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 Volume 69, Number 11, September 11, 2007
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NEUROLOGY 2007;69:1136-1141
© 2007 American Academy of Neurology

Primary systemic amyloidosis with ischemic stroke as a presenting complication

Alexander Y. Zubkov, MD, PhD, Alejandro A. Rabinstein, MD, Angela Dispenzieri, MD and Eelco F.M. Wijdicks, MD

From the Mayo Clinic, Department of Neurology (A.Y.Z., A.A.R., E.F.M.W.) and Division of Hematology (A.D.), Rochester, MN.

Address correspondence and reprint requests to Dr. Eelco Wijdicks, Mayo Clinic, Division of Critical Care Neurology, Department of Neurology, 200 First Street SW, Rochester, MN 55901 wijde{at}mayo.edu

Background: Amyloidosis is an uncommon disorder that ultimately leads to fatal multiorgan failure. Ischemic strokes have been sporadically described but are not well characterized. The purpose of this study was to review the pathophysiologic relationship between primary systemic amyloidosis and ischemic stroke, and to determine how often stroke is the first defining manifestation.

Methods: Retrospective study of 49 patients with confirmed primary amyloidosis and ischemic stroke. All included patients had biopsy proven amyloidosis.

Results: Forty patients were included in the study. Ischemic strokes occurred in 13 patients (32.5%) as the initial presentation of amyloidosis. Patients with initial stroke presentation had the worst outcome, with average survival of 6.9 months after established diagnosis with amyloidosis; strokes developed 9.6 months before diagnosis with primary amyloidosis. Thirty-seven percent experienced recurrent ischemic stroke. The majority (70%) of patients had cardioembolic infarctions.

Conclusions: Ischemic stroke is an underappreciated complication of primary amyloidosis. In the absence of obvious clinical and cardiogenic manifestations, primary amyloidosis should be considered when echocardiography demonstrates thickening of the valves, restrictive pattern, and increased echogenicity. Ischemic strokes as an initial presentation of primary amyloidosis carries a worse prognosis.

Abbreviations: AA = secondary amyloidosis; AF = familial amyloidosis; AL = immunoglobulin light-chain amyloidosis; CHF = congestive heart failure; GI = gastrointestinal; LV = left ventricular; NS = not significant; PE = pericardial effusion; TOAST = Trial of Org 10172 in Acute Stroke Treatment; VS = ventricular septum.

Disclosure: The authors report no conflicts of interest.

Received November 13, 2006. Accepted in final form April 11, 2007.






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