Neuro Disorders: Alexander Disease

Introduction

Alexander Disease is a type of leukodystrophy or white matter disease of the CNS (brain & spinal cord). It is a progressive (degenerative) neurological disease.

There are usually 3 types; infantile, juvenile and adult forms and as the name implies they present around these particular ages.

For a detailed article on Leukodystrophy please click on the link.

What causes it?

A mutation in the gene that codes for GFAP or Glial Fibrillary Acidic Protein on chromosome 17 causes this disease. So it is a genetic disease, however it is not hereditary in nature since parents do not carry the abnormal gene.

Diagnosis

This disease shows certain characteristic radiological findings including white matter changes especially in the frontal area, enlargement of the lateral ventricles etc. Histopathology show characteristic Rosenthal fibers. A genetic test may identify the gene mutation responsible for this disease.

Treatment

This is an example of degenerative diseases of the nervous system and accordingly there is no cure possible at this moment. So supportive & symptomatic treatments are only the options. Certain resarch level treaments are going on e.g. bone marrow transplantation etc.

A neurologist, pediatric and/or adult is commonly involved in the management of this type of leukdystrophy.



Alexander Disease to Neurology Articles

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