Idiopathic very late-onset cerebellar ataxia: a Brazilian case series

ABSTRACTThe authors present a Brazilian case series of eight patients with idiopathic very-late onset (mean 75.5 years old) cerebellar ataxia, featuring predominantly gait ataxia, associated with cerebellar atrophy.Method: 26 adult patients with a diagnosis of idiopathic late onset cerebellar ataxia were analyzed in a Brazilian ataxia outpatient clinic and followed regularly over 20 years. Among them, 8 elderly patients were diagnosed as probable very late onset cerebellar ataxia. These patients were evaluated with neurological, ophthalmologic and Mini-Mental Status examinations, brain MRI, and EMG.Results: 62.5% of patients were males, mean age was 81.9 years-old, and mean age of onset was 75.5 years. Gait cerebellar ataxia was observed in all patients, as well as, cerebellar atrophy on brain MRI. Mild cognitive impairment and visual loss, due to macular degeneration, were observed in 50% of cases. Chorea was concomitantly found in 3 patients.Conclusion: We believe that this condition is similar the one described by Marie-Foix-Alajouanine presenting with mild dysarthria, associated with gait ataxia, and some patients had cognitive dysfunction and chorea.

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Main Authors: Teive,Hélio A. G., Moscovich,Mariana, Moro,Adriana, Farah,Marina, Arruda,Walter O., Munhoz,Renato P.
Format: Digital revista
Language:English
Published: Academia Brasileira de Neurologia - ABNEURO 2015
Online Access:http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2015001100903
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spelling oai:scielo:S0004-282X20150011009032015-10-22Idiopathic very late-onset cerebellar ataxia: a Brazilian case seriesTeive,Hélio A. G.Moscovich,MarianaMoro,AdrianaFarah,MarinaArruda,Walter O.Munhoz,Renato P. late-onset cerebellar ataxia cerebellar atrophy idiopathic late-onset cerebellar ataxia cerebellar atrophy of Marie-Foix-Alajouanine ABSTRACTThe authors present a Brazilian case series of eight patients with idiopathic very-late onset (mean 75.5 years old) cerebellar ataxia, featuring predominantly gait ataxia, associated with cerebellar atrophy.Method: 26 adult patients with a diagnosis of idiopathic late onset cerebellar ataxia were analyzed in a Brazilian ataxia outpatient clinic and followed regularly over 20 years. Among them, 8 elderly patients were diagnosed as probable very late onset cerebellar ataxia. These patients were evaluated with neurological, ophthalmologic and Mini-Mental Status examinations, brain MRI, and EMG.Results: 62.5% of patients were males, mean age was 81.9 years-old, and mean age of onset was 75.5 years. Gait cerebellar ataxia was observed in all patients, as well as, cerebellar atrophy on brain MRI. Mild cognitive impairment and visual loss, due to macular degeneration, were observed in 50% of cases. Chorea was concomitantly found in 3 patients.Conclusion: We believe that this condition is similar the one described by Marie-Foix-Alajouanine presenting with mild dysarthria, associated with gait ataxia, and some patients had cognitive dysfunction and chorea.info:eu-repo/semantics/openAccessAcademia Brasileira de Neurologia - ABNEUROArquivos de Neuro-Psiquiatria v.73 n.11 20152015-11-01info:eu-repo/semantics/articletext/htmlhttp://old.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2015001100903en10.1590/0004-282X20150139
institution SCIELO
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country Brasil
countrycode BR
component Revista
access En linea
databasecode rev-scielo-br
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region America del Sur
libraryname SciELO
language English
format Digital
author Teive,Hélio A. G.
Moscovich,Mariana
Moro,Adriana
Farah,Marina
Arruda,Walter O.
Munhoz,Renato P.
spellingShingle Teive,Hélio A. G.
Moscovich,Mariana
Moro,Adriana
Farah,Marina
Arruda,Walter O.
Munhoz,Renato P.
Idiopathic very late-onset cerebellar ataxia: a Brazilian case series
author_facet Teive,Hélio A. G.
Moscovich,Mariana
Moro,Adriana
Farah,Marina
Arruda,Walter O.
Munhoz,Renato P.
author_sort Teive,Hélio A. G.
title Idiopathic very late-onset cerebellar ataxia: a Brazilian case series
title_short Idiopathic very late-onset cerebellar ataxia: a Brazilian case series
title_full Idiopathic very late-onset cerebellar ataxia: a Brazilian case series
title_fullStr Idiopathic very late-onset cerebellar ataxia: a Brazilian case series
title_full_unstemmed Idiopathic very late-onset cerebellar ataxia: a Brazilian case series
title_sort idiopathic very late-onset cerebellar ataxia: a brazilian case series
description ABSTRACTThe authors present a Brazilian case series of eight patients with idiopathic very-late onset (mean 75.5 years old) cerebellar ataxia, featuring predominantly gait ataxia, associated with cerebellar atrophy.Method: 26 adult patients with a diagnosis of idiopathic late onset cerebellar ataxia were analyzed in a Brazilian ataxia outpatient clinic and followed regularly over 20 years. Among them, 8 elderly patients were diagnosed as probable very late onset cerebellar ataxia. These patients were evaluated with neurological, ophthalmologic and Mini-Mental Status examinations, brain MRI, and EMG.Results: 62.5% of patients were males, mean age was 81.9 years-old, and mean age of onset was 75.5 years. Gait cerebellar ataxia was observed in all patients, as well as, cerebellar atrophy on brain MRI. Mild cognitive impairment and visual loss, due to macular degeneration, were observed in 50% of cases. Chorea was concomitantly found in 3 patients.Conclusion: We believe that this condition is similar the one described by Marie-Foix-Alajouanine presenting with mild dysarthria, associated with gait ataxia, and some patients had cognitive dysfunction and chorea.
publisher Academia Brasileira de Neurologia - ABNEURO
publishDate 2015
url http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2015001100903
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AT farahmarina idiopathicverylateonsetcerebellarataxiaabraziliancaseseries
AT arrudawaltero idiopathicverylateonsetcerebellarataxiaabraziliancaseseries
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