Pattern of Peripheral Nerve Involvement in Machado-Joseph Disease: Neuronopathy or Distal Axonopathy? A Clinical and Neurophysiological Evaluation

Pattern of Peripheral Nerve Involvement in Machado-Joseph Disease: Neuronopathy or Distal Axonopathy? A Clinical and Neurophysiological Evaluation

Author Escorcio Bezerra, Marcio Luiz Autor UNIFESP Google Scholar
Pedroso, Jose Luiz Autor UNIFESP Google Scholar
Pinheiro, Denise Spinola Autor UNIFESP Google Scholar
Braga-Neto, Pedro Autor UNIFESP Google Scholar
Barsottini, Orlando Graziani Povoas Autor UNIFESP Google Scholar
Oliveira Braga, Nadia Iandoli de Autor UNIFESP Google Scholar
Manzano, Gilberto Mastrocola Autor UNIFESP Google Scholar
Institution Universidade Federal de São Paulo (UNIFESP)
Abstract Objective: Neuropathy is a well-recognized feature in spinocerebellar ataxia type 3 (SCA3) or Machado-Joseph disease (MJD), but the pattern of neuropathy is still a matter of debate. This study aimed to evaluate peripheral nerve involvement in MJD patients. Neurophysiological and clinical data were analyzed to distinguish neuronopathy from length-dependent distal axonopathy. Methods: in the present study we evaluated 26 patients with clinical and molecular-proven MJD and investigated their peripheral nerve involvement. Neurophysiological and clinical data were compared and correlated aiming to distinguish neuronopathy from distal axonopathy. Results: the neurophysiological evaluation showed that 42.3% of the patients had polyneuropathy. Among these patients, 81.8% presented neuronopathy. Conclusion: We concluded that neuronopathy is the most common form of peripheral nerve involvement in MJD patients. Copyright (C) 2012 S. Karger AG, Basel
Keywords Machado-Joseph disease
Spinocerebellar ataxia type 3
Neuronopathy
Neuropathy
Length-dependent distal axonopathy
Language English
Date 2013-01-01
Published in European Neurology. Basel: Karger, v. 69, n. 3, p. 129-133, 2013.
ISSN 0014-3022 (Sherpa/Romeo, impact factor)
Publisher Karger
Extent 129-133
Origin http://dx.doi.org/10.1159/000345274
Access rights Closed access
Type Article
Web of Science ID WOS:000316589900001
URI http://repositorio.unifesp.br/handle/11600/35843

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