Date: Monday, June 5, 2017
Session Title: Parkinsonism, MSA, PSP (Secondary and Parkinsonism-Plus)
Session Time: 1:45pm-3:15pm
Location: Exhibit Hall C
Objective: To identify clinical features and investigations which predict PSP pathology during lifetime in the largest pathology PSP cohort reported to date compared to autopsy cases with relevant differential diagnoses.
Background: Progressive supranuclear palsy (PSP) is a neuropathologically defined disease entity. Current criteria for the clinical diagnosis of PSP are not sensitive in the early disease stages for manifestations other than Richardson’s syndrome. However, early diagnosis is important, particularly for the development of disease-modifying therapies.
Methods: We performed a systematic review of the literature published since 1996 to identify clinical features and investigations that may predict PSP pathology. We then extracted standardized data from clinical charts of patients with pathologically diagnosed PSP, as well as relevant disease controls, and calculated sensitivity, specificity and positive predictive value of key clinical features for PSP in this cohort.
Of N=4166 identified articles identified by an automated database inquiry, N=254 met the predefined standards. The literature review identified clinical features predictive for PSP, including features of the four functional domains “ocular motor dysfunction”, “postural instability”, “akinesia”, and “cognitive dysfunction”. However, no biomarker or genetic findings were confirmed to predict PSP. No biomarker or genetic findings were found reliable to predict definite PSP.
High-quality original natural history data was available from patients with pathologically diagnosed PSP (N=206), CBD (N=54), MSA-P (N=51), PD (N=53), and FTLD (N=73). We identified clinical features of differential sensitivity and specificity that predict PSP pathology, including phenotypes other than PSP-RS.
Conclusions: The evidence from this extensive literature review and autopsy cohort presents a valuable basis for the revision of the diagnostic criteria for PSP.
To cite this abstract in AMA style:G. Respondek, C. Kurz, T. Arzberger, E. Gelpi, D. Irwin, W. Meissner, A. Pantelyat, A. Rajput, J. Van Swieten, C. Troakes, K. Josephs, A. Lang, U. Mueller, C. Nilsson, Y. Bordelon, J.-C. Corvol, A. Boxer, L. Golbe, I. Litvan, M. Stamelou, G. Hoeglinger. Which clinical features predict progressive supranuclear palsy pathology? A clinicopathological study on 437 autopsy cases and a literature review [abstract]. Mov Disord. 2017; 32 (suppl 2). https://www.mdsabstracts.org/abstract/which-clinical-features-predict-progressive-supranuclear-palsy-pathology-a-clinicopathological-study-on-437-autopsy-cases-and-a-literature-review/. Accessed December 3, 2023.
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