An Evaluation of the Progressive Supranuclear Palsy Speech/Language Variant

Jennifer Lynn Whitwell, Chase A. Stevens, Joseph R. Duffy, Heather Clark, Mary Margaret Machulda, Edythe A. Strand, Peter R. Martin, Rene L. Utianski, Hugo Botha, Anthony J. Spychalla, Matthew L. Senjem, Christopher G. Schwarz, Clifford R Jr. Jack, Farwa Ali, Anhar Hassan, Keith Anthony Josephs

Research output: Contribution to journalArticle

Abstract

Background: The Movement Disorder Society clinical criteria for progressive supranuclear palsy (PSP) provide a framework for assessing the presence/severity of clinical symptoms and define a speech/language variant of PSP. Objectives: To evaluate the clinical criteria in a cohort of speech/language patients with longitudinal follow-up. Methods: A total of 52 patients presenting with progressive apraxia of speech and/or agrammatic aphasia were followed longitudinally for up to 6 visits with clinical assessments and magnetic resonance imaging. We assessed oculomotor, postural instability, and akinesia diagnostic levels and determined whether patients met criteria for possible PSP-speech/language or probable PSP at each visit. Kaplan-Meier curves assessed time-to-event probabilities according to age. Statistical parametric mapping and midbrain volume were assessed according to disease progression. Results: Few PSP symptoms were observed early in the disease, with oculomotor abnormalities and falls first observed 2 years after onset. Falls were more common than vertical supranuclear gaze palsy. Bradykinesia and rigidity commonly developed but axial was rarely greater than appendicular rigidity. During follow-up, 54% met criteria for possible PSP-speech/language, 38% for probable PSP-Richardson's syndrome, and 38% for probable PSP-parkinsonism, most commonly 6 to 6.9 years after onset. The probability of developing PSP was greater when onset was at an age older than 70 years. Patients who progressed to probable PSP had more parkinsonism and oculomotor impairment at baseline and greater midbrain atrophy when compared with those who did not develop probable PSP. Conclusions: Symptoms typical of PSP commonly develop in patients presenting with a progressive speech/language disorder. Older age appears to be an important prognostic factor in these patients.

Original languageEnglish (US)
JournalMovement Disorders Clinical Practice
DOIs
StatePublished - Jan 1 2019

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Progressive Supranuclear Palsy
Language
Parkinsonian Disorders
Mesencephalon
Language Disorders
Speech Disorders
Apraxias
Hypokinesia
Aphasia
Age of Onset
Paralysis
Atrophy
Disease Progression

Keywords

  • agrammatism
  • aphasia
  • apraxia of speech
  • midbrain
  • PSP

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

Cite this

An Evaluation of the Progressive Supranuclear Palsy Speech/Language Variant. / Whitwell, Jennifer Lynn; Stevens, Chase A.; Duffy, Joseph R.; Clark, Heather; Machulda, Mary Margaret; Strand, Edythe A.; Martin, Peter R.; Utianski, Rene L.; Botha, Hugo; Spychalla, Anthony J.; Senjem, Matthew L.; Schwarz, Christopher G.; Jack, Clifford R Jr.; Ali, Farwa; Hassan, Anhar; Josephs, Keith Anthony.

In: Movement Disorders Clinical Practice, 01.01.2019.

Research output: Contribution to journalArticle

Whitwell, Jennifer Lynn ; Stevens, Chase A. ; Duffy, Joseph R. ; Clark, Heather ; Machulda, Mary Margaret ; Strand, Edythe A. ; Martin, Peter R. ; Utianski, Rene L. ; Botha, Hugo ; Spychalla, Anthony J. ; Senjem, Matthew L. ; Schwarz, Christopher G. ; Jack, Clifford R Jr. ; Ali, Farwa ; Hassan, Anhar ; Josephs, Keith Anthony. / An Evaluation of the Progressive Supranuclear Palsy Speech/Language Variant. In: Movement Disorders Clinical Practice. 2019.
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abstract = "Background: The Movement Disorder Society clinical criteria for progressive supranuclear palsy (PSP) provide a framework for assessing the presence/severity of clinical symptoms and define a speech/language variant of PSP. Objectives: To evaluate the clinical criteria in a cohort of speech/language patients with longitudinal follow-up. Methods: A total of 52 patients presenting with progressive apraxia of speech and/or agrammatic aphasia were followed longitudinally for up to 6 visits with clinical assessments and magnetic resonance imaging. We assessed oculomotor, postural instability, and akinesia diagnostic levels and determined whether patients met criteria for possible PSP-speech/language or probable PSP at each visit. Kaplan-Meier curves assessed time-to-event probabilities according to age. Statistical parametric mapping and midbrain volume were assessed according to disease progression. Results: Few PSP symptoms were observed early in the disease, with oculomotor abnormalities and falls first observed 2 years after onset. Falls were more common than vertical supranuclear gaze palsy. Bradykinesia and rigidity commonly developed but axial was rarely greater than appendicular rigidity. During follow-up, 54{\%} met criteria for possible PSP-speech/language, 38{\%} for probable PSP-Richardson's syndrome, and 38{\%} for probable PSP-parkinsonism, most commonly 6 to 6.9 years after onset. The probability of developing PSP was greater when onset was at an age older than 70 years. Patients who progressed to probable PSP had more parkinsonism and oculomotor impairment at baseline and greater midbrain atrophy when compared with those who did not develop probable PSP. Conclusions: Symptoms typical of PSP commonly develop in patients presenting with a progressive speech/language disorder. Older age appears to be an important prognostic factor in these patients.",
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author = "Whitwell, {Jennifer Lynn} and Stevens, {Chase A.} and Duffy, {Joseph R.} and Heather Clark and Machulda, {Mary Margaret} and Strand, {Edythe A.} and Martin, {Peter R.} and Utianski, {Rene L.} and Hugo Botha and Spychalla, {Anthony J.} and Senjem, {Matthew L.} and Schwarz, {Christopher G.} and Jack, {Clifford R Jr.} and Farwa Ali and Anhar Hassan and Josephs, {Keith Anthony}",
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T1 - An Evaluation of the Progressive Supranuclear Palsy Speech/Language Variant

AU - Whitwell, Jennifer Lynn

AU - Stevens, Chase A.

AU - Duffy, Joseph R.

AU - Clark, Heather

AU - Machulda, Mary Margaret

AU - Strand, Edythe A.

AU - Martin, Peter R.

AU - Utianski, Rene L.

AU - Botha, Hugo

AU - Spychalla, Anthony J.

AU - Senjem, Matthew L.

AU - Schwarz, Christopher G.

AU - Jack, Clifford R Jr.

AU - Ali, Farwa

AU - Hassan, Anhar

AU - Josephs, Keith Anthony

PY - 2019/1/1

Y1 - 2019/1/1

N2 - Background: The Movement Disorder Society clinical criteria for progressive supranuclear palsy (PSP) provide a framework for assessing the presence/severity of clinical symptoms and define a speech/language variant of PSP. Objectives: To evaluate the clinical criteria in a cohort of speech/language patients with longitudinal follow-up. Methods: A total of 52 patients presenting with progressive apraxia of speech and/or agrammatic aphasia were followed longitudinally for up to 6 visits with clinical assessments and magnetic resonance imaging. We assessed oculomotor, postural instability, and akinesia diagnostic levels and determined whether patients met criteria for possible PSP-speech/language or probable PSP at each visit. Kaplan-Meier curves assessed time-to-event probabilities according to age. Statistical parametric mapping and midbrain volume were assessed according to disease progression. Results: Few PSP symptoms were observed early in the disease, with oculomotor abnormalities and falls first observed 2 years after onset. Falls were more common than vertical supranuclear gaze palsy. Bradykinesia and rigidity commonly developed but axial was rarely greater than appendicular rigidity. During follow-up, 54% met criteria for possible PSP-speech/language, 38% for probable PSP-Richardson's syndrome, and 38% for probable PSP-parkinsonism, most commonly 6 to 6.9 years after onset. The probability of developing PSP was greater when onset was at an age older than 70 years. Patients who progressed to probable PSP had more parkinsonism and oculomotor impairment at baseline and greater midbrain atrophy when compared with those who did not develop probable PSP. Conclusions: Symptoms typical of PSP commonly develop in patients presenting with a progressive speech/language disorder. Older age appears to be an important prognostic factor in these patients.

AB - Background: The Movement Disorder Society clinical criteria for progressive supranuclear palsy (PSP) provide a framework for assessing the presence/severity of clinical symptoms and define a speech/language variant of PSP. Objectives: To evaluate the clinical criteria in a cohort of speech/language patients with longitudinal follow-up. Methods: A total of 52 patients presenting with progressive apraxia of speech and/or agrammatic aphasia were followed longitudinally for up to 6 visits with clinical assessments and magnetic resonance imaging. We assessed oculomotor, postural instability, and akinesia diagnostic levels and determined whether patients met criteria for possible PSP-speech/language or probable PSP at each visit. Kaplan-Meier curves assessed time-to-event probabilities according to age. Statistical parametric mapping and midbrain volume were assessed according to disease progression. Results: Few PSP symptoms were observed early in the disease, with oculomotor abnormalities and falls first observed 2 years after onset. Falls were more common than vertical supranuclear gaze palsy. Bradykinesia and rigidity commonly developed but axial was rarely greater than appendicular rigidity. During follow-up, 54% met criteria for possible PSP-speech/language, 38% for probable PSP-Richardson's syndrome, and 38% for probable PSP-parkinsonism, most commonly 6 to 6.9 years after onset. The probability of developing PSP was greater when onset was at an age older than 70 years. Patients who progressed to probable PSP had more parkinsonism and oculomotor impairment at baseline and greater midbrain atrophy when compared with those who did not develop probable PSP. Conclusions: Symptoms typical of PSP commonly develop in patients presenting with a progressive speech/language disorder. Older age appears to be an important prognostic factor in these patients.

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KW - aphasia

KW - apraxia of speech

KW - midbrain

KW - PSP

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