M. Elia et al., LEIGH-SYNDROME AND PARTIAL DEFICIT OF CYTOCHROME-C-OXIDASE ASSOCIATEDWITH EPILEPSIA PARTIALIS CONTINUA, Brain & development, 18(3), 1996, pp. 207-211
Epilepsia partialis continua (EPC) has previously been reported in onl
y two patients with Leigh syndrome (LS). We report here a subject in w
hom LS and partial deficit of cytochrome c oxidase (COX) were associat
ed with EPC. Epilepsy in this subject appeared when neurologic impairm
ent was clearly evident and MRI showed cortical lesions typically asso
ciated with congenital lactic acidosis.