[Multifocal motor neuropathies with conduction blocks. 39 cases].
Clinical, biological and electrophysiological features from a cohort of 39 multifocal motor neuropathies with conduction blocks (NMM with CB) have been studied. There were 29 males and 10 females with an average of 47.3. At the first evaluation, the mean duration of the symptoms was of 8 years with extremes between 1 and 28. Pain and paresthesias were present in respectively 10 and 18 p. 100 of the patients. Fasciculations and cramps were observed in more than 2/3 of the cases. Three patients had tremor at rest. Upper limb muscular weakness was the predominant initial symptom (84.6 p. 100). The weakness always affected distal and unilateral muscles. Radial and cubital nerve distribution are mainly affected and in half of the cases an unilateral motor deficit in the lower limb was associated. Muscle atrophy was frequent (74 p. 100) and rapidly developed in the first 2 years. Reflexes were decreased or absent in 64 p. 100. In 78 p. 100 of cases, biological study showed normal serum immunoelectrophoresis and CSF. IgM anti-GM1 antibodies were found in 24/36 patients. Very high titres were found in 5 cases. All patients had CB in upper limbs. The preferential localizations of the CB were equally at the median and ulnar nerves. Only 7 patients had CB localized to the lower limbs. In many cases, marked reduction of the motor amplitude prevented the detection of CB, marked reduction of the motor amplitude prevented the detection of CB. Moderate fibrillation potentials were found in 28 p. 100 of patients. Giant muscular unit potentials were frequent (21/39). F-waves in nerve with CB were always abnormal with marked increased latencies. Late responses sometimes seemed to be repeater F-waves. Axon reflexes were detected in 5 cases. The late responses abnormalities could precede the block. Clinical, biological and electrophysiological described arguments could may distinguish NMM with CB from motor neuron disease and relate them to the group of chronic demyelinating neuropathies.
Service d'Explorations fonctionnelles, Neurologie, Hôpital de la Salpêtrière, Paris.
N
N
Le Forestier
B
B
Chassande
A
A
Moulonguet
T
T
Maisonobe
S
S
Schaeffer
N
N
Birouk
N
N
Baumann
D
D
Adams
J M
JM
Léger
V
V
Meininger
G
G
Said
P
P
Bouche
D000328:Adult; D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000276:immunology / Q000503:physiopathology; D001132:Arm / Q000294:innervation; D003711:Demyelinating Diseases / Q000276:immunology / Q000503:physiopathology; D004594:Electrophysiology; D005260:Female; D006801:Humans; D007866:Leg / Q000294:innervation; D008297:Male; D008875:Middle Aged; D016472:Motor Neuron Disease / Q000276:immunology / Q000503:physiopathology*; D009431:Neural Conduction; D012189:Retrospective Studies; D013997:Time Factors