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A Rare Genetic Disorder!!??

 

ALEXANDERS DISEASE

For Updated Info you should check with the ULF this is the info that was available to us                                                                                                                                                                                                               Alexanders disease belongs to a group of progressive neurological disorders in which the destruction of white matter in the brain is accompanied by the formation of fibrous, eosinophilic deposits known as Rosenthal fibers. The majority of cases of Alexanders are sporadic; that is, without a known family history of the disorder. There are, however, a number of families with more than one affected child. Alexanders disease begins in infancy, affects mostly males, and results in retarded development and dementia. It is quickly progressive and is the rarest of the identified leukodystrophies.

The onset of the infantile form of Alexanders disease is usually around six months, but may occur between 0 and 24 months of age. Children who develop Alexanders in infancy generally do not survive past the age of 5 or 6 years. Physical and mental development is retarded, and there is progressive enlargement of the brain and head, increasing spasticity, and seizures in some cases. Histologically, demyelination of both sensory and motor fiber tracts is found. In addition, the eosinophilic Rosenthal fibers are found evenly distributed throughout the brain near blood vessels, and on the surface of the brain. The demyelinated areas do not correspond to the distribution of the Rosenthal fibers.

Alexanders disease is thought to be an autosomal recessive disorder which can affect both males and females. The metabolic error is unknown, and the demyelination and fiber formation seem to be otherwise unrelated parallel expressions of the disorder. It is the degeneration of the astrocytic glial cells which seems to lead to demyelination. An exact diagnosis may not be possible without study of postmortem tissues or brain or nerve biopsy.

In addition to the infantile form of the disease, juvenile and adult onset forms are recognized, occurring less frequently and with a longer course of progression. These individuals have in common the widespread formation of Rosenthal fibers throughout the central nervous system. Older patients have less white matter loss and have correspondingly milder symptoms. It is not clear, however, that the disorders described in older children and adults are all the same disorder and should be called Alexanders disease.

Treatment of Alexanders disease is symptomatic and supportive.

Further extensive in-depth research information is available on these subjects and more through the ULF.


 

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