What is Alexander Disease
Alexander disease is one of a group of neurological conditions known as the leukodystrophies disorders that are the result of abnormalities in myelin, the “white matter” that protects nerve fibers in the brain. The disease occurs in both males and females, and there are no ethnic, racial, geographic, or cultural/economic differences in its distribution.
[list style=”arrow” color=”blue” el_position=”first”]Symptoms
- Adult-onset forms of Alexander disease are less common. The symptoms sometimes mimic those of Parkinson’s disease or multiple sclerosis, or may present primarily as a psychiatric disorder
- The juvenile form of Alexander disease is less common and has an onset between the ages of two and thirteen. These children may have excessive vomiting, difficulty swallowing and speaking, poor coordination, and loss of motor control
- Alexander disease is a progressive and often fatal disease
- It has an onset during the first two years of life
- Rosenthal fibers are sometimes found in other disorders, but not in the same amount or area of the brain that are featured in Alexander disease
- The destruction of white matter is accompanied by the formation of Rosenthal fibers, which are abnormal clumps of protein that accumulate in non-neuronal cells of the brain called astrocytes
- The infantile form is the most common type of Alexander disease
- Usually there are both mental and physical developmental delays, followed by the loss of developmental milestones, an abnormal increase in head size, and seizures
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Treatments
- There is no cure for Alexander disease
- There a standard course of treatment
- Treatment of Alexander disease is symptomatic and supportive
- The prognosis for individuals with Alexander disease is generally poor.
- Most children with the infantile form do not survive past the age of 6.
- Juvenile and adult onset forms of the disorder have a slower, more lengthy course
- Biomarkers, if found, would be a major advantage for evaluating the response to any treatments that are developed in the future.
[list style=”arrow” color=”blue” el_position=”first last”]Support Groups
- http://www.rarediseases.org
- United Leukodystrophy Foundation – www.ulf.org
Credit to the NINDS or the NIH for contained information[/list]
