Thiamine triphosphate levels and histopathology. Correlation in Leigh disease
- PMID: 985153
- DOI: 10.1001/archneur.1976.00500110027005
Thiamine triphosphate levels and histopathology. Correlation in Leigh disease
Abstract
Thiamine and thiamine triphosphate (TTP) values were assayed in various brain regions in 11 controls and 13 patients with subacute necrotizing encephalomyelopathy (SNE, Leigh disease). The TTP values of normal brain were 5% of the total thiamine value. The relative TTP (or % TTP) level was consistently low in the pons, midbrain, and cerebellum of all the SNE brains. Twenty-five percent of the SNE brains had normal TTP levels in the frontal region. The TTP values correlated with the degrees of pathologic involvement in all sampled regions of the brain except the cerebellum. The concentration of thiamine in the mammillary bodies exceeded its concentration elsewhere in both control and SNE brains. The finding of low TTP levels in morphologically abnormal regions supports the hypothesis that TTP deficiency is etiologically related to SNE.
Comment in
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Significance of rostral brain lesions of Leigh syndrome associated with the mitochondrial DNA 8993T > G mutation.J Neurol Sci. 2016 Jul 15;366:248. doi: 10.1016/j.jns.2016.05.023. Epub 2016 May 12. J Neurol Sci. 2016. PMID: 27206685 No abstract available.
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