3,4-diaminopyridine base effectively treats the weakness of Lambert-Eaton myasthenia.
Muscle Nerve
; 57(4): 561-568, 2018 04.
Article
en En
| MEDLINE
| ID: mdl-29280483
ABSTRACT
INTRODUCTION:
3,4-diaminopyridine has been used to treat Lambert-Eaton myasthenia (LEM) for 30 years despite the lack of conclusive evidence of efficacy.METHODS:
We conducted a randomized double-blind placebo-controlled withdrawal study in patients with LEM who had been on stable regimens of 3,4-diaminopyridine base (3,4-DAP) for ≥ 3 months. The primary efficacy endpoint was >30% deterioration in triple timed up-and-go (3TUG) times during tapered drug withdrawal. The secondary endpoint was self-assessment of LEM-related weakness (W-SAS).RESULTS:
Thirty-two participants were randomized to continuous 3,4-DAP or placebo groups. None of the 14 participants who received continuous 3,4-DAP had > 30% deterioration in 3TUG time versus 72% of the 18 who tapered to placebo (P < 0.0001). W-SAS similarly demonstrated an advantage for continuous treatment over placebo (P < 0.0001). Requirement for rescue and adverse events were more common in the placebo group.DISCUSSION:
This trial provides significant evidence of efficacy of 3,4-DAP in the maintenance of strength in LEM. Muscle Nerve 57 561-568, 2018.Palabras clave
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Síndrome Miasténico de Lambert-Eaton
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Debilidad Muscular
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Deprescripciones
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Amifampridina
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Fármacos Neuromusculares
Tipo de estudio:
Clinical_trials
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Etiology_studies
Límite:
Adult
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Aged
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Aged80
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Female
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Humans
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Male
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Middle aged
Idioma:
En
Revista:
Muscle Nerve
Año:
2018
Tipo del documento:
Article
País de afiliación:
Estados Unidos