Alexander Disease

Alexander disease (leukodystrophy) is a rare genetic disorder of the brain (central nervous system). Alexander disease affects growth of the myelin sheath. This is the fatty covering which acts as an insulator on nerve fibers in the brain. There are 3 forms of Alexander disease:

  • Infant. Symptoms of Alexander disease begin within the first 2 years of life.

  • Juvenile. The onset of symptoms begins between the ages of 4 and 10.

  • Adult. This is the most rare form.

SYMPTOMS

  • Mental and physical retardation.

  • Confusion or loss of memory (dementia).

  • Progressive enlargement of the head (macrocephaly).

  • Stiffness of arms or legs (spasticity).

  • Convulsions (seizures).

TREATMENT

There is no cure for Alexander disease. Treatment of Alexander disease is based on symptoms.The outcome (prognosis) for individuals with Alexander disease is generally poor. Most children do not survive past the age of 6.