Kyusik Kang1, Kon Chu, Dong-Eog Kim, Sang-Wuk Jeong, Jun-Won Lee, Jae-Kyu Roh. 1. Department of Neurology and the Clinical Research Institute, Seoul National University Hospital, Neuroscience Research Institute of Seoul National University Medical Research Center, Korea.
Abstract
BACKGROUND: A syndrome variously combining peripheral neuropathy, visceromegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS syndrome) is a rare variant of plasma cell dyscrasia with multisystemic manifestations. Acute ischemic strokes in patients with POEMS syndrome have rarely been reported, and the pathophysiologic mechanism of this disease is unknown. Fibrinogen is reported to be an independent risk factor for cerebrovascular disease and is correlated with the interleukin 6 level in the plasma. The serum level of interleukin 6 is high in the active stage of POEMS syndrome. OBJECTIVE: To describe the neuroimaging findings and fibrinogen levels in patients with POEMS syndrome. DESIGN: Case series. SETTING: The neurology department of a tertiary referral center. METHODS: Three patients with an acute cerebral infarction associated with POEMS syndrome underwent magnetic resonance imaging, diffusion-weighted imaging, magnetic resonance angiography, transcranial Doppler ultrasonography, and serum fibrinogen level and serum C-reactive protein level analysis. The serum fibrinogen level before the stroke was collected retrospectively from the hospital medical records. RESULTS: There was an elevated fibrinogen level in all of the patients. In 2 patients, unilateral or bilateral end artery border-zone infarcts were observed on the brain magnetic resonance imaging scan. The serum fibrinogen level was high before the stroke in 2 patients. CONCLUSIONS: The POEMS syndrome can be associated with stroke, particularly end artery border-zone infarctions. We suggest that an elevated fibrinogen level might play a role in the pathogenesis of stroke.
BACKGROUND:A syndrome variously combining peripheral neuropathy, visceromegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS syndrome) is a rare variant of plasma cell dyscrasia with multisystemic manifestations. Acute ischemic strokes in patients with POEMS syndrome have rarely been reported, and the pathophysiologic mechanism of this disease is unknown. Fibrinogen is reported to be an independent risk factor for cerebrovascular disease and is correlated with the interleukin 6 level in the plasma. The serum level of interleukin 6 is high in the active stage of POEMS syndrome. OBJECTIVE: To describe the neuroimaging findings and fibrinogen levels in patients with POEMS syndrome. DESIGN: Case series. SETTING: The neurology department of a tertiary referral center. METHODS: Three patients with an acute cerebral infarction associated with POEMS syndrome underwent magnetic resonance imaging, diffusion-weighted imaging, magnetic resonance angiography, transcranial Doppler ultrasonography, and serum fibrinogen level and serum C-reactive protein level analysis. The serum fibrinogen level before the stroke was collected retrospectively from the hospital medical records. RESULTS: There was an elevated fibrinogen level in all of the patients. In 2 patients, unilateral or bilateral end artery border-zone infarcts were observed on the brain magnetic resonance imaging scan. The serum fibrinogen level was high before the stroke in 2 patients. CONCLUSIONS: The POEMS syndrome can be associated with stroke, particularly end artery border-zone infarctions. We suggest that an elevated fibrinogen level might play a role in the pathogenesis of stroke.