MELAS Syndrome

What is MELAS Syndrome?

This is an uncommon and rare type of dementia. MELAS is an acronym that stands for:


ME – Mitochondrial Encephalopathy

LA – Lactic Acidosis

S – “Stroke-like” episodes.

This usually is a syndrome that arises in childhood; commonly between the ages of two (2) and fifteen (15) years of age and mainly affects muscles and the nervous system.

Symptoms

The more common symptoms in the beginning include:

  • Seizures
  • Recurrent headaches
  • Recurrent vomiting
  • Loss of appetite

Stroke-like incidents with momentary weakness of muscle on only one side of the body – hemiparesis – might likewise occur and this frequently leads to:

  • Hearing and vision loss
  • Altered consciousness
  • Motor skills loss
  • Intellectual disability

Causes

MELAS syndrome is triggered by transmutations in the gene material – DNA – in the mitochondria. While the majority of DNA is situated in the chromosomes in the cell nucleus, some DNA is in another important structure referred to as the mitochondrion – plural mitochondria.

Mitochondria are situated outside the nucleus in the cytoplasm of the cell. Every mitochondrion owes a chromosome consisting of DNA and is very diverse from the best identified chromosomes located in the nucleus. The chromosome in mitochondrial is very small and round – where the normal chromosomes are rod shaped – there are numerous copies of the “mitochondrial chromosome” in each cell; and no matter if it is female or male, we receive our mitochondrial chromosome from our mother.

Most DNA in the mitochondria is needed to create proteins intricate in the vital function of mitochondria – creating energy and powering the cells of our body.

Treatment

There is not any recognized treatment for the disease that is underlying and is normally progressive and fatal. Individuals are treated by which areas of the body are affected at a specific time. Vitamins and antioxidants have been used, but there has been no successes consistently reported.

Prognosis

There is currently no known treatment for the underlying disease which is usually progressive and fatal. Individuals are managed according to what areas of the body are affected at any particular time. Enzymes, amino acids, vitamins and antioxidants have been used but there has been no reliable successes described.

There have been no controlled studies on long-term aids of dietary manipulations, but the below supplements have presented some promise and have offered hope to MELAS individuals.

  • CoQ10 has been helpful. Nicotinamide has been used due to complex acceptance of electrons from NADH with ultimate transference of electrons to CoQ10.
  • Riboflavin has been described as improving the patient functioning with complex/deficiency and the 3350T-C mutation.
  • L-arginine has been administrated during the serious as well as the interictal periods and might represent a probable new management for this syndrome to diminish brain damage because of diminished vasodilation in intracerebral arteries owing to nitric oxide reduction.

There has been no evidence from studies with animals that resveratrol or other SIRT1 activators can be used to treat this syndrome by the increase of mitochondrial biogenesis and function.

There is also a case study where succinate provided successful when used to treat out of control convulsions is MELAS patients, but this treatment modality is yet to be investigated or widely recommended.

Life Expectancy

It is not at this time known how many individuals have developed MELAS thru out the world. The syndrome touches all ethnic groups and both females and males. It is believed that the genetic flaw is most often complex in MELAS and is passed down thru the mother’s side of the family.

Current life expectancy for individuals with MELAS at the onset of symptoms is approximately five (5) years.

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