Ischemic stroke in patients with POEMS syndrome: a case report and comprehensive analysis of literature

Abstract

Background: POEMS syndrome is a rare multi-systemic disease characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. Arterial or venous thrombosis is a less-common complication of POEMS syndrome. Ischemic stroke has also been reported sporadically. However, the association between POEMS syndrome and ischemic stroke has not been entirely understood.

Methods: A case of ischemic stroke caused by cerebral vasculitis in a patient with POEMS syndrome was presented. Then a comprehensive review and analysis of the literature were performed.

Results: A total of 28 patients were identified. The common clinical manifestations of POEMS syndrome were rather non-specific in patients with ischemic stroke compared with those of patients without ischemic stroke. Twenty patients were found with multiple ischemic lesions (71.5%). In the 25 patients who had undergone the evaluation of cerebral arteries, nineteen patients (76.0%) were found with cerebral vasculopathy. Twelve patients (48.0%) had more than one cerebral artery involved. Ischemic events were documented in 8 patients even when they were undergoing all the therapy for ischemic stroke. Ten (55.6%) of the 18 patients who had survival data died within two years after stroke events.

Conclusion: Comprehensive analysis of literature revealed several trends in patients with ischemic stroke and POEMS syndrome including a low survival rate and a preponderance of cerebral vasculopathy and multiple cerebral arteries affected. Ischemic stroke may be a poor outcome predictor in patients with POEMS syndrome. Further researches focusing on a larger cohort may help in better characterizing and treating this rare complication of POEMS syndrome.

Keywords: POEMS syndrome; ischemic stroke; vascular endothelial growth factor; vasculopathy; vessel wall imaging.

Figure 1. CT scan of the thorax showed multiple axillary (Figure 1A, green arrow), and mediastinal lymphadenopathy (Figure 1A, yellow arrow) in combination with right pleural effusion (Figure 1B, white arrow) and pericardial effusion (Figure 1B, red arrow)

Figure 2. Serum immunofixation electrophoresis revealed an IgG kappa monoclonal protein(Figure 2A) with increased IgG and free kappa chain (Figure 2B)

Figure 3. Axial DWI sequences showed multiple acute infarction lesions in end artery border-zone of left cerebral hemisphere

Figure 4. CT angiography showed a great aortic arch and normal artery (Figure 4A) except diffuse and moderate stenosis in the large vessels from the origin of left ICA to the M1 segment of the left MCA (Figure 4B and 4C, red arrow)

Figure 5. High-resolution T2-weighted imaging showed concentric hyperintensity and thickening in the vessel wall of left ICA and (Figure 5A, green circle)

High-resolution, black-blood MRI before (Figure 5B, yellow circle) and after (Figure 6C, red circle) gadolinium injection demonstrating a strong, smooth, and concentric vessel wall enhancement in left ICA.

Figure 6. Histological examination of cervical lymph node revealed infiltration of plasma cells in the interfollicular area (original magnification A: ×200, B: ×400)