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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References
Brignolio F, Leone M, Tribolo A, Rosso MG, Meineri P, Schiffer D (1986) Prevalence of hereditary ataxias and paraplegias in the province of Torin, Italy. Ital J Neurol Sci 7:431–435
Campanella G, Filla A, De Falco FA, Mansi D, Durivage A, Barbeau A (1980) Friedreich's ataxia in the south of Italy: a clinical and biochemical survey of 23 patients. Can J Neurol Sci 7:351–358
Filla A, De Michele G, Caruso G, Marconi R, Campanella G (1990) Genetic data and natural history of Friedreich's disease: a study of 80 Italian patients. J Neurol 237:345–351
Harding AE (1981) Early onset cerebellar ataxia with retained tendon reflexes: a clinical and genetic study of a disorder distinct from Friedreich's ataxia. J Neurol Neurosurg Psychiatry 44:871–883
Harding AE (1984) The hereditary ataxias and related disorders. Churchill Livingstone, Edinburgh
Leone M, Brignolio F, Rosso MG, Curtoni ES, Moroni A, Tribolo A, Schiffer D (1990) Friedreich's ataxia: a descriptive epidemiological study in an Italian population. Clin Genet 38:161–169
Polo GM, Calleja J, Combarros O, Berciano J (1991) Hereditary ataxias and paraplegias in Cantabria, Spain. An epidemiological and clinical study. Brain 114:855–866
Romeo G, Menozzi P, Ferlini A, Fadda S, Di Donato S, Uziel G, Lucci B, Capodaglio L, Filla A, Campanella G (1983) Incidence of Friedreich's ataxia in Italy estimated from consanguineous marriages. Am J Hum Genet 35:523–529
Sedgwick RP, Boder E (1972) Ataxia-telangiectasia. In: Vinken PJ, Bruyn GW (eds) Handbook of clinical neurology, vol 14. North-Holland, Amsterdam, pp 267–339
Sjögren T (1943) Klinische und erbbiologische Untersuchungen über die Heredoataxien. Acta Psychiatr Neurol Scand 27 [Suppl]:1–200
Skre H (1980) Epidemiology of spinocerebellar degeneration in Western Norway: hereditary ataxias. In: Sobue I (ed) Spinocerebellar degenerations. University of Tokyo Press, Tokyo, pp 103–119
Sridharan R, Radhakrishnan K, Ashok PP, Mousa ME (1985) Prevalence and pattern of spinocerebellar degeneration in Northeastern Libya. Brain 108:831–843
Werdelin L (1986) Hereditary ataxias. Occurrence and clinical features. Acta Neurol Scand 73 [Suppl 106]:1–124
Winter RM, Harding AE, Baraitser M, Bravery MB (1981) Intrafamilial correlation in Friedreich's ataxia. Clin Genet 20:419–427
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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