Cree Leukoencephalopathy

Cree Leukoencephalopathy (CLE) is a rare genetic and fatal disease that affects mostly babies. Even though this is a rare disorder, it is the most common inherited disorder that affects the white matter of the brain. In our brain we have “white matter”, now CLE causes our white matter to disappear by liquefying (turn into fluid) our white matter. This white matter turning into fluid affects our brain and spinal cord, which makes up our nervous system. The nervous system takes care of our entire body and everything we do everyday. Our brain is our control centre, it makes sure we breath, blink and makes our heart pump for us to stay alive. When something happens to our brain like CLE and turns our control centre into liquid, then we can no longer breath, think and/or our hearts wouldn’t pump anymore.

There are no signs at birth but the signs will appear between 3 to 9 months old. 

                        
the baby’s muscles may feel soft and doughy
                        muscles may tighten up
                         the baby may have a history of viral illnesses (when the baby gets a virus)
                         may have seizures
                         diarrhea
                        vomiting
                         slow-moving
                        hyperventilation

The signs may not be fully right and this is not a complete list of signs since this disease is still being researched.

As mentioned above Cree leukoencephalopathy is a genetic disease, where a person has a mutated gene (irregular gene). Even if someone is a carrier of the mutated gene they can still live a healthy life. But for a baby to have CLE, both parents must have this mutated gene and they will have a 1 in 4 chance of having a baby that has CLE. Researchers are still unsure of what causes this mutated gene in Cree people; they just know that it is passed down from parents, through generations.

To find out if you are a carrier of this mutated gene, you have to get a blood test done. Go to your clinic and ask to have it done. You can also contact Helene Denoncourt from the Cree Health Board at 819 855 9001 ext 4412 or by email at, hdenoncourt@ssss.gouv.qc.ca  

Website/Bibliography

“Cree Leukoencephalopathy”, Radiology
http://radiology.rsna.org/content/213/2/400.full

Denoncourt, Helene. Educational and Carrier Screening Program for Cree Leukoencephalopathy and Cree Encephalitis. (May 2010)

“Leukoencephalopathy with vanishing white matter”, Genetics Home Reference
http://ghr.nlm.nih.gov/condition/leukoencephalopathy-with-vanishing-white-matter

“Leukoencephalopathy with vanishing white matter”, Wikipedia  
http://en.wikipedia.org/wiki/Leukoencephalopathy_with_vanishing_white_matter

“What is Leukoencephalopathy?”, WD
http://www.wrongdiagnosis.com/l/leukoencephalopathy/basics.htm

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