DIFFUSE LEUKOENCEPHALOPATHY WITHOUT SCLEROSIS

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Abstract
Schilder's disease and diffuse sclerosis are terms which have frequently been used in the neurologic and pediatric literature to designate a condition characterized pathologically by diffuse and symmetric degeneration of the white substance of the brain. The disease usually occurs in children, but it may also appear in adults, the patients showing signs of disease of the brain, such as muscular hypertonicity, spastic paralysis, tonic and epileptic fits, choreoathetoid movements and mental deterioration. The onset of the disorder is usually slow, and the course is variable but always progressive, leading to a fatal termination. The terms Schilder's disease and diffuse sclerosis are popular among certain neuropathologists and neurologists. Many have not liked the former designation because Schilder, in describing his case, used the term "encephalitis periaxialis diffusa." In the majority of cases, however, signs of inflammation are not shown, and the disease is axial, as well as periaxial, since axis-cylinders,

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