Multifocal motor neuropathy.
We present a review of the literature on multifocal motor neuropathy (MMN), a rare neurological disorder which has features in common with both chronic inflammatory demyelinating neuropathy and lower motor neuron disease. Clinically, MMN is characterised by slowly progressive asymmetrical limb weakness, usually most prominent in the forearms. Weakness may be associated with muscle wasting, fasciculations and decreased tendon reflexes. Serum anti-GM1 ganglioside antibody titres may be increased. The diagnostic hallmark of MMN is the electrophysiological demonstration of persistent localised motor conduction blocks, with otherwise normal or near-normal motor and sensory conduction velocities. The pathogenesis of MMN has not been elucidated completely. There is, however, substantial evidence for an autoimmune mechanism. Based on the possible involvement of the immune system in the pathogenesis of MMN the therapeutic efficacy of several immunomodulatory drugs has been tested. Treatment of MMN patients with human immunoglobulin or cyclophosphamide is usually followed by a marked improvement of strength. The finding that MMN is a potentially treatable disorder underscores the importance of distinguishing MMN from lower motor neuron disease, for which no effective therapy is currently available.
Biessels
G J
GJ
Department of Neurology, Rudolf Magnus Research School in the Neurosciences, University Hospital, Utrecht, The Netherlands.
Franssen
H
H
van den Berg
L H
LH
Gibson
A
A
Kappelle
L J
LJ
Venables
G S
GS
Wokke
J H
JH
D001706:Biopsy; D002908:Chronic Disease; D019411:Clinical Laboratory Techniques; D003131:Combined Modality Therapy; D003711:Demyelinating Diseases / Q000175:diagnosis* / Q000209:etiology / Q000503:physiopathology / Q000628:therapy; D003937:Diagnosis, Differential; D006801:Humans; D016472:Motor Neuron Disease / Q000175:diagnosis* / Q000209:etiology / Q000503:physiopathology / Q000628:therapy; D009431:Neural Conduction / Q000502:physiology