Although the intracranial and optic nerve pathology in Schilder's disease is well known, the writers have been able to find only a rare report of its intraocular pathology.1,2 It is the purpose of this paper to report the intraocular pathology in a proven case of this disease.
Schilder's disease, also known as encephalitis periaxialis, or progressive degenerative subcortical encephalopathy, is one of the demyelinating diseases affecting the white matter of the cerebral hemispheres. It is characterized by spastic paralysis, mental deterioration, blindness, deafness, and eventually death. It is most often seen in infants and young children. The course may be acute, subacute, or chronic. The development of the signs mentioned above depends on which area of the brain is affected. In children epileptiform seizures are also seen. Blindness usually occurs late in the disease and a majority of the patients go on to partial or complete blindness. Optic nerve
TAMLER E, EISSLER R. The Intraocular Pathology in Schilder's Disease. Arch Ophthalmol. 1961;65(4):514-516. doi:10.1001/archopht.1961.01840020516009