Case/sex Age at the Underlying Epilepsy Seizure Seizure Efficacy Reasons of beta-hydroxy-
  start of MAD disease classification phenotype frequency at 3 months discontinuation butyrate at 3 months
1/Male 1y6m leukodystrophy SLRE complex partial weekly discontinued reject by patient -
2/Female 1y6m Trisomy 21 IS spasms daily excellent   4929 umol/L
3/Male 1y11m   IS spasms, tonic daily excellent   3986 umol/L
4/Male 2y2m   IS spasms, tonic daily unchanged   5470 umol/L
5/Female 3y   Doose MAS, myoclonic daily discontinued reject by family -
6/Female 3y   IS spasms, tonic daily unchanged   not done
7/Female 3y   IS spasms, tonic daily unchanged   not done
8/Male 3y   IS spasms, tonic daily unchanged   6174 umol/L
9/Female 3y tuberous sclerosis LGS tonic, atypical absence daily discontinued reject by patient -
10/Female 5y band heterotopia unclassified NCSE, GTCs, drop daily excellent   2376 umol/L
11/Female 5y   SLRE NCSE, tonic, hypermotor daily good   2795 umol/L
12/Male 7y   SLRE tonic weekly discontinued reject by family -
13/Female 11y tuberous sclerosis LGS tonic, atypical absence daily discontinued reject by patient -
14/Female 17y tuberous sclerosis LGS tonic, atypical absence daily discontinued reject by family -
15/Female 29y 15q inv dup syndrome SLRE   daily discontinued reject by family -
16/Female 32y   SLRE complex partial, GTCs weekly discontinued reject by patient -
Table 1: Profiles of patients who started the MAD in our hospital and the short-term efficacy and tolerability after 3 months on the diet Abbreviations: SLRE, symptomatic localization-related epilepsy; IS, infantile spasms; LGS, Lennox-Gastaut syndrome; Doose, Doose syndrome; NCSE, nonconvulsive status epilepticus; GTCs, generalized tonic clonic seizure; excellent, seizure-free; good, >50% seizure reduction; unchanged; <50% seizure reduction.