Autonomic dysfunction in progressive supranuclear palsy

Abstract

Background: The degree of involvement of the autonomic nervous system in progressive supranuclear palsy (PSP) has been investigated in several studies, often providing conflicting results. There is a need for a better characterization of autonomic dysfunction in PSP, to enhance our understanding of this highly disabling neurodegenerative disease including patients' needs and possibly be of value for clinicians in the differential diagnosis among Parkinsonian syndromes.

Methods: We applied a systematic methodology to review existing literature on Pubmed regarding autonomic nervous system involvement in PSP.

Results: PSP reported quite frequently symptoms suggestive of autonomic dysfunction in all domains. Cardiovascular autonomic testing showed in some cases a certain degree of impairment (never severe). There was some evidence suggesting bladder dysfunction particularly in the storage phase. Dysphagia and constipation were the most common gastrointestinal symptoms. Instrumental tests seemed to confirm sudomotor and pupillomotor disturbances.

Conclusions: PSP patients frequently reported visceral symptoms, however objective testing showed that not always these reflected actual autonomic impairment. Further studies are needed to better delineate autonomic profile and its prognostic role in PSP.

Keywords: Autonomic dysfunction; Orthostatic hypotension; Photophobia; Progressive supranuclear palsy; Urinary incontinence.