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Potentially fata1 cardiac dysrhythmia and hyperkalemic periodic paralysis

Departments of Pediatrics (Drs. Steeg and De Vivo) and Neurology (Drs. Gould, Eastwood, Penn, Rowland, and De Vivo) and the Divisions of Pediatric Neurology and Pediatric Cardiology, Columbia-Presbyterian Medical Center, New York, NY.

An 11-year-old boy was evaluated for mild periodic muscular weakness exacerbated on separate occasions by disopyramide phosphate and procainamide. He and his mother both had bidirectional ventricular tachydys-rhythmia (BVT), short stature, microcephaly, and clinodactyly. The mother, but not the child, had lingual myotonia. The two antiarrhythmic drugs worsened the muscular weakness without benefiting the cardiac dysrhythmia. Potassium loading produced skeletal muscle weakness and transient conversion of the BVT to normal sinus rhythm. Hypokalemia aggravated the BVT without causing weakness. Acetazolamide had no effect. The patient suffered a nonfatal cardiac arrest after several days of increased carbohydrate intake. Imipramine controlled the dysrhythmia without inducing weakness. Periodic paralysis should be considered as the diagnosis in children with BVT, a potentially fatal condition.

Address correspondence and reprint requests to Dr. De Vivo, Neurological Institute, 710 West 168th Street, New York, NY 10032.

Presented in part at the American Neurological Association meeting in San Francisco, CA, September 1981.

Accepted for publication December 4, 1984.

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