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Table of Contents    
LETTER TO EDITOR
Year : 2016  |  Volume : 64  |  Issue : 6  |  Page : 1318-1319

Stroke and POEMS syndrome: More than a chance association


1 Department of Neurology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
2 Department of Radiodiagnosis, Postgraduate Institute of Medical Education and Research, Chandigarh, India
3 Department of Hematology, Postgraduate Institute of Medical Education and Research, Chandigarh, India

Date of Web Publication11-Nov-2016

Correspondence Address:
Sahil Mehta
Department of Neurology, Postgraduate Institute of Medical Education and Research, Chandigarh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.193842

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How to cite this article:
Rajan R, Wilson V, Das B, Singh P, Ahluwalia J, Mehta S, Lal V. Stroke and POEMS syndrome: More than a chance association. Neurol India 2016;64:1318-9

How to cite this URL:
Rajan R, Wilson V, Das B, Singh P, Ahluwalia J, Mehta S, Lal V. Stroke and POEMS syndrome: More than a chance association. Neurol India [serial online] 2016 [cited 2019 Jul 16];64:1318-9. Available from: http://www.neurologyindia.com/text.asp?2016/64/6/1318/193842


Sir,

A 36-year-old lady without any comorbidity presented with bilateral lower extremity weakness and numbness, which started distally and progressed in two months to the extent that she started requiring support to walk. Three months prior to this episode, she had an episode of headache followed by facial deviation to the left side and inability to speak which spontaneously improved over 1 week duration. her general physical examination was notable for lower limb pitting edema, hyperpigmentation of the distal extremities, and a palpable liver [Figure 1]a. Neurological examination revealed Medical Research Council grade 3 power in hips, knees, grade 1 power at ankles, with normal power in upper limbs. There was glove and stocking sensory loss up to the knees with diffuse areflexia. Fundus examination revealed bilateral papilloedema. Nerve conduction studies revealed a demyelinating motor neuropathy in the upper limbs; the lower limb sensory, and motor nerves were non stimulable. Cerebrospinal fluid showed no cells, elevated protein (246 mg/dl), normal glucose, and no malignant cells. Her hemogram was remarkable for persistent polycythemia (hemoglobin: 15 mg/dl) and thrombocytosis (6.97 × 106/dL). Abdominal computed tomography (CT) scan showed hepatosplenomegaly and moderate ascites. No fluorodeoxyglucose avid lesions were noted on positron emission tomography-CT. Magnetic resonance imaging (MRI) of the brain revealed multiple acute-subacute lacunar infarcts in left periventricular, occipital, and posterior temporal regions [Figure 1]b and [Figure 1]c. MR angiogram revealed diffusely attenuated bilateral supraclinoid, petrous and cavernous internal carotid arteries, absence of flow-related enhancement in the left posterior cerebral artery, and a few collaterals in the perimesencephalic cistern [Figure 1]d and [Figure 1]e. Cardiac work-up was negative. Immunofixation electrophoresis revealed IgA lambda monoclonal band. Bone marrow examination showed mildly hypercellular marrow spaces with 9% plasma cells; megakaryocytes were increased with focal clustering [Figure 1]f. JAK-2 V617F mutation was negative.
Figure 1: (a) Edema and hyperpigmentation of bilateral feet; (b and c) Magnetic resonance imaging (MRI) of the brain diffusion weighted images showing diffusion restriction in the left periventricular and posterior parietal regions; (d and e) MR angiography demonstrating attenuation of supraclinoid, petrous, and cavernous portion of bilateral internal carotid arteries; (f) Bone marrow aspirate showing a small cluster of five plasma cells, including a multinucleate form. May–Grunwald–Giemsa, magnification ×100

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She was diagnosed as a case of POEMS syndrome and was treated with thalidomide, dexamethasone, and antiplatelets.

POEMS syndrome is characterized by peripheral neuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes.[1] Thrombotic events are a well-known complication of POEMS syndrome, with stroke reported in around 10% of the patients.[2] A study by Dupont et al., suggested that the median duration to stroke after the onset of neuropathy is 23 months.[2] However, in our patient, stroke preceded the onset of neuropathic symptoms. Vascular disease in POEMS syndrome is attributed to the prothrombotic state contributed by circulating paraproteins, high levels of proinflammatory cytokines such as interleukin- 1 beta, IL-6, tumor necrosis factor alpha, and vascular endothelial growth factor.[3],[4]

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  References Top

1.
Dispenzieri A, Kyle RA, Lacy MQ, Rajkumar SV, Therneau TM, Larson DR, et al. POEMS syndrome: Definitions and long- term outcome. Blood 2003;101:2496-506.  Back to cited text no. 1
    
2.
Dupont SA, Dispenzieri A, Mauermann ML, Rabinstein AA, Brown RD. Cerebral infarction in POEMS syndrome: Incidence, risk factors and imaging characteristics. Neurology 2009;73:1308-12.  Back to cited text no. 2
    
3.
Garcia T, Dafer R, Hocker S, Schneck M, Barton K, Biller J. Recurrent strokes in two patients with POEMS syndrome and Castleman's disease. J Stroke Cerebrovasc Dis 2007;16:278-84.  Back to cited text no. 3
    
4.
D'Souza A, Hayman SR, Buadi F, Mauermann M, Lacy MQ, Gertz MA, et al. The utility of plasma vascular endothelial growth factor levels in the diagnosis and follow-up of patients with POEMS syndrome. Blood 2011;118:4663-5.  Back to cited text no. 4
    


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