Alexander Syndrome: Alexander disease is a rare, genetic, degenerative disorder of the nervous system. It is one of a group of genetic disorders called the ... more about Alexander Syndrome.
Alexander Syndrome: Brain myelin disorder causing mental degeneration. More detailed information about the symptoms, causes, and treatments of Alexander Syndrome is available below.
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Prognosis for Alexander Syndrome: Poor. Life-shortening. Children often die before 6 years.
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Alexander disease is a rare, genetic, degenerative disorder of the nervous system. It is one of a group of genetic disorders called the leukodystrophies that affect growth of the myelin sheath — the fatty covering which acts as an insulator — on nerve fibers in the brain. (Source: excerpt from NINDS Alexander Disease Information Page: NINDS)
A rare inherited disorder of myelin formation. Alexander disease is a progressive leukoencephalopathy whose hallmark is the widespread accumulation of cytoplasmic inclusions called Rosenthal fibers. The fibers contain GLIAL FIBRILLARY ACIDIC PROTEIN in association with ALPHA-CRYSTALLIN B CHAIN. Rosenthal fibers are found predominantly in ASTROCYTES located in the subependymal, subpial, and periventricular areas of the BRAIN. - (Source - Diseases Database)
Alexander Syndrome is listed as a "rare disease" by the Office of
Rare Diseases (ORD) of the National Institutes of Health
(NIH). This means that Alexander Syndrome, or a subtype of Alexander Syndrome,
affects less than 200,000 people in the US population.
Source - National Institutes of Health (NIH)
Ophanet, a consortium of European partners,
currently defines a condition rare when it affects 1 person per 2,000.
They list Alexander Syndrome as a "rare disease".
Source - Orphanet
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