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August 1979

Paroxysmal Kinesigenic Choreoathetosis and Brain-Stem Atrophy

Author Affiliations

The Medical Center Clinic 8333 N Davis Hwy Pensacola, FL 32591

Arch Neurol. 1979;36(8):522. doi:10.1001/archneur.1979.00500440092023

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To the Editor.—  We read with interest the excellent article by Goodenough and coauthors (Archives 35:827-831, 1978). We recently saw a 22-year-old Lithuanian Jewish man who has classic paroxysmal kinesigenic choreoathetosis (PKC).Computerized tomography scans showing severe enlargement of the fourth ventricle (top) and moderate enlargement of the circummesencephalic cisterns (bottom).

Report of a Case.—  This patient has had attacks since he was 14 years old. The attacks are more likely to occur if the patient is anxious. They can be suppressed by 5 to 10 s of voluntary ankle movement or by standing immobile for 5 to 10 s before walking. Results of physical and neurologic examinations were normal. After being distracted from rest with the request to quickly walk, he suffers grotesque dystonic posturing with facial grimacing and choreoathetoid movements of all limbs, lasting 15 to 20 s. He is perfectly lucid, although embarrassed, throughout. He is refractory

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