BACKGROUND: Conduction block is considered an essential finding for the distinction between motor neuropathies and lower motor neuron disorders. Only a small number of reports describe patients with multifocal motor neuropathies who lack overt conduction block, although in these cases other features of demyelination still suggest the presence of a demyelinating disorder. In contrast, a purely axonal multifocal motor neuropathy has not been described. METHODS: This report describes nine patients with slowly or nonprogressive multifocal motor neuropathies who had purely axonal electrodiagnostic features. RESULTS: GM1 antibodies titers were normal in all nine cases. Six patients were treated with either prednisone or IV immunoglobulin and three showed convincing improvement. CONCLUSIONS: These findings suggest an immune-mediated motor neuropathy with axonal electrophysiologic features that appears to be distinct from both multifocal motor neuropathy and established motor neuron disorders.
BACKGROUND:Conduction block is considered an essential finding for the distinction between motor neuropathies and lower motor neuron disorders. Only a small number of reports describe patients with multifocal motor neuropathies who lack overt conduction block, although in these cases other features of demyelination still suggest the presence of a demyelinating disorder. In contrast, a purely axonal multifocal motor neuropathy has not been described. METHODS: This report describes nine patients with slowly or nonprogressive multifocal motor neuropathies who had purely axonal electrodiagnostic features. RESULTS:GM1 antibodies titers were normal in all nine cases. Six patients were treated with either prednisone or IV immunoglobulin and three showed convincing improvement. CONCLUSIONS: These findings suggest an immune-mediated motor neuropathy with axonal electrophysiologic features that appears to be distinct from both multifocal motor neuropathy and established motor neuron disorders.
Authors: Nidhi Garg; Susanna B Park; Steve Vucic; Con Yiannikas; Judy Spies; James Howells; William Huynh; José M Matamala; Arun V Krishnan; John D Pollard; David R Cornblath; Mary M Reilly; Matthew C Kiernan Journal: J Neurol Neurosurg Psychiatry Date: 2016-12-21 Impact factor: 10.154
Authors: Todd D Levine; Jonathan S Katz; Richard Barohn; Leslie J Vaughan; Mazen M Dimachkie; David S Saperstein; Tahseen Mozaffar; Gil I Wolfe; Matthew S Mayo; Gary J Badger; Lara Katzin; Elissa Ritt; Michelle Greer; Joseph DiStefano; Patrick M Schmidt Journal: Neurol Clin Pract Date: 2018-10