Adrenoleukodystrophy is a disease of the Adrenal Gland, Brain, Spinal Cord and the Nervous System.
The genetic disorder that causes damage to the Adrenal Gland, Brain, Spinal Cord and the Nervous System is Adrenoleukodystrophy.
In Adrenoleukodystrophy, the body fails to break down the fatty substance. There is a defect in the specific Enzymes that beak down the fatty acids in the Body. Then the fatty substance builds up in the Brain and the Adrenal Cortex. This causes damage to the Adrenal gland and the Myelin Sheath. The Adrenal Gland is placed above the Kidney and the Myelin sheath covers the Nerve Fibers. The Myelin sheath also helps in insulating the Brain and the Spinal Cord Neurons. This disrupts the signal coordination between the Brain and the Nerves. The malfunction of Adrenal Gland may lead to
Addison Disease Addison's Disease. It is dealt with separately on this portal.
Types of Adrenoleukodystrophy Childhood Cerebral ALD : It affects in the childhood between the age of 2 to 10. It is a form of X linked ALD. It prognosis very fast if not diagnosed in time. In this, it damages the white matter of the Brain, which may lead to Difficulty in Speaking, Reading and Coordination in children. If it is not treated, it may lead to death.
Adrenomyelopathy : It affects in the childhood but progresses gradually. It shows results in the adult age. It is common in Males. It causes dysfunctions of the Gallbladder and the Bowel Function.
Addison’s Disease : It is the third form of ALD and is caused by Adrenal insufficiency. In this the Adrenal Gland fails to produce enough Hormones.
Causes of Adrenoleukodystrophy It is caused by mutations in the genes. It is linked with X chromosomes. The defect is in X chromosomes. There are two X chromosomes in a woman and only one X chromosome in a man. That is why, it is more common in men. The Women have two copies of X chromosomes. If one copy is defected, the other one would be normal. The woman shows mild symptoms in case if she is a carrier. However, men have only one copy of X chromosomes and shows more symptoms.
If not treated properly, Adrenoleukodystrophy may cause Coma
Death
Paralysis