A case of cerebral infarction caused by cerebral artery occlusion with the onset of POEMS syndrome

Abstract

A 75-year-old man was admitted to our hospital for the assessment of progressive weakness of four extremities. In the past 1 year, he suffered from cerebral infarction two times. No cervical and intracerebral vascular stenosis was seen, and no obvious embolic sources were found. He was diagnosed as vitamin B12 deficiency polyneuropathy due to low value of vitamin B12 and abnormal nerve conduction study (NCS). Vitamin B12 was administered but symptoms progressed. Lower limb distal muscle weakness, areflexia of four extremities, glove and stocking type sensory disorder, gynecomastia, hepatosplenomegaly, pigmentation and edema in the skin were observed. A few days after hospitalization, dysarthria and left upper limb paralysis appeared and MRI showed infarction of right middle cerebral artery (MCA) and right MCA occlusion. Serum vascular endothelial growth factor (VEGF) was markedly elevated, and NCS showed demyelination and axonal degeneration. He was eventually diagnosed as POEMS syndrome. After starting thalidomide therapy, the progression of symptoms temporarily stopped, but recurred. After changing to bortezomib therapy, the progression of symptoms stopped. In this case, initially no major artery stenosis was observed, but sudden occlusion of MCA occurred in the early stage of POEMS syndrome. We report this case regarding the relation among POEMS syndrome, Vitamin B12 deficiency, and cerebrovascular disorder with some literature review.