Ischemic Stroke as First Sign of Primary
Amyloidosis Worsens Prognosis

BY DIANA MAHONEY
Elsevier Global Medical News

BOSTON (EGMN) - Patients with primary systemic amyloidosis who present initially with ischemic stroke have a worse prognosis than do patients who develop the cerebrovascular complication later in the disease course, according to study results presented on May 1 at the annual meeting of the American Academy of Neurology.

Although ischemic strokes have been sporadically linked with primary amyloidosis, the connection between stroke and the rare plasma cell dyscrasia has not been well characterized, said Dr. Alexander Zubkov, of the department of neurology at the Mayo Clinic in Rochester, Minn.

To gain insight into the pathophysiologic relationship between the two conditions, Dr. Zubkov and colleagues conducted a retrospective study of 40 patients with confirmed primary amyloidosis and ischemic stroke.

Of the 40 patients, 13 presented initially with ischemic stroke at an average 9.6 months prior to amyloidosis diagnosis, Dr. Zubkov reported. Initial presentations of the remaining 27 were: cardiac symptoms (12), renal problems (9), GI symptoms (4), and peripheral neuropathy (2). The 13 patients who presented with ischemic stroke initially had the worst outcome, with an average survival of 6.9 months vs. 15.6 months for the 12 patients who had initial cardiac presentation and 44 months for the remaining 15 patients.

"It's possible that the decreased survival was due to delayed diagnosis and treatment of primary amyloidosis," said Dr. Zubkov.

A total of 37% of the full sample experienced recurrent ischemic stroke, and 70% had cardioembolic infarctions.

The results suggest that ischemic stroke is an underappreciated complication of primary amyloidosis, said Dr. Zubkov. Even in the absence of obvious clinical and cardiogenic manifestations of the plasma cell dyscrasia, primary amyloidosis should be considered if there is echocardiographic evidence of valve thickening, restrictive pattern, and increased echogenicity.

"Evidence of thickening of the valves on echocardiography should lead to myocardial biopsy for definitive diagnosis," said Dr. Zubkov. "Diagnosing the condition early in its course leaves more room for treatment and may improve outcome."

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