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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).

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