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Tribute To Nathan, Our Little Man

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Published on Oct 19, 2008

This is Nathan and his family. He had a rare disease called Leigh's Disease. He died October 15. 2008. He was 10 months old.




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The following is from Web MD on Leighs Disease:

Synonyms * Leigh Necrotizing Encephalopathy * Leigh's Syndrome * Necrotizing Encephalomyelopathy of Leigh's * SNE * Subacute Necrotizing Encephalopathy

Disorder Subdivisions * Infantile Necrotizing Encephalopathy * Classical Leigh's Disease * X-Linked Infantile Nectrotizing Encephalopathy * Adult-Onset Subacute Necrotizing Encephalomyelopathy

General Discussion

Leigh's disease is a rare genetic neurometabolic disorder. It is characterized by the degeneration of the central nervous system (i.e., brain, spinal cord, and optic nerve). The symptoms of Leigh's disease usually begin between the ages of three months and two years. Symptoms are associated with progressive neurological deterioration and may include loss of previously acquired motor skills, loss of appetite, vomiting, irritability, and/or seizure activity. As Leigh's disease progresses, symptoms may also include generalized weakness, lack of muscle tone (hypotonia), and episodes of lactic acidosis, which may lead to impairment of respiratory and kidney function.




There appear to be several different types of genetically determined enzyme defects that can cause Leigh's disease. Most individuals with Leighs disease have defects of mitochondrial energy production, such as deficiency of an enzyme of the mitochondrial respiratory chain complex or the pyruvate dehydrogenase complex. In most cases, Leigh's disease is inherited as an autosomal recessive trait. However, X-linked recessive and mitochondrial inheritance have also been noted.

As noted the above is copied from Web MD and all rights belong to that site.

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