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Alexander’s disease – a leukodystrophy

What exactly is this monster we face?

Axd is a neurodegenerative fatal disease. It is a genetic disorder affecting the central nervous system. It causes delays in development of physical, psychological and behavioural skill and a gradual loss of bodily functions.

Axd belongs to a group of diseases known as leukodystrophies. These affect the growth and development of the myelin sheath (the fatty, white substance surrounding our nerve cells.) Consequently, the white matter of the brain is gradually destroyed. In Axd fibrous, eosinophilic deposits also form in the astrocytes of the brain. These are known as rosenthal fibres. The build up of these  can form gliomas/tumours, which can vary in being low grade and slow growing, to more rapid, resulting in a need to use therapy or surgery.

Currently statistics suggest that approximately 500 cases of Alexander’s disease are reported. Most of these cases are of infantile and juvenile onset, with adult onset being less common. There is no cure at present, with prognosis being poor. Generally an early onset of death is expected, usually within 10 years from the onset. Typically, the earlier in life the onset occurs, the quicker its course will run.

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