[Molecular pathological aspects in spongy encephalopathies]

Spongy encephalopathies are rare diseases of infancy and childhood. Apart from cerebral spongiosis in the forms described as Canavan's disease and the Kearns-Sayre syndrome, necrosis in the cerebral tissue is also found in Alpers' disease and Leigh's syndrome. The common pathogenetic factor may be sought in the sphere of mitochondrial energy metabolism, as suggested by the results of molecular pathological investigations. Biochemical enzyme deficiencies in energy metabolism obviously provoke structural alterations of mitochondria.