Moyamoya Disease-like Cerebrovascular Stenotic Lesions Are an Important Phenotype of POEMS Syndrome-associated Vasculopathy

Abstract

A 41-year-old woman was diagnosed with polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome based on polyneuropathy, hepatosplenomegaly, sclerotic bone lesions, IgA-λ M-protein, and an elevated level of serum vascular endothelial growth factor. One month after the initiation of lenalidomide-dexamethasone with prophylactic aspirin, she developed facial paralysis, dysarthria, and left hemiplegia. Multiple cerebral infarctions and internal carotid artery stenosis were detected. Five months after switching to pomalidomide-dexamethasone, she again developed cerebral infarction. Progressed stenotic lesions in the bilateral internal carotid artery terminal portions were detected, showing a moyamoya disease-like appearance. Quasi-moyamoya disease can be an important phenotype of systemic vasculopathies of POEMS syndrome.

Keywords: IMiDs; POEMS syndrome; cerebral infarction; quasi-moyamoya disease; vasculopathy.

Figure 1.

Histopathological picture of a sural nerve biopsy (toluidine blue staining). The histological findings were highly suggestive of POEMS syndrome, such as myelin spheres, indicated distal axonal degeneration due to proximal demyelination, and subperineurial edema (§). Axonal loss was unclear. There was no evident thinning of the myelin sheath or onion bulb formation. Scale bar =100 μm.

Figure 2.

Diffusion-weighted brain magnetic resonance imaging (MRI) (A, B, D, E) and magnetic resonance angiography (MRA) (C, F, G). (A-C). Images taken at the first episode of stroke. Multiple infarctions in the right middle cerebral artery area (A, B) and stenotic lesion of bilateral internal carotid artery (ICA) in the terminal portion (C) are shown. (D-G). Images taken at the second episode of stroke 5 months after the first episode. These images revealed new cerebral infarctions in both frontal lobes (D, E) and significantly progressed stenotic lesions in the bilateral ICA terminal portions, showing moyamoya disease-like appearance (F). Although coronal maximum intensity projection (MIP) picture focusing on ICA terminal portion was also examined (G), typical collateral vessels (moyamoya vessels) were not evident.

Figure 3.

Axial rainbow color scale images of ¹²³I-IMP cerebral blood flow scintigraphy. The results indicated a diffuse and severe decrease of the cerebral blood flow especially in the frontotemporal lobe area.

Figure 4.

Digital subtraction angiography (DSA) images of bilateral common carotid artery. DSA images of right (A, C) and left (B, D) common carotid angiography. Images of pre- (C, D) and post- (A, B) bilateral superficial temporal artery to MCA (STA-MCA) bypass operations are presented.