Successful Treatment of Three Lambert-Eaton Myasthenic Syndrome Patients with 3,4-diaminopyridine
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Accession number;06A0654494
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| Title;Successful Treatment of Three Lambert-Eaton Myasthenic Syndrome Patients with 3,4-diaminopyridine |
| Author;
YABE ICHIRO
(Hokkaido Univ.)
SATO KAZUNORI
(Hokkaido Univ.)
MIYAZAKI YUSEI
(Hokkaido Univ.)
TSUJI SACHIKO
(Hokkaido Univ.)
SASAKI HIDENAO
(Hokkaido Univ.)
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Journal Title;Neurological Therapeutics
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Journal Code:X0110A
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ISSN:0916-8443
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VOL.23;NO.4;PAGE.433-438(2006)
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| Figure&Table&Reference;FIG.2, REF.20 |
| Pub. Country;Japan |
| Language;Japanese |
| Abstract;We report the effectiveness of 3,4-diaminopyridine (3,4-DAP) in three patients with Lembert-Eaton myasthenic syndrome (LEMS). All three patients exhibited proximal muscle weakness, generalized hyporeflexia, and autonomic dysfunction (especially dry mouth), and also displayed the waxing phenomenon following electrical stimulation at 50Hz. Since none of the patients had malignant tumors, the diagnosis was LEMS without any associated tumor. Pyridostigmine therapy achieved marginal improvement in all three patients, while steroid therapy only showed a temporary effect in one of them. Because of the poor response to these medications, we opted to treat the three patients with 3,4-DAP. The symptoms of all three patients were initially improved by administration of 3,4-DAP, but became refractory to the drug with continued use. To overcome this problem, the daily dose was increased and the drug was administered 4-6 times daily; a regimen that proved effective. While 3,4-DAP is not yet approved as a pharmaceutical agent in Japan, our results suggest that this policy may need to be reconsidered. (author abst.) |
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