Abstract

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

Highlights

  • The authors present a Brazilian case series of eight patients with idiopathic very-late onset cerebellar ataxia, featuring predominantly gait ataxia, associated with cerebellar atrophy

  • Gait cerebellar ataxia was observed in all patients, as well as, cerebellar atrophy on brain MRI

  • Alternative terms have been used to describe to this group of patients, such as sporadic adult onset ataxia of unknown etiology (SAOA), or idiopathic late-onset pure cerebellar ataxia (ILOPCA), among others[1,2,6,7,8]

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Summary

Introduction

The 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. 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. Resumo Os autores apresentam uma série de casos incluindo oito pacientes com ataxia cerebellar de início muito tardio (média de 75,5 anos de idade) apresentando ataxia de marcha, associada à atrofia cerebelar. Late-onset cerebellar atrophy of Marie-Foix-Alajouanine is a sub-group of ILOCA patients with very late onset (IVLOCA)[5]. The objective of this study is to analyze the follow-up of 8 Brazilian patients with IVLOCA

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