Leigh's subacute necrotizing encephalopathy: clinical and biochemical study, with special reference to therapy with lipoate.

The family consisted of 4 children, of whom 3 have been clinically affected. J.R. (male) is now 11 years old and clinically normal. P.R. (male) developed symptoms at 7 months and died at 3 years 7 months. F.R. (female) developed symptoms at 11 months and died at 3 years 10 months. H.R. (female) developed symptoms at 10 months and died at 6 years 6 months.