Classification and clinicoradiologic features of primary progressive aphasia (PPA) and apraxia of speech

Hugo Botha, Joseph R. Duffy, Jennifer L. Whitwell, Edythe A. Strand, Mary M. Machulda, Christopher G. Schwarz, Robert I. Reid, Anthony J. Spychalla, Matthew L. Senjem, David T. Jones, Val Lowe, Clifford R. Jack, Keith A. Josephs

Research output: Contribution to journalArticle

37 Citations (Scopus)

Abstract

The consensus criteria for the diagnosis and classification of primary progressive aphasia (PPA) have served as an important tool in studying this group of disorders. However, a large proportion of patients remain unclassifiable whilst others simultaneously meet criteria for multiple subtypes. We prospectively evaluated a large cohort of patients with degenerative aphasia and/or apraxia of speech using multidisciplinary clinical assessments and multimodal imaging. Blinded diagnoses were made using operational definitions with important differences compared to the consensus criteria. Of the 130 included patients, 40 were diagnosed with progressive apraxia of speech (PAOS), 12 with progressive agrammatic aphasia, 9 with semantic dementia, 52 with logopenic progressive aphasia, and 4 with progressive fluent aphasia, while 13 were unclassified. The PAOS and progressive fluent aphasia groups were least impaired. Performance on repetition and sentence comprehension was especially poor in the logopenic group. The semantic and progressive fluent aphasia groups had prominent anomia, but only semantic subjects had loss of word meaning and object knowledge. Distinct patterns of grey matter loss and white matter changes were found in all groups compared to controls. PAOS subjects had bilateral frontal grey matter loss, including the premotor and supplementary motor areas, and bilateral frontal white matter involvement. The agrammatic group had more widespread, predominantly left sided grey matter loss and white matter abnormalities. Semantic subjects had bitemporal grey matter loss and white matter changes, including the uncinate and inferior occipitofrontal fasciculi, whereas progressive fluent subjects only had left sided temporal involvement. Logopenic subjects had diffuse and bilateral grey matter loss and diffusion tensor abnormalities, maximal in the posterior temporal region. A diagnosis of logopenic aphasia was strongly associated with being amyloid positive (46/52 positive). Our findings support consideration of an alternative way of identifying and categorizing subtypes of degenerative speech and language disorders.

Original languageEnglish (US)
Pages (from-to)220-236
Number of pages17
JournalCortex
Volume69
DOIs
StatePublished - Aug 1 2015
Externally publishedYes

Fingerprint

Primary Progressive Aphasia
Apraxias
Aphasia
Wernicke Aphasia
Semantics
Consensus
Anomia
Multimodal Imaging
Language Disorders
Speech Disorders
Frontotemporal Dementia
Motor Cortex
Temporal Lobe
Apraxia of Speech
Amyloid
Grey Matter
Gray Matter
White Matter

Keywords

  • Amyloid PET imaging
  • Diffusion tensor imaging
  • Primary progressive aphasia
  • Progressive apraxia of speech
  • Volumetric based morphometry

ASJC Scopus subject areas

  • Clinical Neurology
  • Neurology
  • Arts and Humanities (miscellaneous)
  • Developmental and Educational Psychology

Cite this

Botha, H., Duffy, J. R., Whitwell, J. L., Strand, E. A., Machulda, M. M., Schwarz, C. G., ... Josephs, K. A. (2015). Classification and clinicoradiologic features of primary progressive aphasia (PPA) and apraxia of speech. Cortex, 69, 220-236. https://doi.org/10.1016/j.cortex.2015.05.013

Classification and clinicoradiologic features of primary progressive aphasia (PPA) and apraxia of speech. / Botha, Hugo; Duffy, Joseph R.; Whitwell, Jennifer L.; Strand, Edythe A.; Machulda, Mary M.; Schwarz, Christopher G.; Reid, Robert I.; Spychalla, Anthony J.; Senjem, Matthew L.; Jones, David T.; Lowe, Val; Jack, Clifford R.; Josephs, Keith A.

In: Cortex, Vol. 69, 01.08.2015, p. 220-236.

Research output: Contribution to journalArticle

Botha, H, Duffy, JR, Whitwell, JL, Strand, EA, Machulda, MM, Schwarz, CG, Reid, RI, Spychalla, AJ, Senjem, ML, Jones, DT, Lowe, V, Jack, CR & Josephs, KA 2015, 'Classification and clinicoradiologic features of primary progressive aphasia (PPA) and apraxia of speech', Cortex, vol. 69, pp. 220-236. https://doi.org/10.1016/j.cortex.2015.05.013
Botha, Hugo ; Duffy, Joseph R. ; Whitwell, Jennifer L. ; Strand, Edythe A. ; Machulda, Mary M. ; Schwarz, Christopher G. ; Reid, Robert I. ; Spychalla, Anthony J. ; Senjem, Matthew L. ; Jones, David T. ; Lowe, Val ; Jack, Clifford R. ; Josephs, Keith A. / Classification and clinicoradiologic features of primary progressive aphasia (PPA) and apraxia of speech. In: Cortex. 2015 ; Vol. 69. pp. 220-236.
@article{71366ffed9ba45a98430dbedf86269f4,
title = "Classification and clinicoradiologic features of primary progressive aphasia (PPA) and apraxia of speech",
abstract = "The consensus criteria for the diagnosis and classification of primary progressive aphasia (PPA) have served as an important tool in studying this group of disorders. However, a large proportion of patients remain unclassifiable whilst others simultaneously meet criteria for multiple subtypes. We prospectively evaluated a large cohort of patients with degenerative aphasia and/or apraxia of speech using multidisciplinary clinical assessments and multimodal imaging. Blinded diagnoses were made using operational definitions with important differences compared to the consensus criteria. Of the 130 included patients, 40 were diagnosed with progressive apraxia of speech (PAOS), 12 with progressive agrammatic aphasia, 9 with semantic dementia, 52 with logopenic progressive aphasia, and 4 with progressive fluent aphasia, while 13 were unclassified. The PAOS and progressive fluent aphasia groups were least impaired. Performance on repetition and sentence comprehension was especially poor in the logopenic group. The semantic and progressive fluent aphasia groups had prominent anomia, but only semantic subjects had loss of word meaning and object knowledge. Distinct patterns of grey matter loss and white matter changes were found in all groups compared to controls. PAOS subjects had bilateral frontal grey matter loss, including the premotor and supplementary motor areas, and bilateral frontal white matter involvement. The agrammatic group had more widespread, predominantly left sided grey matter loss and white matter abnormalities. Semantic subjects had bitemporal grey matter loss and white matter changes, including the uncinate and inferior occipitofrontal fasciculi, whereas progressive fluent subjects only had left sided temporal involvement. Logopenic subjects had diffuse and bilateral grey matter loss and diffusion tensor abnormalities, maximal in the posterior temporal region. A diagnosis of logopenic aphasia was strongly associated with being amyloid positive (46/52 positive). Our findings support consideration of an alternative way of identifying and categorizing subtypes of degenerative speech and language disorders.",
keywords = "Amyloid PET imaging, Diffusion tensor imaging, Primary progressive aphasia, Progressive apraxia of speech, Volumetric based morphometry",
author = "Hugo Botha and Duffy, {Joseph R.} and Whitwell, {Jennifer L.} and Strand, {Edythe A.} and Machulda, {Mary M.} and Schwarz, {Christopher G.} and Reid, {Robert I.} and Spychalla, {Anthony J.} and Senjem, {Matthew L.} and Jones, {David T.} and Val Lowe and Jack, {Clifford R.} and Josephs, {Keith A.}",
year = "2015",
month = "8",
day = "1",
doi = "10.1016/j.cortex.2015.05.013",
language = "English (US)",
volume = "69",
pages = "220--236",
journal = "Cortex",
issn = "0010-9452",
publisher = "Masson SpA",

}

TY - JOUR

T1 - Classification and clinicoradiologic features of primary progressive aphasia (PPA) and apraxia of speech

AU - Botha, Hugo

AU - Duffy, Joseph R.

AU - Whitwell, Jennifer L.

AU - Strand, Edythe A.

AU - Machulda, Mary M.

AU - Schwarz, Christopher G.

AU - Reid, Robert I.

AU - Spychalla, Anthony J.

AU - Senjem, Matthew L.

AU - Jones, David T.

AU - Lowe, Val

AU - Jack, Clifford R.

AU - Josephs, Keith A.

PY - 2015/8/1

Y1 - 2015/8/1

N2 - The consensus criteria for the diagnosis and classification of primary progressive aphasia (PPA) have served as an important tool in studying this group of disorders. However, a large proportion of patients remain unclassifiable whilst others simultaneously meet criteria for multiple subtypes. We prospectively evaluated a large cohort of patients with degenerative aphasia and/or apraxia of speech using multidisciplinary clinical assessments and multimodal imaging. Blinded diagnoses were made using operational definitions with important differences compared to the consensus criteria. Of the 130 included patients, 40 were diagnosed with progressive apraxia of speech (PAOS), 12 with progressive agrammatic aphasia, 9 with semantic dementia, 52 with logopenic progressive aphasia, and 4 with progressive fluent aphasia, while 13 were unclassified. The PAOS and progressive fluent aphasia groups were least impaired. Performance on repetition and sentence comprehension was especially poor in the logopenic group. The semantic and progressive fluent aphasia groups had prominent anomia, but only semantic subjects had loss of word meaning and object knowledge. Distinct patterns of grey matter loss and white matter changes were found in all groups compared to controls. PAOS subjects had bilateral frontal grey matter loss, including the premotor and supplementary motor areas, and bilateral frontal white matter involvement. The agrammatic group had more widespread, predominantly left sided grey matter loss and white matter abnormalities. Semantic subjects had bitemporal grey matter loss and white matter changes, including the uncinate and inferior occipitofrontal fasciculi, whereas progressive fluent subjects only had left sided temporal involvement. Logopenic subjects had diffuse and bilateral grey matter loss and diffusion tensor abnormalities, maximal in the posterior temporal region. A diagnosis of logopenic aphasia was strongly associated with being amyloid positive (46/52 positive). Our findings support consideration of an alternative way of identifying and categorizing subtypes of degenerative speech and language disorders.

AB - The consensus criteria for the diagnosis and classification of primary progressive aphasia (PPA) have served as an important tool in studying this group of disorders. However, a large proportion of patients remain unclassifiable whilst others simultaneously meet criteria for multiple subtypes. We prospectively evaluated a large cohort of patients with degenerative aphasia and/or apraxia of speech using multidisciplinary clinical assessments and multimodal imaging. Blinded diagnoses were made using operational definitions with important differences compared to the consensus criteria. Of the 130 included patients, 40 were diagnosed with progressive apraxia of speech (PAOS), 12 with progressive agrammatic aphasia, 9 with semantic dementia, 52 with logopenic progressive aphasia, and 4 with progressive fluent aphasia, while 13 were unclassified. The PAOS and progressive fluent aphasia groups were least impaired. Performance on repetition and sentence comprehension was especially poor in the logopenic group. The semantic and progressive fluent aphasia groups had prominent anomia, but only semantic subjects had loss of word meaning and object knowledge. Distinct patterns of grey matter loss and white matter changes were found in all groups compared to controls. PAOS subjects had bilateral frontal grey matter loss, including the premotor and supplementary motor areas, and bilateral frontal white matter involvement. The agrammatic group had more widespread, predominantly left sided grey matter loss and white matter abnormalities. Semantic subjects had bitemporal grey matter loss and white matter changes, including the uncinate and inferior occipitofrontal fasciculi, whereas progressive fluent subjects only had left sided temporal involvement. Logopenic subjects had diffuse and bilateral grey matter loss and diffusion tensor abnormalities, maximal in the posterior temporal region. A diagnosis of logopenic aphasia was strongly associated with being amyloid positive (46/52 positive). Our findings support consideration of an alternative way of identifying and categorizing subtypes of degenerative speech and language disorders.

KW - Amyloid PET imaging

KW - Diffusion tensor imaging

KW - Primary progressive aphasia

KW - Progressive apraxia of speech

KW - Volumetric based morphometry

UR - http://www.scopus.com/inward/record.url?scp=84934989005&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84934989005&partnerID=8YFLogxK

U2 - 10.1016/j.cortex.2015.05.013

DO - 10.1016/j.cortex.2015.05.013

M3 - Article

AN - SCOPUS:84934989005

VL - 69

SP - 220

EP - 236

JO - Cortex

JF - Cortex

SN - 0010-9452

ER -