3,4-Diaminopyridine in the treatment of congenital (hereditary) myasthenia.

Congenital or hereditary myasthenia describes a heterogeneous group of disorders in which the immune system is not implicated. Treatment has previously depended on anticholinesterase medication. The effectiveness of 3,4-diaminopyridine (3,4-DAP), a preparation that enhances acetylcholine release fro...

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Main Authors: Palace, J, Wiles, C, Newsom-Davis, J
Formato: Journal article
Idioma:English
Publicado: 1991
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author Palace, J
Wiles, C
Newsom-Davis, J
author_facet Palace, J
Wiles, C
Newsom-Davis, J
author_sort Palace, J
collection OXFORD
description Congenital or hereditary myasthenia describes a heterogeneous group of disorders in which the immune system is not implicated. Treatment has previously depended on anticholinesterase medication. The effectiveness of 3,4-diaminopyridine (3,4-DAP), a preparation that enhances acetylcholine release from motor nerve terminals, has been evaluated using a series of standardised strength measures. Sixteen patients (aged seven to 47 years) were studied in an open prospective trial, and four of them in a double blind crossover trial; existing anticholinesterase medication was continued. For the group as a whole, there was a highly significant increase in muscle strength (p less than 0.001; n = 16). In individual paired comparisons, 13 out of 16 showed significant improvement in the open trial and four out of four in the blind crossover trial. In conclusion, 3,4-DAP, either alone or combined with anticholinesterase medication, may be a useful additional treatment in congenital myasthenia.
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spelling oxford-uuid:672e6f78-445e-4da3-b8ab-b153387838592022-03-26T18:36:37Z3,4-Diaminopyridine in the treatment of congenital (hereditary) myasthenia.Journal articlehttp://purl.org/coar/resource_type/c_dcae04bcuuid:672e6f78-445e-4da3-b8ab-b15338783859EnglishSymplectic Elements at Oxford1991Palace, JWiles, CNewsom-Davis, JCongenital or hereditary myasthenia describes a heterogeneous group of disorders in which the immune system is not implicated. Treatment has previously depended on anticholinesterase medication. The effectiveness of 3,4-diaminopyridine (3,4-DAP), a preparation that enhances acetylcholine release from motor nerve terminals, has been evaluated using a series of standardised strength measures. Sixteen patients (aged seven to 47 years) were studied in an open prospective trial, and four of them in a double blind crossover trial; existing anticholinesterase medication was continued. For the group as a whole, there was a highly significant increase in muscle strength (p less than 0.001; n = 16). In individual paired comparisons, 13 out of 16 showed significant improvement in the open trial and four out of four in the blind crossover trial. In conclusion, 3,4-DAP, either alone or combined with anticholinesterase medication, may be a useful additional treatment in congenital myasthenia.
spellingShingle Palace, J
Wiles, C
Newsom-Davis, J
3,4-Diaminopyridine in the treatment of congenital (hereditary) myasthenia.
title 3,4-Diaminopyridine in the treatment of congenital (hereditary) myasthenia.
title_full 3,4-Diaminopyridine in the treatment of congenital (hereditary) myasthenia.
title_fullStr 3,4-Diaminopyridine in the treatment of congenital (hereditary) myasthenia.
title_full_unstemmed 3,4-Diaminopyridine in the treatment of congenital (hereditary) myasthenia.
title_short 3,4-Diaminopyridine in the treatment of congenital (hereditary) myasthenia.
title_sort 3 4 diaminopyridine in the treatment of congenital hereditary myasthenia
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AT wilesc 34diaminopyridineinthetreatmentofcongenitalhereditarymyasthenia
AT newsomdavisj 34diaminopyridineinthetreatmentofcongenitalhereditarymyasthenia