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January 26, 1935

Diffuse Sclerosis (Encephalitis Periaxialis Diffusa)

JAMA. 1935;104(4):342. doi:10.1001/jama.1935.02760040074031

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Diffuse sclerosis, also known as Schilder's disease and under many other names, has attracted much attention during the last twenty years, though it has been known for more than fifty years. Strümpell, for instance, mentions it even in the oldest editions of his textbook, but the pathologic basis was first emphasized clearly and properly by Schilder. In diffuse sclerosis a vast destruction of the subcortical white substance, mainly of the occipital and temporal lobes, takes place, producing a clinical picture that can sometimes be diagnosed at the bedside. Blindness, and deafness setting in acutely or subacutely, combined with speech, motor and often psychic disturbances are the frequent symptoms and signs. In many cases, however, neither the clinical nor the histopathologic picture is characteristic enough to enable one to make a correct diagnosis. Nor is it always possible to make a definite diagnosis post mortem, as the main histopathologic feature, demyelinization

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