Potentially fatal cardiac dysrhythmia and hyperkalemic periodic paralysis

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 tachydysrhythmia (BVT), short stature, microcephaly, and clinodactyly. The mother, but not the child...

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Bibliographic Details
Published inNeurology Vol. 35; no. 8; p. 1208
Main Authors Gould, R J, Steeg, C N, Eastwood, A B, Penn, A S, Rowland, L P, De Vivo, D C
Format Journal Article
LanguageEnglish
Published United States 01.08.1985
Subjects
Arrhythmias, Cardiac - complications
Arrhythmias, Cardiac - drug therapy
Arrhythmias, Cardiac - genetics
Biopsy
Child
Electromyography
Humans
Hyperkalemia - complications
Imipramine - therapeutic use
Male
Muscles - pathology
Muscles - physiopathology
Paralysis - etiology
Paralysis - pathology
Paralysis - physiopathology
Periodicity
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Summary: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 tachydysrhythmia (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.
ISSN:0028-3878
DOI:10.1212/WNL.35.8.1208