Renal lesions in subacute encephalomyelopathy (Leigh's disease)

A case of subacute necrotizing encephalomyelopathy (SNE; Leigh's disease) in an infant aged 19 months is reported. In addition to the typical central nervous lesions, hyperlactatemia and hyperpyruvatemia and morphological changes in the kidney were documented and have been identified as a tubulo-interstitial disease with a focal sclerosing glomerulopathy.