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November 8, 2009
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MELAS Syndrome

Medical Author: William C. Shiel, Jr., MD, FACP, FACR
Medical Editor: Frederick Hecht, M.D., F.A.A.P., F.A.C.M.G.

What is MELAS?

MELAS is a rare form of dementia. MELAS is an abbreviation that stands for Mitochondrial Encephalopathy, Lactic Acidosis, and Stroke-like episodes.

What causes MELAS?

MELAS syndrome is caused by mutations in the genetic material (DNA) in the mitochondria. While most of our DNA is in the chromosomes in the cell nucleus, some of our DNA is in another important structure called the mitochondrion (plural: mitochondria).

The mitochondria are located outside the nucleus in the cell's cytoplasm. Each mitochondrion has a chromosome made of DNA that is quite different from the better known chromosomes in the nucleus. The mitochondrial chromosome is much smaller; it is round (whereas the chromosomes in the nucleus are normally shaped like rods); there are many copies of the mitochondrial chromosome in every cell; and no matter whether we are male or female, we inherit all of our mitochondrial chromosome from our mother.

Much of the DNA in our mitochondria is used to manufacture proteins involved in the key function of mitochondria -- to produce energy and power the cells in our body.



Next: What are the symptoms of MELAS? »

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MELAS Syndrome

What is a stroke?

A stroke, or cerebrovascular accident (CVA), occurs when blood supply to part of the brain is disrupted, causing brain cells to die. When blood flow to the brain is impaired, oxygen and glucose cannot be delivered to the brain. Blood flow can be compromised by a variety of mechanisms.

Blockage of an artery

  • Narrowing of the small arteries within the brain can cause a so-called lacunar stroke, (lacune=empty space). Blockage of a single arteriole can affect a tiny area of brain causing that tissue to die (infarct).
  • Hardening of the arteries (atherosclerosis) leading to the brain. There are four major blood vessels that supply the brain with blood. The anterior circulation of the brain that controls most motor, activity, sensation, thought, speech, and emotion is supplied by the carotid arteries. The posterior circulation, which supplies the brainstem and the cerebellum, con...

Read the Stroke article »










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