ALD Foundation: 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.