Adrenoleukodystrophy – Causes and Synptoms of Adrenoleukodystrophy
Adreunoleukodystrophy (ALD) is a rare disorder which causes severe damage to the brain as the myelin sheath is damaged because of a defective gene and the covering of nerve fibers is called myelin sheath. This sheath is very much important as the nerves do not function well without them. In this disease the myelin sheath gets damaged, as this is a X-linked chromosomal disorder and this results in the damage of the nerve cells which is irreversible and may prove to be fatal. The average life span of a child suffering from ALD is 10 years approximately.
The main causes include:
- Adrenoleukodystrophy is a disorder of the brain which is passed down from parents to their children.
- It is an X-linked genetic trait and affects man, although few women who can be carriers can have this disorder in milder form.
- It affects 1 in 20,000 people from all the races.
- ALD is mainly caused by a mutation in the gene called the ALD gene.
- The ALD gene makes a protein called ALD protein and these proteins when put together forms the organs and tissues in the body like myelin sheath.
- In this disorder, the myelin sheath is and the nerve signals are disrupted and it is slowed down or stopped completely.
- ALD can lead to mental disorders, weakness, muscle spasms and hearing loss.
Symptoms of Adrenoleukodystrophy
The list of signs and symptoms includes the following:
- Impaired vision
- Attention problems
- Behavioral problems
- Mental deterioration
- Apraxia (causes tremors)
- Difficulty in reading
- Difficulty in understanding speech in children
- Leg weakness
- Increased skin pigmentation
- Lack of voluntary movements in the leg
- Difficulty in walking
- Retinal Degeneration
- Reduced secretion of hormones by testes and ovaries
- Cortical blindness
- Urinary disorders
- Difficulty in reading and writing in the case of children
- Adrenal Dystrophy
- Swallowing difficulties
- Progressive blindness
- Leg stiffness
- Brain sclerosis and demyelination of the brain tissues and cells
- Hypo tension
- Muscle weakness on one side of the body
- Adrenal insufficiency
When to consult a Medical Professional?
Call your health care provider if your child suffers from these symptoms of X-linked chromosomal disorder adrenoleukodystrophy before the symptoms worsen.