Distal acquired demyelinating symmetric neuropathy associated with anti-GM1 antibodies: is this a CIDP variant?
Distal acquired demyelinating symmetric (DADS) neuropathy is clinically characterised by distal motor and sensory disturbances. Typically DADS does not respond or responds poorly to intravenous immunoglobulins (IVIg). We report the case of a 58-year-old patient who developed distal paraparesis. Serum electrophoresis demonstrated monoclonal IgM paraproteinemia having an anti-GM1 but no anti-MAG activity. Conduction velocities showed demyelinating pattern. Work-up excluded a lymphoproliferative disorder After IVIg treatment we observed a clinical and neurophysiological improvement. Regarding these peculiar findings, we suggest that DADS needs to be splitted in several forms determined among others by clinical, neurophysiological and antiganglioside profile and therapeutic response. We advocate to perform systematic antiganglioside antibodies assay additionnaly to anti-MAG when DADS is suspected in order to improve dysimmune neuropathies classification.
Department of Neurology, Hôpital Erasme, Université Libre de Bruxelles, Brussels, Belgium. gremiche@ulb.ac.be
Gauthier
G
Remiche
Alain
A
Kentos
Nicolas
N
Mavroudakis
D000888:Antibodies, Anti-Idiotypic / Q000097:blood*; D003711:Demyelinating Diseases / Q000097:blood* / Q000175:diagnosis* / Q000188:drug therapy / Q000503:physiopathology; D005677:G(M1) Ganglioside / Q000276:immunology*; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000494:pharmacology / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D009431:Neural Conduction / Q000187:drug effects / Q000502:physiology; D020277:Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / Q000097:blood / Q000145:classification* / Q000276:immunology
D000888:Antibodies, Anti-Idiotypic; D016756:Immunoglobulins, Intravenous; D005677:G(M1) Ganglioside