Literature DB >> 26852139

Amifampridine phosphate (Firdapse(®)) is effective and safe in a phase 3 clinical trial in LEMS.

Shin J Oh1, Natalya Shcherbakova2, Anna Kostera-Pruszczyk3, Mohammad Alsharabati1, Mazen Dimachkie4, Jose Munoz Blanco5, Thomas Brannagan6, Dragana Lavrnić7, Perry B Shieh8, Christophe Vial9, Andreas Meisel10, Samuel Komoly11, Benedikt Schoser12, Kumaraswamy Sivakumar13, Yuen So14.   

Abstract

OBJECTIVE: We evaluated the efficacy and safety of amifampridine phosphate (Firdapse(®)) for symptomatic treatment in Lambert-Eaton myasthenic syndrome (LEMS).
METHODS: Phase 3, randomized, double-blind, study. Patients were treated initially with amifampridine phosphate for 7-91 days, followed by randomization to continue amifampridine phosphate for 14 days or placebo (7-day taper, 7-day placebo). The primary efficacy endpoints were changes from baseline at day 14 in Quantitative Myasthenia Gravis and Subject Global Impression scores.
RESULTS: The coprimary efficacy end points and 1 of the secondary efficacy end points were met, showing a significant benefit of aminfampridine phosphate over placebo at Day 14. All 5 primary, secondary, and tertiary endpoints achieved statistical significance at Day 8. Amifampridine phosphate was well tolerated; the most common adverse events were oral and digital paresthesias, nausea, and headache.
CONCLUSIONS: This study provides Class I evidence of efficacy of amifampridine phosphate as a symptomatic treatment for LEMS.
© 2016 Wiley Periodicals, Inc.

Entities:  

Keywords:  3,4-DAP; 3,4-diaminopyridine; Amifampridine phosphate; LEMS; Lambert-Eaton myasthenic syndrome; Potassium channel blockers; Small cell lung cancer (SCLC)

Mesh:

Substances:

Year:  2016        PMID: 26852139     DOI: 10.1002/mus.25070

Source DB:  PubMed          Journal:  Muscle Nerve        ISSN: 0148-639X            Impact factor:   3.217


  9 in total

1.  Low specificity of voltage-gated calcium channel antibodies in Lambert-Eaton myasthenic syndrome: a call for caution.

Authors:  Rodica Di Lorenzo; Karin Mente; Jianbo Li; Luay Shayya; Alexander Rae-Grant; Yuebing Li; Adham Jammoul
Journal:  J Neurol       Date:  2018-07-09       Impact factor: 4.849

2.  Amifampridine safety and efficacy in spinal muscular atrophy ambulatory patients: a randomized, placebo-controlled, crossover phase 2 trial.

Authors:  Silvia Bonanno; Riccardo Giossi; Riccardo Zanin; Valentina Porcelli; Claudio Iannacone; Giovanni Baranello; Gary Ingenito; Stanley Iyadurai; Zorica Stevic; Stojan Peric; Lorenzo Maggi
Journal:  J Neurol       Date:  2022-06-28       Impact factor: 6.682

3.  3,4-diaminopyridine base effectively treats the weakness of Lambert-Eaton myasthenia.

Authors:  Donald B Sanders; Vern C Juel; Yadollah Harati; A Gordon Smith; Amanda C Peltier; Tessa Marburger; Jau-Shin Lou; Robert M Pascuzzi; David P Richman; Tai Xie; Valentin Demmel; Laura R Jacobus; Kathy L Aleš; David P Jacobus
Journal:  Muscle Nerve       Date:  2018-02-02       Impact factor: 3.217

4.  Five years experience on 3,4-diaminopyridine phosphate in Lambert-Eaton syndrome: Case reports.

Authors:  Simona Portaro; Teresa Brizzi; Stefano Sinicropi; Alberto Cacciola; Maria Cristina De Cola; Alessia Bramanti; Demetrio Milardi; Antonino Lupica; Placido Bramanti; Antonio Toscano; Carmelo Rodolico
Journal:  Medicine (Baltimore)       Date:  2017-09       Impact factor: 1.889

Review 5.  Recent Advances and Therapeutic Options in Lambert-Eaton Myasthenic Syndrome.

Authors:  Arsalan Anwar; Sidra Saleem; Mirza Fawad Ahmed; Sara Ashraf; Sameen Ashraf
Journal:  Cureus       Date:  2019-08-21

Review 6.  Lambert-Eaton Myasthenic syndrome: early diagnosis is key.

Authors:  Trajche Ivanovski; Francesc Miralles
Journal:  Degener Neurol Neuromuscul Dis       Date:  2019-05-13

7.  The European Lambert-Eaton Myasthenic Syndrome Registry: Long-Term Outcomes Following Symptomatic Treatment.

Authors:  Andreas Meisel; Jörn P Sieb; Gwendal Le Masson; Ville Postila; Sabrina Sacconi
Journal:  Neurol Ther       Date:  2022-05-05

8.  α-Synuclein-induced Kv4 channelopathy in mouse vagal motoneurons drives nonmotor parkinsonian symptoms.

Authors:  Wei-Hua Chiu; Lora Kovacheva; Ruth E Musgrove; Hadar Arien-Zakay; James B Koprich; Jonathan M Brotchie; Rami Yaka; Danny Ben-Zvi; Menachem Hanani; Jochen Roeper; Joshua A Goldberg
Journal:  Sci Adv       Date:  2021-03-10       Impact factor: 14.136

9.  Population Pharmacokinetics/Pharmacodynamics of 3,4-Diaminopyridine Free Base in Patients With Lambert-Eaton Myasthenia.

Authors:  Nilay Thakkar; Jeffrey T Guptill; Kathy Aleš; David Jacobus; Laura Jacobus; Charles Peloquin; Michael Cohen-Wolkowiez; Daniel Gonzalez
Journal:  CPT Pharmacometrics Syst Pharmacol       Date:  2017-07-24
  9 in total

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