Table 3

Efficacy of DCA in 53 patients with CLA as a function of the biochemical or clinical syndrome

DiagnosisNo of casesBiochemical response (yes/no)Clinical response (yes/no)Reference
PDC (unspecified)107/32/223, 24, 27,3-150
PDC E1 42/20/425-28,3-150
PDC E2 11/00/129
PDC E3 21/11/121,3-150
Complex I11/01/030
Complex IV63/31/412, 27, 31, 32, 46
Complex I, IV11/00/132
PC, PEPCK22/00/233
Leigh’s syndrome22/00/134,3-150
MELAS3-151 54/13/119, 20, 35, 36,3-150
MERRF44/01/237, 38, 46
Pearson’s syndrome11/01/0 3-150
Unknown1211/15/512, 37, 39-44, 47,3-150
  • 3-150 Personal communication.

  • 3-151 In two patients with MELAS, there was insufficient information to determine either biochemical or clinical response to DCA.