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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.

journal_name

J Neurol Sci

journal_title

Journal of the neurological sciences

authors

Hayat GR,Kulkantrakorn K,Campbell WW,Giuliani MJ

doi

10.1016/s0022-510x(00)00407-x

subject

Has Abstract

pub_date

2000-12-01 00:00:00

pages

38-43

issue

1-2

eissn

0022-510X

issn

1878-5883

pii

S0022-510X(00)00407-X

journal_volume

181

pub_type

杂志文章

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