Adrenoleukodystrophy (ALD) is one of a group of genetic disorders called the Leukodystrophies. Those with the condition accumulate high levels of saturated fatty acids (VLCFA) in the brain and adrenal cortex. The genetic disorders inflict damage to the myelin sheath, an insulating membrane that surrounds nerve cells in the brain. The loss of myelin and the progressive dysfunction of the adrenal gland are the primary characteristics of ALD.
Those with the condition may experience behavioural changes such as abnormal withdrawal or aggression, poor memory, and poor school performance. 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 the patients with ALD, adrenal function must be tested periodically. The treatment symptomatic and supportive treatment options are physical therapy, psychological support and special education. The treatment with adrenal hormones can be lifesaver. The studies have found that a mixture of oleic acid and erucic acid, known as "Lorenzo's Oil," can also reduce or delay the appearance of symptoms.
Bone marrow transplants may render long-term benefits to patients. In infants and children, oral administration of docosahexanoic acid (DHA) may prove helpful. However, the treatment options do carry the risk of mortality and morbidity.
The prognosis for patients with ALD is not quite bright because of the progressive neurological deterioration. After the onset of symptoms, the condition becomes life-threatening.
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