Midbrain/pons area ratio and clinical features predict the prognosis of progressive Supranuclear palsy
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RESEARCH ARTICLE
Open Access
Midbrain/pons area ratio and clinical features predict the prognosis of progressive Supranuclear palsy Shi-Shuang Cui1,2, Hua-Wei Ling3, Juan-Juan Du1, Yi-Qi Lin1, Jing Pan1, Hai-Yan Zhou1, Gang Wang1, Ying Wang1, Qin Xiao1, Jun Liu1, Yu-Yan Tan1* and Sheng-Di Chen1*
Abstract Background: Progressive supranuclear palsy (PSP) is a rare movement disorder with poor prognosis. This retrospective study aimed to characterize the natural history of PSP and to find predictors of shorter survival and faster decline of activity of daily living. Method: All patients recruited fulfilled the movement disorder society (MDS) clinical diagnostic criteria for PSP (MDS-PSP criteria) for probable and possible PSP with median 12 years. Data were obtained including age, sex, date of onset, age at onset (AAO), symptoms reported at first visit and follow-up, date of death and date of institutionalization. Magnetic resonance imaging was collected at the first visit. Endpoints were death and institutionalization. Kaplan-Meier method and Cox proportional hazard model were used to explore factors associated with early death and institutionalization. Results: Fifty-nine patients fulfilling MDS-PSP criteria were enrolled in our study. Nineteen patients (32.2%) had died and 31 patients (52.5%) were institutionalized by the end of the follow-up. Predictors associated with poorer survival were late-onset PSP and decreased M/P area ratio. Predictors associated with earlier institutionalization were older AAO and decreased M/P area ratio. Conclusion: Older AAO and decreased M/P area ratio were predictors for earlier dearth and institutionalization in PSP. The neuroimaging biomarker M/P area ratio was a predictor for prognosis in PSP. Keywords: Progressive Supranuclear palsy, Survival analysis, M/P area ratio
Background Progressive supranuclear palsy (PSP) is a rare movement disorder with an estimated prevalence of 6.4 per 100, 000, characterized by the accumulation of abnormally phosphorylated tau protein in the basal ganglia, frontal lobe and the brainstem [1]. The cardinal clinical features of PSP are early postural instability, supranuclear gaze * Correspondence: [email protected]; [email protected] 1 Department of Neurology & Institute of Neurology, Ruijin Hospital affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai, China Full list of author information is available at the end of the article
palsy, akinesia, cognitive impairment and behavior changes [2]. The prognosis of PSP is poor with increased risks of falls, dysphagia, aspiration pneumonia and pressure ulcer, leading to institutionalization and short survival time [3]. The mean survival is only 3 to 4 years after the diagnosis and 5.3 to 13.0 years at the onset of the disease [1]. Previous studies indicated that male, older age at onset (AAO), early falls, dementia and clinical phenotypes were predictors of poor survival [1, 4–9]. Midbrain atrophy is commonly observed in PSP. A number of midbrain metrics including “hummingbird”
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