Clinical aspects of neuromuscular transmission disorders

Acta Neurologica Scandinavica. Supplementum
Amelia Evoli

Abstract

Autoimmune disorders of neuromuscular transmission are caused by antibodies (abs) directed against membrane proteins at the motor end-plate. Myasthenia gravis (MG) is due, in most cases, to abs against the nicotinic acetylcholine receptor (AChR). Anti-AChR-positive MG actually includes different disease entities: weakness can be confined to extrinsic ocular muscles or can be generalized; patients with generalized MG (G-MG) can be subdivided on the basis of age of onset, HLA association and thymic pathology. About 15% of G-MG patients are anti-AChR-negative; in a proportion of these cases serum abs against the muscle- specific kinase (MuSK) are found. Anti-MuSK-positive MG is characterized by predominant involvement of bulbar muscles and very low frequency of thymic pathology. The Lambert-Eaton myasthenic syndrome (LEMS) is caused by abs against voltage-gated calcium channels at nerve terminal. LEMS is characterized by muscle weakness and autonomic disturbances and it is paraneoplastic in over 50% of the cases. In neuromyotonia and cramp-fasciculation syndrome, that are thought to be due to anti-voltage-gated potassium channel abs, signs of peripheral nerve hyperexcitability can be associated with CNS features.

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Citations

Mar 30, 2013·Autoimmunity Reviews·Sonia Berrih-AkninRobert P Lisak
Jan 29, 2013·Journal of Clinical Neuroscience : Official Journal of the Neurosurgical Society of Australasia·Rawiphan WitoonpanichAngela Vincent
Mar 13, 2009·Journal of Hand Therapy : Official Journal of the American Society of Hand Therapists·Sarah B GoldmanAmanda Antczak
Sep 22, 2007·Anesthesiology Clinics·Ansgar M Brambrink, Jeffrey R Kirsch
Mar 5, 2014·Acta Neurologica Scandinavica. Supplementum·J B AndersenN E Gilhus
Dec 9, 2014·Biochimica Et Biophysica Acta·James C Ha, David P Richman
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Aug 1, 2007·Arquivos de neuro-psiquiatria·Maria das Graças Wanderley S CoriolanoOtávio Gomes Lins

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