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Article
February 1984

Ataxia-Telangiectasia

Author Affiliations

USN
Department of Pediatrics Clinical Investigation Department Naval Hospital San Diego, CA 92134

Arch Neurol. 1984;41(2):137. doi:10.1001/archneur.1984.04050140035014
Abstract

To the Editor.  —There is no treatment for the movement disorder associated with ataxia-telangiectasia. In the examination of a 6-year-old boy with this disorder, a debilitating motor disturbance was apparent. Test results for intellectual and behavior functions were normal.

Report of a Case.  —A school referral issued a complaint that ambulation by the patient was no longer safe, handwriting was illegible, and speech was difficult to understand. Drooling complicated his social acceptance.Motor examination was characterized by findings of moderate hypotonia, brisk symmetric reflexes, and bilateral extensor plantar responses. Appendicular, trunkal atactic, and choreoathetoid movements complicated ambulation. The boy needed to touch the wall for stability.Reports that methylphenidate hydrochloride administration improved motor performance in familial choreoathetosis and children with cerebral dysfunction prompted a trial of this medication in this patient.1-3A 10-mg morning and noon dosage of methylphenidate was initially selected. The result was dramatic. Safe ambulation was

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