What Is White Matter Disease?

White matter disease is associated with the development of infants and their brain. It often is hard to detect at first because symptoms may not appear until a head injury occurs. There are different forms of white matter disease with different symptoms. The treatment for the disease is supportive, as there is no known cure.

Significance

• White matter is associated with the brain. It is one of three parts of the central nervous system and contains the cells that connect various regions of the brain to each other. In white matter disease, lesions occur when the blood supply in the region is cut off. Often this is due to hardening of the artery. It causes problems with nerve cells and associated brain functioning. The cells are called axons. Symptoms often appear in very young children with developing brains. It is also caused by genetic problems.

Function

• The disease is believed to be caused by mutation in the five genes that make proteins in the body. They are known as EIF2b. The reduction in functioning becomes a problem when someone has an infection, fever or head trauma. It is often diagnosed by looking at the symptoms and having a test called an MRI. It is called childhood ataxia and vanishing white matter leukodystrophy.

Types

• White matter disease affects the baby's early brain development. It attacks the myelin of the brain, which is the fatty white substance that protects the neural fibers. Alexander Disease is another form of the disease in babies and occurs when the myelin in the brain does not form at all. Krabble Disease is another form where the myelin forms but not properly. Often afflicted infants do not gain weight or grown normally. They do not keep up with other infants mentally, and some have seizures or swollen heads.

Features

• The symptoms of the disease often appear in early childhood and include episodes of fever, muscle spasms and involuntary contractions. This means muscles can be stiff and movement awkward or limited in range. There is lethargy and indifference to stimuli. Death may result after a few years. This disease can cause problems with vision and seizures. Often, there is fever and coma.

Diagnoisis

• There are many test used to find white matter diseases, including X-rays and CT and MRI scans. Some tests examine the blood because it is a defect in the genes that causes the illness. The disease is treated with surgery to relieve pressure and sometimes brain cell transplants. Overall, there is no cure or consistent treatment for the disease.