Author, Year, Country

Participants

Study Design

Intervention

Reported Benefits

Level of Evidence

Quality Appraisal

OECBM/SIGN

Benedetti-Isaac, et al. [25] Colombia

1 female, 10-years-old

Case report

VNS stimulation for drug-resistant epilepsy

Reduced seizure frequency at one-year follow-up

-

Poor validity (20%)

Besterman & Hendren [26] USA

1 female, 5-years-old

Case report

Medical treatment for problem behaviours

^*Improved sleep

-

Poor validity (40%)

^*Improved behaviour and learning in school

^*Decrease in tantrum intensity and frequency

Dagorno, et al. [27] France

10: 5 females, 5 males, aged 2-42 months

Retrospective observational single cohort study

Surgery for Hirschsprung’s disease

Surgical complications were common, mixed results at follow-ups

3

Poor validity (42%)

Nosaki, et al. [28] Japan

1 male, 45-years-old

Case report

Medical treatment for drug-resistant epilepsy

Seizure free at nine-month follow-up

-

Valid (75%)

Patel, et al. [29] UK

1 male, three-months-old

Case report, longitudinal

Surgery for

Normal bowel function at ten-year follow-up

-

Poor validity (20%)

Hirschsprung’s disease

Ricci, et al. [6] Italy

40: 22 females, 18 males, 2-31 years-old

Observational longitudinal cohort study

Medical treatment and VNS for epilepsy

Valproic acid is the most widely used and effective antiepileptic drug in MWS, followed by Levetiracetam

3

Valid (75%)