrome D. Gradenigo’ syndrome E. Wallenberg syndrome
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解答： A
題目之出處：Neurology in Clinical Practice 5th edition P2063
( ) 41. Which electrodiagnostic feature on needle EMG is suggestive of a chronic neurogenic injury?
A. Myokymia discharge B. Early recruitment C. Motor unit potentials with long duration and high amplitude D. Neuromyotonia discharge E. Positive sharp waves and/or fibrillation potentials
解答： C
題目之出處：Merritt’s textbook of neurology 12th edition P.86
( ) 42. The commonly evaluated parameters of brainstem auditory evoked potentials (BAEPs) include all of the following EXCEPT:
A. I – III interpeak latency B. III–V interpeak latency C. I–V interpeak latency D. Wave III amplitude E. Wave I latency
解答： D
題目之出處：Neurology in Clinical Practice 5th edition P474
( ) 43. Mutism with gait disturbance is associated with lesions located in?
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A. Medial frontal lobe B. Lateral frontal lobe C. Cingulate D. Parietal lobe E. Temporal lobe
解答： A
題目之出處：Merritt's textbook of neurology 12th edition P.271
( ) 44. A 30-year-old woman was admitted for ascending weakness and numbness in her feet for 36 hours. She also reports severe back pain. On examination, she has distal and proximal weakness, diffuse areflexia, and distal sensory loss. Which of the following electrodiagnostic findings is MOST likely to be abnormal in this patient?
A. Sural sensory response B. Peroneal motor response C. F wave D. Blink responses E. Needle EMG
解答：C
題目之出處：Neurology in Clinical Practice 5th edition P2288etic stimulation: Report of an IFCN
committee. Clin Neurophysiol 2012;123:858-882.
( ) 45. Which description about insertional activity on EMG is FALSE?
A. Endplate spike is irregular firing pattern on EMG and generated from muscle fiber B. Myotonia has the characteristic of waxing and waning on EMG. C. Myokymia is bursting firing pattern on EMG and generated from motor neuron or axon. D. Fasciculation is regular firing pattern on EMG and generated from motor unit.
第 19 頁，共 51 頁 E. Neuromyotonia is waning firing pattern on EMG and generated from motor unit.
解答： (D)
題目之出處：Neurology in Clinical Practice, 6th Edition, p.406 (Table 32B.1)
( ) 46. Which of the following reflexes may be present in brainstem death?
A. Ciliopinal reflex B. Pupillary light reflex C. Oculocephalic reflex D. Corneal reflex E. Cough reflex
解答： A 出處：Merritt’s 12th Ed, p.28-29
( ) 47. Which neurological examination is LESS indicated when vestibular dysfunction is suspected?
A. Barany’s past-pointing test B. Head thrust test C. Caloric testing D. Fukuda stepping test E. Postural reflex
解答： E
題目之出處： DeJong’s The Neurological Examination, 7th edition, p.290
第 20 頁，共 51 頁 ( ) 48. Which of the following about CSF analysis is TRUE?
A. Bilirubin in CSF persist for 1 week B. High CSF protein could be caused by CSF leak related to traumatic dural fistula. C. CSF glucose level may be low in patients with carcinomatous meningitis D. The normal IgG index is less than about 0.5. E. Xanthochromia always suggest subarachnoid hemorrhage for 1 week or longer.
解答： C 題目之出處：Merritt’s Neurology (12th ed.) p112-114 題目難易：中
考古
( ) 49. Which of the following about chorea is FALSE?
A. Sydenham chorea is often an autoimmune disorder in consequence of infection with group A β–hemolytic streptococcus. B. In Huntington disease, the numbers of extra copies of trinucleotide repeats of CAG is not associated with the prevalence and severity of disease. C. Huntington disease is the most common cause of hereditary chorea. D. Systemic autoimmune disease such as systemic lupus erythematosus and antiphospholipid syndrome may cause chorea. E. All of above are true.
解答： B
題目之出處：Merritt’s Neurology, 12th edition; chap.117; P.727-730
( ) 50. A 12-year-old boy suffered from writer’s cramp since his age of 10 and both postural and action tremors were noticed. Unfortunately, torticollis and trunk dystonia developed gradually. About this boy, what of the following statement is TRUE?
A. Dystonic tremor in arm is uncommon.
第 21 頁，共 51 頁 B. The most possible diagnosis is an autosomal recessive disorder with GAG deletion in exon 5 on chromosome 9. C. The first symptom in most patients is torticollis. D. There may be great intra-familial variability, ranging from no dystonia to severe generalized dystonia. E. None of above
解答： D
題目之出處：Merritt’s Neurology, 12th edition; chap.120; P.739-741
( ) 51. Which of the following about dystonia is FALSE?
A. Onset of dystonia in a handis the most important predictive factor for a more rapidly progressive course. B. Sensory trick (geste antagoniste) is a characteristic and almost unique feature for dystonia. C. Cervical dystonia is the most common focal dystonia, followed by dystonias of cranial muscles such as blepharospasm, spasmodic dysphonia, or oromadibular dystonia. D. Dopa-responsive dystonia responds well to levodopa, without the development of long-term motor fluctuations. E. Anticholinergics, baclofen, benzodiazepine, and antidopaminergics have been reported to be effective in dystonia.
解答： A
題目之出處：Merritt’s 12th edition p.738-7486
( ) 52. Which statement about essential tremor (ET) is FALSE?
A. The tremor is a postural and kinetic tremor with an intentional component. B. Some ET patients have tremor at rest. C. ET is a chronic, non-progressive neurologic disease. D. Some ET patients have mild cognitive impairment, or impaired olfaction and hearing.
第 22 頁，共 51 頁 E. PET and MRS studies and neuropathologic examination suggest that dysfunction of cerebellum and its outflow tracts may be the pathogenesis of ET.
解答： C
題目之出處：From Merritt's Neurology, 12th Edition, 2010; Chapter 121 - Essential tremor
( ) 53. About Braak’s staging system of Parkinson’s disease (PD), which of the following statement is FALSE?
A. In the initial stage, Lewy pathology is observed in dorsal motor nucleus of vagal nerve B. Sympathetic and parasympathetic projection neurons are also involved in the early stages (stage 1) C. In stage 3, Lewy pathology involves midbrain tegmentum and basal forebrain, and Lewy pathology of substantia nigra also begins in stage 3 D. Anterior olfactory structures involvement in Lewy pathology is often seen to begin in stages 4 E. Neocortical involvement of Lewy pathology usually begins in stage 5 and 6
解答： D
題目之出處：Parkinson’s disease, non motor and non dopaminergic features, 2011, pp16-26
( ) 54. A 17-year-old girl developed paroxysmal chorea (dyskinesia) and myoclonus of her limbs, neck and face 8 years ago. Dyskinesias are often exacerbated by anxiety. Facial twitching (myokymia) of the periorbital and perioral muscles was also present. Her intelligence and cognitive function were intact. Her family members also have similar clinical features in an autosomal dominant pattern. Which of the following is MOST likely?
A. Huntington’s disease B. ADCY5-related dyskinesia C. Wilson’s disease D. Benign familial chorea E. Sialidosis type I
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解答： B 題目之出處： Basic Movement Disorder CME course 2 August 2015, Taipei
( ) 55. About paroxysmal movement disorders, which of the following is FALSE?
A. There are three main types of paroxysmal dyskinesia, all of which consist of bouts of any combination of dystonic postures, chorea, athetosis and ballism. B. Paroxysmal nonkinesigenic dyskinesia may be predisposed by ethanol or caffeine consumption. C. The duration of each attack in paroxysmal kinesigenic dyskinesia usually lasts seconds, and always within 5 minutes. D. There is never alteration of consciousness in the attack of paroxysmal movement disorders. E. All paroxysmal movement disorders respond well to anticonvulsants.
解答： E
題目之出處：Merritt’s Neurology, 12th edition; chap.124; P.775-777
( ) 56. A 65-year-old woman presents with progressive clumsiness of left hand. Which of the following features would MOST likely suggest corticobasal degeneration?
A. Hummingbird sign on brain MRI B. Sustained response to levodopa C. 4-Hz resting tremor D. Marked slowing of vertical saccades E. Asymmetric cortical sensory loss
解答： E
題目之出處： Merritt’s Neurology, 12 ed. P. 773
第 24 頁，共 51 頁 ( ) 57. An 80-year-old Taiwanese female with treated NIDDM presents with acute onset of hemichorea-hemiballism in her right limbs. There was no contributory family history. Her T1-weighted MRI showed a hyperintensity lesion at the left striatum. Which of the following acquired cause is MOST likely?
A. Brain tumor B. Cerebral hemorrhage C. Cerebral tuberculosis D. Non-ketotic hyperglycemia E. Syndenham’s chorea
解答： D
題目之出處： Merritt’s Neurology 12th edition, 2010 Chapter 117, P729
( ) 58. Which of the following is NOT characteristic of Dopa-responsive dystonia?
A. Female predominance B. Prominent diurnal fluctation C. Motor fluctuation and dyskinesia after chronic L-dopa therapy D. 18F-DOPA PET is normal E. Childhood-onset foot dystonia
解答： C 題目難易：易
考古
( ) 59. Which one is NOT the “Red Flags” in the diagnosis of idiopathic Parkinson’s disease? (ps.“Red Flags” means seldom manifest at the early stage of Parkinson’s disease)
A. Inspiratory stridor B. Falls within the first year C. Symmetrical signs
第 25 頁，共 51 頁 D. Wheelchair dependence within 5 years E. No resting tremor
解答： E
題目之出處：Neurology in clinical practice, 5th edition, Page 2088
( ) 60. Which of the following about multiple system atrophy is FALSE?
A. Laryngeal stridor, caused by paresis of vocal cords, may be found. B. Facial dystonia commonly develops after low-dosage L-dopa therapy. C. Orthostatic hypotension is a major disabling symptom. D. A common pathologic feature is the presence of widespread glial cytoplasmic inclusions in oligodendroglia. E. Lewy bodies and neurofirillary tangles are frequently found in the zona compacta of the substantia nigra.
解答：E 題目之出處： Merritt's Neurology, 12th Edition, Chapter 123 Parkinson-plus syndromes 題目難易：中
考古
( ) 61. Which of the following is the MOST common electrolyte imbalance in critically ill neurological patients?
A. Hypercalcemia B. Hypokalemia C. Hyperkalemia D. Hyponatremia. E. Hypocalcemia
解答：(D)
第 26 頁，共 51 頁 題目之出處：NICP 6th, p815
( ) 62. About central fever in critically ill neurological patients, which is FALSE?
A. It occurs most frequently in patients with SAH and is associated with increased risk of vasospasm B. Patients with central fever often have spikes of fever rather than prolonged hyperthermia C. Among patients with traumatic brain injury, high fever may be accompanied by paroxysmal sympathetic activity D. Mechanical cooling methods must be added if refractory hyperthermia E. Endovascular cooling devices may offer great control but require placement of central venous catheter
解答：(B)
題目之出處：NICP 6th, p818
( ) 63. Which of the following about Hashimoto encephalopathy is FALSE?
A. The clinical manifestation could be stroke-like episodes, dementia, psychiatric symptom or seizure B. CSF protein level and cell count are often normal C. ANA is usually abnormal D. Antithyroid peroxidase and antithyroglobulin level are elevated E. In general, patients respond to steroid therapy.
解答： C 題目之出處：Merritt’s Neurology (12th ed.) p.1051-1052
考古
( ) 64. Raise the blood PaCO2 will cause the followings, EXCEPT:
第 27 頁，共 51 頁
A. Decrease the pH of the CSF B. Cerebral vasodilator C. Intracranial hypertension D. Increase in cerebral blood flow E. Treatment of acutely raised ICP
解答： E
題目之出處：Adams and Victor’s PRINCIPLES OF NEUROLOGY 9th ed. P 593
( ) 65. Which of the following statement about tetanus is FALSE?
A. When clostridium tetani are introduced into a wound, they are converted into vegetative forms, which produce the exotoxin tetanospasmin. B. In developing countries, tetanus is still a common disease. The mortality is higher among young patients. C. The toxin interferes with the function of the reflex arc by the blockade of inhibitory transmitters, mainly GABA, at presynaptic sites in the spinal cord and brainstem. D. The toxin could act directly on skeletal muscle. E. The tetanus toxin is a zinc-dependent protease.
解答： B
題目之出處：Principles of Neurology 9th Edition, p1160~1161
( ) 66. Which of the following about botulism is TRUE?
A. There are three types of botulinum toxins—A, B, and E. B. Food-borne illness caused by the endotoxin of Clostridium botulinum. C. The primary site of action of toxin is at a neuromuscular junction, more specifically on the postsynaptic membrane. D. Ciliary body paralysis has the feature of the opposite of the Argyll Robertson reaction. E. Consciousness disturbance is the early clinical presentation.
第 28 頁，共 51 頁
解答： A
題目之出處：Principles of Neurology 9th Edition, p1162~1163
( ) 67. Which of the following about hepatic encephalopathy is FALSE?
A. The severity of the encephalopathy can be classified into four stages: mild confusion, lethargy, somnolence, and coma. B. An increase in large Alzheimer type II astrocytes. C. MRI usually shows increased signal in the globus pallidus in T1-weighted studies. D. Decerebrate and decorticate postures and diffuse spasticity of the limbs frequently accompany early stage of the encephalopathy. E. Ammonia and facilitation of GABA-mediated neurotransmission are considered the two most important factors.
解答： D
題目之出處：Merritt’s Textbook of Neurology 12th Edition 2010, p1011~1013
( ) 68. Which of the following is NOT symptoms/signs of opioid poisoning?
A. Hyperthermia B. Cyanotic and skin and mucous membranes C. Bradycardia D. Periodic breathing E. Pin-point pupils
解答： A
題目之出處：Adams and Victor's principles of Neurology, 9th p.11475
( ) 69. Which of the following about neuroleptic malignant syndrome is FALSE?
第 29 頁，共 51 頁
A. May be caused by phenothiazines B. Can be treated with bromocriptine C. The syndrome consists of hyperthermia, rigidity, stupor, unstable blood pressure, diaphoresis and high serum creatine kinase (CK) values. D. It may occur days, weeks or months after neuroleptic treatment is begun. E. A genetic factor may underlie most of cases of the neuroleptic malignant syndrome (in the D2 receptor gene) possibly provoked by fatigue and dehydration.
解答： E
題目之出處： Adams and Victor's principles of Neurology, 9th p.1155-1156
( ) 70. Which of the following is NOT associated with reversible posterior leukoencephalopathy?
A. Severe hypertension B. Hydrocortisone C. Methotrexate D. Interferon E. Intravenous immunoglobulin
解答： B
題目之出處： Adams and Victor's principles of Neurology, 9th p.1173
( ) 71. Which of the following about manifestations of alcohol withdrawal syndrome is FALSE?
A. Tremulousness is the most common B. Hallucinations may include purely visual in type, mixed visual and auditory, tactile, or olfactory C. Withdrawal seizures occur during the 7- to 48-h period following the cessation of drinking,
第 30 頁，共 51 頁 with a peak incidence between 13 and 24 h D. Pinpoint pupils E. Psychomotor overactivity
解答： D 題目之出處 Adams and Victor's principles of Neurology, 9th p.1135, 11372
( ) 72. Which of the following about prion disease is FALSE?
A. Human prion proteinaceous (PrP) is encoded by a gene currently designated PRNP on the short arm of chromosome 20. B. Gerstmann-Sträussler-Scheinker Syndrome inherited as an autosomal dominant trait. C. Kuru has gradually disappeared, apparently because of the cessation of ritual cannibalism. D. An immunoassay for the 14-3-3 protein was positive in 50 % of Creutzfeldt-Jakob disease patients E. CSF tau protein may also be increased, especially later in the disease.
解答： D
考古
( ) 73. Which one is NOT belong to the lower motor neuron syndrome?
A. Adrenomyeloneuropathy B. Poliomyelitis C. Kennedy disease D. Multifocal motor neuropathy with conduction block E. Hirayama disease
解答： A
題目之出處： Bradley’s neurology in clinical practice 6th ed. P1861
第 31 頁，共 51 頁 ( ) 74. A 55-year-old female presents with limb-girdle pattern of progressive weakness, winging of the scapula, and orthopnea for the last 5 years. Needle electromyography of the paraspinal muscles reveals myotonic potentials. The next most appropriate step is to perform which test:
A. Forearm exercise test B. Single fiber electromyography C. Assay of alpha-glucosidase enzyme activity D. Mutation analysis of glycogen brancher enzyme E. A trial of corticosteroids
解答： C
Re: American Association of Neuromuscular & Electrodiagnostic Medicine. Diagnostic criteria for
late-onset (childhood and adult) Pompe disease. Muscle Nerve 2009;40:149-160.
( ) 75. Which of the following is TRUE regarding the neuropathy associated with antibodies to myelin-associated glycoprotein?
A. Patients usually have an immunoglobulin G monoclonal gammopathy B. The treatment of choice is intravenous corticosteroids C. Clinical manifestations include sensory ataxia and tremor D. Nerve conduction studies show depressed compound muscle action potential amplitudes with relatively normal motor latencies and conduction velocities E. Cranial nerves are preferentially affected
解答： C
Re: Dyck PJ, Thomas PK. Peripheral neuropathy, 4th edition. Philadelphia: Elsevier Saunders;
2005. P596-597, 2187-2188
( ) 76. Which of the following membrane ion channel disorders is related to Isaac’s syndrome?
第 32 頁，共 51 頁 A. Chloride ion channel B. Calcium ion channel C. Potassium ion channel D. Sodium ion channel E. GABA receptor
解答： C
題目之出處： 2015 summer NMD conference
( ) 77. Which of the following is NOT an Anti-Hu antibody related neuromuscular disorders?
A. Sensory neuronopathy B. Autonomic neuropathy C. Sensorimotor polyneuropathy D. Neuromyotonia E. Lambert-Eaten syndrome
解答：D
題目之出處：2015 summer NMD conference
( ) 78. Which statement about amyloid neuropathy with transthyretin mutations is TRUE?
A. Amyloid (TTR) formation and deposition is found in peripheral nerve, kidney, eye and leptomeninges B. Usually cause progressive cranial neuropathies C. Autonomic function is usually preserved. D. Usually spared heart involvement E. Mainly autosomal recessive inheritance
解答：(A) 題目之出處：Neurology in clinical practice, 6th ed., P.1948-1949
第 33 頁，共 51 頁
( ) 79. Which of the following regarding the symptoms of Refsum disease is FALSE?
A. Ichthyosis B. Overriding toes C. Retinitis pigmentosa D. Axonal polyneuropathy E. Alopecia
解答：(E) 題目之出處：Neurology in clinical practice, 6th ed., P. 1954
( ) 80. Distal myopathies are heritable diseases with features of a predominantly distal pattern weakness. Which of the following statements is FALSE?
A. Welander myopathy is the most common form with AD inheritance. B. Udds myopathy is an AD myopathy is a vacuolar myopathy with anterior compartment predominantly involved. C. Miyoshi myopathy is a vacuolar myopathy with gastrocnemius spared. D. Nonaka myopathy shares the same GNE mutation with hIBM. E. Laing myopathy is an AD myopathy, characterized by weakness of the anterior tibial muscle groups and the neck flexors.
解答：(C)
題目之出處：Merritt's Neurology, 12th Ed p.873-874; Neurology in Clinical Practice, 6th Ed
p.2085
( ) 81. Which of the following about Kennedy disease is FALSE?
A. Limb weakness is usually more severe proximally B. Tendon reflexes are usually lost C. It is an X-linked recessive disease
第 34 頁，共 51 頁 D. Sensory nerve conduction studies are usually normal E. The mutation is an expansion of a CAG repeat
解答：(D)
題目之出處、：Merritt's Neurology, 12th Ed p802
( ) 82. Which of the following about McArdle disease is FALSE?
A. Myophosphorylase deficiency, Glycogen storage disease type V B. EMG silence during ischemic contraction C. Measuring the ammonia concentrations during exercise forearm test ensure the patient was sufficiently exercising. D. The measured lactate and ammonia level of exercise forearm test was elevated compared with baseline E. May develop second-wind phenomenon
解答：(D)
題目之出處：Neurology in Clinical Practice, 6th Ed p. 2093-94
( ) 83. Which of the following about spinal muscular atrophies is FALSE?
A. Werdnig-Hoffmann syndrome (infantile SMA type I) is evident always before age 6 months B. SMA type II patients have clinical onset from 6 months to 1 year and learn to sit but never walk C. Distal muscles are affected before proximal muscles, but ultimately flaccid quadriplegia results in Werdnig-Hoffmann syndrome D. The onset of Kugelberg-Welander syndrome is followed by proximal arm weakness and wasting, and fasciculation of limb muscles E. Three SMA types all are autosomal recessive
解答：(C)
第 35 頁，共 51 頁 題目之出處：Merritt’s Neurology, 12th Ed p.810
( ) 84. Which of the following about critical illness myopathy is FALSE?
A. Patient may have been exposed to high doses of corticosteroids for a brief period. B. Use of neuromuscular blocking agents appears to play a important role in the genesis of this myopathy C. Serum CPK was elevated. D. EMG revealed myopathy pattern; there are fibrillations as well E. Muscle biopsy disclosed inflammatory changes affecting mainly type 2 fibers
解答：(E)
題目之出處：Adams and Victor’s Principles of Neurology p.1453
( ) 85. Which of the following is LEAST likely the differential diagnosis of spastic paraplegia in middle aged adults?
A. Multiple sclerosis B. Amyotrophic lateral sclerosis C. Hereditary spastic paraplegia D. Kennedy disease E. Adrenoleukodystrophy
解答：D
考古
( ) 86. Which of the following about acute disseminated encephalomyelitis (ADEM) is FALSE?
A. It is an acute inflammatory and demyelinating disease B. Demyelinating course in brain white matter, brain stem, spinal cord and peripheral nerves
第 36 頁，共 51 頁 C. Common involve children follows a febrile illness by days or weeks D. Initial few days with onset of the exanthema of measles, rubella, smallpox, or chickenpox. E. May also be noted in post vaccine injection state (rabies vaccine)
解答： B
考古
( ) 87. Which of the following about the diagnostic test of multiple sclerosis (MS) is TRUE?
A. The pleocytosis found in CSF study is not correlated with the disease activity. B. The necrotic myelopathy and Devic disease variants are usually lack of oligoclonal band in CSF. C. Dawson fingers, which are oval or linear regions of demyelination, oriented parallel to the ventricle, are the characteristic brain MRI findings in typical MS. D. Gadolinium enhancing lesion on the brain MRI usually last for 6 to 8 months following the development of the acute lesion. E. Regarding the evoked potential studies (e.g., SSEP, BAEP, VEP) in MS patient, SSEP has the highest abnormal response rate.
解答： B
考古
( ) 88. Which of the following about treatment of multiple sclerosis is FALSE?
A. The therapeutic effects of interferon may be due to limit trafficking of T lymphocytes into the CNS. B. The beneficial effects of glatiramer acetate may result from reactive Th1 cells that cross the blood-brain barrier and downregulate inflammatory activity within the CNS. C. Natalizumab is a humanized monoclonal antibody that seems to be more effective in relapsing-remitting MS D. Periodic pulses of IV methylprednisolone are not effective in preventing disability in patients with secondary progressive MS
第 37 頁，共 51 頁 E. Mitoxantrone is an anthracendione and an effective therapy for secondary progressive MS
解答： B
考古
( ) 89. Which of the following about neuromyelitis optica (NMO) and multiple sclerosis (MS) is FALSE?
A. NMO has higher age at onset B. NMO has female predominance C. MS has marked pleocytosis and neutrophilia in cerebrospinal fluid (CSF) D. NMO has longitudinally extensive (≥3 vertebral segments) cord lesions E. Of NMO patients, 10–20% has monophasic course
解答： C
考古
( ) 90. MRI findings strongly suggest multiple sclerosis (MS) EXCEPT?
A. 4 or more white matter lesions (each > 3mm) B. Ovoid lesions, oriented parallel to ventricles. C. Corpus callosum lesions. D. 3 white matter lesions, 1 periventricular E. Open ring appearance on contrast-enhanced MRI
解答： B
考古
( ) 91. Which of the following regarding familial hemiplegic migraine (FHM) is FALSE?
A. Hemiparesis or hemiplegia occurring before migraine attacks is in fact a form of migraine
第 38 頁，共 51 頁 aura. B. Most FHMs are autosomal dominant. C. FHM1 is caused by mutations within the CACNA1A gene, which encodes an N type calcium channel subunit. D. FHM2 is caused by mutations leading to functional disturbances of a Na+-K+ ATPase pump. E. FHM3 results from mutations causing increased Na+ influx into neurons.
解答：C
題目之出處：Merritt’s Neurology, 11th edition, p. 952
( ) 92. Which of the following regarding the pathophysiology of migraine is FALSE?
A. Cortical spreading depression (CSD) is widely believed to correspond to the clinical phenomenon of migraine aura. B. CSD is characterized by a transient oligemia followed by prolonged cortical hyperperfusion. C. The propagation of CSD is slower than the spreading of cortical electrical activities in seizure disorders. D. Head pain in migraine results from the stimulation of meningeal nociceptive terminals by sterile neurogenic inflammation. E. Electrical stimulation near dorsal raphe neurons could trigger migraine-like headaches.
解答：B
題目之出處：Merritt’s Neurology, 11th edition, p. 953
( ) 93. Which of the following medication is LEAST likely to cause headache?
A. Magnesium sulfate B. Nitroglycerin C. Prazosin D. Amlodipine E. Cephalexin
第 39 頁，共 51 頁 解答：A
題目之出處：Merritt’s Neurology, 11th edition, p. 959
( ) 94. Which of the following medications has the WEAKEST evidence to support its use as a migraine prophylactic agent?
A. Propranolol B. Valproic acid C. Amitriptyline D. Carbamazepine E. Flunarizine
解答：D
題目之出處：Acta Neurol Taiwan 2008;17:132-148
( ) 95. Which of the following about Tolosa-Hunt syndrome is FALSE?
A. Cranial nerve III, IV, VI, V1, V2 and sympathetic nerve are involved. B. Elevated ESR is the mandatory criteria for the diagnosis of Tolosa-Hunt syndrome C. Occasionally, it may lead to vision loss or impaired visual acuity. D. Differential diagnosis of painful opthalmoplegia should include cavernous sinus lymphoma and cavernous-carotid fistula E. Steroid response could be seen both in Tolosa-Hunt syndrome or lymphoma
解答： B
題目之出處： NICP 6th, p 1747
( ) 96. Which of the following about meralgia paresthetica is FALSE?
A. Nerve compression by inguinal ligament
第 40 頁，共 51 頁 B. NCV could be a diagnostic tool C. Pregnancy is a risk factor D. May be confused with L2 radiculopathy E. Caused by femoral nerve compression
解答： E
題目之出處： Merritt’s Neurology 12th Ed. p.1047
( ) 97. Which of the following about Alzheimer disease (AD) is FALSE?
A. Memory impairment for newly acquired information is the usual initial complaint B. CSF with decreased Abeta42 and increased tau is a strong predictor for Alzheimer disease C. Two major features characterize AD: intracellular plaques consisting of the 42-amino acid amyloid-β peptide and extracellular neurofibrillary tangles D. The incidence rates for AD are also slightly higher in women, especially after age 85 years E. Cholinesterase inhibitors are currently the most prominent FDA approved treatments for AD
解答： (C)
題目之出處：Merritt’s Neurology, 12th edition, P 713- 716
( ) 98. Which of the following diseases is MOST likely to present with a clinical syndrome resembling behavioral variant frontotemporal dementia (bvFTD)?
A. Late-onset Alzheimer disease B. Posterior cortical atrophy C. Dementia with Lewy bodies D. Progressive supranuclear palsy E. Huntington disease
解答： D 題目之出處：Merritt’s Neurololgy (12th edition), p718-719
第 41 頁，共 51 頁
( ) 99. Which of the following is NOT the usual symptom for posterior cortical atrophy?
A. Severe visuospatial difficulty B. Prosopagnosia C. Dyslexia D. Aphasia E. Bálint’s syndrome
解答： D
題目之出處：Principles of Neurology, 10e. Chap. 39
( ) 100. Which of the following is the ignored cognitive domain of Mini-Mental State Examination (MMSE)?
A. Executive function B. Orientation C. Attention D. Memory E. Language
解答： A
題目之出處： Burrell JR, et al. J Neurol Neurosurg Psychiatry 2015;0:1-9.
doi:10.1136/jnnp-2013-307483
( ) 101. According to the recent International consensus criteria for behavioral variant frontotemporal dementia (bvFTD), which of the following symptom is NOT included for the diagnosis possible behavioral variant FTD?
A. Impulsive, rash or careless actions.
第 42 頁，共 51 頁 B. Altered food preferences. C. Diminished response to other people’s needs and feelings. D. Severe impairment of episodic memory. E. Complex, compulsive or ritualistic behaviors.
解答： D
題目之出處：Bradley’s Neurology in Clinical Practice, Sixth Edition Page:1550-1551.Brain. 2011
Sep;134(Pt 9):2456-77
( ) 102. Which of the following descriptions about alien hand syndrome (AHS) is FALSE?
A. Intermanual conflict is frequently observed in frontal type AHS B. Callosal type AHS may be caused by lesion located at isthmus of corpus callosum C. The dominant hand is typically involved in frontal type AHS D. The frontal type AHS may be caused by lesion located at anterior corpus callosum E. Reflexive grasping may be observed in frontal type AHS
解答： A
題目之出處： NICP (2012, V6) P59, 123
( ) 103. Which of the followings is the best description about the feature of Parkinson’s disease with dementia (PDD)?
A. The occurrence of dementia is not correlated with the duration and severity of Parkinson’s disease. B. Increase the speed of thought is the characteristic feature of PDD. C. PDD is also associated with APOE4. D. Dopamine deficiency is the major cause of PDD. E. PDD usually associated with visual hallucination.
第 43 頁，共 51 頁 解答：(E) 題目之出處：Clinical Neurology for Psychiatrists, 7th ed., p 403
考古
( ) 104. Which of the following is characteristic of Kluver-Bucy syndrome?
A. Bilateral frontal lobectomy B. Diminished fear response C. Hyposexuality D. Poor food intake E. Auditory agnosia
解答： B 題目之出處：Principles of Neurology, 9th ed., p. 500
考古
( ) 105. Which of the following is related to tauopathy?
A. Frontotemporal dementia with ubiquitin (FTD-U) B. Progressive supranuclear palsy (PSP) C. Parkinson’s disease with dementia (PDD) D. Dementia with Lewy body (DLB) E. Creutzfeldt-Jakob disease (CJD)
解答： B 出處：Merritt’s Textbook of Neurology, 12th Edition, Chap. 112, p710-711
考古
( ) 106. A 65-year-old woman presents with acute onset akinetic mutism, lack of motivation, apathy, leg weakness, and incontinence. You suspect she may have a stroke. Which of the following could explain her symptoms?
第 44 頁，共 51 頁
A. Pontine infarct B. Dominant temporal lobe infarct C. Nondominant parietal lobe infarct D. Nondominant temporal lobe infarct E. Bilateral ACA infarct
解答：E
( ) 107. A 32-year-old alcoholic woman presents to the emergency department. She says she cannot remember how she got there or even what her name is. On examination, you find that she has intact new learning ability with loss of remote memory, including autobiographical memory. What is your diagnosis?
A. Wernicke’s encephalopathy B. Korsakoff’s disease C. Transient global amnesia D. Psychogenic amnesia E. Retrograde amnesia
解答：D
( ) 108. An 87-year-old man who is legally blind due to severe macular degeneration reports seeing images of people and colorful animals. He realizes that he is blind and clearly understands that these are hallucinations. This man has?
A. Anton’s syndrome B. Balint’s syndrome C. Psychosis with visual hallucinations D. Charles Bonnet syndrome E. Delusional disorders
第 45 頁，共 51 頁
解答：D
( ) 109. Accumulation of very long chain fatty acids is seen in:
A. X-linked adrenoleukodystrophy B. Alexander disease C. Canavan disease D. Cerebrotendinos xanthomatosis E. Ataxia with selective vitamin E deficiency
解答：A
( ) 110. Genetic predisposition is an important factor for the following epilepsy syndromes/seizure, EXCEPT:
A. Febrile seizure B. West syndrome C. Benign rolandic epilepsy D. Juvenile myoclonic epilepsy E. Childhood absence epilepsy
解答：B
( ) 111. Which of the following feaatures is more common in autosomal dominant ataxia compared to autosomal recessive type?
A. Early onset B. Loss of function protein C. Associated with non-neurologic manifestation D. Anticipation
第 46 頁，共 51 頁 E. Lack of family history
解答：D
題目之出處：Merritt’s Neurology (12th ed.) p. 782
( ) 112. A 23-year-old man has one-year history of progressive ptosis and ophthalmoplegia. Evaluations for myasthenia gravis are negative. Lactic acid levels are normal at rest but increase remarkably after exercise. A muscle biopsy shows ragged red fibers. Which of the following is the MOST likely cause of this patient's presentations?
A. Mutation of the LMNA gene on chromosome 1q B. Expanded, unstable CTG repeat on chromosome 19q C. Mutation of nuclear DNA encoding mitochondrial proteins D. Mutation of mitochondrial DNA E. Mutation of Capain-3 gene
解答：D
題目之出處：Merritt's Neurology 12ed p689-692
( ) 113. Which of the following diseases is NOT caused by nucleotide repeat expansion disorder?
A. Myotonic dystrophy type 1 B. Familial spastic paraplegia (Strumpell Lorrain disease) C. Spinocerebellar ataxia 1 D. Huntington disease E. Kennedy disease (bulbospinal muscular atrophy)
解答：B 題目之出處：NICP 6th ed. P712. Table 40.2
考古
第 47 頁，共 51 頁
( ) 114. For genetic diagnostic tests for hereditary diseases, which one is FALSE?
A. Site-specific polymerase chain reaction is a useful diagnosis of unequal crossing-over at the hot spot of CMT1A in some population. B. Fluorescence in situ hybridization is the diagnosis of choice of hereditary neuropathy with liability of pressure with large deletion. C. Direct DNA sequencing is the only diagnostic test of Duchenne muscular dystrophy. D. The amplicon is a diagnostic indicator of Huntingon’s disease E. Linkage study base on single nucleotide polymorphism is the initial approach to a genetic test with an unknown gene
解答： C
( ) 115. A 48 year-old woman has suffered from vigorous, agitated and often dangerous motor activity during sleep accompanied by vivid dreams. One year after the sleep problem, she has developed progressive cerebellar ataxia. She also had episodic loss of consciousness associated with brief jerking movement of her body and profound orthostatic hypotension. Which of the following is MOST likely?
A. The habitual attacks usually happen in the first half of the night B. The EEG often shows paroxysmal low voltage fast activity or voltage suppression over frontal areas C. The PSG often shows augmented surface electromyography activity during rapid eye movement sleep D. The treatment of choice is serotonin-norepinephrine reuptake inhibitor E. The sleep disorder has a strong link to tauopathy
解答： C
題目之出處：Adams and Victor’s Principles of Neurology, 10th edition, Chap.19, Sleep and its
Abnormalities, REM Sleep Behavior Disorder
第 48 頁，共 51 頁 ( ) 116. A 47 year-old woman presented with nocturnal attacks for 10 years characterized by screaming during sleep, sometimes accompanied by sitting up and attempting to get out of the bed or standing up with tachycardia, diaphoresis, a fearful gaze for 1-2 minutes. These episodes rarely provoked a full awakening with awareness. If she had a full awakening, she was not confused at all but could not clearly remember the event. Which of the following is MOST likely?
A. The drug of choice is Alprazolam B. The electroencephalography during the attack is usually normal C. The polysomnography during the attack usually shows abnormal arousal patterns from slow wave sleep (stage N3) D. There is a strong link to alpha-synucleinopathy E. This is a sporadic disorder without any possible genetic linkage
解答： B
題目之出處：Adams and Victor’s Principles of Neurology, 10th edition, Chap.19, Sleep and its
Abnormalities, Nocturnal Frontal Lobe Epilepsy
( ) 117. Which of the following about restless leg syndrome is FALSE?
A. Patient may complain of unpleasant aching and drawing sensations in the calves and thighs B. Symptoms tend to be better in warm weather C. Dopamine agonists are useful for this condition D. Ferrum supplementation may be needed in some patients E. The urge to move or unpleasant sensations are relieved by movements
解答： B
( ) 118. Which of the following tests is used to evaluate parasympathetic function?
A. Blood pressure response to Valsalva maneuver B. Quantitative sudomotor axon reflex test (QSART)
第 49 頁，共 51 頁 C. Sympathetic skin responses (SSR) D. Heart rate variability to cyclic deep breathing E. Blood pressure response to orthostatic stress (such as head-up tilt)
解答： D 題目之出處： Principles of Neurology, 9e. Chap. 26, table 26-1
考古
( ) 119. Which of the following treatment maneuvers for orthostatic hypotension is FALSE?
A. Squatting, leg crossing B. Abdominal and leg compression C. Night-time upright tilt D. Isometric exercise E. Small, frequent meals low in carbohydrates
解答： D 題目之出處： Merritt’s Neurology, 12th Edition, p. 976
考古
( ) 120. Which of the following about diabetic autonomic neuropathy (DAN) is FALSE?
A. DAN is the most common and important cause of autonomic neuropathy. B. Ewing et al found 56% of 73 patients with DAN died within 5 years, many owing to non-autonomic complications such as renal failure. C. Later studies showed less ominous DAN mortality (23% at 8 years) without other initial complications D. The added autonomic dysfunction risk of DAN is dependent of coronary perfusion deficits. E. Upper GI dysmotility, impotence, constipation and episodic diarrhea are a few common manifestations.
解答： D
第 50 頁，共 51 頁 題目之出處：Merritt’s textbook of Neurology 12th ed., Chap. 159, p978
考古
第 51 頁，共 51 頁