Skip navigation | en español search this site: search you are here: home » disorders a - z » adrenoleukodystrophy » skip secondary menu home disorders a - z adrenoleukodystrophy information page publications organizations news research literature other related groups organizations professional societies government resources ninds clinical trials en español order brochures news from ninds recovery act funding information training & career awards diversity programs research programs find people about ninds email this page print-friendly version contact ninds adobe reader microsoft word viewer ninds adrenoleukodystrophy information page table of contents (click to jump to sections) what is adrenoleukodystrophy? Is there any treatment? What is the prognosis? What research is being done? Clinical trials organizations what is adrenoleukodystrophy? Adrenoleukodystrophy (ald) is one of a group of genetic disorders called the leukodystrophies that cause damage to the myelin sheath, an insulating membrane that surrounds nerve cells in the brain. People with ald accumulate high levels of saturated, very long chain fatty acids (vlcfa) in the brain and adrenal cortex 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. Ald has two subtypes. The most common is the x-linked form (x-ald), which involves an abnormal gene located on the x-chromosome. Women have two x-chromosomes and are the carriers of the disease, but since men only have one x-chromosome and lack the protective effect of the extra x-chromosome, they are more severely affected. Onset of x-ald can occur in childhood or in adulthood. viagra without a doctor prescription classicmotocrossimages.com/mbs-canadian-pharmacy-viagra-no-prescription-ex/ floridalighttacklecharters.com/thq-secure-canadian-pharmacy-online-cheap-viagra-iq/ buy cheap viagra cheap viagra online viagra online generic viagra viagra without a doctor prescription cheap generic viagra http://classicmotocrossimages.com/mbs-order-cheap-viagra-online-jt/
The childhood form is the most severe, with onset between ages 4 and 10. The most common symptoms are usually behavioral changes such as abnormal withdrawal or aggression, poor memory, and poor school performance. Other symptoms include 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 the milder adult-onset form, which typically begins between ages 21 and 35, symptoms may include progressive stiffness, weakness or paralysis of the lower limbs, and ataxia. Although adult-onset ald progresses more slowly than the classic childhood form, it can also result in deterioration of brain function. A mild form of ald is occasionally seen in women who are carriers of the disorder. Symptoms include progressive stiffness, weakness or paralysis of the lower limbs, ataxia, excessive muscle tone, mild peripheral neuropathy, and urinary problems. Is there any treatment? Adrenal function must be tested periodically in all patients.
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