Objective:

To examine possible autonomic changes associated with progressive supranuclear palsy in response to a strong, unanticipated stimulus.

Background:

Progressive supranuclear palsy (PSP) is a neurodegenerative disorder that causes serious problems in eye movements, walking, and balance, and is also often associated with cognitive, emotional, and behavioral changes. In other movement disorders such as Parkinson’s disease, similar symptoms have often been associated with changes in autonomic nervous system (ANS) functioning. Despite this, previous studies have rarely examined ANS functioning in PSP. .In the present study, we determined whether ANS responses to an emotionally provocative stimulus were altered in PSP. We tested the hypothesis that ANS responses to an unanticipated aversive auditory stimulus (i.e., a brief, loud burst of white noise) designed to induce a startle response would differ between patients with PSP compared to healthy controls (HC).

Design/Methods:

Participants included 33 PSP patients and 38 HC. Participants sat still for a 60 second baseline and heard a brief (100-ms) loud (115-dB) burst of white noise without warning. Continuous measures of cardiac interbeat intervals (IBI; decreases in IBI correspond to increases in heart rate) and skin conductance levels (SCL) were obtained throughout the task. 

Results:

Compared to HC, PSP patients showed significantly less decrease in IBI (i.e., less increase in heart rate) and less increase in SCL from baseline during the 10 seconds after stimulus onset.

Conclusions:

Less decrease in IBI and less increase in SCL in the face of a strong external stimulus suggests a diminished sympathetic nervous system response. In light of previous research suggesting that peripheral sympathetic responses are relatively preserved in the setting of orthostatic postural testing in PSP, we suspect this relates to a brainstem degeneration in a pontine circuit that is critical for startle reflexes during emotional challenges.