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Abstract In this report we are describing a progressive brain disease featured by supranuclear ophthalmoplegia affecting chiefly vertical gaze, pseudobulbar palsy, dysarthria, dystonic rigidity of the neck and upper trunk, and other less constant cerebellar and pyramidal symptoms. Dementia has usually remained mild. This disease would appear to be predominantly a nerve cell degeneration centered chiefly in the brain stem. The fully developed clinical picture presented by this disease seems to follow a fairy definitive pattern and does not conform to the classical system degenerations such as motor neurone disease, paralysis agitans, cerebellar degeneration, Creuzfeldt-Jakob disease, or the presenile dementias. Yet it would seem unlikely that the disease shown by our cases is a new one, and similar earlier cases may well have been accepted as arteriosclerotic parkinsonism when that diagnosis was used in a very broad sense such as in Critchley's monograph of 1929.[1] There are some resemblances to Lhermitte's pyramidopallidal syndrome and to the cases with rigidity and dementia sometimes called Jakob's disease. However, none of these older writings seem to describe the peculiar combination of oculomotor, dystonic, pseudobulbar, and mental signs that has seemed to develop in a common mold in our cases. Moreover, the pathological findings which were provided by our case were clearly not arteriopathic and seem to show a specific type and pattern of nerve cell change. Verhaart[2] has described one case with pathological features very similar to ours, though the clinical findings differ, and has provided the term “heterogeneous system degeneration of the central nervous system.” Brusa[3] has presented a case difficult to classify with the same type of pathological changes as shown in our cases and has presented a useful classification of transitional form, or multisystem, degeneration of the nervous system. The one case which does bear striking clinical and pathological resemblance to the ones here reported was published by Chavany et al,[4] and this will be considered further in the comment after the presentation of cases. Presented at the meeting of the American Neurological Association, Atlantic City, June, 1963 (clinical observations) and the meeting of the American Association of Neuropathologists, Atlantic City, June, 1963 (neuropathological findings). Supported in part by a grant from the Canadian Medical Research Council to Dr. Olszewski. This is a second publication. This article is reprinted with permission from Arch Neurol 1964;10(4):333-359. Copyright © (1964) American Medical Association. All rights reserved.