Myasthenia gravis (MG) with antibodies to the muscle-specific tyrosine kinase receptor (MuSK) is characterized by prominent cranial and bulbar weakness. The disease course is often severe and some patients develop permanent, disabling symptoms despite long-term immunosuppression. 1 In patients with MuSK MG, the administration of acetylcholinesterase inhibitors (AChE-I) is poorly effective and often associated with clinical and electrophysiologic signs of cholinergic hypersensitivity. 2 3,4-Diaminopyridine (3,4-DAP) improves neuromuscular transmission (NMT) by increasing quantal release. We report the case of an adult patient with MuSK MG who was responsive to and tolerant of 3,4-DAP.

3,4-Diaminopyridine may improve myasthenia gravis with M u SK antibodies / Evoli A.; Alboini P.E.; Damato V.; Iorio R.. - In: NEUROLOGY. - ISSN 0028-3878. - ELETTRONICO. - 86:(2016), pp. 1070-1071. [10.1212/WNL.0000000000002466]

3,4-Diaminopyridine may improve myasthenia gravis with M u SK antibodies

Damato V.;
2016

Abstract

Myasthenia gravis (MG) with antibodies to the muscle-specific tyrosine kinase receptor (MuSK) is characterized by prominent cranial and bulbar weakness. The disease course is often severe and some patients develop permanent, disabling symptoms despite long-term immunosuppression. 1 In patients with MuSK MG, the administration of acetylcholinesterase inhibitors (AChE-I) is poorly effective and often associated with clinical and electrophysiologic signs of cholinergic hypersensitivity. 2 3,4-Diaminopyridine (3,4-DAP) improves neuromuscular transmission (NMT) by increasing quantal release. We report the case of an adult patient with MuSK MG who was responsive to and tolerant of 3,4-DAP.
2016
86
1070
1071
Evoli A.; Alboini P.E.; Damato V.; Iorio R.
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Utilizza questo identificatore per citare o creare un link a questa risorsa: https://hdl.handle.net/2158/1257924
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