NECROTIC ASPECTS OF MULTIPLE-SCLEROSIS AND SCHILDERS DISEASE

  • 1 January 1981
    • journal article
    • research article
    • Vol. 137  (10) , 589-600
Abstract
Two anatomo-clinical cases of a necrotic form of demyelinating disease are reported. The disease occurred in 2 women, had a late onset (patients were about 50 yr old) and had a relapsing-remitting course during more than 10 yr. The CSF displayed a high protein level over 125 mg/100 ml whereas the .gamma.-globulin level was normal. The anatomical study found symmetrical cavitations involving both hemispheres and optic tracts with clear-cut limits. Axons and myelin were both destroyed, only the vascular network being partially spared. At the lesion''s border-line mononuclear cell infiltrates and some phagocytes with sudanophilic inclusions were found. The scarcity of the compound granular corpuscules suggest an old pathological process. A narrow zone of myelin-axonal dissociation was also observed. Astrocytic proliferation was unimportant. Blood vessels were normal. In 1 case plaques of multiple sclerosis were found in the spinal cord. Those 2 cases are unusual forms of a diffuse disseminated sclerosis: multiple sclerosis and Schilder''s disease are considered as 2 anatomo-clinical variants of the same pathological process. The observed necrotic lesions are different from acute necrotic forms of multiple sclerosis as the latter rapidly developed. The long lasting course of the disease, over 10 yr, allowed a complete resolution of the lesions explaining the cavitations. The late onset of the disease and the high CSF protein level are pointed out. The significance of the high protein level and normal .gamma.-globulin level in the CSF is discussed.

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