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May 1980

Valproic Acid and Normal Computerized Tomographic Scan in Kinesiogenic Familial Paroxysmal Choreoathetosis

Arch Neurol. 1980;37(5):327. doi:10.1001/archneur.1980.00500540105032

To the Editor.—  Phenytoin, phenobarbital, carbamazepine, and primidone give much relief to more than 90% of the patients with kinesiogenic familial paroxysmal choreoathetosis (FPC). Previous studies including rare autopsy data have failed to show consistent anatomic lesions.1,2 There is no previous report of a normal computerized tomographic (CT) scan. We studied a patient with kinesiogenic FPC with a normal CT scan and resolution of symptoms with valproic acid after incomplete response to phenobarbital.

Report of a Case.—  For 12 years, a 17-year-old woman had had paroxysms of dystonic flexion of the right arm and hand provoked by voluntary movements of the same extremity. If relaxation could not be attained or if she were anxious, the paroxysmal "spasm" would spread to involve the face, left extremity, and the legs, causing her to fall. Attacks rarely lasted more than a few minutes, but occurred several times a week. They could be