Surgical revascularization for quasi-moyamoya disease associated with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome: a case report and literature review

Abstract

POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome is a rare multisystem disease characterized by plasma cell dyscrasia and overproduction of vascular endothelial growth factor, which is related to disease activity. Recent treatment strategies have improved survival of patients suffering from this disorder; however, ischemic stroke remains a poor prognostic factor. POEMS patients with ischemic stroke frequently develop cerebral large artery stenosis/occlusion, followed by progressive stroke. Post literature review, we present an ischemic stroke case of quasi-moyamoya disease linked with this syndrome that was successfully treated with surgical revascularization. A 41-year-old woman diagnosed with POEMS syndrome developed progressive ischemic stroke due to quasi-moyamoya disease, despite decreased vascular endothelial growth factor level with lenalidomide and dexamethasone treatment. She underwent superficial temporal artery to middle cerebral artery bypass with encephalo-duro-myo-synangiosis bilaterally. The postoperative course was uneventful. Two years and five months after the stroke, neuroimaging demonstrated bypass patency, neovascularization after encephalo-duro-myo-synangiosis, and no recurrence of stroke. Our case is the first to report successful surgical revascularization for a POEMS patient. Surgical revascularization may be a useful treatment option for patients with quasi-moyamoya disease associated with POEMS syndrome, especially for those who develop refractory ischemic stroke despite reduced vascular endothelial growth factor level.

Keywords: Crow–Fukase syndrome; STA-MCA anastomosis; cerebral infarction; cerebral revascularization; vascular endothelial growth factor.

Fig. 1

Preoperative magnetic resonance imaging including diffusion-weighted imaging, apparent diffusion coefficient maps, fluid-attenuated inversion recovery imaging, and magnetic resonance angiography (MRA) Fig. 1A: Magnetic resonance imaging at the first stroke onset. MRA demonstrates stenosis at the bilateral terminal internal carotid arteries (ICA). Fig. 1B: Magnetic resonance imaging at the second stroke onset. MRA shows loss of signal intensity at the bilateral terminal ICA, proximal anterior cerebral arteries, and proximal middle cerebral arteries.

Fig. 2

Preoperative cerebral angiography (A, B) and N-isopropyl-p-[¹²³I]-iodoamphetamine single photon emission computed tomography (¹²³I-IMP SPECT) imaging (C) Fig. 2A, 2B: Preoperative common carotid angiography shows the terminal internal carotid artery (ICA) occlusion and presence of the basal moyamoya vessels bilaterally. Fig. 2C: Preoperative ¹²³I-IMP SPECT imaging shows decreased cerebral blood flow in the bilateral ICA territories, predominantly on the left.

Fig. 3

Histopathological pictures of resected superficial temporal artery (A, B: Hematoxylin-Eosin stain, C: Elastica van Gieson stain, D: Alcian Brue PAS stain) Stenotic superficial temporal artery without evident infiltration of inflammatory cells is shown (A). Dotted rectangle in A indicates the area of enlarged view shown in pictures B, C, and D. Asterisks in pictures B, C, and D indicate significantly thickened intima. Internal elastic membrane (arrowheads) was preserved (C). Alcian Brue-positive acid mucopolysaccharide deposition in the thickened intima was detected (D).

Fig. 4

Postoperative magnetic resonance imaging (A), cerebral angiography (B), and N-isopropyl-p-[¹²³I]-iodoamphetamine single photon emission computed tomography (¹²³I-IMP SPECT) imaging two years and five months after the last stroke Fig. 4A: Postoperative magnetic resonance imaging shows no recurrence of ischemic stroke. In magnetic resonance angiography, bilateral superficial temporal arteries and right middle meningeal artery were observed. Fig. 4B: Postoperative lateral views of right external carotid artery angiography and three-dimensional rotational angiography. Bypass patency (white arrows) and neovascularization via the right middle meningeal artery (white arrowheads) were identified. Fig. 4C: Postoperative lateral views of left external carotid artery angiography and three-dimensional rotational angiography. Bypass patency (yellow arrows) and neovascularization via the left deep temporal artery (yellow arrowheads) were identified. Fig. 4D: Postoperative ¹²³I-IMP SPECT imaging shows improved cerebral blood flow in the bilateral middle cerebral artery territories.