Autoantibodies in Neurological Diseases by G. Martino, A. Vincent (auth.), Angela Vincent, Gianvito

By G. Martino, A. Vincent (auth.), Angela Vincent, Gianvito Martino (eds.)

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Similar clinical symptoms can be caused by mutations in VGKC genes, particularly in episodic ataxia type I, which is due to a mutation in the shaker-like VGKC KCNAI, or by neurotoxins that block VGKCs, or by those that reduce inactivation of voltage-gated sodium channels (reviewed in [24]). Antibodies to VGKCs, detected by immunoprecipitation of 125I-dendrotoxin VGKCs, are found in about 40% of patients with NMT and in 25% of those with CFS. Plasma exchange and other immunotherapies are frequently effective clinically, and passive transfer of disease to mice shows evidence of peripheral nerve hyperexcitability, including repetitive action potentials in sensory nerve axons [24].

Casele HL, Laifer SA (1998) Association of pregnancy complications and choice of immunosuppressant in liver transplant patients. Transplantation 27:581-583 54. Present DH, Meltzer SJ, Krumholz MP et al (1989) 6-mercaptopurine in the managment of inflammatory bowel disease: short and long term toxicity. Ann Intern Med 111:641-649 55. Watson WJ, Katz VL, Bowes WA (1984) Plasmapheresis during pregnancy. Obstet GynecoI76:451-457 56. Kaaja R, Julkunen A, Ammala P et al (1993) Intravenous immunoglobulin treatment of pregnant patients with recurrent pregnancy losses associated with antiphospholipid antibodies.

In addition, these limbic encephalitis patients did not have symptoms of NMT, suggesting perhaps that the specificity of their antibodies is different to those in Morvan's syndrome and acquired neuromyotonia. VGKCs are highly pleomorphic, being formed as tetramers of variable proportions of up to six different gene products (KCNAl-6); thus, specificity for different oligomers might underlie some of the differences in clinical expression of diseases associated with VGKC antibodies. Alternatively, it is possible that the peripheral tissue can up regulate other VGKCs or in some other way compensate for their loss.

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