We are independent & ad-supported. We may earn a commission for purchases made through our links.
Advertiser Disclosure
Our website is an independent, advertising-supported platform. We provide our content free of charge to our readers, and to keep it that way, we rely on revenue generated through advertisements and affiliate partnerships. This means that when you click on certain links on our site and make a purchase, we may earn a commission. Learn more.
How We Make Money
We sustain our operations through affiliate commissions and advertising. If you click on an affiliate link and make a purchase, we may receive a commission from the merchant at no additional cost to you. We also display advertisements on our website, which help generate revenue to support our work and keep our content free for readers. Our editorial team operates independently of our advertising and affiliate partnerships to ensure that our content remains unbiased and focused on providing you with the best information and recommendations based on thorough research and honest evaluations. To remain transparent, we’ve provided a list of our current affiliate partners here.
Health

Our Promise to you

Founded in 2002, our company has been a trusted resource for readers seeking informative and engaging content. Our dedication to quality remains unwavering—and will never change. We follow a strict editorial policy, ensuring that our content is authored by highly qualified professionals and edited by subject matter experts. This guarantees that everything we publish is objective, accurate, and trustworthy.

Over the years, we've refined our approach to cover a wide range of topics, providing readers with reliable and practical advice to enhance their knowledge and skills. That's why millions of readers turn to us each year. Join us in celebrating the joy of learning, guided by standards you can trust.

What is Leigh Disease?

By V. Cassiopia
Updated: May 17, 2024
Views: 7,302
Share

Leigh disease, commonly referred to as Leigh’s disease, is a rare neurometabolic disorder, which is a disorder that prevents cells in the central nervous system from producing energy. The cells are unable to produce energy for growing and maturing because they are unable to convert nutrients from the bloodstream into a form of energy that can be used by the cell as fuel. Since the cells are unable to produce the energy to grow and maintain themselves, energy depletion accumulates, and the cells begin to deteriorate and die. Since Leigh disease strikes primarily at the time of maximum growth — from birth to early childhood — and since it affects the brain at a vital period of development, the disease is invariably fatal.

Leigh disease was discovered in the early 1950s by Dr. Denis Leigh, and at that time it was termed a “deadly” disease, due to its prevalence for fatalities among the very young. It is characterized by a rapid failure of the body to develop, along with seizures and loss of coordination. The disease can also occur during the adolescent or early adult years, but when it does so, it is then termed subacute necrotizing encephalomyelopathy (SNEM). The tendency for cellular dysfunction is thought by many medical specialists to be inherited on the maternal side, and it has been found to be linked to mutation of the cellular DNA, the genetic material of a cell that gets passed on to newly created cells.

In neurology, an area of medicine that studies the brain and nervous system, Leigh disease is classified as being one of the most severe forms of known mitochondrial diseases caused by defective cell mitochondria — the part of the cell that produces energy. Other less lethal forms exist, depending upon how the energy production of cells is affected. Smaller groups of cells with abnormal genetic material can exist, producing milder forms of mitochondrial disease, since higher numbers of normal energy-producing cells can mitigate the severity of the disease. Both nerve and muscle cells are particularly subject to mitochondrial disease, due to their high energy requirements, and the condition caused by deteriorating muscle fiber cells is known as mitochondrial myopathy.

Leigh disease is actually a syndrome, in that it affects both the muscles and the brain, and is also called mitochondrial encephalomyopathy. There is no known cure for the disease, and treatment depends upon its severity. Food supplements have been tried in an effort to help replace substances needed by the body that are unable to be produced by the affected cells. Combined forms of natural enzymes and amino acids — such as as creatine, L-carnitine and coQ10 supplements — have been used, and although they have not been shown to make a medical improvement in mitochondrial disease, they are generally believed to be beneficial.

Share
WiseGeek is dedicated to providing accurate and trustworthy information. We carefully select reputable sources and employ a rigorous fact-checking process to maintain the highest standards. To learn more about our commitment to accuracy, read our editorial process.

Editors' Picks

Discussion Comments
Share
https://www.wisegeek.net/what-is-leigh-disease.htm
Copy this link
WiseGeek, in your inbox

Our latest articles, guides, and more, delivered daily.

WiseGeek, in your inbox

Our latest articles, guides, and more, delivered daily.