What is Alexander Syndrome?
What is Alexander Syndrome?
- Alexander Syndrome: Brain myelin disorder causing mental degeneration.
- Alexander Syndrome: 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
Alexander Syndrome: Introduction
Types of Alexander Syndrome:
Types of Alexander Syndrome:
- Infantile Alexander Syndrome - most common form
- Juvenile Alexander Syndrome
- Adult-onset Alexander Syndrome
- more types...»
Broader types of Alexander Syndrome:
How many people get Alexander Syndrome?
Prevalance of Alexander Syndrome: rare
Who gets Alexander Syndrome?
Patient Profile for Alexander Syndrome: Symptoms usually at 6 months.
Profile for Alexander Syndrome:
Alexander disease, which affects mostly males, is usually nonfamilial and
begins at about 6 months of age.
(Source: excerpt from NINDS Alexander Disease Information Page: NINDS)
Gender Profile for Alexander Syndrome: Mostly males.
How serious is Alexander Syndrome?
Prognosis of Alexander Syndrome: Poor. Life-shortening. Children often die before 6 years.
Complications of Alexander Syndrome:
see complications of Alexander Syndrome
Prognosis of Alexander Syndrome:
The
prognosis for individuals with Alexander disease is generally poor. Most
children with the infantile form do not survive past the age of 6. In the
juvenile form death usually occurs within 10 years after the onset of
symptoms.
(Source: excerpt from NINDS Alexander Disease Information Page: NINDS)
What causes Alexander Syndrome?
Causes of Alexander Syndrome: see causes of Alexander Syndrome
What are the symptoms of Alexander Syndrome?
Symptoms of Alexander Syndrome:
see symptoms of Alexander Syndrome
Complications of Alexander Syndrome:
see complications of Alexander Syndrome
Can anyone else get Alexander Syndrome?
Contagion of genetic conditions:
generally not; see details in contagion of genetic diseases.
Inheritance of genetic conditions: see details in inheritance of genetic diseases.
Alexander Syndrome: Testing
Diagnostic testing: see tests for Alexander Syndrome.
Misdiagnosis: see misdiagnosis and Alexander Syndrome.
How is it treated?
Treatments for Alexander Syndrome:
see treatments for Alexander Syndrome
Organs Affected by Alexander Syndrome:
Organs and body systems related to Alexander Syndrome include:
Name and Aliases of Alexander Syndrome
Main name of condition: Alexander Syndrome
Class of Condition for Alexander Syndrome: genetic
Other names or spellings for Alexander Syndrome:
Alexander Disease, Alexanders leukodystrophy, Megalencephaly in infancy accompanied by progressive spasticity and dementia, Infantile Alexander disease (subtype)
Fibrinoid leukodystrophy
Source - Diseases Database
Adult Alexander disease (subtype), Alexanders leukodystrophy, Infantile Alexander disease (subtype), Megalencephaly in infancy accompanied by progressive spasticity and dementia
Source - Office of Rare Diseases (ORD) of the National Institutes of Health (NIH)
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