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Hypokalemic periodic paralysis exacerbated by acetazolamide

Departments of Neurology and Pediatrics of the University of Rochester School of Medicine and Dentistry (Drs Torres, Griggs, and Moxley) and the Department of Neurology, Mount Sinai School of Medicine (Dr. Bender)

Address correspondence and reprint requests to Dr. Torres, Department of Neurology, Box 673, University of Rochester. School of Medicine and Dentistry, 601 Elmwood Avenue, Rochester, NY 14642.

Although acetazolamide usually prevents paralytic attacks in hypokalemic periodic paralysis, not all patients benefit from this treatment. We studied a father and two sons in whom attack frequency and severity increased on acetazolamide. Administration of triamterene virtually abolished attacks in three separate single-blind trials totaling more than 12 months.

Spontaneous and glucose-insulin provoked attacks occurred with only slight hypokalemia. Acetazolamide produced slight hypokalemia and provoked attacks of weakness whereas triamterene increased potassium levels significantly. Certain patients with hypokalemic periodic paralysis are worsened by acetazolamide, perhaps because of its kaliopenic effect. Triamterene may be effective in some of these patients.

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