Abstract
A descriptive epidemiological survey of hereditary ataxias and spastic paraplegias was conducted in the province of Torino, Italy (2 327 996 inhabitants). On prevalence day (31 December 1982) 142 patients were alive. Total prevalence was 6.1 cases/100,000 inhabitants (95% confidence limits=5.1–7.0). The prevalence rate was 2.6 for recessive or sporadic juvenile ataxias, 1.3 for spastic paraplegias, 1.2 for autosomal dominant cerebellar ataxias, and 1.1 for late onset cerebellar ataxias. The prevalence of hereditary ataxias is similar in our province to that recorded in other populations, but hereditary spastic paraplegias are less frequent than in other populations.
Sommario
È stato condotto uno studio di epidemiologia descrittiva sulle atassie e paraplegie spastiche ereditarie nella provincia di Torino (2.327.996 abitanti). Al giorno di prevalenza (31 dicembre 1982), 142 pazienti erano vivi. La prevalenza totale era di 6.1 casi/100.000 abitanti (limiti di confidenza al 95%=5.1–7.0). La prevalenza era di 2.6 per le atassie recessive o sporadiche giovanili, 1.3 per le paraplegie spastiche, 1.2 per le atassie cerebellari dominanti, e 1.1 per le atassie cerebellari ad inizio tardivo. La prevalenza delle eredoatassie nella nostra provincia è simile a quella riscontrata in altre popolazioni, mentre meno frequenti sono le paraplegie spastiche ereditarie.
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References
Barbeau A.:A tentative classification of recessively inherited ataxias. Can J Neurol Sci 9: 95–98, 1982.
Berkson J.:Limitations of the application of fourfold table analysis to hospital data. Biometrics Bulletin 2: 47–53, 1946.
Brewis MR, Poskanzer DC, Rolland C, Miller H:Neurological diseases in an English city. Acta Neurol Scand Suppl 24: 9–89, 1966.
Chen K, Brody JA, Kurland LT:Patterns of neurological diseases on Guam. Arch Neurol 19: 573–578, 1968.
Currier RD:A classification of ataxia. Ital J Neurol Sci Suppl 4: 55–64, 1984.
Filla A, Campanella G:Studio epidemiologico delle eredoatassie spino-cerebellari in Campania. In “Atti del II Convegno Nazionale di Neuroepidemiologia” p. 81–82. Ed. Boeri R. e Filippini G. Milano, 1980.
Geoffroy G, Barbeau A, Breton G et al:Clinical description and roentgenologic evaluation of patients with Friedreich's ataxia. Can J Neurol Sci 3: 279–286, 1976.
Gudmundsson KR:Prevalence and occurrence of some rare neurological disease in Iceland, Acta Neurol Scand 45: 114–118, 1969.
Hanhart E:Beitrage zur Konstitutions und Vererbungsforschung an Hand von Studien uber hereditare Ataxien (46 neue Falle Friedreichscher Krankheit). Schweitz Med Wschz 4: 139–143, 1923.
Harding AE:Early onset cerebellar ataxia with retained tendon reflexes: a clinical and genetic study of a disorder distinct from Freidreich's ataxia. J Neurol Neurosurg Psychiatry 44: 503–508, 1981.
Harding AE:“Idiopathic” late onset cerebellar ataxia: a clinical and genetic study of 36 cases. J Neurol Sci 51: 259–271, 1981.
Harding AE:The clinical features and classification of the late onset autosomal dominant cerebellar ataxias. A study of 11 families, including descendants of “the Drew family of Walworth”. Brain 105: 1–28, 1982.
Harding AE:Classification of the hereditary ataxias and paraplegias. Lancet 1: 1151–1155, 1983.
Harding AE:Genetic aspects of inherited ataxias. Ital J Neurol Sci Suppl. 4: 112–117, 1984.
Istat:Censimento generale della popolazione italiana, 1981. Roma, 1975.
Koeppen AH, Hans MB, Sheperd DI, Best PV:Adult-onset hereditary ataxia in Scotland. Arch Neurol 34: 611–618, 1977.
Kreindler A, Ionasescu V, Drinca-Ionescu M:Repartitia bolilor neurologiee erdofamiliale in Rominia. Stud Cercet Neurol 9: 401–410, 1964.
Kurland LT:Descriptive epidemiology of selected neurologic and myopathic disorders with particular reference to a survey in Rochester. Minn. J Chronic Dis 8: 378–418, 1958.
Lucci B, Motti B, Guidotti D, Zucco R:Indagine epidemiologica descrittiva delle eredoatassie nella provincia di Reggio-Emilia. In “Atti del III Convegno Nazionale di Neuroepidemiologia” p. 169–174. Ed Filippini G., Boeri R., Milano, 1982.
Mortara P, Leone M, Schiffer D, Bardelli D:Studio epidemiologico delle eredoatassie spino-cerebellari in provincia di Torino. Risultati preliminari. In “Atti del III Convegno Nazionale di Neuroepidemiologia” p. 227–238. Ed. Filippini G., Boeri R. Milano, 1982.
Sjögren T.:Klinische end Erbbiologische untersuchungen uber die heredoataxien. Acta Psychiatr Neurol Suppl. 27: 1–200, 1943.
Skre H:Hereditary spastic pareplegia in Western Norway. Clin Genet 6: 165–183, 1974.
Skre H.:Spino-cerebellar ataxia in Western Norway. Clin Genet 6: 265–288, 1974.
Skre H:Friedreich's ataxia in Western Norway. Clin Genet 7: 287–298, 1975.
Trizio M, Serlenga L, Margari L, Pozio G:Le atassie spino-cerebellari in Puglia. In “Atti del II Congresso Nazionale di Neuroepidemiologia” p. 83–85. Ed. Boeri R., Filippini G. Milano, 1980.
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Brignolio, F., Leone, M., Tribolo, A. et al. Prevalence of hereditary ataxias and paraplegias in the province of Torino, Italy. Ital J Neuro Sci 7, 431–435 (1986). https://doi.org/10.1007/BF02283021
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DOI: https://doi.org/10.1007/BF02283021