Klinische Neurophysiologie 2013; 44 - P93
DOI: 10.1055/s-0033-1337234

Ganglioside Antibodies in Amyotrophic Lateral Sclerosis

K Kollewe 1, U Wurster 1, T Sinzenich 1, B Mohammadi 2, S Körner 1, R Dengler 1, S Petri 1
  • 1Medizinische Hochschule Hannover, Neurologie mit klinischer Neurophysiologie, Hannover, Deutschland
  • 2International Neuroscience Institute Hannover, Neurologie, Hannover, Deutschland

Introduction: Amyotrophic Lateral Sclerosis (ALS) is a devastating neurodegenerative disorder with typical onset in the 5th- 6th decade of life. The earlier hypothesis of an autoimmune origin of ALS receives less attention today, but immunological phenomena still seem to be involved and mechanisms such as protective autoimmunity may be important. Antibodies against a variety of gangliosides also occur in ALS, but widely differing frequencies and titers have been reported. We investigated the presence of IgG and IgM antibodies ALS patients.

Methods: IgG and IgM antibodies to the six gangliosides asialoGM1 (GA1), GM1, GM2, GD1a, GD1b, GQ1b were determined by GanglioCombi ELISA in sera of 84 ALS patients (45 men, 39 women) with a mean age of 58 (28 – 81). Results were expressed as a % ratio of a highly positive GM1 control and categorized as negative (100%). The values obtained from 220 Swiss blood donors (mean age: 52, 18 – 70 years, 128 men, 92 women) served as a reference group.

Results: Twenty-two (26.2%) out of 84 ALS-patients showed elevated ganglioside antibodies: Taking all subspecific antibodies together, IgG antibodies were found in 9/84 (10,7%) and IgM in 15/84 (17.9%) patients. As 2 patients exhibited both isotypes, the combined frequency is 22/84 (26.2%). Three simultaneous antibodies occurred in 1 patient each for IgG (GA1, GD1a, GQ1b) and IgM (GM1, GD1a, GD1b). However, the frequencies for the individual antibodies were rather low, with maximal 7.1% (6/84) for IgG GD1a and 8.3% (7/84) for IgM GA1. Strongly positive values were found in 4 patients, 2 for IgG (GA1, GD1a) and 2 for IgM (GA1 and GM1). There was no statistically significant difference to the collective of normal blood donors. Within the 84 ALS patients, there were no statistically significant differences in age, gender, site of onset, clinical presentation and survival, between patients with or without elevated ganglioside-antibody-titres.

Conclusions: Several studies with controversial results have assessed a potential association between elevated ganglioside-antibody-titres in ALS patients and specific disease phenotypes. While the existing literature, mainly GM1- ganglioside antibodies were analysed, we used a novel assay to detect six subspecific IgM and IgG antibodies. Even with this more thorough approach, ganglioside antibody frequencies and patterns in our ALS cohort closely resemble the values observed in healthy controls, and the presence of ganglioside antibody was not correlated with age, gender, ALS phenotype or survival.