POEMS Syndrome Associated with Ischemic Stroke
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ORIGINAL CONTRIBUTION POEMS Syndrome Associated With Ischemic Stroke Kyusik Kang, MD; Kon Chu, MD; Dong-Eog Kim, MD; Sang-Wuk Jeong, MD; Jun-Won Lee, MD; Jae-Kyu Roh, MD, PhD Background: A syndrome variously combining periph- Methods: Three patients with an acute cerebral infarc- eral neuropathy, visceromegaly, endocrinopathy, mono- tion associated with POEMS syndrome underwent mag- clonal gammopathy, and skin changes (POEMS syn- netic resonance imaging, diffusion-weighted imaging, drome) is a rare variant of plasma cell dyscrasia with magnetic resonance angiography, transcranial Doppler multisystemic manifestations. Acute ischemic strokes in ultrasonography, and serum fibrinogen level and serum patients with POEMS syndrome have rarely been re- C-reactive protein level analysis. The serum fibrinogen ported, and the pathophysiologic mechanism of this dis- level before the stroke was collected retrospectively from ease is unknown. Fibrinogen is reported to be an inde- the hospital medical records. pendent risk factor for cerebrovascular disease and is correlated with the interleukin 6 level in the plasma. The Results: There was an elevated fibrinogen level in all of serum level of interleukin 6 is high in the active stage of the patients. In 2 patients, unilateral or bilateral end ar- POEMS syndrome. tery border-zone infarcts were observed on the brain mag- netic resonance imaging scan. The serum fibrinogen level Objective: To describe the neuroimaging findings was high before the stroke in 2 patients. and fibrinogen levels in patients with POEMS syn- drome. Conclusions: The POEMS syndrome can be associated with stroke, particularly end artery border-zone infarc- Design: Case series. tions. We suggest that an elevated fibrinogen level might play a role in the pathogenesis of stroke. Setting: The neurology department of a tertiary refer- ral center. Arch Neurol. 2003;60:745-749 HE ACRONYM POEMS was rienced an acute ischemic stroke underwent brain first suggested by Bard- magnetic resonance imaging (MRI) and diffu- wick and colleagues1 to de- sion-weighted imaging (DWI) using previ- 4 scribe a syndrome vari- ously described methods. Magnetic resonance ously combining peripheral angiography of the intracranial and extracra- nial vessels and/or transcranial Doppler ultra- Tneuropathy, visceromegaly, endocrinopa- sonography was used to document the pres- thy, monoclonal gammopathy, and skin ence of large-artery disease. All subjects changes. Ischemic diseases of the coro- underwent a structured clinical interview, physi- From the Department of nary and lower limb arteries have often cal and neurologic examinations, electrocardio- Neurology and the Clinical been reported in patients with POEMS syn- graphy, echocardiography, and laboratory test- Research Institute, Seoul 2,3 ing, including serum fibrinogen level and National University Hospital, drome. However, acute ischemic strokes C-reactive protein (CRP) level analysis. The se- Neuroscience Research Institute in patients with POEMS syndrome have rum fibrinogen level from before the onset of the of Seoul National University rarely been reported, and the pathophysi- stroke, if available, was collected retrospec- Medical Research Center, Seoul ologic mechanism of this disease is un- tively from the hospital medical records. The epi- (Drs Kang, Chu, Jeong, and known. We report 3 cases of acute cere- demiologic and clinical characteristics of the pa- Roh), and the Department of bral ischemic stroke associated with tients are summarized in the Table. Neurology, College of Medicine, POEMS syndrome, and suggest a pos- Kwandong University, sible pathologic mechanism. Kangwon-do (Dr Lee), South RESULTS Korea; and the Center for Molecular Imaging Research, METHODS PATIENT 1 Massachusetts General Hospital, Harvard Medical From June 1, 2000, to May 31, 2001, 3 consecu- This patient was a 42-year-old man with School, Boston (Dr Kim). tive patients with POEMS syndrome who expe- a history of sensorimotor polyneuropa- (REPRINTED) ARCH NEUROL / VOL 60, MAY 2003 WWW.ARCHNEUROL.COM 745 ©2003 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/24/2021 Summary of Patient Characteristics* Patient Characteristic 123 Clinical symptoms Slurred speech, right-sided facial palsy, Tetraparesis Slurred speech and monoparesis of the right arm Visceromegaly Hepatosplenomegaly and Castleman Hepatosplenomegaly and Hepatomegaly and lymphadenopathy disease Castleman disease Monoclonal gammopathy Absent Present Present Risk factors for stroke Old myocardial infarction History of smoking Hypertension Brain MRI and DWI findings BZI in the left ACA-MCA and MCA-PCA Bilateral end artery BZIs and BZI in the left MCA-PCA territory, left territories and a left MCA infarction bilateral PCA infarctions end artery BZI, and stenosis of the left intracranial ICA MRA findings Hypoplastic right precommunicating Stenosis of the left MCA Not performed ACA segment and fetal type circulation in the right PCA TCD findings Increased MFV of the left ACA, the Not performed Increased MFV in the left ICA siphon, MCA, and the right ICA siphon the right MCA, both VAs, and the basilar artery Serum fibrinogen level, before/after 0.557/0.603 0.706/0.594 Not obtained/0.361 the stroke, g/dL† CRP level, mg/dL‡ 1.1 0.9 1.6 Hemoglobin, g/dL 12.2 12.8 11.7 Platelet count, ϫ103/µL§ 695 232 280 LA Absent Absent Not performed APL Absent Absent Not performed APC Absent Absent Not performed Echocardiographic findings Akinesia of the anterior, anterolateral, Mildly decreased systolic function Pulmonary hypertension and and inferolateral wall of the left ventricle and pericardial effusion pericardial effusion Abbreviations: ACA, anterior cerebral artery; APC, activated protein C resistance; APL, anti−phospholipid antibody; BZI, border-zone infarction; CRP, C-reactive protein; DWI, diffusion-weighted imaging; ICA, internal carotid artery; LA, lupus anticoagulant; MCA, middle cerebral artery; MFV, mean flow velocity; MRA, magnetic resonance angiography; MRI, magnetic resonance imaging; PCA, posterior cerebral artery; TCD, transcranial Doppler ultrasonography; VA, vertebral artery. SI units: To convert fibrinogen level to micromoles per liter, multiply by 29.41. *All patients had a peripheral neuropathy, endocrinopathy (hypothyroidism), and skin changes (hyperpigmentation). †The reference range for serum fibrinogen level in our laboratory is 0.17 to 0.35 g/dL. ‡The reference range for CRP level in our laboratory is 0 to 0.5 mg/dL. §The reference range for platelet count in our laboratory is 130 ϫ 103/µL to 400 ϫ 103/µL. thy, splenomegaly, lymphadenopathy, hypothyroid- anterior cerebral artery, the left MCA, and the right ism, hyperpigmentation, and myocardial infarction. internal carotid artery siphon. He was admitted to the hospital with weakness of the right arm. A neurologic examination revealed right- PATIENT 2 sided central type facial palsy, dysarthria, and mono- paresis of the right arm. Deep tendon reflexes were This patient was a 48-year-old woman with a history of diminished in all limbs, and sensation was impaired in polyneuropathy, hepatomegaly, hypothyroidism, M pro- both legs. The serum fibrinogen and CRP levels were tein, hyperpigmentation, gangrene of the left foot, and 0.603 g/dL (17.7 µmol/L) and 1.1 mg/dL, respectively, human herpesvirus 8–associated Castleman disease. She on day 3. Four months before onset, the serum had a 5 pack-year history of smoking. She was admitted fibrinogen level had been 0.557 g/dL (16.4 µmol/L). to the hospital because of acute weakness in all limbs. This patient did not have polycythemia. Lupus antico- The findings on physical examination included quad- agulant, anti–phospholipid antibody, and activated riplegia, hypoesthesia involving the distal parts of both protein C resistance were not detected. This patient legs, and areflexia in all extremities. Her fibrinogen level had mild thrombocythemia. Echocardiography was 0.594 g/dL (17.5 µmol/L) on day 3. It had been 0.706 showed akinesia of the anterior, anterolateral, and g/dL (20.8 µmol/L) 1 month before admission. Her CRP inferolateral wall. On the MRI and DWI scans, there level was 0.9 mg/dL on day 10. Polycythemia, thrombo- were left anterior cerebral artery–middle cerebral cythemia, lupus anticoagulant, anti–phospholipid anti- artery (MCA) border-zone infarctions (BZIs) and body, and activated protein C resistance were not ob- MCA–posterior cerebral artery BZIs (Figure 1). Mag- served. Echocardiography showed a mildly decreased netic resonance angiography showed a hypoplastic systolic function of the left ventricle and a small amount right precommunicating anterior cerebral artery seg- of pericardial effusion. An MRI scan revealed bilateral pos- ment and fetal type circulation in the right posterior terior cerebral artery infarcts and bilateral end artery BZIs cerebral artery. Transcranial Doppler ultrasonography (Figure 2). On magnetic resonance angiography, there revealed increased mean flow velocities of the left was stenosis of the left proximal MCA. (REPRINTED) ARCH NEUROL / VOL 60, MAY 2003 WWW.ARCHNEUROL.COM 746 ©2003 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/24/2021 A B Figure 1. Brain magnetic resonance imaging scan of patient 1. Fluid-attenuated inversion recovery images show high signal intensity in the left frontoparietal lobe (A) and the left anterior and posterior border-zone areas (B). A B Figure 2. Brain magnetic resonance imaging scan