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Alexander disease

Background

Alexander disease: Alexander Leukodystrophy

Alexander disease is a rare genetic, degenerative disorder of the nervous system with effects on the mid brain and cerebellum; in the most common infantile form of the disease, the frontal white matter is involved. It is one of a group of genetic disorders called the leukodystrophies. The term leukodystrophy comes from the Greek words, ‘leuko’ meaning white and referring to the ‘white matter’ of the nervous system and ‘dystrophy’ meaning imperfect growth or development. In Alexander disease, the growth of the white matter of the brain, or myelin sheath, is affected. The brain stem in particular may be involved in rare adult forms of the condition resulting in unusual signs such as palatal myoclonus (flapping movement of the palate), limb paralysis and clumsy movements. Cerebellar disease is also known to occur.

What are the symptoms? View What are the symptoms?

Medical text written November 2000 by the Alzheimer’s Society. Approved November 2000 by Professor T M Cox. Last updated January 2008 by Professor T M Cox, Professor of Medicine, University of Cambridge, Cambridge, UK.

 

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