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 | |
| State | Published - Oct 1 2019 |
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Keywords
- Amyloid imaging
- Frontotemporal dementia
- Hypometabolism
- PET imaging
- Primary progressive aphasia
ASJC Scopus subject areas
- Experimental and Cognitive Psychology
- Language and Linguistics
- Linguistics and Language
- Cognitive Neuroscience
- Speech and Hearing
Cite this
Clinical and neuroimaging characteristics of clinically unclassifiable primary progressive aphasia. / Utianski, Rene; Botha, Hugo; Martin, Peter R.; Schwarz, Christopher; Duffy, Joseph R.; Clark, Heather; Machulda, Mary Margaret; Butts, Alissa; Lowe, Val; Jack, Clifford R Jr.; Senjem, Matthew L.; Spychalla, Anthony J.; Whitwell, Jennifer Lynn; Josephs, Keith Anthony.
In: Brain and Language, Vol. 197, 104676, 01.10.2019.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Clinical and neuroimaging characteristics of clinically unclassifiable primary progressive aphasia
AU - Utianski, Rene
AU - Botha, Hugo
AU - Martin, Peter R.
AU - Schwarz, Christopher
AU - Duffy, Joseph R.
AU - Clark, Heather
AU - Machulda, Mary Margaret
AU - Butts, Alissa
AU - Lowe, Val
AU - Jack, Clifford R Jr.
AU - Senjem, Matthew L.
AU - Spychalla, Anthony J.
AU - Whitwell, Jennifer Lynn
AU - Josephs, Keith Anthony
PY - 2019/10/1
Y1 - 2019/10/1
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.
KW - Amyloid imaging
KW - Frontotemporal dementia
KW - Hypometabolism
KW - PET imaging
KW - Primary progressive aphasia
UR - http://www.scopus.com/inward/record.url?scp=85070652080&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85070652080&partnerID=8YFLogxK
U2 - 10.1016/j.bandl.2019.104676
DO - 10.1016/j.bandl.2019.104676
M3 - Article
VL - 197
JO - Brain and Language
JF - Brain and Language
SN - 0093-934X
M1 - 104676
ER -