Long term follow up of multifocal motor neuropathy with conduction block under treatment.
Eighteen patients (15 men, three women; age range 30 to 71 years, mean 45.8 years) with multifocal motor neuropathy treated with high dose intravenous immunoglobulin (IVIg) were evaluated for nine to 48 months (mean follow up 25.3 months). The median time between onset of multifocal motor neuropathy and treatment was 5.8 years. The dose of IVIg was 0.4 g/day for three to five days. The interval between each treatment was determined for each patient by the evaluation of the effect of the first course. Muscle strength was evaluated by a computerised analyser. Clinical improvement was seen in 12 patients treated with IVIg (67%). Isometric strength increased from 32% to 97% (mean 54.5%) of the initial value. Functional scales corroborated these findings. No clear predictive factors of response to IVIg was found except the presence of high titres of IgM anti-GM1 antibodies. Often, patients needed repeated courses of IVIg to maintain the improvement. In two patients, IVIg infusions were stopped without signs of relapse after one year. Four patients were initially treated with prednisone (1 mg/kg/day), without any clear improvement. Five patients with no response to IVIg or who were IVIg dependent were treated with cyclophosphamide, but only one showed improvement. These results show the long term benefits and safety of IVIg in multifocal motor neuropathy but also the transient effect of this expensive treatment in most patients.
Azulay
J P
JP
Service de Neurologie et Maladies Neuromusculaires, CHU de la Timone, Marseille, France.
Rihet
P
P
Pouget
J
J
Cador
F
F
Blin
O
O
Boucraut
J
J
Serratrice
G
G
D000328:Adult; D000368:Aged; D000906:Antibodies / Q000032:analysis; D005260:Female; D005500:Follow-Up Studies; D005677:G(M1) Ganglioside / Q000276:immunology; D006801:Humans; D007075:Immunoglobulin M / Q000032:analysis; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D007537:Isometric Contraction / Q000187:drug effects; D008297:Male; D008875:Middle Aged; D016472:Motor Neuron Disease / Q000503:physiopathology / Q000628:therapy*; D009431:Neural Conduction; D010523:Peripheral Nervous System Diseases / Q000503:physiopathology / Q000628:therapy*; D013718:Tensile Strength / Q000187:drug effects / Q000502:physiology; D016896:Treatment Outcome
D000906:Antibodies; D007075:Immunoglobulin M; D016756:Immunoglobulins, Intravenous; D005677:G(M1) Ganglioside