Leukodystrophy

Summarized by Plex Health
Last Updated: 04 May 2022
medpix case - x-linked adrenoleukodystrophy "medpix case - x-linked adrenoleukodystrophy", by Original Source: Dawn Light, MD, MPH; Author: Ronald M Erdelyi (Childrens Hospital of Dayton, OH); Approved By: Dawn E Light (Childrens Hospital of Dayton, OH);. T1-weighted MRI image showing significant low signal cerebral white matter abnormalities consistent with X-ALD....

Leukodystrophy refers to hereditary diseases that mainly influence the white matter of the central nervous system. White matter is tissue comprised of bundles of nerve fibers that link nerve cells. Myelin provides nutritional support to nerve cells and assists speed up signals between them, permitting them to send out and obtain messages swiftly. It is generally described as the myelin sheath. Leukodystrophy is not a single disorder. It is a group of rare, mostly inherited neurological disorders referred to as the leukodystrophies that arise from the abnormal production, processing, or development of myelin and other components of CNS white matter, such as the cells called oligodendrocytes and astrocytes. Astrocytes and oligodendrocytes belong to a group of cells called glial cells which border, sustain, and shield nerve cells. Each type of leukodystrophy is triggered by a flaw in among the genes that control the framework or amount of among the proteins or lipids in myelin. Mutations in any one of these genetics can influence just how the lipid or protein works and can disturb the normal development, processing, and advancement of myelin and the function of white matter. Damages to or damage of myelin can delay the rate or slow down of brain signals or keep them from reaching their destination. Symptoms of leukodystrophy vary according to the specific type and might be challenging to recognize in the beginning of the disorder. Each type of leukodystrophy impacts myelin in different ways and in different parts of the CNS, leading to a series of symptoms.

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