Nissenkorn A, Hassin-Baer S, Lerman SF, Levi YB, Tzadok M,
Ben-Zeev B. Movement disorder in ataxia-telangiectasia: treatment with
amantadine sulfate. J Child Neurol. 2013 Feb;28(2):155-60.
Abstract
Ataxia-telangiectasia is a cerebellar neurodegenerative
disorder presenting with ataxia, chorea, myoclonus, and bradykinesia.
Literature on treatment of movement disorders is scarce. We treated 17 children
(aged 11.2 ± 3.9 years) for 8 weeks with the dopaminergic and
anti-N-methyl-d-aspartate (NMDA) agent amantadine sulfate 6.3 ± 0.87 mg/kg/d.
Ataxia was assessed by using the International Cooperative Ataxia Scale,
parkinsonism by the Unified Parkinson Disease Rating Scale, and
chorea/myoclonus by the Abnormal Involuntary Movement Scale. Responders were
considered those patients who had at least 20% improvement in the summation of
the 3 scales. Overall, 76.5% of patients were responders, with a mean 29.3%
improvement. Ataxia, involuntary movements, and parkinsonism improved
significantly (25.3%, 32.5%, and 29.5%, respectively); (P < .001, t test).
Side effects were mild and transient, and they did not lead to drug
discontinuation. Amantadine is a well-tolerated and effective treatment for
motor symptoms in ataxia telangiectasia. Assessment of long-term effects and a
double-blind study should follow.
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