Guillain-Barré syndrome with antibody to a ganglioside, N-acetylgalactosaminyl GD1a.
A retrospective case study of 33 Guillain-Barré syndrome (GBS) patients with the antibody to the ganglioside N-acetylgalactosaminyl GD1a (GalNAc-GD1a) was made to investigate the clinical features of GBS with this antibody. Patients were classified into three groups: (i) 25 with IgG antibody (group G, titre >/= 1 : 40); (ii) 16 with high-titre IgG antibody (group G-high, titre >/=1:320; selected from group G patients), and (iii) eight with IgM antibody but without elevation of IgG (group M, normal range <1:40 for both IgM and IgG). The control group consisted of 72 GBS patients without anti-GalNAc-GD1a antibody. Compared with the control group, the G-high and G group patients were characterized as having had antecedent gastrointestinal infection (87% and 72% versus 31%, both P < 0.001), uncommon cranial nerve involvement (19% and 36% versus 54%, P = 0.02 and 0.2, respectively), distal-dominant weakness (94% and 68% versus 36%, P < 0.001 and P = 0.01, respectively) and no sensory signs (81% and 60% versus 25%, P < 0.001 and P = 0.003, respectively). Electrophysiological findings indicative of axonal dysfunction were significantly more common in the G-high and G group patients (63% and 52% versus 14%, both P < 0.001). The pure motor variant that showed neither sensory signs nor abnormalities in sensory conduction studies was also more frequent in these groups (44% and 32% versus 9%, both P < 0.001). IgG anti-GalNAc-GD1a antibody may be a marker of the pure motor and the axonal variants of GBS, and therefore it, as well as anti-GM1 antibody, must be investigated in these forms in order to diagnose and understand the variants. By contrast, mild weakness, frequent facial palsy (75%) and a high incidence of IgM anti-GM2 antibody reactivity (88%) were characteristic of group M, indicating that the GBS in that group resulted from a different immune mechanism from that in the G group.
Kaida
K
K
Third Department of Internal Medicine, National Defense Medical College, University of Tokyo,Tokyo, Japan.
Kusunoki
S
S
Kamakura
K
K
Motoyoshi
K
K
Kanazawa
I
I
D000328:Adult; D001323:Autoantibodies / Q000097:blood*; D001369:Axons / Q000502:physiology; D002169:Campylobacter Infections / Q000150:complications; D016123:Campylobacter jejuni; D002240:Carbohydrate Sequence; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D005732:Gangliosides / Q000737:chemistry / Q000276:immunology*; D020275:Guillain-Barre Syndrome / Q000097:blood / Q000276:immunology* / Q000503:physiopathology; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood*; D007075:Immunoglobulin M / Q000097:blood*; D008297:Male; D008969:Molecular Sequence Data; D009046:Motor Neurons / Q000502:physiology; D009431:Neural Conduction; D012016:Reference Values; D012141:Respiratory Tract Infections / Q000150:complications
D001323:Autoantibodies; D005732:Gangliosides; D007074:Immunoglobulin G; D007075:Immunoglobulin M; C055253:IV(4)-galactosyl-N-acetylganglioside GD1a