Cerebral infarction in POEMS syndrome: incidence, risk factors, and imaging characteristics.
OBJECTIVE
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.
Department of Neurology, Mayo Clinic, Rochester, MN 55905, USA. dupont.stefan@mayo.edu
S A
SA
Dupont
A
A
Dispenzieri
M L
ML
Mauermann
A A
AA
Rabinstein
R D
RD
Brown
D000328:Adult; D000368:Aged; D001853:Bone Marrow / Q000473:pathology / Q000503:physiopathology; D001921:Brain / Q000000981:diagnostic imaging / Q000473:pathology; D049109:Cell Proliferation; D002544:Cerebral Infarction / Q000453:epidemiology* / Q000209:etiology / Q000473:pathology*; D015331:Cohort Studies; D016208:Databases, Factual; D005260:Female; D006801:Humans; D015994:Incidence; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D016878:POEMS Syndrome / Q000453:epidemiology* / Q000473:pathology*; D010950:Plasma Cells / Q000473:pathology / Q000502:physiology; D010976:Platelet Count; D012189:Retrospective Studies; D012307:Risk Factors; D020521:Stroke / Q000453:epidemiology* / Q000209:etiology / Q000473:pathology*; D014057:Tomography, X-Ray Computed