What are the signs and symptoms of progressive supranuclear palsy (PSP)?

Updated: Sep 24, 2018
  • Author: Stephen M Bloomfield, MD; Chief Editor: Selim R Benbadis, MD  more...
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Answer

Onset of PSP typically begins in the sixth or seventh decade of life. The patient develops bradykinesia, rigidity, dysarthria, dysphagia, and dementia, as in patients with idiopathic PD. However, tremor is rare, and the patient has severe postural instability. Axial rigidity appears to be more prominent than limb rigidity. Consistently, patients have downgaze ophthalmoparesis and pseduobulbar palsy. Eyelid problems are present including eyelid freezing and difficult with either opening or closing the eyes. The association of dementia in PSP is contentious. Dystonia is present in about 13% of patients with pathologically proven PSP. [23]

The supranuclear component of the disorder is ocular paresis, which can be overcome by vertical doll's-eyes maneuvers. The combination of vertical paresis and a history of frequent falls due postural instability is central to the diagnosis of PSP. Some patients develop severe palilalia, emotional incontinence, and other evidence of bilateral frontal lobe dysfunction. Occasionally, patients present with akinesia of gait, speech, and handwriting, without rigidity, tremor, dementia, or gaze paresis. Blepharospasm and dry eyes have been reported. Dubois et al noted that the applause sign was useful in the clinical diagnosis of PSP. [24]


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