Stages

Classic childhood form (X-linked adrenoleukodystrophy)

In this case of ALD, the child usually develops normally usually up to 3 or 4 years of age. The first noticeable problems are behavioral changes such as hyperactivity, difficulty paying attention, aggression, poor academic performance, difficulty reading and comprehending and abnormal withdrawal. In this stage, children are most often misdiagnosed with ADHD. As the disease progresses, more serious difficulties occur such as loss of hearing and vision. The child is unable to discriminate sounds and has difficulty understanding speech. Also, they may develop an inability to see visual information on the left or right side, may not be able to see clearly, and double vision. This eventually leads to deafness and blindness. ALD patients will then experience difficulty walking, swallowing, have learning impairments such as poor memory, difficulty in speech articulation due to the inability to control muscles involved in speech, fatigue and seizures. About 33% of patients with ALD develop seizures, which may be the first sign of ALD. 90% of children develop insufficient production of hormones by adrenal glands by the time signs of the disease are first noticed. From the onset of the initial symptoms, patients can be in a vegetative state within 6 months to 2 years, although rate of progression varies in different patients. Death can occur anytime after symptoms begin, but usually occur within 1 to 10 years.

Adult-onset form (adrenomyeloneuropathy)

This form of ALD usually occurs between the ages of 21 and 35. It progresses more slowly than the childhood form, with the first symptoms being stiffness and weakness in legs that worsen over time. Patients develop ataxia (impairment in coordinating movements), difficulty walking, sensory loss, and loss of bladder control (no sphincter control). About half of patients show brain damage, with 33% having myelin loss, and 10-20% showing severe brain damage. This usually results in total disability and death. 70% have insufficient production of hormones by the adrenal glands. This type of ALD usually progresses over a 5 to 15 year period, but can go progressing for decades.

Nevena V.

References:

http://www.medfriendly.com/adrenoleukodystrophy.php5

Moser MW, Mahmood A, Raymond GV (2007) X-liked adrenoleukodystrophy. Nature Review 3(3): 140-151.