Autopsy Validation of Progressive Supranuclear Palsy‐Predominant Speech/Language Disorder Criteria
Fatma Ozlem Hokelekli, Joseph R. Duffy, Heather M. Clark, Rene L. Utianski, Hugo Botha, Farwa Ali, Julie A. G. Stierwalt, Mary M. Machulda, R. Ross Reichard, Dennis W. Dickson, Jennifer L. Whitwell, Keith A. Josephs
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.