Autopsy Validation of Progressive Supranuclear Palsy-Predominant Speech/Language Disorder Criteria

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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.

Original languageEnglish (US)
JournalMovement Disorders
DOIs
StateAccepted/In press - 2021

Keywords

  • PSP
  • criteria
  • language
  • pathology
  • speech

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

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