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[Relationship between heroin spongiform leucoencephalopathy and respiratory chain complex I deficiency].

[canavan disease]

To investigate the relationship between heroin spongiform leucoencephalopathy and respiratory chain complex I deficiency.The activity of respiratory chain complex I in peripheral white blood cell mitochondria was compared between 36 cases of heroin spongiform leucoencephalopathy and 36 healthy subjects using enzyme-linked immunosorbent assay (ELISA).The activity of respiratory chain complex I was 5.62.4 U/ml in patients with heroin spongiform leucoencephalopathy, significantly higher than that in the normal subjects (4.22.1 U/ml, t=2.634, P<0.05).In patients with heroin spongiform leucoencephalopathy, mitochondrial dysfunction results in energy metabolism disorder to cause extensive demyelination of the cerebral white matter. Respiratory chain complex I deficiency of the mitochondria plays a significant role in the pathogenesis of heroin spongiform leucoencephalopathy.