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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Formato: | Journal article |
Idioma: | English |
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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. |
first_indexed | 2024-03-06T23:16:06Z |
format | Journal article |
id | oxford-uuid:672e6f78-445e-4da3-b8ab-b15338783859 |
institution | University of Oxford |
language | English |
last_indexed | 2024-03-06T23:16:06Z |
publishDate | 1991 |
record_format | dspace |
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 |
work_keys_str_mv | AT palacej 34diaminopyridineinthetreatmentofcongenitalhereditarymyasthenia AT wilesc 34diaminopyridineinthetreatmentofcongenitalhereditarymyasthenia AT newsomdavisj 34diaminopyridineinthetreatmentofcongenitalhereditarymyasthenia |