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

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

Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).

Type

Journal Article

Citation List Month

Backlog

Pages

358-362

Issue

5

Volume

88

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 19, 2021, https://pedpalascnetlibrary.omeka.net/items/show/11923.

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