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From the Department of Neurology, University of Munich, Germany.
Address correspondence and reprint requests to Dr. M. Strupp, Department of Neurology, University of Munich, Klinikum Grosshadern, Marchioninistrasse 15, 81377 Munich, Germany; e-mail: mstrupp{at}nefo.med.uni-muenchen.de
Patients with episodic ataxia type 2 (EA2) can often be successfully treated with acetazolamide. The authors report three patients with EA2 (two with proven mutations in the CACNA1A gene) whose attacks were prevented with the potassium channel blocker 4-aminopyridine (4-AP; 5 mg tid). Attacks recurred after treatment was stopped; subsequent treatment alleviated the symptoms (mean follow-up time 6 months). These effects might be due to an improvement of the impaired functioning of Purkinje cells.
Received October 20, 2003. Accepted in final form February 23, 2004.
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