Ataxia without telangiectasia masquerading as benign hereditary chorea

C. Klein, G. K. Wenning, N. P. Quinn, C. D. Marsden*

*Corresponding author for this work
37 Citations (Scopus)


We report a nonconsanguineous family in whom two (of three) sons developed isolated chorea in early childhood, suggesting a diagnosis of benign hereditary chorea (BHC). However, cerebellar ataxia and oculomotor apraxia, without telangiectasia, subsequently developed. Chromosome analysis showed increased radiosensitivity in both brothers and translocations in the younger one. We conclude that ataxia with chromosomal instability may masquerade as BHC in some patients.

Original languageEnglish
JournalMovement Disorders
Issue number2
Pages (from-to)217-220
Number of pages4
Publication statusPublished - 25.03.1996


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