Autopsy Validation of Progressive Supranuclear Palsy‐Predominant Speech/Language Disorder Criteria. Issue 1 (10th October 2021)

• Record Type: Journal Article
• Authors: Hokelekli, Fatma Ozlem; Duffy, Joseph R.; Clark, Heather M.; Utianski, Rene L.; Botha, Hugo; Ali, Farwa; Stierwalt, Julie A.; Machulda, Mary M.; Reichard, R. Ross; Dickson, Dennis W.; Whitwell, Jennifer L.; Josephs, Keith A.
• Is Part Of: Movement disorders. Volume 37:Issue 1(2022)
• Page Start: 213
• Page End: 218
• Publication Date: 2021-10-10
• Subjects: PSP -- pathology -- speech -- language -- criteria; Movement disorders -- Periodicals; 610
• DOI: 10.1002/mds.28822
• Languages: English
• ISSNs: 0885-3185

Abstract

ABSTRACT: Background: Progressive supranuclear palsy (PSP) may present as a speech/language disorder (PSP‐SL). Objective: We assessed pathological correlates of patients with PSP‐SL who retained the suggestive of PSP‐SL (s.o. PSP‐SL) diagnosis versus those who progressed to possible/probable (poss./prob.) PSP. Methods: Thirty‐four prospectively recruited patient with s.o. PSP‐SL completed comprehensive speech/language and neurological assessments longitudinally, died, and underwent autopsy. Results: Twelve patients (35%) evolved to poss./prob PSP, while 22 (65%) remained as s.o. PSP‐SL. Pathological diagnoses differed across the groups ( P = 0.025). Patients with s.o. PSP‐SL had four different neuropathologies (corticobasal degeneration [59%], PSP [13%], Pick's disease [14%], and frontotemporal lobar degeneration with TDP‐43 [14%]), while all patients with poss./prob. PSP had a 4R‐tauopathy (PSP [67%] and corticobasal degeneration [33%]). Development of poss./prob. PSP increased the chance of having PSP pathology by 2.38 times. Conclusions: PSP‐SL is associated with heterogenous pathologies. Evolution of PSP‐SL into poss./prob. PSP is more predictive of underlying PSP pathology than s.o. PSP‐SL. © 2021 International Parkinson and Movement Disorder Society