The multiplicity of terms applied to Schilder's disease (encephalitis periaxialis diffusa)1 and similar clinicopathologic conditions is an indication of the nebulous state in which the problem of unifying the demyelinating diseases of the central nervous system remains. With each contribution to the literature there emerges a clearer concept of the possible etiologic factors. The apparent diversity of histopathologic appearance in reality may merely represent different phases of a fundamentally similar process.
The clinical manifestations of Schilder's disease are so multiform that Bouman2 stated, in effect, that no uniform symptom complex could be formulated. Ferraro,3 in his unique article in which he essayed the difficult task of classifying the primary demyelinating processes of the central nervous system, gave a review of the literature and a comprehensive outline of the clinical and pathologic observations in cases of Schilder's disease and related disorders.
Because of the varied mode of onset
Winkelman NW, Moore MT. CHRONIC PROGRESSIVE DEGENERATIVE ENCEPHALOPATHY: A Clinicopathologic Study of an Unusual Case of Schilder's Disease. Arch NeurPsych. 1939;41(4):773–787. doi:10.1001/archneurpsyc.1939.02270160129010
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