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ROBERT F. JOHNSTON, M.D.; ROBERT A. GREEN, M.D.; JOHN T. HEADINGTON, M.D.

Arch Intern Med. 1963;111(3):353-362.

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In 1958, Åström, Mancall, and Richardson described a demyelinating disease distinct from multiple sclerosis and other demyelinating disorders which they called progressive multifocal leukoencephalopathy.¹ The condition is characterized by progressive and fatal neurologic deterioration with "dementia, alterations in the state of consciousness, language disorders, visual impairment and evidence of pyramidal tract involvement."¹ Pathologically, the brain shows widespread focal destruction of myelin sheaths in all gradations of size and states of evolution with relative sparing of the axis cylinders. "These foci have shown a pronounced tendency to become confluent so that large plaquelike lesions may occur. Associated with the myelin destruction is a distinctive cytological reaction consisting of hypertrophy of astrocytes into bizarre gigantic forms and alteration of oligodendrocytes with the production of large, round, densely basophilic nuclei."¹ Recently, Richardson reviewed the subject and accepted a total of 24 cases as progressive multi focal leukoencephalopathy. The additional material . . . [Full Text PDF of this Article]

Author Affiliations
ANN ARBOR, MICH.

Instructor, Department of Medicine, University of Michigan Medical School, Ann Arbor, and Physician, Department of Medicine, Wayne County General Hospital, Eloise, Mich. (Dr. Johnston); Assistant Professor of Medicine, University of Michigan Medical School, Ann Arbor, and Chief, Pulmonary Disease Section, Veterans Administration Hospital, Ann Arbor (Dr. Green); Formerly an Instructor, Department of Pathology, University of Michigan Medical School, Ann Arbor (Dr. Headington).; From the Medical and Pathology Services, Veterans Administration Hospital, and the Departments of Medicine and Pathology, University of Michigan Medical Center.

Footnotes
Received for publication Sept. 27, 1962; accepted Nov. 19.

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