MELAS Syndrome

Judy MacDonald jnmacdon at stfx.ca
Mon Apr 17 08:08:30 PDT 2000


Interesting. I checked the Merck Manual (professional edition) - the full-text
is finally on-line- and they quote a different name for MELAS:
"MELAS (Mitochondrial Encephalomyopathy, Lactic Acidosis, Strokelike episodes)"
http://www.merck.com/pubs/mmanual/section21/chapter286/286f.htm
Little detail on the genetic disorder - no nutritional component mentioned.
Thank you for sharing your findings!
Judy

Claire Chinkel wrote:


> After reading the post from Mona Lisa, I did a little research and found

> this response to a question about MELAS on the consumer website

> www.medhelp.org : (Copied directly from the posted response from Cleveland

> Clinic Foundation)

>

> "MELAS syndrome, an acronym for the full name of the disease, which is

> called Myoclonic Epilepsy with Lactic Acidosis and Stroke-like episodes, is

> a rare inherited genetic disorder in which there is a mutation in the DNA of

> the mitochondria. Mitochondria are organelles, or subcompartments of all

> cells in the body, and are responsible for the ultimate use of almost all

> energy in the body that is derived from the cellular respiratory chain,

> which is the final common pathway for the use of oxygen in every

> multicellular organism in the world. When the mitochondria are abnormal or

> become sick, the organs in the body that have the highest metabolic demands

> and greatest oxygen use in the body are affected, and if the mitochondria

> are so sick that they cannot deliver enough energy to the cells of the

> affected organs, damage to the organs occurs. In the human body, the organs

> that use the most energy are the brain and the muscles (especially eye

> muscles), and it is these organs that are thus most often affected by

> disorders like MELAS. In MELAS, as the brain cells are strained for energy,

> they may produce seizure activity. Unfortunately the seizure activity itself

> then further increases demand for oxygen, and causes actual death of cells

> and often irreversible brain damage, the symptoms of which are stroke-like

> events."

>

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