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.
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 MJDpatients. 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 MJDpatients.
Authors: Camila N Fahl; Lucas Melo T Branco; Felipe P G Bergo; Anelyssa D'Abreu; Iscia Lopes-Cendes; Marcondes C França Journal: Cerebellum Date: 2015-04 Impact factor: 3.847
Authors: Marcio Luiz Escorcio Bezerra; José Luiz Pedroso; Pedro Braga-Neto; Agessandro Abrahao; Marcus Vinicius Cristino de Albuquerque; Franklin Roberto Pereira Borges; Maria Luiza Saraiva-Pereira; Laura Bannach Jardim; Nadia Iandoli de Oliveira Braga; Gilberto Mastrocola Manzano; Orlando G P Barsottini Journal: Cerebellum Date: 2016-12 Impact factor: 3.847
Authors: Gabriel Vasata Furtado; Camila Maria de Oliveira; Gabriela Bolzan; Jonas Alex Morales Saute; Maria Luiza Saraiva-Pereira; Laura Bannach Jardim Journal: Genet Mol Biol Date: 2019-06-10 Impact factor: 1.771