Vanishing white matter disease

Vanishing white matter disease (VWM) is one of the most prevalent inherited childhood white-matter disorders. It was described in 1962 by Eicke. VWM is caused by mutations in any of the five genes encoding the subunits of eukaryotic translation initiation factor eIF2B, and is manifested by chronic progressive neurological deterioration with cerebellar ataxia and mild mental decline. The disease is usually revealed at an early age of two to six years, and most of the patients die within several years after diagnosis.

VWM disease stems from a severe deterioration of white matter, which shows myelin loss, abnormal formation of myelin sheaths, vacuolation; the white matter degenerates and appears cystic; cavities are often observed. Around these cavities a pronounced loss of oligodendrocytes is detected; many oligodendrocytes also have an abnormal morphological appearance. The astrocytes are dysmorphic with blunt broad processes rather than their typical delicate arborizations.

There is no specific treatment for VWM.

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