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. 2014 Dec 17;2014(12):CD010028.
doi: 10.1002/14651858.CD010028.pub2.

Ephedrine for myasthenia gravis, neonatal myasthenia and the congenital myasthenic syndromes

Charlotte Vrinten  1 , Angeli M van der Zwaag, Stephanie S Weinreich, Rob J P M Scholten, Jan J G M Verschuuren
Affiliations

Ephedrine for myasthenia gravis, neonatal myasthenia and the congenital myasthenic syndromes

Charlotte Vrinten et al. Cochrane Database Syst Rev. .

Abstract

Background: Myasthenia is a condition in which neuromuscular transmission is affected by antibodies against neuromuscular junction components (autoimmune myasthenia gravis, MG; and neonatal myasthenia gravis, NMG) or by defects in genes for neuromuscular junction proteins (congenital myasthenic syndromes, CMSs). Clinically, some individuals seem to benefit from treatment with ephedrine, but its effects and adverse effects have not been systematically evaluated.

Objectives: To assess the effects and adverse effects of ephedrine in people with autoimmune MG, transient neonatal MG, and the congenital myasthenic syndromes.

Search methods: On 17 November 2014, we searched the Cochrane Neuromuscular Disease Group Specialized Register, the Cochrane Central Register of Controlled Trials (CENTRAL), MEDLINE and EMBASE. We also searched reference lists of articles, conference proceedings of relevant conferences, and prospective trial registers. In addition, we contacted manufacturers and researchers in the field.

Selection criteria: We considered randomised controlled trials (RCTs) and quasi-RCTs comparing ephedrine as a single or add-on treatment with any other active treatment, placebo, or no treatment in adults or children with autoimmune MG, NMG, or CMSs.

Data collection and analysis: Two review authors independently assessed study design and quality, and extracted data. We contacted study authors for additional information. We collected information on adverse effects from included articles, and contacted authors.

Main results: We found no RCTs or quasi-RCTs, and therefore could not establish the effect of ephedrine on MG, NMG and CMSs. We describe the results of 53 non-randomised studies narratively in the Discussion section, including observations of endurance, muscle strength and quality of life. Effects may differ depending on the type of myasthenia. Thirty-seven studies were in participants with CMS, five in participants with MG, and in 11 the precise form of myasthenia was unknown. We found no studies for NMG. Reported adverse effects included tachycardia, sleep disturbances, nervousness, and withdrawal symptoms.

Authors' conclusions: There was no evidence available from RCTs or quasi-RCTs, but some observations from non-randomised studies are available. There is a need for more evidence from suitable forms of prospective RCTs, such as series of n-of-one RCTs, that use appropriate and validated outcome measures.

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Conflict of interest statement

Jan Verschuuren (JJGMV) has been involved in a thymectomy trial sponsored by the NIH, and in a FP7 European grant which involves testing a vaccine to treat AChR myasthenia gravis with Curavac. The Neurology department of the LUMC has received fees from BioMarin Ltd in 2009 to 2010, because of consultancies by JJGMV in the field of Lambert‐Eaton myasthenic syndrome. JJGMV did not receive any personal payments. He has no known conflict of interest related to this review.

From 2008 to 2010 Stephanie Weinreich worked at the Erasmus MC Medical Center on a project funded through the Top Institute Pharma, Leiden, The Netherlands. Project partners were a public‐private consortium including two pharmaceutical companies. The research concerned newborn screening for Pompe disease; this is unrelated to the Cochrane review at hand. She has no known conflict of interest related to this review.

Rob Scholten: none known. Angeli van der Zwaag: none known. Charlotte Vrinten: none known.

Figures

1
1
Study flow diagram (a supplementary search shortly before publication resulted in 16 references, no RCTs or quasi‐RCTs and seven additional studies to be assessed for inclusion in the Discussion at the next update).
See this image and copyright information in PMC

Update of

  • doi: 10.1002/14651858.CD010028

References

References to ongoing studies

NCT00541216 {published data only (unpublished sought but not used)}
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