Leukodystrophy Sudanophilic

Leukodystrophies are a specific form of diffuse demyelinating damage to the central nervous system, characterized by a hereditary predisposition and a gradual progressive course without obvious periods of remission. According to the course of the process, A. N. Nasonov distinguishes the leukodikisticoform variety (with predominant damage to the lateral ventricles of the brain), as well as the spinocerebellar one. Among the latter, the bulbar-spinal variety of the cerebrovascular type is distinguished (in the absence of a vascular history). With the bulbar variety of the cerebral form of leukodystrophic disease, cerebellar disorders (ataxia) and damage to the brain stem (ophthalmoplegia, pseudobulbar syndrome) are observed, dilation of the dilated ventricles of the brain and their asymmetry are observed (in the presence of cerebrospinal fluid in the meninges). Diagnosed at the age of 3-7 years, the peak incidence occurs at the age of 5-6 years. Death is possible during the first year of life.

Pathomorphologically, the disease is manifested by changes from atrophy of the hemispheres of the telencephalon with expansion of the ventricles of the cerebral type, atrophy of the meninges and cerebellar vallecules, or the absence of signs.