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Amifampridines are the Most Effective Drugs for Treating Lambert-Eaton Myasthenic Syndrome With a Focus on Pediatric Lambert-Eaton Myasthenic Syndrome.
J Clin Neurol
July 2024
Department of Neurology, University of Alabama at Birmingham, Birmingham, Alabama, USA.
In 1983, the first successful trial of 3,4-diaminopyridine (3,4-DAP) in Lambert-Eaton myasthenic syndrome (LEMS) was reported. Efficacy of amifampridine (3,4-DAP and 3,4-diaminopyridine phosphate [3,4-DAPP]) for symptomatic treatment in LEMS was proven by seven randomized studies in 3,4-DAP and two randomized studies in 3,4-DAPP. US Food Drug Administration approved 3,4-DAPP usage for adult LEMS in 2018 and for pediatric LEMS in 2022.
View Article and Find Full Text PDFSafety, efficacy and steroid-sparing effect of amifampridine in Lambert-Eaton myasthenic syndrome patients - real world data.
Neurol Neurochir Pol
October 2024
Department of Neurology, Medical University of Warsaw, Warsaw, Poland, ERN EURO NMD.
Introduction: Lambert-Eaton myasthenic syndrome (LEMS) is an ultrarare neuromuscular disease with a triad of symptoms: muscle paresis, dysautonomy, and areflexia. Amifampridine is the symptomatic treatment of LEMS.
Aim Of Study: To assess the effectiveness and safety of treatment in the real world.
Updated consensus statement: Intravenous immunoglobulin in the treatment of neuromuscular disorders report of the AANEM ad hoc committee.
Muscle Nerve
October 2023
Neurology Clinic, Vanderbilt University, Nashville, Tennessee, USA.
Article Synopsis
- Intravenous immune globulin (IVIG) is a therapy being used for neuromuscular disorders, but there is limited high-quality evidence for many specific diseases, leading to the AANEM's 2009 consensus on its use.
- Since then, new research and FDA approvals have prompted an update to the guidelines, categorizing recommendations from Class I (strong evidence) to Class IV (weak evidence).
- IVIG is strongly recommended for conditions like chronic inflammatory demyelinating polyneuropathy and Guillain-Barré Syndrome but not recommended for others like inclusion body myositis, with uncertain support for various other disorders.
Nationwide survey of Lambert-Eaton myasthenic syndrome in Japan.
BMJ Neurol Open
September 2022
Neurology, Kanazawa Medical University, Kahoku-gun, Ishikawa, Japan.
Background: There was no nationwide epidemiological study of Lambert-Eaton myasthenic syndrome (LEMS) in Japan; therefore, we conducted a nationwide survey.
Methods: For the first survey, we sent survey sheets to randomly selected medical departments (n=7545) to obtain the number of LEMS who visited medical departments between 1 January 2017 and 31 December 2017. For the second survey, we sent survey sheets to the corresponding medical departments to obtain clinical information on LEMS.
Lambert-Eaton myasthenic syndrome in a young girl.
BMJ Case Rep
February 2022
Department of Neurology, Yale-New Haven Hospital, New Haven, Connecticut, USA.
Lambert-Eaton Myasthenic Syndrome (LEMS) is a neuromuscular junction defect that results in weakness. It is rarely seen in children and most commonly is associated with small-cell lung cancer. Diagnosis of LEMS requires characteristic electromyogram (EMG) findings and the detection of antibodies directed against voltage-gated calcium channels.
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