[Multifocal motor neuropathy with persistent conduction blocks: 18 years on].
Multifocal motor neuropathy with persistent conduction blocks was firstly reported in 1986 and outlined from the group of purely motor diseases of the peripheral nervous system. The main criterion is the presence of conduction blocks located only on the motor nerves; additionally 30 percent of patients have IgM subclass serum antibodies directed against GM1 ganglioside. The clinical picture is a multifocal, asymmetrical, neuropathy, starting and predominant in the upper limbs, occurring in males aged 50 years and more, and having a progressive course. There is no biological sign besides elevated anti-GM1 antibodies. CSF analysis discloses mild increased protein count. The course is unpredictable, the neuropathy may be strictly limited to one or two motor nerves, or spread to other motor nerves in the four limbs. There is no involvement of the sensory and the cranial nerves, no involvement of the autonomic and the central nervous system. The pathophysiology is unknown, animal models do not allow to confirm the role of humoral immunity, and the role of anti-GM1 antibodies is controversial. Randomized controlled trials have assessed the efficacy of intravenous immunoglobulins which dramatically improve strength in 70-80 percent of patients in the short term, but remain unable to prevent motor deterioration in most patients, together with the occurrence of new conduction blocks. Corticosteroids and plasma exchanges do not improve the patients and may be followed by transient worsening. Long-term efficacy of immunosuppressive agents is not known.
Léger
J-M
JM
Groupe Neuropathies Périphériques Pitié-Salpêtrière, Hôpital Pitié-Salpêtrière, Paris. jean-marc.leger@psl.ap-hop-paris.fr
D003937:Diagnosis, Differential; D018450:Disease Progression; D004568:Electrodiagnosis; D006801:Humans; D016472:Motor Neuron Disease / Q000175:diagnosis / Q000276:immunology / Q000503:physiopathology* / Q000628:therapy; D009046:Motor Neurons / Q000502:physiology; D009431:Neural Conduction / Q000502:physiology*