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Revue Neurologique
Vol 160, N° 5-C2  - mai 2004
pp. 85-89
Doi : RN-05-2004-160-5-0035-3787-101019-ART12
Neuromyotonia
 

J. Newsom-Davis
[1]  Department of Clinical Neurology, University of Oxford, Radcliffe Infirmary, Oxford OX2 6HE, U.K.

Tirés à part : J. Newsom-Davis

[2]  , Department of Clinical Neurology, University of Oxford, Radcliffe Infirmary, Oxford OX2 6HE, U.K., tel: +44 1865 224940, fax: +44 1865 224273,

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Neuromyotonie.

Plusieurs arguments plaident en faveur d'un mécanisme auto-immun impliqué dans la pathogénie du syndrome d'hypererexcitabilité périphérique (neuromyotonie et syndrome crampes-fasciculations) et de la maladie de Morvan où des anomolies du système nerveux central sont aussi présentes. Les trois affections peuvent être associées à la présence d'un thymone, d'une myasthénie ou d'autres désordres auto-immuns et peuvent être améliorées par les échanges plasmatiques. Les transferts de plasma ou d'IgG provenant de patients atteints de neuromyotonie conduisent à des anomalies électrophysiologiques chez la souris et une réduction du courant des canaux potassiques voltage dépendant in vitro . Des anticorps dirigés contre les canaux potassiques voltage dépendant sont détectés dans le sérum de la plupart des parents atteints d'hyperexcitabilité périphérique ou de maladie de Morvan, au cours de laquelle des signes cliniques d'encéphalite lumbique peuvent survenir. La mise en évidence du caractère auto-immun de ces affections devrait en faciliter le diagnostic et ouvrir de nouvelles voies thérapeutiques.

Abstract

There is increasing evidence that autoimmunity is implicated in the pathogenesis of peripheral nerve hyperexcitability (neuromyotonia, NMT and Cramp-fasciculation syndrom C-FS ) and in Maladie de Morvan in which CNS features are also present. All three conditions can associate with thymoma, myasthenia gravis and other autoimmune disorders, and can often respond to plasma exchange. In NMT, patient's plasma or IgG can transfer the electrophysiological features to mice, and can reduce voltage-gated potassium channel currents in vitro. Antibodies to voltage-gated potassium channels can be detected in the serum of many patients who have peripheral nerve hyperexcitability, and also in those with Maladie de Morvan. These latter patients have clinical features similar to limbic encephalitis in which VGKC antibodies can also occur. Thus neuromyotonia, cramp-fasciculation syndrome and Maladie de Morvan can occur as antibody-mediated autoimmune ion channelopathies like myasthenia gravis and the Lambert-Eaton myasthenic syndrome. These discoveries should aid diagnosis and offer new approaches to treatment.


Mots clés : Neuromyotonie , Canal potassium , EMG

Keywords: Neuromyotonia , Potassium channel , EMG






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