X-linked Adrenoleukodystrophy

(ALD; Sudanophilic Leukodystrophy; Schilder’s Disease)


X-linked adrenoleukodystrophy (ALD) is a rare inherited genetic disorder. There are 40 subtypes of leukodystrophy. X-linked ALD is the most common category. ALD results in degeneration of the:
  • Myelin sheath—the fatty insulation covering on nerve fibers in the brain
  • Adrenal gland and surrounding adrenal cortex—they produce vital hormones
Myelin Sheath Around Nerve Fiber
AX00010 97870 1 myelin sheath
Copyright © Nucleus Medical Media, Inc.
There are 6 subtypes of X-linked ALD:
  • Childhood cerebral ALD
  • Adolescent ALD
  • Adrenomyeloneuropathy
  • Adult cerebral ALD
  • Adrenal insufficiency-only
  • Symptomatic heterozygotes


Adrenoleukodystrophy (ALD) is caused by an inherited defective gene.In people with ALD, the body cannot properly break down fatty acids. This results in a build up of saturated fatty acids in the brain and the adrenal cortex. This causes damage to the myelin sheath in the brain and adrenal gland.

Risk Factors

This condition is most common in males, although females may also be affected. It is also more common in children and young adults. Having a mother who carries the defective X-linked adrenoleukodystrophy (ALD) gene may increase your risk.


Symptoms can vary within the types of ALD.

X-linked ALD (Childhood Cerebral ALD)

This form is the most severe. It only affects boys. Symptoms usually begin between 2–10 years of age. About 35% of boys can have severe symptoms during the early phase. On average, death results in 2 years, though some may live a couple of decades.Initial symptoms include:
  • Behavioral changes
  • Poor memory
  • A slowing down of thought processes and physical activity
  • Developmental regression
As the disease progresses, more serious symptoms develop. These include:
  • Vision loss
  • Seizures
  • Hearing loss
  • Difficulty swallowing and speaking
  • Difficulty with walking and coordination
  • Vomiting
  • Fatigue
  • Increased pigmentation of the skin, due to adrenal hormone deficiency—Addison’s disease
  • Progressive dementia
  • Vegetative state or death

X-linked ALD (Adolescent Cerebral ALD)

This type is similar to the childhood type. It begins around 11-21 years of age. The progression is usually slower.

Adrenomyeloneuropathy (AMN)

This is the most common form. Symptoms of AMN can present in the 20's. It progresses slowly. Symptoms can include:
  • Weakness, clumsiness, weight loss, and nausea
  • Emotional disturbances or depression
  • Motor/movement problems, such as walking problems
  • Urinary problems or impotence

X-linked ALD (Adult Cerebral ALD)

With this type, symptoms usually do not appear until young adulthood (20's) or middle age (50's). It causes symptoms similar to schizophrenia and dementia. It usually progresses quickly. Death or a vegetative state can occur in 3-4 years.

Symptomatic Heterozygotes

This form is only seen in women. Symptoms may be mild or severe. It usually does not affect the adrenal gland function.

leave comments
Did you like this? Share with your family and friends.
Related Topics:
Current Research From Top Journals

Music May Improve Sleep Quality in Adults with Insomnia
October 2015

Insomnia can make your days miserable and a cure can be hard to find. There are some medications but there is some worry with side effects and the potential for addiction. Music is side effect free and according to this study may help you find sleep.

dot separator
previous editions

CPAP May Help Older Adults with Obstructive Sleep Apnea
September 2015

Water Before Meals May Promote Weight Loss
August 2015

Fecal Transplants Induce Ulcerative Colitis Remission
July 2015

dashed separator


Our Free Newsletter
click here to see all of our uplifting newsletters »