Progressive Supranuclear Palsy
OBJECTIVES
To illustrate the clinical presentation of progressive supranuclear palsy.
To discuss progressive supranuclear palsy and pathologies presenting with a similar syndrome.
To highlight the importance of oculomotor exam in the diagnosis of this form of parkinsonism.
VIGNETTE
This 59-year-old woman reported balance impairment and falls (at least four), within the last year. Her husband also noted slowness in the movement of the eyes and walking while “looking at the floor,” which she never did before. More recently, her speech became slurred, slow, and with poor enunciation, and she started having choking spells. She admitted to lifelong anosmia but denied constipation or urinary problems, skin discoloration, excessive sweating, or postural light-headedness. She used to play golf but felt that her game was not up to expectations. She was involved in three motor vehicle accidents within this year, which raised concerns regarding her driving safety.
CASE SUMMARY
The presence of postural impairment leading to falls close to symptom onset, associated with a tremorless symmetric parkinsonism, with axial-predominant rigidity, facial dystonia (furrowing the forehead and deepening of the nasolabial folds are obvious on inspection), dysphagia, dysarthria with “hypobradyphonia,” apraxia of eyelid opening, square-wave jerks, supranuclear vertical gaze palsy, and a positive “applause sign” (an evidence of motor perseveration) were highly suggestive of the classical form of progressive supranuclear palsy (PSP) or Richardson syndrome. There was no apraxia or cortical sensory loss, nor dysautonomic features. Although no overt cognitive impairment was documented at the initial visit (MMSE = 28/30; Frontal Assessment Battery = 16/18; normal clock drawing test), a dys-executive frontal-predominant form of dementia eventually developed. The patient succumbed to her illness within 5 years from symptom onset. PSP was confirmed at autopsy.