Adrenoleukodystrophy (ALD) is one of a group of genetic disorders called the Leukodystrophies. These genetic disorders inflict damage to the myelin sheath, an insulating membrane that surrounds nerve cells in the brain.
Those with the condition accumulate high levels of saturated fatty acids (VLCFA) in the brain and adrenal cortex. It happens because they do not produce the enzyme that breaks down these fatty acids in the normal manner. The loss of myelin and the progressive dysfunction of the adrenal gland are the primary characteristics of ALD.
Behavioural changes such as abnormal withdrawal or aggression, poor memory, and poor school performance are the two major symptoms. Among other symptoms of adrenoleukodystrophy are visual loss, learning disabilities, seizures, poorly articulated speech, difficulty swallowing, deafness, disturbances of gait and coordination, fatigue, intermittent vomiting, increased skin pigmentation, and progressive dementia.
In all patients with ALD, adrenal function must be tested periodically. The treatment with adrenal hormones can be lifesaver. The symptomatic and supportive treatment options are physical therapy, psychological support and special education.
There is scientific evidence to suggest that a mixture of oleic acid and erucic acid, known as "Lorenzo's Oil," can reduce or delay the appearance of symptoms. Bone marrow transplants can provide long-term benefits. The treatment options also carry the risk of mortality and morbidity. In some cases, oral administration of docosahexanoic acid (DHA) may help infants and children with neonatal ALD.
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