Rochelle A Yanofsky, Sashi S Seshia, Angelika J Dawson, Kent Stobart, Cheryl R Greenberg, Frances A Booth, Chitra Prasad, Marc R Del Bigio, Jens J Wrogemann, Francesca Fike, Richard A Gatti
BACKGROUND: The onset of progressive cerebellar ataxia in early childhood is considered a key feature of ataxia-telangiectasia (A-T), accompanied by ocular apraxia, telangiectasias, immunodeficiency, cancer susceptibility and hypersensitivity to ionizing radiation. METHODS: We describe the clinical features and course of three Mennonite children who were diagnosed with A-T following the completion of therapy for lymphoid malignancies. RESULTS: Prior to cancer therapy, all had non-progressive atypical neurological abnormalities, with onset by age 30 months, including dysarthria, dyskinesia, hypotonia and/or dystonia, without telangiectasias...
July 2009: Canadian Journal of Neurological Sciences. le Journal Canadien des Sciences Neurologiques