[Peripheral neuropathies simulating amyotrophic lateral sclerosis in gammopathies].
Particular clinical pictures of ALS may occur during the course of some "benign gammapathies". We observed 12 patients (age range 57 to 74 years; 9 men-3 women) with initially benign gammapathy (9 IgG, 1 IgA, 2 IgM) associated to a clinical picture of progressive anterior horn and pyramidal tract involvement. These cases led us to recognize some particularities of gammapathy-associated ALS: Relative frequency of asymmetrical clinical manifestations, rarity of bulbar signs; Decrease of sensory nerve conduction velocities without evidence of sensory clinical symptoms (8 out 9); Increase CSF protein content including the monoclonal component, axonal degeneration and immunostaining evidence of the paraprotein fixation observed on nerve biopsies (5 out 7 cases). Association of ALS and gammapathy is not fortuitous as shown by epidemiology, experiments, pathology and effects of different immunological treatments as related in this study. Demonstration of infra-clinical neuropathy face to a clinical syndrome of ALS should prompt too careful screening for a gammapathy.
Bady
B
B
Service d'Electromyographie, Hôpital Neurologique de Lyon.
Vial
C
C
Brudon
F
F
Lapras
J
J
Kopp
N
N
Trillet
M
M
D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000150:complications* / Q000473:pathology; D005260:Female; D006801:Humans; D006942:Hypergammaglobulinemia / Q000150:complications*; D007070:Immunoglobulin A; D007074:Immunoglobulin G; D007075:Immunoglobulin M; D008297:Male; D008875:Middle Aged; D008998:Monoclonal Gammopathy of Undetermined Significance / Q000150:complications* / Q000473:pathology
D007070:Immunoglobulin A; D007074:Immunoglobulin G; D007075:Immunoglobulin M
Neuropathies périphériques simulant une sclérose latérale amyotrophique au cours des gammapathies.