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Clinical Heterogeneity of Anti-GM2-Ganglioside-Antibody Syndrome
#MMPMID29971981
Kim JK
; Kim YH
; Yoon BA
; Cho JY
; Oh SY
; Shin HY
; Kim JS
; Park KH
; Kim SY
; Suh BC
; Seok HY
; Yoo JH
; Bae JS
J Clin Neurol
2018[Jul]; 14
(3
): 401-406
PMID29971981
show ga
BACKGROUND AND PURPOSE: Antiganglioside antibodies are known to play a pathogenic
role in Guillain-Barré syndrome (GBS). Either an immunoglobulin (Ig)G- or
IgM-type anti-GM2 antibody is detected in rare cases in GBS patients. However,
the specific pathogenic role of these antibodies in GBS has not been reported
previously. This study aimed to define and characterize the clinical spectrum of
GBS with anti-GM2 positivity. METHODS: We reviewed the database of the Dong-A
University Neuroimmunology Team, which has collected sera of GBS and its variants
from more than 40 general and university-based hospitals in Korea. Detailed
information about the involved patients was often obtained and then corrected by
the charge doctor applying additional questionnaires. RESULTS: Four patients with
acute monophasic peripheral neuropathy or cranial neuropathy with isolated
IgM-type anti-GM2-antibody positivity were recruited. In addition, IgG-type
anti-GM2 antibody was solely detected in the sera of another four patients. The
IgM-positive group comprised heterogeneous syndromes: two cases of acute motor
axonal neuropathy, one of acute inflammatory demyelinating polyneuropathy, and
one of isolated facial diplegia. In contrast, all of the cases enrolled in the
IgG-positive group manifested with dizziness with or without oculomotor palsy due
to cranial neuropathy syndrome. CONCLUSIONS: This study has identified that
anti-GM2 antibody can be found in various subtypes of GBS and its variants in
rare cases. Compared to the clinical heterogeneity of the IgM-positive group, the
IgG-positive group can be characterized by cranial-dominant GBS variants
presenting mainly with oculomotor and vestibular dysfunctions.
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