What is Leigh Syndrome, French-Canadian Type?
Leigh syndrome, French-Canadian type, is an inherited disease that causes degeneration of the brain as well as metabolic abnormalities. It is caused by a deficiency in the cytochrome oxidase (COX) enzyme complex and is usually fatal by the age of 5 or 6. The disease primarily affects people from the Saguenay Lac-Saint-Jean region of Quebec, Canada and is rare elsewhere. The French-Canadian form of Leigh syndrome causes developmental delay, poor muscle tone, crossed eyes, characteristic facial features, and a tendency toward life-threatening metabolic crisis and coma. It causes characteristic lesions on the brain and spinal cord. The disease shares symptoms with other forms of Leigh syndrome, which cause progressive degeneration in brain tissues. Metabolic crises can cause low blood sugar and an extreme lack of energy and can very quickly turn fatal. Often illness can precipitate a metabolic crisis.
