Cerebral infarction in POEMS syndrome: incidence, risk factors, and imaging characteristics
- PMID: 19841383
- PMCID: PMC2764416
- DOI: 10.1212/WNL.0b013e3181bd136b
Cerebral infarction in POEMS syndrome: incidence, risk factors, and imaging characteristics
Abstract
Objectives: To determine the risk factors and incidence of cerebral infarction associated with POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome.
Methods: The Mayo Clinic dysproteinemia database was queried to identify patients with coded diagnosis of POEMS syndrome. Patients with cerebral infarction, occurring after the onset of POEMS-related symptoms, were selected. A retrospective observational study design was used to evaluate potential predictors of stroke in patients with POEMS syndrome.
Results: A total of 9 patients (10%; 95% confidence interval 5.4-17.9) with cerebral infarction were identified (2 women, 22%). Traditional stroke risk factors were not significantly different between the stroke and nonstroke subgroups, but hematologic abnormalities such as elevated platelet count and bone marrow plasmacytosis differed between the 2 groups. Cerebral infarction occurrence after successful treatment of the underlying condition was not observed. CT and MRI data demonstrated a wide spectrum of infarct topography in these patients. Common stroke etiologies comprised suspected vascular structural abnormalities leading to vessel dissection and stenosis, in addition to embolism from a proximal source.
Conclusions: The 5-year risk of cerebral infarction in patients with POEMS syndrome is 13.4%. Evidence of plasma cell proliferation within the bone marrow and elevated serum platelet count led to increased risk of cerebral infarction in this population. We conclude that known modifiable stroke risk factors should be aggressively managed. Treatment of thrombocytosis should be considered in patients without a contraindication. Treatment of the syndrome may be the best approach to decreasing risk of cerebral infarction in these patients.
References
-
- Dispenzieri A, Kyle RA, Lacy MQ, et al. POEMS syndrome: definitions and long-term outcome. Blood 2003;101:2496–2506. - PubMed
-
- Santoro L, Manganelli F, Bruno R, Nolano M, Provitera V, Barbieri F. Sural nerve and epidermal vascular abnormalities in a case of POEMS syndrome. Eur J Neurol 2006;13:99–102. - PubMed
-
- Bova G, Pasqui AL, Saletti M, Bruni F, Auteri A. POEMS syndrome with vascular lesions: a role for interleukin-1beta and interleukin-6 increase: a case report. Angiology 1998;49:937–940. - PubMed
-
- 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;16:278–284. - PubMed
-
- Huang J, Wang L, Zhou W, Jin J. Hyaline vascular Castleman disease associated with POEMS syndrome and cerebral infarction. Ann Hematol 2007;86:59–61. - PubMed
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