Objective: To characterize the clinical features and prevalence of patients with progressive supranuclear palsy (PSP) in the recently implemented longitudinal Parkinson’s cohort (HELP-PD).

Background: PSP is a rare sporadic tauopathy with a prevalence of 6.5 per 100.000 (Respondek and Hoglinger 2016). Regional clusters of PSP have previously been described, for instance in northern France (Caparros-Lefebvre 2015). In light of the emergence of disease modifying drugs and the constantly increasing subtypes, clinical syndromes need to be defined precisely.

Methods: A cohort with a yearly follow-up was implemented in Luxembourg, recruiting all types of parkinsonism, which are expected to be approximately 1000 patients. Patient’s history, detailed clinical and neuropsychological assessments, as well as self-evaluation questionnaires were collected on a web-based electronic data-capture. Patients with PSP were compared to age- and sex-matched patients with idiopathic Parkinson’s disease (IPD), with a ratio of 1:2, to evaluate between-group differences.

Results: 125 patients with parkinsonism were recruited in the first 8 months, including 6 with PSP and 105 with IPD. Sex ratio was 5:1, mean disease duration 66.26±33.75 months, mean age at onset 61.93±5.30 years, mean Hoehn and Yahr score 3.42±1.43 and mean Sniffin’ Stick score 10.67±3.98. No evident family history of parkinsonism was found, 2 were exposed to pesticides, 4 were consuming alcohol regularly and none reported exposure to metal dust or fumes. Compared to the IPD-group, PSP-patients performed significantly worse in the MDS-UPDRS-II [35.50(19.00-45.00) vs. 11.50(1.00-22.00), p=0.001]. A statistical trend was observed for a worse MDS-UPDRS-I [18.67±6.65 vs. 12.33±6.58, p=0.073] and MDS-UPDRS-III [52.50±17.92 vs. 34.67±17.83, p=0.063], predominantly for akinetic-rigid symptoms. They performed worse at the MoCA [20.00(8.00-23.00) vs. 26.00(19.00-29.00), p=0.008], especially in the visiospatial/executive (p=0.013) and attention subsections (p=0.042) and scored less in the REM-sleep behavior disorder screening questionnaire [2.80±1.10 vs. 7.00±4.03, p=0.007].

Conclusions: Our preliminary findings suggests a higher prevalence for PSP in Luxembourg and the Greater Region, but this remains to be determined during the long-term follow-up, as this could still be a referral bias. Establishing a prospective PSP-cohort in Luxembourg will give further disclosure.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/initial-clinical-description-of-progressive-supranuclear-palsy-in-the-luxembourg-parkinsons-study/