Summary
An epidemiological survey of hereditary ataxias and paraplegias was conducted in Molise, a region of Italy (335, 211 inhabitants on 1 January 1989). Total prevalence was 7.5 x 10−5 inhabitants (95% confidence limits 4.8–11.1). There were 7 patients with Friedreich's disease, 5 with early onset cerebellar ataxia with retained tendon reflexes, 4 with ataxia-telangiectasia, 9 with hereditary spastic paraplegias (2 autosomal dominant and 7 autosomal recessive cases). There was no patient with autosomal dominant cerebellar ataxia.
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Filla, A., De Michele, G., Marconi, R. et al. Prevalence of hereditary ataxias and spastic paraplegias in Molise, a region of Italy. J Neurol 239, 351–353 (1992). https://doi.org/10.1007/BF00867594
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DOI: https://doi.org/10.1007/BF00867594