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Title: Pattern of Peripheral Nerve Involvement in Machado-Joseph Disease: Neuronopathy or Distal Axonopathy? A Clinical and Neurophysiological Evaluation
Authors: Escorcio Bezerra, Marcio Luiz [UNIFESP]
Pedroso, Jose Luiz [UNIFESP]
Pinheiro, Denise Spinola [UNIFESP]
Braga-Neto, Pedro [UNIFESP]
Barsottini, Orlando Graziani Povoas [UNIFESP]
Oliveira Braga, Nadia Iandoli de [UNIFESP]
Manzano, Gilberto Mastrocola [UNIFESP]
Universidade Federal de São Paulo (UNIFESP)
Keywords: Machado-Joseph disease
Spinocerebellar ataxia type 3
Length-dependent distal axonopathy
Issue Date: 1-Jan-2013
Publisher: Karger
Citation: European Neurology. Basel: Karger, v. 69, n. 3, p. 129-133, 2013.
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
ISSN: 0014-3022
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