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X-linked Adrenoleukodystrophy

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by Alan R

(ADL; Sudanophilic Leukodystrophy; Schilder’s Disease)

 

Symptoms

Symptoms can vary within the types of ALD.

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 20s. It progresses slowly. They can include:
  • Weakness, clumsiness, weight loss, 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 (20s) or middle age (50s). 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.
 

Treatment

There is no known cure for the brain damage of ALD. However, the adrenal deficiency can be treated with cortisone replacement. ALD often causes death within 10 years of the onset of symptoms.
Some therapies can help manage the symptoms of ALD. These include:
  • Physical therapy
  • Psychological therapy
  • Special education for children
There are also some experimental treatments. Some treatments that are still be investigated that you may want to talk to your doctor about include:
  • Bone marrow transplantation —this procedure may be most helpful when given early to boys with X-linked child-onset ALD
  • Dietary therapy, which includes consumption of:
    • A very low fat diet
    • Lorenzo’s oil—dietary supplements of glycerol trioleate and glycerol trierucate
  • Lovastatin—an anti-cholesterol medication
  • Medications that affect interactions between DNA and other proteins
 

RESOURCES

National Institute of Neurological Disorders and Stroke
http://www.ninds.nih.gov

United Leukodystrophy Foundation
http://www.ulf.org

 

CANADIAN RESOURCES

Health Canada
http://www.hc-sc.gc.ca

The Myelin Project of Canada
http://www.myelin.org

 

References


Berger J, Pujol A, Aubourg P, Forrs-Petter S. Current and future pharmacologic treatment strategies in X-linked adrenoleukodystrophy. Brain Pathol . 2010;20(4):845-856.


Moser HW. Therapy of X-linked adrenoleukodystrophy (review). NeuroRx. 2006 . Apr;3(2):246-53.


Moser HW, Raymond GV, Dubey P. Adrenoleukodystrophy: new approaches to a neurodegenerative disease. JAMA . 2005 Dec 28;294(24):3131-4.


Moser HW, Raymond GV, Lu SE, Muenz LR, Moser AB, Xu J, et al. Follow-up of 89 asymptomatic patients with adrenoleukodystrophy treated with Lorenzo's oil. Arch Neurol . 2005 Jul;62(7):1073-1080.


NINDS adrenoleukodystrophy information page. National Institute of Neurological Disorders and Stroke website. Available at: http://www.ninds.nih.gov/disorders/adrenoleukodystrophy/adrenoleukodystrophy.htm . Updated October 22, 2012. Accessed July 16, 2013.

 

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