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Disorders of the Neuromuscular Junction: Outcome Measures and Clinical Scales

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Clinical Trials in Neurology

Abstract

In this chapter disorders of the neuromuscular junction are described. The main symptom of these diseases is fluctuating weakness of voluntary muscles associated with fatigability. Myasthenia gravis (MG), the most well-known disorder in this group, is caused by a postsynaptic disturbance, while the Lambert-Eaton myasthenic syndrome is a prototype of a presynaptic defect. In addition a number of rare diseases of the neuromuscular junction are recognised: kinetic abnormalities of the acetylcholine receptor (the slow channel syndrome), or structural deficits such as end-plate acetyl-cholinesterase deficiency, paucity of synaptic vesicles, and others. Presynaptic and congenital disorders will not be considered here. The same rules as for MG will hold for trials in patients with these syndromes.

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© 2001 Springer-Verlag London

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Kuks, J.B.M., Oosterhuis, H.J.G.H. (2001). Disorders of the Neuromuscular Junction: Outcome Measures and Clinical Scales. In: Guiloff, R.J. (eds) Clinical Trials in Neurology. Springer, London. https://doi.org/10.1007/978-1-4471-3787-0_38

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  • DOI: https://doi.org/10.1007/978-1-4471-3787-0_38

  • Publisher Name: Springer, London

  • Print ISBN: 978-1-84996-856-0

  • Online ISBN: 978-1-4471-3787-0

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