The morbid anatomy of the demyelinative diseases

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Abstract

A demyelinative disease is one in which there is destruction of myelin sheaths with relative sparing of axis cylinders, nerve cells and supporting tissues, occurring usually in multiple foci within the central nervous system. The diseases which conform to these criteria are acute and chronic multiple sclerosis, acute disseminated encephalomyelitis, acute necrotizing hemorrhagic encephalomyelitis and Schilder's diffuse periaxial encephalitis. Brain purpura and metachromatic leucoencephalopathy resemble the demyelinative diseases in some respects but because of significant pathologic differences probably should not be included in the group. The pathology of these diseases is described. Each of the demyelinative diseases exhibits distinctive pathologic changes from which the diagnosis can usually be made without difficulty. There are, however, rather impressive similarities that may be overlooked. The essential process in these diseases is a focal necrobiosis of nervous tissue which varies in degree from degeneration of myelin sheaths with sparing of the axis cylinders to an almost complete degeneration or necrosis of all elements of nervous tissue. Common to all of the demyelinative diseases is a tendency for this focal necrobiosis to occur in relation to small veins and to involve the white matter in particular. This latter feature suggests that some property of myelin determines the localization of the pathology. Exudates of inflammatory cells in the perivascular spaces is another important feature. Theories as to the etiology and pathogenesis of these diseases are evaluated in the light of the morbid anatomy.

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    This study was aided by a Grant from the National Multiple Sclerosis Society.

    1

    From the Neurological Service, Massachusetts General Hospital and the Department of Neurology, Harvard Medical School, Boston, Mass.

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