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<a href="http://doi.org/10.1111/j.1600-0404.1993.tb05358.x" target="_blank" rel="noreferrer">http://doi.org/10.1111/j.1600-0404.1993.tb05358.x</a>
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Title
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Reduced life expectancy in 40 cases of early onset cerebellar ataxia with retained tendon reflexes: a population-based study
Publisher
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Acta Neurologica Scandinavica
Date
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1993
Subject
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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
Creator
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Chio A; Orsi L; Mortara P; Schiffer D
Description
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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
Identifier
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<a href="http://doi.org/10.1111/j.1600-0404.1993.tb05358.x" target="_blank" rel="noreferrer">10.1111/j.1600-0404.1993.tb05358.x</a>
Rights
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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
The nature or genre of the resource
Journal Article
1993
Acta Neurologica Scandinavica
Adolescent
Adult
Aged
Backlog
Cerebellar Ataxia/genetics/mortality/physiopathology
Child
Chio A
Chromosome Aberrations/genetics
Chromosome Disorders
Female
Follow-up Studies
Genes
Humans
Italy/epidemiology
Journal Article
Life Expectancy
Male
Middle Aged
Mortara P
Neurologic Examination
Orsi L
Outcomes
Preschool
Recessive/genetics
Reflex
Schiffer D
Spinocerebellar Degenerations/genetics/mortality/physiopathology
Stretch/genetics/physiology
Survival Analysis
Survival Rate