Symptom-led staging for semantic and non-fluent/agrammatic variants of primary progressive aphasia. Alzheimer's & Dementia

Paul Camic; C.J.D. Hardy; C. Taylor-Rubin; H. Harding; A. Suárez-Gonzalez; J. Jiang; L Thompson; K. Kingma; A. Chokesuwattanaskul; F. Walker; S. Barker; E.V. Brotherhood; C. Waddington; O. Wood; N. Zimmerman; N. Kupeli; K.X.X. Yong; J. Scott; C.R. Marshall; N.P. Oxtoby; J.D. Rohrer; A. Volkmer; S.J. Crutch; J.D. Warren

doi:10.1002/alz.13415

Symptom-led staging for semantic and non-fluent/agrammatic variants of primary progressive aphasia. Alzheimer's & Dementia

Paul Camic
, C.J.D. Hardy
, C. Taylor-Rubin
, H. Harding
, A. Suárez-Gonzalez
, J. Jiang
, L Thompson
, K. Kingma
, A. Chokesuwattanaskul
, F. Walker
, S. Barker
, E.V. Brotherhood
, C. Waddington
, O. Wood
, N. Zimmerman
, N. Kupeli
, K.X.X. Yong
, J. Scott
, C.R. Marshall
, N.P. Oxtoby

J.D. Rohrer, A. Volkmer, S.J. Crutch, J.D. Warren

Research output: Contribution to journal › Article › peer-review

43 Citations (Scopus)

Abstract

INTRODUCTION: Here we set out to create a symptom-led staging system for the
canonical semantic and non-fluent/agrammatic variants of primary progressive aphasia (PPA), which present unique diagnostic and management challenges not well captured by functional scales developed for Alzheimer’s disease and other dementias.

METHODS: An international PPA caregiver cohort was surveyed on symptom development under six provisional clinical stages and feedback was analyzed using a mixed-methods sequential explanatory design.

RESULTS: Both PPA syndromes were characterized by initial communication dysfunction and non-verbal behavioral changes, with increasing syndromic convergence and functional dependency at later stages. Milestone symptoms were distilled to create a prototypical progression and severity scale of functional impairment: the PPA Progression Planning Aid (“PPA-Squared”).

DISCUSSION: This work introduces a symptom-led staging scheme and functional
scale for semantic and non-fluent/agrammatic variants of PPA. Our findings have implications for diagnostic and care pathway guidelines, trial design, and personalized prognosis and treatment for PPA.

Original language	English
Journal	Alzheimer's & Dementia
DOIs	https://doi.org/10.1002/alz.13415
Publication status	Published - 7 Aug 2023

Keywords

Frontotemporal dementia
Primary progressive aphasia
Primary progressive non-fluent/ agrammatic aphasia,
Progression planning aid
Semantic dementia
Staging

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Camic, P., Hardy, C. J. D., Taylor-Rubin, C., Harding, H., Suárez-Gonzalez, A., Jiang, J., Thompson, L., Kingma, K., Chokesuwattanaskul, A., Walker, F., Barker, S., Brotherhood, E. V., Waddington, C., Wood, O., Zimmerman, N., Kupeli, N., Yong, K. X. X., Scott, J., Marshall, C. R., ... Warren, J. D. (2023). Symptom-led staging for semantic and non-fluent/agrammatic variants of primary progressive aphasia. Alzheimer's & Dementia. Alzheimer's & Dementia. https://doi.org/10.1002/alz.13415

@article{327d173944414fa198cf20c03b10bb8a,

title = "Symptom-led staging for semantic and non-fluent/agrammatic variants of primary progressive aphasia. Alzheimer's \& Dementia",

abstract = "INTRODUCTION: Here we set out to create a symptom-led staging system for the canonical semantic and non-fluent/agrammatic variants of primary progressive aphasia (PPA), which present unique diagnostic and management challenges not well captured by functional scales developed for Alzheimer{\textquoteright}s disease and other dementias. METHODS: An international PPA caregiver cohort was surveyed on symptom development under six provisional clinical stages and feedback was analyzed using a mixed-methods sequential explanatory design. RESULTS: Both PPA syndromes were characterized by initial communication dysfunction and non-verbal behavioral changes, with increasing syndromic convergence and functional dependency at later stages. Milestone symptoms were distilled to create a prototypical progression and severity scale of functional impairment: the PPA Progression Planning Aid (“PPA-Squared”). DISCUSSION: This work introduces a symptom-led staging scheme and functional scale for semantic and non-fluent/agrammatic variants of PPA. Our findings have implications for diagnostic and care pathway guidelines, trial design, and personalized prognosis and treatment for PPA. ",

keywords = "Frontotemporal dementia, Primary progressive aphasia, Primary progressive non-fluent/ agrammatic aphasia,, Progression planning aid, Semantic dementia, Staging",

author = "Paul Camic and C.J.D. Hardy and C. Taylor-Rubin and H. Harding and A. Su{\'a}rez-Gonzalez and J. Jiang and L Thompson and K. Kingma and A. Chokesuwattanaskul and F. Walker and S. Barker and E.V. Brotherhood and C. Waddington and O. Wood and N. Zimmerman and N. Kupeli and K.X.X. Yong and J. Scott and C.R. Marshall and N.P. Oxtoby and J.D. Rohrer and A. Volkmer and S.J. Crutch and J.D. Warren",

year = "2023",

month = aug,

day = "7",

doi = "10.1002/alz.13415",

language = "English",

journal = "Alzheimer's \& Dementia",

issn = "1552-5260",

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Camic, P, Hardy, CJD, Taylor-Rubin, C, Harding, H, Suárez-Gonzalez, A, Jiang, J, Thompson, L, Kingma, K, Chokesuwattanaskul, A, Walker, F, Barker, S, Brotherhood, EV, Waddington, C, Wood, O, Zimmerman, N, Kupeli, N, Yong, KXX, Scott, J, Marshall, CR, Oxtoby, NP, Rohrer, JD, Volkmer, A, Crutch, SJ & Warren, JD 2023, 'Symptom-led staging for semantic and non-fluent/agrammatic variants of primary progressive aphasia. Alzheimer's & Dementia', Alzheimer's & Dementia. https://doi.org/10.1002/alz.13415

TY - JOUR

T1 - Symptom-led staging for semantic and non-fluent/agrammatic variants of primary progressive aphasia. Alzheimer's & Dementia

AU - Camic, Paul

AU - Hardy, C.J.D.

AU - Taylor-Rubin, C.

AU - Harding, H.

AU - Suárez-Gonzalez, A.

AU - Jiang, J.

AU - Thompson, L

AU - Kingma, K.

AU - Chokesuwattanaskul, A.

AU - Walker, F.

AU - Barker, S.

AU - Brotherhood, E.V.

AU - Waddington, C.

AU - Wood, O.

AU - Zimmerman, N.

AU - Kupeli, N.

AU - Yong, K.X.X.

AU - Scott, J.

AU - Marshall, C.R.

AU - Oxtoby, N.P.

AU - Rohrer, J.D.

AU - Volkmer, A.

AU - Crutch, S.J.

AU - Warren, J.D.

PY - 2023/8/7

Y1 - 2023/8/7

N2 - INTRODUCTION: Here we set out to create a symptom-led staging system for the canonical semantic and non-fluent/agrammatic variants of primary progressive aphasia (PPA), which present unique diagnostic and management challenges not well captured by functional scales developed for Alzheimer’s disease and other dementias. METHODS: An international PPA caregiver cohort was surveyed on symptom development under six provisional clinical stages and feedback was analyzed using a mixed-methods sequential explanatory design. RESULTS: Both PPA syndromes were characterized by initial communication dysfunction and non-verbal behavioral changes, with increasing syndromic convergence and functional dependency at later stages. Milestone symptoms were distilled to create a prototypical progression and severity scale of functional impairment: the PPA Progression Planning Aid (“PPA-Squared”). DISCUSSION: This work introduces a symptom-led staging scheme and functional scale for semantic and non-fluent/agrammatic variants of PPA. Our findings have implications for diagnostic and care pathway guidelines, trial design, and personalized prognosis and treatment for PPA.

AB - INTRODUCTION: Here we set out to create a symptom-led staging system for the canonical semantic and non-fluent/agrammatic variants of primary progressive aphasia (PPA), which present unique diagnostic and management challenges not well captured by functional scales developed for Alzheimer’s disease and other dementias. METHODS: An international PPA caregiver cohort was surveyed on symptom development under six provisional clinical stages and feedback was analyzed using a mixed-methods sequential explanatory design. RESULTS: Both PPA syndromes were characterized by initial communication dysfunction and non-verbal behavioral changes, with increasing syndromic convergence and functional dependency at later stages. Milestone symptoms were distilled to create a prototypical progression and severity scale of functional impairment: the PPA Progression Planning Aid (“PPA-Squared”). DISCUSSION: This work introduces a symptom-led staging scheme and functional scale for semantic and non-fluent/agrammatic variants of PPA. Our findings have implications for diagnostic and care pathway guidelines, trial design, and personalized prognosis and treatment for PPA.

KW - Frontotemporal dementia

KW - Primary progressive aphasia

KW - Primary progressive non-fluent/ agrammatic aphasia,

KW - Progression planning aid

KW - Semantic dementia

KW - Staging

U2 - 10.1002/alz.13415

DO - 10.1002/alz.13415

M3 - Article

SN - 1552-5260

JO - Alzheimer's & Dementia

JF - Alzheimer's & Dementia

ER -

Symptom-led staging for semantic and non-fluent/agrammatic variants of primary progressive aphasia. Alzheimer's & Dementia

Abstract

Keywords

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