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[Progressive supranuclear palsy: clinical report on 7 cases and review of literature].

[Progressive supranuclear palsy: clinical report on 7 cases and review of literature].

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Original abstract

Progressive supranuclear palsy is sufficiently rare and difficult to diagnose that it escaped clinical recognition until 1964, when Steele, Richardson, and Olszewski clarified it as a pathologic entity. From January 1981 to June 1989, 7 patients who fulfilled the Golbe's criteria of progressive supranuclear palsy were admitted to the neurological department of National Taiwan University Hospital. The mean age at onset was 60 years (range: 53-69 years), and the mean age at diagnosis was 63 years), and the mean age at diagnosis was 63 years (range: 55-71 years). The study failed to identify any specific risk factors associated with progressive supranuclear palsy. Unsteady gait, vertical gaze palsy, pseudobulbar palsy, parkinsonian features and dementia were noted in all cases. The brain computed tomography revealed mild cortical atrophy and ventricular dilatation in 5 patients. Additionally, electroencephalography revealed diffuse theta waves in 4 patients and temporal theta waves in 2 patients, but these findings were nonspecific. Nystagmograms were performed in 3 patients, and hypometric saccades were noted in all of these patients, and uninhibited neurogenic bladder was proven by cystometry in 2 of these patients. All patients were treated with levodopa, but none of the patients showed any beneficial effects. Two patients died of aspiration pneumonia; the average duration from onset to death was about 4 years.

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