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Further Observations In Progressive Supranuclear Palsy

N. J. David, E. Mackey, J. Smith
Published 1968 · Medicine

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“PROGRESSIVE SUPRANUCLEAR PALSY” is a diagnostic term coined by Steele and associates1 to describe a heterogenous, neuronal degenerative disease, which they were first to characterize adequately with clinicopathological studies. A syndrome was described in nine middle-aged men, the clinical characteristics of which included impressive supranuclear paralysis of gaze movements, especially in the vertical direction; dystonic rigidity of the face, neck, and upper trunk; dementia; and variable corticospinal and cerebellar signs. Of the five patients examined postmortem, all showed loss of neurons, gliosis, and neurofibrillary tangles in a distinctive pattern centered in the brainstem, with surprising lack of cortical involvement. Aside from their thorough tabulation of the topography of the lesion, they made no specific attempt at neuro-ophthalmologic interpretation of the striking defect in ocular motility. The purpose of this report is to describe six additional patients with the disorder, two of whom have been studied at autopsy. All patients conformed to the unmistakable clinical criteria set down by Steele and co-workers.’ Of interest are the four women in our series (since all previously reported patients have been men) and the description of a very similar fatal illness in one patient’s mother.
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