PEDIATRICS Vol. 65 No. 1 January 1980, pp. 149-151
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Paroxysmal Dystonic Choreoathetosis of Mount and Reback

J. A. R. Tibbles MB, MRCP, FRCP(C)1 and S. E. Barnes MB, MRCP1

1 The I.W.K. Hospital for Children, Halifax, Nova Scotia

Paroxysmal dystonic choreoathetosis is a rare and intriguing familial disorder that was first described in 1940 by Mount and Reback.1 Their patient was a young, male adult textile worker whose attacks had begun in infancy. They were characterized by an aura of a feeling of tightness in the neck and waist, together with a sensation of tiredness. This was followed by involuntary flexion of one or both arms and posturing of the legs, and was succeeded by choreoathetoid movements and dysarthria. The attacks lasted for up to two hours, during which time the patient was conscious, and recurred on an average of one to three times per day.




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