Cognitive dysfunction in hereditary spastic paraplegias and other motor neuron disorders

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Main Author: Ingrid Faber
Format: Artículo científico
Language:en
Published: Associação Neurologia Cognitiva e do Comportamento 2016
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author Ingrid Faber
author_facet Ingrid Faber
contents Cognitive dysfunction in hereditary spastic paraplegias and other motor neuron disorders Ingrid Faber Lucas Melo T. Branco Marcondes Cavalvante França Júnior Medicina dementia cognition mental retardation Hereditary spastic paraplegia Hereditary spastic paraplegia (HSP) is a diverse group of single-gene disorders that share the predominant clinical feature of progressive lower limb spasticity and weakness. More than 70 different genetic subtypes have been described and all modes of inheritance are possible. Intellectual dysfunction in HSP is frequent in recessive forms but rare in dominant families. It may manifest by either mental retardation and/or cognitive decline. The latter may be subtle, restricted to executive dysfunction or may evolve to severe dementia. The cognitive profile is thought to depend largely on the genetic subtype of HSP, although wide phenotypic variability within the same genetic subtype and also within the same family can be found. 2016 artículo científico 1980-5764 https://www.redalyc.org/articulo.oa?id=339548909005 en http://www.redalyc.org/revista.oa?id=3395 Dementia & Neuropsychologia application/pdf Associação Neurologia Cognitiva e do Comportamento Dementia & Neuropsychologia (Brasil) Num.4 Vol.10
format Artículo científico
id redalyc_339548909005
language en
publishDate 2016
publisher Associação Neurologia Cognitiva e do Comportamento
spellingShingle Cognitive dysfunction in hereditary spastic paraplegias and other motor neuron disorders
Ingrid Faber
Medicina
dementia
cognition
mental retardation
Hereditary spastic paraplegia
Cognitive dysfunction in hereditary spastic paraplegias and other motor neuron disorders Ingrid Faber Lucas Melo T. Branco Marcondes Cavalvante França Júnior Medicina dementia cognition mental retardation Hereditary spastic paraplegia Hereditary spastic paraplegia (HSP) is a diverse group of single-gene disorders that share the predominant clinical feature of progressive lower limb spasticity and weakness. More than 70 different genetic subtypes have been described and all modes of inheritance are possible. Intellectual dysfunction in HSP is frequent in recessive forms but rare in dominant families. It may manifest by either mental retardation and/or cognitive decline. The latter may be subtle, restricted to executive dysfunction or may evolve to severe dementia. The cognitive profile is thought to depend largely on the genetic subtype of HSP, although wide phenotypic variability within the same genetic subtype and also within the same family can be found. 2016 artículo científico 1980-5764 https://www.redalyc.org/articulo.oa?id=339548909005 en http://www.redalyc.org/revista.oa?id=3395 Dementia & Neuropsychologia application/pdf Associação Neurologia Cognitiva e do Comportamento Dementia & Neuropsychologia (Brasil) Num.4 Vol.10
title Cognitive dysfunction in hereditary spastic paraplegias and other motor neuron disorders
topic Medicina
dementia
cognition
mental retardation
Hereditary spastic paraplegia
url https://www.redalyc.org/articulo.oa?id=339548909005