Maternally inherited Leigh syndrome.

Ciafaloni, E; Santorelli, F M; Shanske, S; Deonna, T; Roulet, E; Janzer, C; Pescia, G; DiMauro, S · J Pediatr · 1993

case_report · Level V

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Abstract

A 6 1/2-year-old girl had developmental regression, and Leigh syndrome was diagnosed. A second girl born to the same mother after heterologous artificial insemination also lost acquired skills and died at 2 1/2 years of age; neuropathologic examination confirmed the diagnosis of Leigh syndrome. Tissues from both children and from the mother had a point mutation at nucleotide 8993 in the adenosinetriphosphatase 6-gene of mitochondrial DNA. This family illustrates that Leigh syndrome can be transmitted by maternal inheritance.

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