Objective: Understanding the common clinical features of PSP.

Background: PSP is a relatively rare progressive neurodegenerative disease, which can often be difficult to distinguish from idiopathic Parkinson’s disease (PD). PSP can exhibit rapid deterioration, and can be non-responsive to PD pharmacological treatments. Further understanding of the presentation and progression of PSP is essential, in order to optimise patient care and education.

Method: The clinical notes of all patients diagnosed with PSP managed by the elderly care movement disorders team at Royal Berkshire Hospital, UK were reviewed from 2008 to present. Using excel, data regarding presenting symptoms, motor and non-motor features, clinical signs, response to levodopa, residence and duration of disease was collected.

Results: Notes of 22 patients with PSP were reviewed. The age at onset of symptoms ranged from 61 to 95 (mean of 81 years). 59% (13/22) were male. 31.8% resided in nursing homes either presently, or at time of death. The majority (68.2%) managed at home with either the support of family or a package of care.

Reason for specialist referral was commonly recurrent falls (54.5%), followed by poor mobility (9.1%). Non-motor symptoms varied. 68.1% of patients displayed a degree of cognitive impairment. Low mood and anxiety were described in 45.6% of patients. 54.5% of patients experienced dysphagia or sialorrhoea. Visual disturbance was also a key feature, with blurred vision or intolerance of bright lights in 31.8% of patients. Features such as REM sleep behavior disorder or hallucinations were rare. Only 50% of patients displayed upper gaze palsy on examination. 

81.8% of patients trialed levodopa therapy at some point during their illness, with doses ranging from 150mg to 1000mg levodopa/24 hours. Only one patient reported a significant benefit in their motor symptoms. The majority (83%) reported nil to minimal improvement. 63.6% (14/22) have now died. The time spanning from symptom onset to death ranged from 1 to 16 years, with a mean duration of 4.7 years.

Conclusion: The features of PSP in older patients can be varied, but early and recurrent falls are common. Cognitive impairment, anxiety, depression and dysphagia also feature heavily. Further research is required to optimise management for these patients, both pharmacological and supportive. Given the diversity of symptoms, a multidisciplinary approach is required.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/a-retrospective-review-of-clinical-features-of-progressive-supranuclear-palsy-psp-in-a-movement-disorders-clinic/