i(3)so3-galactosylceramide and Cryoglobulinemia

Mixed cryoglobulinemia (MC) is an immunological disorder characterized by immune-complex-mediated systemic vasculitis involving small vessels, which may present with renal, cutaneous, rheumatologic, and/or neurological manifestations. Until recently, the possible appearance of anti-neuronal autoantibodies in peripheral neuropathy occurring in the context of hepatitis C virus (HCV)-associated IgMk/IgG MC has not been extensively addressed. Therefore, a sample of these patients were evaluated by means of immuno-enzyme methods of anti-neuronal autoantibody detection. A significant increase in plasma titers of both anti-GM1 ganglioside and anti-sulfatide was observed. Abnormal titers were associated with evidence of active neuropathy as assessed by electrophysiologic studies. While peripheral neuropathy was traditionally thought to result from axonal ischemic damage caused by deposits of cryoprecipitable immune complexes in the vasa nervorum, a significant association between anti-GM1 and anti-sulfatide antibodies and involvement of the peripheral nervous system was observed in HCV-associated mixed IgMk/IgG cryoglobulinemia. Anti-neuronal reactivity could be a direct trigger of neurologic injury in this disorder.

Topics: Aged; Antigen-Antibody Complex; Autoantibodies; Cryoglobulinemia; Female; Ganglioside Galactosyltransferase; Hepacivirus; Hepatitis C; Humans; Male; Middle Aged; Paresthesia; Reperfusion Injury; Sulfoglycosphingolipids; Vasculitis

It is well known that polyneuropathy is associated with monoclonal IgM kappa.. We report the case of a 79-year-old man with lymphoma and motor neuron disease at cervical level simulating amyotrophic lateral sclerosis (ALS). Neurological deficit with inflammatory process evolved within 4 months. Electrophysiological findings showed increased and enlarged muscular potentials with neurogenic patterns. Nerve conduction velocities were normal, with neither multifocal neuropathy nor persistent conduction blocks. Besides mixed cryoglobulinemia type II, antisulfatide antibodies issued from monoclonal IgM were found. They were directed against myelin glycosphingolipids. No antiganglioside GM1 antibodies could be detected. This not only evoked ALS but also proximal motor axonopathy related with monoclonal IgM.. This case suggests that antisulfatide antibodies often present in sensitive demyelinating polyneuropathy could also be involved in lower motor neuron syndrome.

Topics: Aged; Amyotrophic Lateral Sclerosis; Antibodies; Cryoglobulinemia; Diagnosis, Differential; Electromyography; Electrophysiology; Humans; Immunoglobulin kappa-Chains; Immunoglobulin M; Leukemia, Lymphocytic, Chronic, B-Cell; Male; Motor Neuron Disease; Sulfoglycosphingolipids