ganglioside--gd1a and Acute-Disease

We describe a patient with acute oropharyngeal-facial diplegia, tongue palsy and albuminocytological dissociation following upper respiratory tract infection. Electrophysiological abnormalities in blink reflex suggested a brainstem lesion. High titers of anti-GM3, GD1a and GT1b IgG class serum antibodies were initially detected. Absorption studies indicated that antibodies were directed to a common terminal epitope NeuNAc(alfa 2-3)Gal. This novel antiganglioside antibody specificity may play a role in this unusual regional form of acute bulbar palsy of possible central origin. These data are supportive for extending the panel of antiganglioside specificities with anti-GM3.

Topics: Acute Disease; Antibody Specificity; Bulbar Palsy, Progressive; Enzyme-Linked Immunosorbent Assay; G(M3) Ganglioside; Gangliosides; Humans; Male; Middle Aged

Recent years have seen major progress in our understanding of the clinical pathophysiology of autoimmune neuropathies particularly with the identification and analysis of antibodies to gangliosides and related glycolipids in the serum of patients. Anti-glycolipid antibodies react with epitopes on the carbohydrate region of glycolipid molecules and can be routinely measured by standard immunoassays. In multifocal motor neuropathy, IgM anti-GM1 antibodies that cross react with GD1b and asialo-GM1 are detectable in around 50p. 100 of cases. This condition may clinically resemble certain forms of lower motor neurone disease. IgM anti-GD1b antibodies are found in IgM paraproteinaemic neuropathy characterised by profound sensory ataxia. In the anti-myelin associated glycoprotein (anti-MAG) IgM paraproteinaemic neuropathy, antibodies also react with the acidic glycolipids, sulphated glucuronyl paragloboside and its higher lactosaminyl homologue (SGPG and SGPLG). Thus a variety of chronic syndromes can be defined by their anti-glycolipid antibody profile. In Guillain-Barré syndrome, anti GM1, GM1b, GD1a and GalNAc-GD1a antibodies are found in patients with acute motor axonal neuropathy (AMAN) and anti-GQ1b IgG antibodies are a very sensitive and specific marker for the Miller Fisher syndrome. Many other anti-glycolipid antibodies are being increasingly identified in other neuropathy subtypes. The article will summarise existing clinical and serological information in this field.

Topics: Acute Disease; Antibodies; Autoimmune Diseases of the Nervous System; beta-Galactosidase; Chronic Disease; Gangliosides; Globosides; Humans

Acute motor axonal neuropathy (AMAN) is associated with high titer anti-GD1a antibodies. We have found that very high titer IgG anti-GD1a antibodies (Ab) from one AMAN patient selectively bind to motor, but not sensory, nerve nodes of Ranvier. Binding is abolished by preadsorption with GD1a. Sera negative for Ab do not immunostain motor and sensory nerve roots. We have also found that botulinum toxin A (BTA), which binds to GD1a, stains both motor and sensory nerve nodes of Ranvier. Our results strongly support the pathogenetic role of anti-GD1a antibodies in AMAN. Why BTA also binds to sensory fibers still remains to be elucidated, although the different size of BTA and its specificity to other gangliosides present in sensory axons might represent important factors.

Topics: Acute Disease; Antibody Specificity; Autoantibodies; Binding, Competitive; Botulinum Toxins, Type A; Fluorescent Antibody Technique; Gangliosides; Humans; Immunoglobulin G; Motor Neuron Disease; Motor Neurons; Neuromuscular Agents; Neurons, Afferent; Ranvier's Nodes; Spinal Nerve Roots

We report cases of 2 patients with pure motor Guillain-Barré syndrome of explosive onset who required mechanical ventilation for more than 2 months. Their electrophysiologic findings and poor clinical recoveries suggested severe axonal degeneration involving the motor nerves. Enzyme-linked immunosorbent assay and thin-layer chromatogram-immunostaining showed the sera of both patients had high IgG antibody titer against GD1a ganglioside. Their titers decreased with the clinical course of the illness. GD1a as well as GM1, appears to be the target pathogenic antigen in motor axon disorders. Elevated IgG anti-GD1a antibody titer may prove useful for predicting severe GBS.

Topics: Acute Disease; Adult; Axons; Campylobacter Infections; Campylobacter jejuni; Chromatography, Thin Layer; Enzyme-Linked Immunosorbent Assay; Gangliosides; Humans; Immunoglobulin G; Male; Nerve Degeneration; Neural Conduction; Polyradiculoneuropathy