title: Neuromyotonia: autoimmune pathogenesis and response to immune modulating therapy.
abstract: BACKGROUND
  Neuromyotonia (NMT) has been postulated to be an autoimmune channelopathy, probably by affecting voltage gated potassium channels (VGKC) leading to excitation and abnormal discharges [Sinha et al., Lancet 338 (1991) 75].
  
  
  OBJECTIVE
  To report three patients with NMT who had other associated immune-mediated conditions, i.e., myasthenia gravis, thymoma and various types of peripheral neuropathies. One patient had peripheral neuropathy and involvement of pre- and post-synaptic neuromuscular junction.
  
  
  RESULTS
  All three patients had evidence of polyneuropathy and neuromyotonic discharges on electrodiagnostic studies. Elevated acetylcholine receptor antibodies were noted in all patients and malignant thymoma was found in two patients with metastasis. All three patients showed moderate to marked response to plasma exchange.
  
  
  CONCLUSIONS
  These findings strongly suggest a humoral autoimmune pathogenesis of NMT, probably by K(+) channel involvement, affecting acetylcholine quantal release and postsynaptic membrane. Clinicians should be aware of this association of immune-mediated conditions in NMT patients and marked improvement with plasma exchange.
authors: Department of Neurology, Saint Louis University, 3635 Vista at Grand Blvd., 63110-0250, St. Louis, MO, USA. hayatg@slucare1.sluh.edu
authors: G R
authors: GR
authors: Hayat
authors: K
authors: K
authors: Kulkantrakorn
authors: W W
authors: WW
authors: Campbell
authors: M J
authors: MJ
authors: Giuliani
mesh_terms: D000328:Adult; D000368:Aged; D006801:Humans; D007167:Immunotherapy; D020386:Isaacs Syndrome / Q000276:immunology* / Q000503:physiopathology / Q000628:therapy; D008297:Male; D008875:Middle Aged; D009157:Myasthenia Gravis / Q000150:complications / Q000276:immunology / Q000628:therapy; D009431:Neural Conduction / Q000502:physiology; D010523:Peripheral Nervous System Diseases / Q000150:complications / Q000175:diagnosis / Q000276:immunology; D010956:Plasmapheresis / Q000706:statistics & numerical data; D015221:Potassium Channels / Q000378:metabolism; D011950:Receptors, Cholinergic / Q000276:immunology / Q000378:metabolism; D013945:Thymoma / Q000150:complications / Q000276:immunology / Q000628:therapy
chemical_list: D015221:Potassium Channels; D011950:Receptors, Cholinergic