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Adrenoleukodystrophy is a rare disease. It causes severe damage to our brain. It is a fatal disease that causes the adrenal gland to completely stop functioning. In this kind of disease the myelin sheath gets totally damaged on account of a defective gene. The covering of fats on the nerve fibers is known as the myelin sheath. The myelin sheath is extremely important as the nerves do not function without them. Thus, in this disease when the myelin sheath gets damaged, there is an irreversible damage caused to the nerve cells which at times may prove to be fatal.
The medical experts have identified the absence of transporter protein in our body metabolism with this kind of disease. The transporter protein is very important for carrying the enzyme which helps in breaking down the fatty acids. Any amount of storage of these fatty acids in the adrenal glands and the brain results in the damage of myelin sheath and consequently the disease.
Causes of Adrenoleukodystrophy
The Adrenoleukodystrophy most commonly appears in X-linked condition. The men are more prone to this disease as they only have one X chromosome, while the women have the luxury of two X chromosomes. So along with the affected ones they also have a spare one. The occurrence of this kind of X linked ALD can happen with three forms. The symptoms show up both in the childhood and the adulthood. The percentage of this disease occurring in a new born baby is very less.
Adrenoleukodystrophy Symptoms
The symptoms usually start to show between the ages of four to ten years. The parents have to be particularly careful during this period. If the child shows signs of memory loss and his or her performance in the school gradually drops, the parents need to take it seriously as this might signal the beginning of Adrenoleukodystrophy. The condition can be followed by pigmentation in the skin, repeated seizures and fatigue. The situation gets worse and slowly becomes the main reason for death.
The adult form of the disease generally occurs during the age of twenty to thirty five years. The symptoms include ataxia or problem with muscle coordination. The weaknesses of limbs are termed by the experts as the early signs of this disease which eventually results in paralyzing them. All of these symptoms develop very slowly compared to the childhood form.
In the third form of ALD abnormality in neurological conditions become very apparent. In case of neonatal Adrenoleukodystrophy which is very rare the baby witnesses unusual facial appearances and weak muscle tones. The liver also gets a bit enlarged on account of this. Compared to the adult form the symptoms generally develop at a rapid pace.
Treatment of Adrenoleukodystrophy
There’s no proper treatment for Adrenoleukodystrophy. Supportive treatments include special help from the society and the school, physiotherapy and replacement of adrenal hormones. A mixture of euric acid and oleic acid delays the disease to some extent. This usually happens in case of boys. Transplantation of the bone marrow is also one effective procedure which has resulted in considerable success. The medical researchers are striving extremely hard for finding a new method of treatment. Overall the support and the attention of the parents in the initial stages of the disease in case of a child have proved to be very vital. Neuroimaging and MRI examination have proved to be essential for the treatment of this disease. Apart from this some dietary treatments have been experimented for the cure of this disease, but they have resulted in limited success.
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