A survival analysis of 40 cases of early onset cerebellar ataxia (EOCA) with retained tendon reflexes was performed.
They represent all cases of EOCA diagnosed between 1945 and 1990 among residents of a defined area of Northwestern Italy, followed up to December 31, 1990.
The survival rates were respectively 92%, 87% and 77% at 10, 20 and 30-years, worse than expected in a disease which is usually considered benign.
The relative death rate was 4 times higher than expected for the general population.
Prognosis was significantly worse for males than for females, whereas the age of onset and the calendar year of onset did not affect survival.
Mots-clés Pascal : Ataxie, Cervelet, Homme, Age apparition, Précoce, Epidémiologie, Italie, Europe, Survie, Système nerveux pathologie, Système nerveux central pathologie, Encéphale pathologie, Cervelet pathologie, Trouble neurologique
Mots-clés Pascal anglais : Ataxia, Cerebellum, Human, Age of onset, Early, Epidemiology, Italy, Europe, Survival, Nervous system diseases, Central nervous system disease, Cerebral disorder, Cerebellar disease, Neurological disorder
Notice produite par :
Inist-CNRS - Institut de l'Information Scientifique et Technique
Cote : 94-0028187
Code Inist : 002B17A01. Création : 199406.