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Pattern of peripheral nerve involvement in Machado-Joseph disease: neuronopathy or distal axonopathy? A clinical and
Marcio Luiz Escorcio Bezerra1, José Luiz Pedroso, Denise Spinola Pinheiro
1Department of Neurology and Neurosurgery, Universidade Federal de São Paulo, São Paulo, Brazil.
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.
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