Neurological Disorders: Adrenoleukodystrophy

Introduction

Adrenoleukodystrophy (ALD) is a type of leukodystrophy of the CNS (central nervous system). CNS or central nervous system is the term used to refer both the brain and spinal cord. Leukodystrophy is the term used for certain type diseases of the white matter of the brain and these are mostly progressive neurological disorder (degenerative neurological disorders). The white matter of the brain and the spinal cord is mainly formed by the myelin tissue - which is a complex of fat and protein and the leukodystrophy results due to the damage to the myelin tissue so it is appropriately considered as an example for white matter diseases. These leukodystrophies mostly involve the myelin of the central nervous system although certain types of leukodystophies may also involve the myelin tissue of the peripheral nervous system too.

For additional details on the basic information, classification, clinical manifestations etc of different white matter diseases (leukodystrophies) please click on Leukodystrophy . For reading further on ALD you may continue below.

What causes ALD?

It is an inherited type of neurological disorder. There are x linked & autosomal recessive forms. X linked is the commonest form. There is problem in the metabolism of very long chain fatty acids.

Types of ALD

The x linked has neonatal, childhood & adult forms but the childhood type is the commonest (manifests around age 3 and 10 usually).

Adrenomyeloneuropathy is a type of x linked ALD and presents in adults and characteristically involves the spinal cord and peripheral nerves without any prominent brain involvement.

The autosomal recessive type presents typically as a neonatal type.

The adrenal gland involvement is common for all these ALDs. Progressive adrenal gland failure will manifest as low blood sodium, low blood pressure (hypotension), high blood potassium, also abnormal pigmentation of skin. This is in addition to the progressive neurological manifestations mentioned in the leukodystrophy article.

Diagnosis

The following tests are usually employed while establishing the diagnosis of ALD;

• VLCFA (very long chain fatty acid) analysis of the blood
• Brain MRI scan will show some typical white matter changes
• Spinal cord MRI scan (especially for adrenomyeloneuropathy type)
• Genetic tests
• EMG/NCS (electromyography & nerve conduction studies) etc

Treatment

There is no cure for this disease as of now. So the management is focused on symptomatic & supportive therapy.

Lorenzo oil, with a diet of low amounts of VLCFA, if started very early in the disease may help some patients.

Bone marrow transplantation, gene therapy etc are some of the research based therapies available for the management of Adrenoleukodystrophy condition.



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