N. Yuki et al., Clinical features and response to treatment in Guillain-Barre syndrome associated with antibodies to GM1b ganglioside, ANN NEUROL, 47(3), 2000, pp. 314-321
GM1b is a minor ganglioside in human peripheral nerves. Serum anti-GM1b ant
ibodies frequently are present in patients with Guillain-Barre syndrome (GB
S). In this collaborative study, we investigated the antecedent infections,
clinical features, and response to treatment of GBS patients with anti-GM1
b antibodies. Of 132 GBS patients who participated in the Dutch GBS trial t
hat compared the effect of intravenous immunoglobulins and plasma exchange,
25 (19%) patients had anti-GM1b antibodies. IgM antibodies were present in
14, IgG antibodies in 15, and both isotypes in 4 patients. The 25 patients
with anti-GM1b antibodies had a clinical pattern distinct from that of the
other 107 GBS patients. They more often had an episode of gastrointestinal
illness and frequently showed serological evidence of recent infection by
Campylobacter jejuni. The anti-GM1b-positive subgroup was marked by more ra
pidly progressive, more severe, and predominantly distal weakness. Cranial
nerve involvement and sensory deficits were less common in the patients wit
h anti-GM1b antibodies. The presence of anti-GM1b antibodies was associated
with slower recovery. The clinical manifestations predominantly were assoc
iated with anti-GM1b antibodies of the IgG isotype. Fourteen (56%) of the 2
5 patients with anti-GM1b antibodies also had anti-GM1 antibodies. The grou
p of patients with both antibodies was clinically more homogeneous and had
a more rapidly progressive, pure motor neuropathy. The subgroup of anti-GM1
b-positive GBS patients responded well to treatment with immunoglobulins bu
t not to plasmapheresis. The distinctive clinical features of the patients
with anti-GM1b antibodies show that acute motor neuropathy represents a spe
cific subgroup within GBS and that recognizing these patients may have cons
equences as to the choice of therapy.