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NEUROLOGY 2009;73:1308-1312
© 2009 American Academy of Neurology

Cerebral infarction in POEMS syndrome

Incidence, risk factors, and imaging characteristics

S. A. Dupont, MD, PhD, A. Dispenzieri, MD, M. L. Mauermann, MD, A. A. Rabinstein, MD and R. D. Brown, Jr, MD, MPH

From the Departments of Neurology (S.A.D., M.L.M., A.A.R., R.D.B.) and Hematology (A.D.), Mayo Clinic, Rochester, MN.

Address correspondence and reprint requests to Dr. Stefan A. Dupont, Department of Neurology, Mayo Clinic, 200 First St SW, Rochester, MN 55905

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.

Abbreviations: CI = confidence interval; FSRP = Framingham Stroke Risk Profile; POEMS = polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes; TOAST = Trial of Org 10172 in Acute Stroke Treatment; VEGF = vascular endothelial growth factor.


dupont.stefan{at}mayo.edu

Disclosure: Author disclosures are provided at the end of the article.

Received March 16, 2009. Accepted in final form July 27, 2009.







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