title: Pattern of peripheral nerve involvement in Machado-Joseph disease: neuronopathy or distal axonopathy? A clinical and neurophysiological evaluation.
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.
  
  
  CONCLUSIONS
  We concluded that neuronopathy is the most common form of peripheral nerve involvement in MJD patients.
authors.affiliation: Department of Neurology and Neurosurgery, Universidade Federal de São Paulo, São Paulo, Brazil.
authors.forename: Marcio Luiz
authors.initials: ML
authors.lastname: Escorcio Bezerra
authors.forename: José Luiz
authors.initials: JL
authors.lastname: Pedroso
authors.forename: Denise Spinola
authors.initials: DS
authors.lastname: Pinheiro
authors.forename: Pedro
authors.initials: P
authors.lastname: Braga-Neto
authors.forename: Orlando Graziani
authors.initials: OG
authors.lastname: Povoas Barsottini
authors.forename: Nadia Iandoli de Oliveira
authors.initials: NI
authors.lastname: Braga
authors.forename: Gilberto Mastrocola
authors.initials: GM
authors.lastname: Manzano
mesh_terms: D000328:Adult; D000368:Aged; D001369:Axons / Q000473:pathology*; D004576:Electromyography; D006801:Humans; D017827:Machado-Joseph Disease / Q000473:pathology* / Q000503:physiopathology; D008875:Middle Aged; D009431:Neural Conduction; D009474:Neurons / Q000473:pathology*; D010525:Peripheral Nerves / Q000473:pathology*