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December 1969

Subacute Multifocal Leukoencephalopathy: With Widespread Intranuclear Inclusions

Author Affiliations

From the Department of Pathology, Cleveland Metropolitan General Hospital and Case Western Reserve University School of Medicine, Cleveland.

Arch Neurol. 1969;21(6):590-602. doi:10.1001/archneur.1969.00480180046003

THE OCCURRENCE of unique demyelinative lesions of the brain associated with malignant diseases of the reticuloendothelial system was first described by Astrom et al.1 This disorder, to which the name progressive multifocal leukoencephalopathy has been applied, is most often associated with leukemia and lymphoma. It has been reported in a variety of other disorders including tuberculosis,2 sarcoidosis,3 carcinomatosis,4 multiple myeloma,5,6 Whipple's disease,7 primary hypersplenism,8 polycythemia vera,9 and coronary artery disease.10 The medical literature contains about 60 pathologically verified cases of progressive multifocal leukoencephalopathy. Included within this group is a case described by Castaigne et al,11 the neuropathological features of which did not conform to those usually observed in progressive multifocal leukoencephalopathy. The case which follows, observed in association with reticulum cell sarcoma, is being presented because of its unusual pathological features which, like those of the French case,11

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