Movement disorder in ataxia-telangiectasia: Treatment with amantadine Sulfate

Andreea Nissenkorn, Sharon Hassin-Baer, Sheera F. Lerman, Yonit Banet Levi, Michal Tzadok, Bruria Ben-Zeev

Research output: Contribution to journalArticlepeer-review

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.

Original languageEnglish (US)
Pages (from-to)155-160
Number of pages6
JournalJournal of child neurology
Volume28
Issue number2
DOIs
StatePublished - Feb 2013
Externally publishedYes

Keywords

  • N-methyl-d-aspartate
  • NMDA antagonists
  • ataxia-telangiectasia
  • bradykinesia
  • chorea

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Clinical Neurology

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