Pathophysiology of multifocal motor neuropathy
نویسنده
چکیده
Multifocal motor neuropathy is a unique disease that not only challenges the electromyographer to make differential diagnosis from a fatal disease but also provides an opportunity to reconsider the mechanism of conduction block in demyelinating neuropathies. Conduction block or slowing is not always a consequence of demyelination, but can be due to membrane hyperpolarisation or depolarisation, as well as sodium channel blockage. Recent pieces of evidence suggest that clinical features of multifocal motor neuropathy, such as fasciculations, muscle fatigue, cold paralysis, sensory sparing and secondary axonal degeneration, can be explained by depolarisation and hyperpolarisation block in addition to focal demyelination.
منابع مشابه
Cytokine profiles in multifocal motor neuropathy and progressive muscular atrophy
Multifocal motor neuropathy (MMN) and progressive muscular atrophy (PMA) are associated with IgM monoclonal gammopathy or the presence IgM anti-GM1-antibodies. To further investigate the pathophysiology of MMN and PMA we determined concentrations of 16 mainly B-cell associated inflammatory markers in serum from 25 patients with MMN, 55 patients with PMA, 25 patients with amyotrophic lateral scl...
متن کاملContactin-1 and Neurofascin-155/-186 Are Not Targets of Auto-Antibodies in Multifocal Motor Neuropathy
Multifocal motor neuropathy is an immune mediated disease presenting with multifocal muscle weakness and conduction block. IgM auto-antibodies against the ganglioside GM1 are detectable in about 50% of the patients. Auto-antibodies against the paranodal proteins contactin-1 and neurofascin-155 and the nodal protein neurofascin-186 have been detected in subgroups of patients with chronic inflamm...
متن کاملMultifocal motor neuropathy.
We report the case of a patient with multifocal motor neuropathy. Electrophysiological studies showed typical multifocal conduction blocks, but the search for anti-GM1 antibodies was negative. This case provide the heterogeneity of this clinical entity.
متن کاملAxonal multifocal motor neuropathy without conduction block or other features of demyelination.
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 ax...
متن کاملMultifocal acquired demyelinating sensory and motor neuropathy: report of a case and review of the literature.
Multifocal acquired demyelinating sensory and motor (MADSAM) neuropathy is characterized by an asymmetric multifocal pattern of motor and sensory loss, and conduction block and other features of demyelination in nerve conduction studies. MADSAM neuropathy needs to be differentiated from chronic inflammatory demyelinating polyneuropathy (CIDP) and multifocal motor neuropathy (MMN). In classic CI...
متن کامل