Objective: To elucidate specific clinical features of progressive supranuclear palsy (PSP) pathology in PSP syndrome.

Background: PSP syndrome (PSPS) is the clinical hallmark of PSP; however, pathological backgrounds of PSPS are varied. It is extremely important to detect PSP pathology in PSPS for clinical trials and treatments.

Methods: We identified all autopsy-confirmed subjects diagnosed as having PSPS according to the NINDS-SPSP clinical criteria. Of 23 subjects with PSPS, 16 had a PSP pathology (69.5%), and 7 had a non-PSP pathology. Of 7 subjects with a non-PSP pathology, 4 had 4R-tauopathy (2 corticobasal degeneration, 1 globular glial tauopathy, and 1 atypical 4R tauopathy), and 3 had non-4R tauopathy (Alzheimer disease, senile dementia of the neurofibrillary tangle type, and Alexander disease). We evaluated clinical features by referring to clinical records and videos. Subjects were divided based on the following two perspectives: 1) presence or absence of PSP pathology, i.e., PSP or non-PSP pathology group and 2) presence or absence of 4R tauopathy, i.e., 4R tauopathy or non-4R tauopathy group. Next, we retrospectively recorded the presence or absence of clinical features both throughout the course of disease and within 2 years after the disease onset. Statistical comparisons of the differences in clinical features were performed using Fisher’s test.

Results: (1) Within 2 years after disease onset, no significant clinical features were observed in either groups. (2) Regarding clinical features throughout the course, the frequency of decreased blinking, disturbance of saccadic eye movement, and abnormal speech was higher in 4R tauopathy patients than in non-4R tauopathy patients (p = 0.016, 0.014, and 0.013, respectively). The frequencies of face touching signs and slurred speech were higher in PSP patients than in non-PSP patients (p = 0.011 and 0.018, respectively), whereas that of abnormal behavior was lower in PSP patients than in non-PSP patients (p = 0.031).

Conclusions: Our data suggest that decreased blinking, disturbance of saccades, and abnormal speech are predictors of 4R-tauopathy. Slurred speech and face touching signs predict PSP pathology, whereas abnormal behavior predicts non-PSP pathology in PSPS. Conclusively, detection of PSP pathology is difficult in early stages.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/clinical-predictors-of-progressive-supranuclear-palsy-psp-pathology-in-psp-syndrome/