Alexander disease is a rare genetic disorder that affects the midbrain and cerebellum. This disease is a progressive and fatal neurodegenerative disease that mostly affects infants and children. The disease is broadly classified as Type I, detected below the age of 4, and Type II, detected above the age of 4. Symptoms of Type I are failure to grow and gain weight at expected rates, delayed growth of physical, mental and behavioral skills and sudden episodes of brain seizures. Abnormal enlargement of the head, abnormal increase in muscle stiffness are other symptoms of Type I. Type II symptoms are display of pseudobulbar signs, ataxia and spasticity.
HOW IS ALEXANDER DISEASE DIAGNOSED?
Alexander disease is diagnosed in various ways including; • magnetic resonance imaging (MRI • genetic testing • clinical symptoms • radiological studies • leukodystrophies
HOW IS ALEXANDER DISEASE TREATED?
There is no cure for Alexander disease. The only way is symptomatic and supportive. Hydrocephalus may be partially treated by surgery. The later the disease occurs the course gets slow and lengthy.
DID YOU KNOW?
Alexander disease is also known as dysmyelogenic leukodystrophy, fibrinoid leukodystrophy and hyaline polyneuropathy