Progressive Dystonia Masking Ataxia in Ataxia-Telangiectasia

John B. Bodensteiner, Randall M. Goldblum, Armond S. Goldman

Research output: Contribution to journalArticlepeer-review

22 Scopus citations


A 10-year-old boy with ataxia-telangiectasia had severe progressive dystonic posturing that masked the ataxia until treatment relieved the dystonia. A younger sister had more classical neurologic manifestations of the disease. However, both children had telangiectasia, immunologicabnormalities, and other features of ataxia-telangiectasia. The pathologic changes that have been found in the basal ganglia at autopsy and the occurrence of choreoathetosis, oculomotor disturbances, and now dystonia indicate that the function of the basal ganglia in patients with ataxia-telangiectasia is abnormal. Children who have basal ganglial abnormalities should be studied for ataxia-telangiectasia.

Original languageEnglish (US)
Pages (from-to)464-465
Number of pages2
JournalArchives of Neurology
Issue number7
StatePublished - Jul 1980

ASJC Scopus subject areas

  • Arts and Humanities (miscellaneous)
  • Clinical Neurology


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