Leigh's necrotizing encephalopathy with pyruvate carboxylase deficiency.

@article{Gilbert1983LeighsNE,
  title={Leigh's necrotizing encephalopathy with pyruvate carboxylase deficiency.},
  author={Enid F. Gilbert and S. Arya and Raymond Y. T. Chun},
  journal={Archives of pathology & laboratory medicine},
  year={1983},
  volume={107 4},
  pages={
          162-6
        }
}
Infants with subacute necrotizing encephalopathy or Leigh's encephalopathy usually are first examined before the age of 2 years with degenerative neurologic disease with variable clinical appearance. Necrotizing lesions of the CNS occur with special predilection of the gray matter. Biochemical defects of thiamine triphosphate associated with an inhibitor of the enzyme thiamine pyrophosphate-adenosine triphosphate phosphoryltransferase and deficiency of the enzyme pyruvate carboxylase have been… CONTINUE READING

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