Adrenoleukodystrophy, or ALD, is a genetically determined neurological disorder that affects 1 in every 17,900 boys worldwide. The presentation of symptoms occurs somewhere between the ages of 4 and 10, and affects the brain with demyelination..
Demyelination is the stripping away of the fatty coating that keeps nerve pulses confined and maintains the integrity of nerve signals. This process inhibits the nerves ability to conduct properly, thereby causing neurological deficits, including visual disturbances, auditory discrimination, impaired coordination, dementia, and seizures. Demyelination is an inflammatory response and nerve cells throughout the brain are destroyed.
Boys develop normally until the onset of symptoms occurs. Symptoms typically rival those of attention deficit disorder before serious neurological involvement becomes apparent. The symptoms progress rapidly and lead to vegetative state within two years, and death anytime thereafter.
Adrenocortical insufficiency, Addison's Disease, is seen in 90 percent of the cases of ALD.