Coexistence of Quasi-moyamoya Disease and POEMS Syndrome in a Patient with Intracranial Hemorrhage: A Case Report and Literature Review

Abstract

POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome is a rare paraneoplastic syndrome elicited by plasma cell dyscrasia. Its clinical manifestations are multiple and stroke is not a recognized feature. A 44-year-old woman with a 3-month history of bilateral lower extremity sensorimotor disturbance was admitted to our hospital. Examinations revealed polyneuropathy, organomegaly, hypothyroidism, monoclonal gammopathy, pelvic plasmacytoma, and elevated serum vascular endothelial growth factor (VEGF) levels. A diagnosis of POEMS was made. Her condition was improved by radiation therapy of her pelvic plasmacytoma and she continued to be seen on an outpatient basis. Five years after her first admission she was re-admitted with sudden-onset right hemiparesis. A brain computed tomography (CT) scan revealed a left intracranial hemorrhage and magnetic resonance (MR) angiography and cerebral angiography showed occlusion of the proximal portion of the bilateral middle cerebral artery and narrowing of the bilateral internal carotid artery at the terminal portion; moyamoya vessels were seen. This is the first report of a patient whose intracranial hemorrhage was attributable to quasi-moyamoya disease associated with POEMS syndrome. We suggest that the POEMS syndrome be ruled out in the differential diagnosis of patients presenting with atypical stroke, multivessel stenotic lesions (moyamoya vessels), and polyneuropathy.

Keywords: POEMS syndrome; VEGF; quasi-moyamoya disease.

Fig. 1

Abdominal CT (A) Osteolytic lesion was detected in right pelvis. (B) Pelvic tumor became smaller and changed into osteosclerotic lesion.

Fig. 2

Pathological features of plasmacytoma (A) Diffuse proliferation of plasma cells (Hematoxylin and eosin 40×) (B) CD138 positivity (40×) (C) Lambda light-chain positivity (40×).

Fig. 3

(A) MR angiography revealed narrowing of the proximal portion of the bilateral middle cerebral artery and the terminal portion of the internal carotid artery. (B) Brain CT scan demonstrating left putaminal hemorrhage. (C–E) MR angiography and cerebral angiography revealed occlusion of the proximal portion of the bilateral middle cerebral artery and narrowing at the terminal portion of the bilateral internal carotid artery. Note Moyamoya vessels. (F) Cerebral angiography demonstrated collateral circulation via leptomeningeal anastomoses in the posterior circulation. (G) SPECT ( ¹²³ I-IMP) showed reduction of the CBF in the left frontoparietal cortex, left regional temporo-occipital cortex, and right cerebellum.