Among ocular symptoms, nystagmus, strabismus, conjugate devi ation, choked disc and atrophy of the optic nerve are observed. The pupils react promptly to light and accommodation.
Disturbances of deglutition, phonation and the movements of the tongue are present in rare eases.
The course of the disease is occasionally interrupted by apoplecti form, rarely epileptic attacks, which leave the child in a much worse condition. Death ultimately results from decubitue, pneumonia or some other terminal condition.
The above-described symptoms are by no means present in every ease. Many- of the symptoms may be wanting and, on the other hand, the course not always steadily progressive as here described. It must be remembered that the clinical picture is as yet but imperfectly defined and an accurate deseription of the symptoms cannot be thought of.
Such pathologic observations as have been made in diffuse sclerosis arc, in the main, quite uniform. Not only the brain, but the medulla oblongata and the spinal cord as well. is diffusely thickened and hard ened, so that cross-sections retain their sharp edges which is not usually the case in the brains of children. The hardening and thickening process
is particularly marked in the white substance and in the basal ganglia. The color of the medullary substance is yellowish white, that of the cortex a pale gray. There are no areas of special thickening. Histo logically a considerable increase of the connective tissue and of the medullary- cells is found, while the ganglionic eleme»ts are intact. Ac cording to Schinaus, the pathologic picture represents the entl-product of an interstitial inflammatory process and his view has been aceepted by most authors.
The term pseudosclerosis is applied to a condition characterized by. symptoms such as are usually ascribed to multiple eclerosis--disturbance of tile speech, tremor, nodding of thc head and disturbance of degluti tion—and by complete absence of all pathologic findings. In children peetaloselerosis appears to be an endogenous disease i Frankl-Hochwart).