Clinical and neuroimaging characteristics of clinically unclassifiable primary progressive aphasia

Rene L. Utianski; Hugo Botha; Peter R. Martin; Christopher G. Schwarz; Joseph R. Duffy; Heather M. Clark; Mary M. Machulda; Alissa M. Butts; V. J. Lowe; Clifford R. Jack; Matthew L. Senjem; Anthony J. Spychalla; Jennifer L. Whitwell; Keith A. Josephs

doi:10.1016/j.bandl.2019.104676

Clinical and neuroimaging characteristics of clinically unclassifiable primary progressive aphasia

Rene L. Utianski, Hugo Botha, Peter R. Martin, Christopher G. Schwarz, Joseph R. Duffy, Heather M. Clark, Mary M. Machulda, Alissa M. Butts, V. J. Lowe, Clifford R. Jack, Matthew L. Senjem, Anthony J. Spychalla, Jennifer L. Whitwell, Keith A. Josephs

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

9 Scopus citations

Abstract

Many patients who meet core/root criteria for Primary Progressive Aphasia (PPA) are not classifiable as a recognized variant and are often excluded from neuroimaging studies. Here, we detail neurological, neuropsychological, speech and language assessments, and anatomic and molecular neuroimaging (MRI, PiB-PET, and FDG-PET) for fifteen (8 female) clinically unclassifiable PPA patients. Median age of onset was 64 years old with median 3 years disease duration at exam. Three patients were amyloid positive on PiB-PET. 14/15 patients had abnormal FDG-PETs with left predominant hypometabolism, affecting frontal, temporal, parietal, and even occipital lobes. Patients had mild to severe clinical presentations. Visualization of the FDG-PETs principal component analysis revealed patterns of hypometabolism similar to those seen in the PPA variants and suggests the brain regions affected in unclassifiable PPA patients are no different from those who are more easily classifiable. These findings may inform future modifications to the diagnostic criteria to improve diagnostic classification.

Original language	English (US)
Article number	104676
Journal	Brain and Language
Volume	197
DOIs	https://doi.org/10.1016/j.bandl.2019.104676
State	Published - Oct 2019

Keywords

Amyloid imaging
Frontotemporal dementia
Hypometabolism
PET imaging
Primary progressive aphasia

ASJC Scopus subject areas

Language and Linguistics
Experimental and Cognitive Psychology
Linguistics and Language
Cognitive Neuroscience
Speech and Hearing

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10.1016/j.bandl.2019.104676

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Utianski, R. L., Botha, H., Martin, P. R., Schwarz, C. G., Duffy, J. R., Clark, H. M., Machulda, M. M., Butts, A. M., Lowe, V. J., Jack, C. R., Senjem, M. L., Spychalla, A. J., Whitwell, J. L., & Josephs, K. A. (2019). Clinical and neuroimaging characteristics of clinically unclassifiable primary progressive aphasia. Brain and Language, 197, [104676]. https://doi.org/10.1016/j.bandl.2019.104676

Clinical and neuroimaging characteristics of clinically unclassifiable primary progressive aphasia. / Utianski, Rene L.; Botha, Hugo; Martin, Peter R.; Schwarz, Christopher G.; Duffy, Joseph R.; Clark, Heather M.; Machulda, Mary M.; Butts, Alissa M.; Lowe, V. J.; Jack, Clifford R.; Senjem, Matthew L.; Spychalla, Anthony J.; Whitwell, Jennifer L.; Josephs, Keith A.

In: Brain and Language, Vol. 197, 104676, 10.2019.

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

Utianski, Rene L. ; Botha, Hugo ; Martin, Peter R. ; Schwarz, Christopher G. ; Duffy, Joseph R. ; Clark, Heather M. ; Machulda, Mary M. ; Butts, Alissa M. ; Lowe, V. J. ; Jack, Clifford R. ; Senjem, Matthew L. ; Spychalla, Anthony J. ; Whitwell, Jennifer L. ; Josephs, Keith A. / Clinical and neuroimaging characteristics of clinically unclassifiable primary progressive aphasia. In: Brain and Language. 2019 ; Vol. 197.

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title = "Clinical and neuroimaging characteristics of clinically unclassifiable primary progressive aphasia",

abstract = "Many patients who meet core/root criteria for Primary Progressive Aphasia (PPA) are not classifiable as a recognized variant and are often excluded from neuroimaging studies. Here, we detail neurological, neuropsychological, speech and language assessments, and anatomic and molecular neuroimaging (MRI, PiB-PET, and FDG-PET) for fifteen (8 female) clinically unclassifiable PPA patients. Median age of onset was 64 years old with median 3 years disease duration at exam. Three patients were amyloid positive on PiB-PET. 14/15 patients had abnormal FDG-PETs with left predominant hypometabolism, affecting frontal, temporal, parietal, and even occipital lobes. Patients had mild to severe clinical presentations. Visualization of the FDG-PETs principal component analysis revealed patterns of hypometabolism similar to those seen in the PPA variants and suggests the brain regions affected in unclassifiable PPA patients are no different from those who are more easily classifiable. These findings may inform future modifications to the diagnostic criteria to improve diagnostic classification.",

keywords = "Amyloid imaging, Frontotemporal dementia, Hypometabolism, PET imaging, Primary progressive aphasia",

author = "Utianski, {Rene L.} and Hugo Botha and Martin, {Peter R.} and Schwarz, {Christopher G.} and Duffy, {Joseph R.} and Clark, {Heather M.} and Machulda, {Mary M.} and Butts, {Alissa M.} and Lowe, {V. J.} and Jack, {Clifford R.} and Senjem, {Matthew L.} and Spychalla, {Anthony J.} and Whitwell, {Jennifer L.} and Josephs, {Keith A.}",

note = "Funding Information: We thank the patients and their families for their time and participation in our research program. We express gratitude to Sarah Boland, psychometrist and study coordinator, for her assistance in data collection and coordinating patient visits. This study was funded by the National Institutes of Health, National Institute on Deafness and Other Communication Disorders [grants R01 DC010367 (PI: Josephs), R01 DC014942 (PI: Josephs), and R01 DC012519 (PI: Whitwell)], National Institute of Neurological Disorders and Stroke [grant R21 NS094684 (PI: Josephs)], and National Institute on Aging [grant R37 AG11378 (PI: Jack)]. Publisher Copyright: {\textcopyright} 2019 Elsevier Inc.",

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doi = "10.1016/j.bandl.2019.104676",

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AU - Utianski, Rene L.

AU - Botha, Hugo

AU - Martin, Peter R.

AU - Schwarz, Christopher G.

AU - Duffy, Joseph R.

AU - Clark, Heather M.

AU - Machulda, Mary M.

AU - Butts, Alissa M.

AU - Lowe, V. J.

AU - Jack, Clifford R.

AU - Senjem, Matthew L.

AU - Spychalla, Anthony J.

AU - Whitwell, Jennifer L.

AU - Josephs, Keith A.

N1 - Funding Information: We thank the patients and their families for their time and participation in our research program. We express gratitude to Sarah Boland, psychometrist and study coordinator, for her assistance in data collection and coordinating patient visits. This study was funded by the National Institutes of Health, National Institute on Deafness and Other Communication Disorders [grants R01 DC010367 (PI: Josephs), R01 DC014942 (PI: Josephs), and R01 DC012519 (PI: Whitwell)], National Institute of Neurological Disorders and Stroke [grant R21 NS094684 (PI: Josephs)], and National Institute on Aging [grant R37 AG11378 (PI: Jack)]. Publisher Copyright: © 2019 Elsevier Inc.

PY - 2019/10

Y1 - 2019/10

N2 - Many patients who meet core/root criteria for Primary Progressive Aphasia (PPA) are not classifiable as a recognized variant and are often excluded from neuroimaging studies. Here, we detail neurological, neuropsychological, speech and language assessments, and anatomic and molecular neuroimaging (MRI, PiB-PET, and FDG-PET) for fifteen (8 female) clinically unclassifiable PPA patients. Median age of onset was 64 years old with median 3 years disease duration at exam. Three patients were amyloid positive on PiB-PET. 14/15 patients had abnormal FDG-PETs with left predominant hypometabolism, affecting frontal, temporal, parietal, and even occipital lobes. Patients had mild to severe clinical presentations. Visualization of the FDG-PETs principal component analysis revealed patterns of hypometabolism similar to those seen in the PPA variants and suggests the brain regions affected in unclassifiable PPA patients are no different from those who are more easily classifiable. These findings may inform future modifications to the diagnostic criteria to improve diagnostic classification.

AB - Many patients who meet core/root criteria for Primary Progressive Aphasia (PPA) are not classifiable as a recognized variant and are often excluded from neuroimaging studies. Here, we detail neurological, neuropsychological, speech and language assessments, and anatomic and molecular neuroimaging (MRI, PiB-PET, and FDG-PET) for fifteen (8 female) clinically unclassifiable PPA patients. Median age of onset was 64 years old with median 3 years disease duration at exam. Three patients were amyloid positive on PiB-PET. 14/15 patients had abnormal FDG-PETs with left predominant hypometabolism, affecting frontal, temporal, parietal, and even occipital lobes. Patients had mild to severe clinical presentations. Visualization of the FDG-PETs principal component analysis revealed patterns of hypometabolism similar to those seen in the PPA variants and suggests the brain regions affected in unclassifiable PPA patients are no different from those who are more easily classifiable. These findings may inform future modifications to the diagnostic criteria to improve diagnostic classification.

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SN - 0093-934X

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ER -

Clinical and neuroimaging characteristics of clinically unclassifiable primary progressive aphasia

Abstract

Keywords

ASJC Scopus subject areas

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