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Alexander's disease

  • Article
  • 2021-03-02

Alexander's disease belongs to a group of neurological conditions known as the leukodystrophies. Leukodystrophies are conditions that result from abnormalities in myelin, the "white matter" that protects nerve fibers in the brain. In Alexander's disease, the destruction of white matter is accompanied by the formation of Rosenthal fibers - abnormal clumps of protein that build up in non-nerve cells (astrocytes) in the brain. The most common form of Alexander's disease is the infantile form, which usually starts during the first two years of life. Symptoms include mental and physical developmental delays, followed by the loss of developmental milestones, an abnormal increase in head size, and seizures. The juvenile form of Alexander disease starts between the ages of two and thirteen. These children may have excessive vomiting, difficulty swallowing and speaking, poor coordination and loss of motor control. Forms of Alexander's disease in adulthood are less common. The symptoms sometimes mimic those of Parkinson's disease or multiple sclerosis, or may present primarily as a psychiatric condition. The disease affects both men and women and there are no ethnic, racial, geographic or cultural / economic differences in its distribution. Alexander's disease is a progressive and often fatal disease. or cultural / economic differences in distribution. Alexander's disease is a progressive and often fatal disease. or cultural / economic differences in distribution. Alexander's disease is a progressive and often fatal disease.

Therapy

There is no cure for Alexander's disease, nor is there any standard treatment. Treatment for Alexander's disease is symptomatic and supportive.

Prognosis

The prognosis for individuals with Alexander's disease is generally poor. Most children with the infantile form do not survive the age of 6 years. Juvenile and adult forms of the disease have a slower and longer-lasting course.


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