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Cochrane Database of Systematic Reviews

Efedrina para la miastenia grave, la miastenia neonatal y los síndromes miasténicos congénitos

Información

DOI:
https://doi.org/10.1002/14651858.CD010028.pub2Copiar DOI
Base de datos:
  1. Cochrane Database of Systematic Reviews
Versión publicada:
  1. 17 diciembre 2014see what's new
Tipo:
  1. Intervention
Etapa:
  1. Review
Grupo Editorial Cochrane:

  1. Grupo Cochrane de Neuromuscular

Copyright:
  1. Copyright © 2014 The Cochrane Collaboration. Published by John Wiley & Sons, Ltd.

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Contraer

Autores

  • Charlotte Vrinten

    Community Genetics Section, Clinical Genetics, VU University Medical Center, Amsterdam, Netherlands

  • Angeli M van der Zwaag

    Community Genetics Section, Clinical Genetics, VU University Medical Center, Amsterdam, Netherlands

  • Stephanie S Weinreich

    Correspondencia a: Community Genetics Section, Clinical Genetics, VU University Medical Center, Amsterdam, Netherlands

    [email protected]

  • Rob JPM Scholten

    Dutch Cochrane Centre, Julius Center for Health Sciences and Primary Care / University Medical Center Utrecht, Utrecht, Netherlands

  • Jan JGM Verschuuren

    Department of Neurology, Leiden University Medical Center, Leiden, Netherlands

Contributions of authors

CV played the lead in writing the protocol and designing the review. She developed criteria for a search strategy in conjunction with the Trials Search Co‐ordinator. Together with AZ, she searched the identified titles and abstracts and obtained copies of papers. She selected which studies to include together with AZ, and checked extracted data. She and AZ carried out the analysis of the results and interpreted the analysis together with SW. She entered data into Review Manager 5 and took the lead in drafting the final review.

AZ searched identified titles and abstracts, obtained full‐text copies of relevant papers, and extracted data in conjunction with CV and SW. Together with CV, she conducted the analysis of the data, entered data into Review Manager 5, and drafted the Results, Discussion, and Conclusion sections.

SW conceived the review and secured funding. Together with AZ, she extracted data and helped to interpret the analysis, in preparation for discussions with RS and JV. She coached CV in drafting the final review with special attention to the consumer and policy perspectives. Together with JV she will update the review.

RS helped design the review and has provided general advice. He contributed to the interpretation of the analysis and to the draft of the final review, especially from a methodological perspective.

JV helped design the review. He contributed to interpretation of the analysis and to the draft of the final review, especially from a clinical perspective. Together with SW he will update the review.

Sources of support

Internal sources

  • No sources of support supplied

External sources

  • ZonMw, the Netherlands Organisation for Health Research and Development, Netherlands.

    CV and SW were paid by a ZonMw grant (project number 152002030)

Declarations of interest

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.

Acknowledgements

We would like to thank Professor Jan Kuks of the University Medical Center Groningen, The Netherlands, for providing us with valuable references for the Background section of this review, and Ilse Jansma, medical information specialist at the University Library of the VU University Medical Center, for her assistance in the development of a preliminary search strategy. Dagmar Ivanyi kindly helped with translation from Czech. We gratefully acknowledge the help of the Cochrane Neuromuscular Disease Group in preparing this review, especially the peer reviewers, Ruth Brassington for her useful comments, and Angela Gunn, Trials Search Co‐ordinator, for developing the final search strategy.

The Editorial base of the Cochrane Neuromuscular Disease Group is supported by the MRC Centre for Neuromuscular Diseases and the Muscular Dystrophy Campaign.

Version history

Published

Title

Stage

Authors

Version

2014 Dec 17

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

Review

Charlotte Vrinten, Angeli M van der Zwaag, Stephanie S Weinreich, Rob JPM Scholten, Jan JGM Verschuuren

https://doi.org/10.1002/14651858.CD010028.pub2

2012 Aug 15

Ephedrine for myasthenia gravis

Protocol

Charlotte Vrinten, Stephanie S Weinreich, Rob JPM Scholten, Jan JGM Verschuuren

https://doi.org/10.1002/14651858.CD010028

Differences between protocol and review

We changed the title of the review protocol to clarify the content. The protocol was originally titled 'Ephedrine for myasthenia gravis'.

Keywords

MeSH

Medical Subject Headings (MeSH) Keywords

Medical Subject Headings Check Words

Adult; Child; Humans; Infant, Newborn;

PICO

Population
Intervention
Comparison
Outcome

El uso y la enseñanza del modelo PICO están muy extendidos en el ámbito de la atención sanitaria basada en la evidencia para formular preguntas y estrategias de búsqueda y para caracterizar estudios o metanálisis clínicos. PICO son las siglas en inglés de cuatro posibles componentes de una pregunta de investigación: paciente, población o problema; intervención; comparación; desenlace (outcome).

Para saber más sobre el uso del modelo PICO, puede consultar el Manual Cochrane.

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).
Figuras y tablas -
Figure 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).

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Table 1. Summaries of findings of non‐randomised studies

Type of myasthenia

Before‐after studies

Case series

Case reports

No. of people in review

Ephedrine dose (orally, unless stated otherwise)

Effect

Autoimmune

AChR MG

(Hashimoto 1981; Macdonald 1984; McAlpine 1988)

‐

‐

3

3

15 to 40 mg 4 times daily

or 4 mg I.V. Not reported in some.

Possible improvement in muscle strength in 3 people. Adverse effects reported.

MuSK MG

(Ehler 2008; Haran 2013)

‐

‐

2

2

12 to 50 mg 3 times daily

Possible improvement in symptoms in 2 people. Adverse effects reported.

Neonatal myasthenia gravis

n/a

n/a

n/a

n/a

n/a

n/a

Congenital myasthenic syndromes

Presynaptic

CHAT

(Kinali 2008 and Robb 2010)

‐

1

‐

1

Not reported

Possible improvement in symptoms in 1 person.

Presynaptic other

(Engel 2003 and Maselli 2001)

‐

1

‐

1

Not reported

Possible improvement in muscle strength and fatigue in 1 person.

Synaptic

AGRN

(Chaouch 2012; Huzé 2009)

‐

2

‐

3

50 mg/day

or 2 mg/kg/day

Possible improvements in muscle strength, endurance and well‐being in 2 people. No change in 1 person.

COLQ

(Adamovičová 2012; Bestue‐Cardiel 2005, Bestué 2006, Brengman 2006 and Engel 2008;

Chaouch 2012; Chillingworth 2009; Edvardson 2007 and NCT00541216; Guven 2012; Kinali 2008; Mihaylova 2008a, Mihaylova 2008b, Mihaylova 2008c and Chaouch 2012; Wargon 2012, Wargon 2011 and Bauduin 2011; Yeung 2010)

‐

9

1

29

50 to 200 mg/day (adults)

or 0.5 to 5 mg/kg/day (children). Not reported in some.

Possible improvements in endurance, muscle strength or both in about half of 29 people. Possible improvements in quality of life and respiration.

No change reported in 3 people. Adverse effects reported.

LAMB2

(Maselli 2009)

‐

‐

1

1

Not reported

Possible improvements in 1 person.

Postsynaptic

CHRNE

(Beeson 2005; Burke 2004; Khan 2011; Kinali 2008; Linzoain 2011; Maselli 2011; Nogajski 2009)

‐

3

4

14

7.5 mg twice daily. Not reported in most.

Possible improvements in 9/14 people. No change in 4 people. Adverse effects suspected.

DOK7

(Anderson 2008; Ben Ammar 2010 and Sarkozy 2010; Burke 2009; Burke 2013 and Burke 2011; Della Marina 2011; Kinali 2008; Lashley 2010, Chaouch 2012, Cossins 2010 and Lashley 2009; Palace 2007 and Slater 2006; Schara 2009 and Schara 2007; Schara 2012; Selcen 2008; Srour 2010)

4

7

1

40

7.5 to 100 mg/day

or 0.5 to 1.0 mg/kg/day (children). Not reported in some.

Possible improvements in endurance, muscle strength, quality of life, or unspecified improvements in majority of 40 people. No response in 4 people. Adverse effects reported in 10 people.

Fast channel

(Palace 2012)

‐

‐

1

1

Not reported

Possibly no effect in 1 person.

Limb‐girdle

(Beeson 2005; Kinali 2008; Slater 2006)

‐

3

5

Not reported

Possible improvements in all 5 people.

MuSK

(Maselli 2010; Mihaylova 2009)

‐

1

1

2

Not reported

No response in 1 person. Not tolerated in the other person.

RAPSN

(Banwell 2004; Burke 2004; Chaouch 2012)

‐

3

4

Not reported

Possible improvements in all 4 people.

Slow channel

(Beeson 2005)

‐

1

‐

1

Not reported

Possible slight improvement in 1 person.

Not genetically characterised CMS

(Beeson 2005; Felice 1996; Kinali 2008; Terblanche 2008)

1

2

1

5

25 ‐ 50 mg oral,

or 25 mg twice daily oral,

or 25 mg I.M. Not reported in some.

No objective improvements in

forced vital capacity, muscle strength, or RNS/CMAP decrement. Possible subjective improvements in strength. Adverse effects reported.

Unknown form of myasthenia

(Chan‐Lui 1984; Dalkara 1988; Edgeworth 1930, Edgeworth 1933 and Boothby 1934; Nelson 1935; Patten 1972; Pearce 2005, Johnston 2005, Walker 1934 and Walker 1935; Schwarz 1955; Simpson 1966; Viets 1939; Wilson 1944; Yahr 1944)

1

7

3

196

15 ‐ 96 mg oral,

or < 64 mg S.C.,

or 3% eye drop

solution. Not reported in some.

Possible improvements in a majority of people. No response in a minority. Adverse effects reported.

AChR: acetylcholine receptor; CMAP: compound muscle action potential; CMS: congenital myasthenic syndrome; I.M.: intramuscular; I.V.: intravenous; MG: myasthenia gravis; MuSK: muscle specific tyrosine kinase; n/a: not applicable; RNS: repetitive nerve stimulation; S.C.: subcutaneous

Figuras y tablas -
Table 1. Summaries of findings of non‐randomised studies
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