Epidemiology of Progressive Supranuclear Palsy: Real World Data from the Second Largest Health Plan in Israel

Abstract

Progressive supranuclear palsy (PSP) is a rare and fatal neurodegenerative movement disorder and no disease modifying therapy (DMT) is currently available. This study aims to assess the epidemiology of PSP in Israel and to describe its clinical features. This retrospective analysis identified patients with PSP between 2000 and 2018 over the age of 40 years at first diagnosis (index date). We identified 209 patients with ≥1 diagnosis of PSP. Of those, 88 patients satisfied the inclusion criteria with a mean age at diagnosis of 72 years (SD = 8) and 53% were female. The 2018 prevalence and incidence rates were 5.3 and 1 per 100,000 persons, respectively. Median survival time was 4.9 years (95% CI 3.6–6.1) and median time from initial symptom to diagnosis was 4.2 years. The most common misdiagnoses were Parkinson’s disease, cognitive disorder and depression. The present study demonstrates that the clinic-epidemiological features of PSP in Israel are similar to PSP worldwide. In light of PSP’s rarity, investigation of PSP cohorts in different countries may create a proper platform for upcoming DMT trials.

Progressive supranuclear palsy’s economical burden: the use and costs of healthcare resources in a large health provider in Israel

“…In addition, these findings are in line with those of resembling conditions; AD [31] and PD [27,32,33]. The higher costs during the year prior index can be explained by the known delayed in PSP's diagnosis [4], and by higher HCRU leads to the diagnosis.…”

“…Additional limitations are those inherit to database research. First, we identified PwPSP only by one diagnosis, which may cause misclassification of the disease; however, in a previous analysis of PSP's epidemiology, using the same criteria, we showed that our results are in line with the other publications [4]. The consequence of such non-differential misclassification of PSP is dilution of the measure of effect size and underestimation of the true economic burden of the disease due to "bias towards the null".…”

“…One publication addressed the economic burden associated with frontotemporal degeneration, while 5.4% of patients were defined as either PSP or corticobasal syndrome, the economic burden was not reported for that group [21]. Due to the scarcity of studies in the field, we compared this study's findings to those of other neurodegenerative diseases: Alzheimer's disease (AD)-another tau based neurodegenerative disease, and PD, the most common misdiagnosis in PwPSP [4,22]. Both conditions demonstrated an increase of medical expenditure with disease progression [23][24][25][26][27][28][29][30].…”

“…Progressive supranuclear palsy (PSP) is a fatal but rare neurodegenerative disease [1] with an estimated annual prevalence of 0.2-8.3 per 100,000 person [2] and median survival time of 5-8 years [3]. PSP's prevalence in Maccabi healthcare services (MHS) is 5.3/100,000 [4]. PSP is characterized by various symptoms, such as mobility difficulties (slowness and unsteadiness which lead to frequent falls), cognitive impairment, behavioral changes, dysarthria and dysphagia, and visual manifestations (blurred vision and vertical supranuclear gaze palsy) [5].…”

Magnetic resonance imaging in the diagnosis of progressive supranuclear palsy: A case report and review of literature

“…1 PSP characteristics include vertical supranuclear palsy, postural instability with unexplained falls, akinesia and frontal cognitive dysfunction. 2 The pathological features of PSP consist of neuronal loss, globose neurofibrillary tangles, tau-positive inclusion found in tufted astrocytes, and gliosis mainly in the basal ganglia, cerebellum, brainstem, and to a lesser extent, cerebral cortex. [3][4][5] Thus, the definitive diagnosis of PSP requires neuropathological examination.…”