THE NEUROMUSCULAR JUNCTION DISORDERS

Neuromuscular junction (NMJ) disorders result from destruction, malfunction, or absence of one or more key proteins involved in neuromuscular transmission, illustrated diagrammatically in fig 1. The most common pathology is antibody-mediated damage or downregulation of ion channels or receptors, resulting in myasthenia gravis (MG), Lambert-Eaton myasthenic syndrome (LEMS), and acquired neuromyotonia (Isaac’s syndrome). *Contents 1 Autoimmune 2 Neuromuscular junction 3 Classification 3.1 Immune-mediated 3.2 Toxic/metabolic 3.3 Congenital 3.4 Presynaptic 3.5 Synapse 3.6 Postsynaptic 4 Most common diseases 4.1 Myasthenia gravis 4.2 Lambert-Eaton myasthenic syndrome (LEMS) 5 Other diseases 5.1 Neuromyotonia 5.2 Congenital myasthenia 5.3 Botulism 6 Diagnosis 6.1 Tests 7 Treatment 7.1 Symptomatic treatment 7.2 Immunosuppressive treatment