Clinical Information

Peripheral neuropathies are a group of disorders that results from lesions on peripheral nerves. Patients with a peripheral neuropathy can have symptoms of weakness, sensory loss, and/or autonomic dysfunction. The causes of acquired peripheral neuropathies are varied, and include vitamin deficiencies, metabolic abnormalities, infections, malignancies (paraneoplastic disorders), and autoimmune diseases. A subset of the autoimmune-mediated peripheral neuropathies is associated with the presence of circulating autoantibodies that bind to specific gangliosides. Gangliosides are glycosphingolipids that contain sialic acid residues. Although present in the plasma membranes of many cell types, gangliosides are particularly abundant in neural tissue.

Guillain-Barre syndrome is one class of autoimmune peripheral neuropathies, and comprises a spectrum of disorders including acute inflammatory demyelinating polyradiculoneuropathy, acute motor axonal neuropathy, and acute motor and sensory axonal neuropathy. This class of autoimmune neuropathies is generally characterized by an acute onset. Although the diagnosis of these disorders is based significantly on clinical evaluation and electrophysiologic studies, assessment of ganglioside antibodies, particularly against GM1, asialo GM1, and GD1b, can provide useful information. It is thought that the Guillain-Barre syndrome disorders are triggered by an infection, which results in production of infection-associated lipooligosaccharide-specific antibodies. These antibodies subsequently bind to endogenous gangliosides, due to molecular mimicry, which leads to immune-mediate damage to the peripheral nerves, ultimately resulting in the clinical symptoms associated with the disorders.(1)