Alexander disease is classed under leukodystrophy. It is caused by destruction of myelin sheath in the brain. Myelin sheath (fatty area around nerves that helps electrical impulses to work efficiently) forms sheath or layers around nerves and spinal cord.
As we mentioned before that alexander disease is a kind of leukodystrophy, we need to discuss here what this term means. The word leukodystrophy is actually a combination of Greek stems leuko, dys and troph. Leuko means “white”, dys “abnormal” and troph means “growth”. Leukodystrophy then is a group of abnormalities caused by lack of the growth of white cells/matter/substance in the brain.
Is Alexander disease a genetic disorder?
Alexander disease is an inherited disorder of the nervous system. The neurological condition is caused when myelin sheath around nerves in the brain is damaged.
It’s a rare abnormality that usually begins before the age of 2. Alexander disease is not very common as only 500 cases are detected since the disease was discovered first.
Different forms of Alexander disease:
Alexander disease carries different forms or types as given below:
- Neonatal form
- Infantile form
- Juvenile form
- Adult form
Symptoms and severity of every form is different. Neonatal form alexander can cause severe disability and death in a short period of time.
- Mental disability
- Severe motor and decreased intellectual abilities.
This is the most common form of alexander and its symptoms are found usually in age under 2. Children with infantile form hardly survive and die before reaching the age 6 mostly.
- Delayed mental development
- Delayed physical growth
- Abnormal head size
- Destruction of developmental milestones
This form of alexander disease is rare and uncommon or less common and children between the ages 2-13 catch this disease.
- Lack of coordination
- Poor swallowing
- Difficulty speaking
- Difficulty in swallowing
- Excessive vomiting
The adult form is less common and the weakest or mild because its course is lengthy and slower and moreover chances of survival are more if compared to other forms.
How is Alexander disease diagnosed?
Most cases of Alexander disease begin under age 2. Patients with alexander disease are generally poor and children especially suffering from infant form don’t survive. But other forms such as adult form and juvenile affect the victim slowly.
Well there is no cure or treatment for alexander disease till now. There isn’t even a standard course treatment and it’s probably the reason that mostly patients can’t survive more than a couple of years.
Till now the strongest conception about alexander disease is that it’s an inherited or genetic disease caused by genes. But it is not clear whether or not even a single research has proved that how genes cause Alexander disease.
There can be a number of causes of alexander disease that are not found or identified yet and no therapy or treatment is there to cure the disease. Doctors may recommend physical therapy after analyzing the condition of the patient.