2012/12/07 by Márcio Luiz Escórcio Bezerra, Marcio Luiz Escorcio Bezerra, José Luiz Pedroso +7
Medicine · Neuroscience · #Amyotrophic Lateral Sclerosis Research #Autoimmune Neurological Disorders and Treatments #Genetic Neurodegenerative Diseases
paper · doi:10.1159/000345274
openalex publication_date 2012/12/07 · openalex created_date 2025/10/10 · openalex updated_date 2026/08/02
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.