Anti-GalNAc-GD1a antibody-associated Guillain-Barre syndrome with a predominantly distal weakness without cranial nerve impairment and sensory disturbance

The serum antibodies to N-acetylgalactosaminyl GD1a (GalNAc-GD1a) and other gangliosides as well as to Campy-lobacter jejuni were determined in 147 pa tients with Guillain-Barre syndrome (GBS), We found a distinctive clinical pattern in patients with anti-GalNAc-GD1a antibodies compared with those wi thout the antibodies, that is, lack of cranial nerve involvement (87% versu s 38%), distal-dominant weakness (80% versus 25%), and no sensory disturban ce (73% versus 22%). The frequency of distal-dominant weakness was signific antly higher in patients with both C jejuni infection and anti-GalNAc-GD1a positivity (100%) than in C jejuni-negative/anti-GalNAc-GD1a-positive (25%) , C jejuni-positive/anti-GalNAc-GD1a-negative (32%) and C jejuni-negative/a nti-GalNAc-GD1a-negative patients (20%). Lack of cranial nerve involvement and sensory disturbance were found in most C jejuni-positive/anti-GalNAc-GD 1a-positive and C jejuni-negative/anti-GalNAc-GD1a-positive patients, but n ot in C jejuni-positive/anti-GalNAc-GD1a-negative and C jejuni-negative/ant i-GalNAc-GD1a-negative patients. Although the anti-GM l-positive/anti-GalNA c-GD1a-negative patients mostly (75%) lacked cranial nerve involvement, dis tal-dominant weakness (38%) and lack of sensory disturbance (13%) were infr equent. These results may indicate that (1) the combination of C jejuni inf ection and anti-GalNAc-GD1a antibodies, but not anti-GalNAc-GD1a, anti-GM1, or C jejuni infection alone, is associated with a predominantly distal wea kness, (2) the presence of anti-GalNAc-GD1a, rather than C jejuni infection or anti-GM1 antibody, is associated with a lack of sensory disturbance, (3 ) both anti-GalNAc-GD1a and anti-GM1 antibodies are independently associate d with a lack of cranial nerve impairment.