J Clin Neurol.  2018 Jul;14(3):401-406. 10.3988/jcn.2018.14.3.401.

Clinical Heterogeneity of Anti-GM2-Ganglioside-Antibody Syndrome

• 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

• KMID: 2415059
• DOI: http://doi.org/10.3988/jcn.2018.14.3.401

Abstract

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.

Keyword

Guillain-Barré syndrome; ganglioside; antibodies; GM2; Korea

MeSH Terms

Antibodies
Axons
Cranial Nerve Diseases
Dizziness
Guillain-Barre Syndrome
Humans
Immunoglobulins
Korea
Paralysis
Peripheral Nervous System Diseases
Population Characteristics*
Antibodies
Immunoglobulins

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