Tuesday, February 17, 2009

Leigh's Disease



Ok so I researched a pathology by the name of Leigh's disease. To be quite honest, the reason why this pathology caught my interest is because my middle name is Leigh! Anyway, from what I understand Leighs disease affects the nerves in your brain, it affects how they function, which obviously makes this a fatal disease. MOST the time the disease will show itself within toddler stages; 6 months to two years old. Rarely the disease can begin in teenage to young adult years, but it is possible.

What happens is the nerve cells die because the mitochondria (powerhouse of each cell) is affected. Some symptons that I think are unique to this disease are a childs sucking inablity, constant crying, and lack of motor skills.More obvious symptons are seizures and vomitting. Lacitic acidosis, which is lactic acid built up in the brain is a sympton as well. This leads to kidney failure.

Unforunately there is no cure for Leigh's disease. The best medicine can do right now is the use of vitamin B-1. Vitamin B-1 is what nerve cells require to maintain function.
It is quickly degenerative. Since mitochondrian ablilities inherited from the mother,Leigh's disease is also inherited from the mothers side by a DNA mutation. Most do not live to their teens, although it is possible. Though the appearance of Leigh's disease is not strikingly obvious, it is pictured below.





http://www.ispub.com/xml/journals/ijra/vol3n1/mri-fig2.jpg
Ok here is a normal T1 weighted image of a brain. Notice the size of the gyrus, ventricles, and cerebellum.








I apolgize about the blurriness but this is a T1 weighted image of a child with Leigh's disease. Although the difference is not dramatic, there is volume loss in the cerebellum and gyri. The ventricles are also abnormally larger.

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