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Lipid composition of brain in a patient with mental retardation due to encephalopathy in an infantile period and one due to homocystinuria.

The lipid compositions of brains of a patient with etiologically unknown encephalopathy (ECP) in an early infantile period and a patient with homocystinuria (HCU) were investigated. In the ECP brain the total lipid content and all lipid classes decreased and the degree of the reduction was more conspicuous in the gray matter than in the white matter. The ganglioside distribution was altered in the ECP brain and the ganglioside long-chain base composition was the infantile pattern. It was shown that damage inflicted on an immature brain leaves persistent effects on lipid metabolism in the course of future brain development, resulting mental and neurological dysfunction. The HCU brain retained almost a normal lipid composition. Among disorders of amino acid metabolism it was found that HCU did not significantly affect lipid metabolism in the brain.