What is the inheritance pattern associated with Leigh Syndrome?

Leigh Syndrome is characterized by a complex inheritance pattern involving both mitochondrial and nuclear genetic contributions. This neurodegenerative disorder primarily affects the central nervous system and can manifest due to mutations in mitochondrial DNA, which is inherited maternally, as well as in nuclear genes that can be inherited in different patterns depending on the specific mutation.

The mitochondrial inheritance emphasizes the role of the mitochondria, which are the energy-producing organelles in cells, and the fact that they are only passed down from mothers to their children. However, many cases of Leigh Syndrome also involve defects in genes located in the nuclear DNA, which can follow autosomal recessive or even some dominant patterns, depending on the specific genetic variant.

This dual inheritance aspect is critical for understanding the wide phenotypic variability seen in Leigh Syndrome, making it vital to consider both mitochondrial and nuclear genes in genetic counseling and diagnosis.