Reduced life expectancy in 40 cases of early onset cerebellar ataxia with retained tendon reflexes: a population-based study
Title
Reduced life expectancy in 40 cases of early onset cerebellar ataxia with retained tendon reflexes: a population-based study
Creator
Chio A; Orsi L; Mortara P; Schiffer D
Identifier
Publisher
Acta Neurologica Scandinavica
Date
1993
Subject
Child; Chromosome Disorders; Female; Humans; Male; Survival Rate; Adult; Follow-Up Studies; Aged; Middle Aged; Survival Analysis; adolescent; Preschool; Outcomes; Life Expectancy; Neurologic Examination; Chromosome Aberrations/genetics; Genes; Recessive/genetics; Cerebellar Ataxia/genetics/mortality/physiopathology; Spinocerebellar Degenerations/genetics/mortality/physiopathology; Reflex; Italy/epidemiology; Stretch/genetics/physiology
Description
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.
1993
Rights
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Type
Journal Article
Citation List Month
Backlog
Citation
Chio A; Orsi L; Mortara P; Schiffer D, “Reduced life expectancy in 40 cases of early onset cerebellar ataxia with retained tendon reflexes: a population-based study,” Pediatric Palliative Care Library, accessed September 22, 2023, https://pedpalascnetlibrary.omeka.net/items/show/11923.