LEIGH disease is due to the accumulation of: (A) Glycogen, (B) Pyridoxine, (C) Sphingomyelin, (D) None of the above
Answer is A. (A) Glycogen # L eigh's disease or, subacute Necrotizing Encephalopathy> Is characterized by an arrest of psychomotor development, feeding problem, seizures, extraocular palsies, and weakness with hypotonia between one and 2 years of age> The cardinal Laboratory finding is lactic Addenda> Various biochemical abnormalities have been found, all of which lie in the mitrochondrial pathway for converting pyruvate to ATP.