Atypical presentation of progressive supranuclear palsy.
Four pathologically documented cases of progressive supranuclear palsy are reported. Two patients exhibited severe dementia and 2 parkinsonism; none had the classic ophthalmoplegia. On retrospective analysis, clues to the diagnosis included early prominent gait disturbance, apraxia of eyelid opening in 1 patient, lack of tremor, poor response to levodopa-carbidopa, and severe rigidity with a posture of neck extension terminally in 1 patient. The clinical presentation of progressive supranuclear palsy, therefore, is not as stereotyped as previously thought, and the diagnosis can be overlooked if one adheres rigidly to the classic diagnostic criteria.
D000368:Aged; D001921:Brain / Q000473:pathology; D010244:Bulbar Palsy, Progressive / Q000175:diagnosis* / Q000473:pathology; D003704:Dementia / Q000473:pathology; D005260:Female; D006801:Humans; D008297:Male; D012189:Retrospective Studies