Leukodystrophy Orthochromatic

Leukodystrophies can occur in both peripheral nerves and internal organs, but in this text I will talk about the type of leukodystrophies that affect peripheral nerves. One of the most common types of such lesions is called Orthochromatic leukodystrophy **(L. Orthochromatica)**, which means “unwelcoming” or “non-first passer”.

Orthochromatic leukodystrophy can be defined as a pathological condition that is accompanied by a deficiency of myelin in peripheral nerve fibers, usually manifests as changes in motor function and can cause loss of sensation and motor function in the extremities. Changes in myelin levels can lead to weakened nerve conduction, leading to impaired joint movement in the fingers, loss of the ability to hold certain objects in the hands, and difficulty swallowing and chewing. This can cause significant difficulties for patients and requires ongoing treatment.

The basis of the disease consists of a mutation in the _APHO_ gene, which encodes the basic neuronal myelin protein. This

Leukodystrophies This is a group of hereditary and acquired diseases of the central nervous system that lead to the development of dystrophic changes in the white matter of the brain and spinal cord. Orthochromatic leukodystrophies (OL) include types of dystrophies in which the destruction of white matter predominates in the periphery and the white matter in the central sections is relatively little affected, which is manifested by the preservation of clear boundaries between the gray and white matter in these sections and the presence of traces of multiple scarring. The article discusses orthochromatic types of leukodystrophies - Von Rechtzamerscheidt-Rubasch disease, Leber disease/amaurotic idiocy and Forrer disease.