Clinical Correlates of Alzheimer's Disease Cerebrospinal Fluid Analytes in Primary Progressive Aphasia
Background: While primary progressive aphasia (PPA) is associated with frontotemporal lobar degeneration (FTLD) pathology due to tau or TDP, clinical-pathological studies also demonstrate many cases have Alzheimer's disease (AD) pathology. The logopenic variant of PPA (lvPPA) is most often asso...
Ausführliche Beschreibung
Autor*in: |
Catherine Norise [verfasserIn] Molly Ungrady [verfasserIn] Amy Halpin [verfasserIn] Charles Jester [verfasserIn] Corey T. McMillan [verfasserIn] David J. Irwin [verfasserIn] Katheryn A. Cousins [verfasserIn] Murray Grossman [verfasserIn] |
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Format: |
E-Artikel |
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Sprache: |
Englisch |
Erschienen: |
2019 |
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Schlagwörter: |
primary progressive aphasia (ppa) |
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Übergeordnetes Werk: |
In: Frontiers in Neurology - Frontiers Media S.A., 2010, 10(2019) |
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Übergeordnetes Werk: |
volume:10 ; year:2019 |
Links: |
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DOI / URN: |
10.3389/fneur.2019.00485 |
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Katalog-ID: |
DOAJ032613539 |
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520 | |a Background: While primary progressive aphasia (PPA) is associated with frontotemporal lobar degeneration (FTLD) pathology due to tau or TDP, clinical-pathological studies also demonstrate many cases have Alzheimer's disease (AD) pathology. The logopenic variant of PPA (lvPPA) is most often associated with AD pathology, but this has proven to be the least reliable PPA to diagnose using published clinical criteria. In this study, we used cerebrospinal fluid (CSF) analytes to identify patients with likely AD pathology, and relate phenotypic features of lvPPA to CSF.Methods: We studied 46 PPA patients who had available CSF analytes, including 26 with a clinical diagnosis of lvPPA, 9 with non-fluent/agrammatic variant (naPPA), and 11 with semantic variant (svPPA). We identified patients with likely AD pathology using amyloid-beta 1–42 (Aβ1−42) < 192 pg/ml and assessed MRI gray matter atrophy in these patients.Results: We found that 23 (49%) of 46 PPA patients have a low CSF Aβ1−42 level consistent with AD pathology. Twenty-one (91%) of 23 patients had a lvPPA phenotype, and 18 (79%) of 23 cases with an elevated CSF Aβ1−42 level did not have a lvPPA phenotype. Patients with a lvPPA phenotype demonstrated GM atrophy in the left lateral temporal lobe, and this was also seen in those with a CSF Aβ1−42 level < 192 pg/ml.Conclusion: The lvPPA clinical phenotype may be a useful screen for CSF analytes that are a surrogate for likely AD pathology, and may help establish eligibility of these patients for disease-modifying treatment trials. | ||
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10.3389/fneur.2019.00485 doi (DE-627)DOAJ032613539 (DE-599)DOAJ7a11e6d2907b4d87ba6a96ac2f098846 DE-627 ger DE-627 rakwb eng RC346-429 Catherine Norise verfasserin aut Clinical Correlates of Alzheimer's Disease Cerebrospinal Fluid Analytes in Primary Progressive Aphasia 2019 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Background: While primary progressive aphasia (PPA) is associated with frontotemporal lobar degeneration (FTLD) pathology due to tau or TDP, clinical-pathological studies also demonstrate many cases have Alzheimer's disease (AD) pathology. The logopenic variant of PPA (lvPPA) is most often associated with AD pathology, but this has proven to be the least reliable PPA to diagnose using published clinical criteria. In this study, we used cerebrospinal fluid (CSF) analytes to identify patients with likely AD pathology, and relate phenotypic features of lvPPA to CSF.Methods: We studied 46 PPA patients who had available CSF analytes, including 26 with a clinical diagnosis of lvPPA, 9 with non-fluent/agrammatic variant (naPPA), and 11 with semantic variant (svPPA). We identified patients with likely AD pathology using amyloid-beta 1–42 (Aβ1−42) < 192 pg/ml and assessed MRI gray matter atrophy in these patients.Results: We found that 23 (49%) of 46 PPA patients have a low CSF Aβ1−42 level consistent with AD pathology. Twenty-one (91%) of 23 patients had a lvPPA phenotype, and 18 (79%) of 23 cases with an elevated CSF Aβ1−42 level did not have a lvPPA phenotype. Patients with a lvPPA phenotype demonstrated GM atrophy in the left lateral temporal lobe, and this was also seen in those with a CSF Aβ1−42 level < 192 pg/ml.Conclusion: The lvPPA clinical phenotype may be a useful screen for CSF analytes that are a surrogate for likely AD pathology, and may help establish eligibility of these patients for disease-modifying treatment trials. primary progressive aphasia (ppa) PPA lvPPA logopenic variant primary progressive aphasia CSF logopenic primary progressive aphasia Neurology. Diseases of the nervous system Molly Ungrady verfasserin aut Amy Halpin verfasserin aut Charles Jester verfasserin aut Corey T. McMillan verfasserin aut David J. Irwin verfasserin aut Katheryn A. Cousins verfasserin aut Murray Grossman verfasserin aut In Frontiers in Neurology Frontiers Media S.A., 2010 10(2019) (DE-627)631498753 (DE-600)2564214-5 16642295 nnns volume:10 year:2019 https://doi.org/10.3389/fneur.2019.00485 kostenfrei https://doaj.org/article/7a11e6d2907b4d87ba6a96ac2f098846 kostenfrei https://www.frontiersin.org/article/10.3389/fneur.2019.00485/full kostenfrei https://doaj.org/toc/1664-2295 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2003 GBV_ILN_2014 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 10 2019 |
spelling |
10.3389/fneur.2019.00485 doi (DE-627)DOAJ032613539 (DE-599)DOAJ7a11e6d2907b4d87ba6a96ac2f098846 DE-627 ger DE-627 rakwb eng RC346-429 Catherine Norise verfasserin aut Clinical Correlates of Alzheimer's Disease Cerebrospinal Fluid Analytes in Primary Progressive Aphasia 2019 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Background: While primary progressive aphasia (PPA) is associated with frontotemporal lobar degeneration (FTLD) pathology due to tau or TDP, clinical-pathological studies also demonstrate many cases have Alzheimer's disease (AD) pathology. The logopenic variant of PPA (lvPPA) is most often associated with AD pathology, but this has proven to be the least reliable PPA to diagnose using published clinical criteria. In this study, we used cerebrospinal fluid (CSF) analytes to identify patients with likely AD pathology, and relate phenotypic features of lvPPA to CSF.Methods: We studied 46 PPA patients who had available CSF analytes, including 26 with a clinical diagnosis of lvPPA, 9 with non-fluent/agrammatic variant (naPPA), and 11 with semantic variant (svPPA). We identified patients with likely AD pathology using amyloid-beta 1–42 (Aβ1−42) < 192 pg/ml and assessed MRI gray matter atrophy in these patients.Results: We found that 23 (49%) of 46 PPA patients have a low CSF Aβ1−42 level consistent with AD pathology. Twenty-one (91%) of 23 patients had a lvPPA phenotype, and 18 (79%) of 23 cases with an elevated CSF Aβ1−42 level did not have a lvPPA phenotype. Patients with a lvPPA phenotype demonstrated GM atrophy in the left lateral temporal lobe, and this was also seen in those with a CSF Aβ1−42 level < 192 pg/ml.Conclusion: The lvPPA clinical phenotype may be a useful screen for CSF analytes that are a surrogate for likely AD pathology, and may help establish eligibility of these patients for disease-modifying treatment trials. primary progressive aphasia (ppa) PPA lvPPA logopenic variant primary progressive aphasia CSF logopenic primary progressive aphasia Neurology. Diseases of the nervous system Molly Ungrady verfasserin aut Amy Halpin verfasserin aut Charles Jester verfasserin aut Corey T. McMillan verfasserin aut David J. Irwin verfasserin aut Katheryn A. Cousins verfasserin aut Murray Grossman verfasserin aut In Frontiers in Neurology Frontiers Media S.A., 2010 10(2019) (DE-627)631498753 (DE-600)2564214-5 16642295 nnns volume:10 year:2019 https://doi.org/10.3389/fneur.2019.00485 kostenfrei https://doaj.org/article/7a11e6d2907b4d87ba6a96ac2f098846 kostenfrei https://www.frontiersin.org/article/10.3389/fneur.2019.00485/full kostenfrei https://doaj.org/toc/1664-2295 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2003 GBV_ILN_2014 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 10 2019 |
allfields_unstemmed |
10.3389/fneur.2019.00485 doi (DE-627)DOAJ032613539 (DE-599)DOAJ7a11e6d2907b4d87ba6a96ac2f098846 DE-627 ger DE-627 rakwb eng RC346-429 Catherine Norise verfasserin aut Clinical Correlates of Alzheimer's Disease Cerebrospinal Fluid Analytes in Primary Progressive Aphasia 2019 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Background: While primary progressive aphasia (PPA) is associated with frontotemporal lobar degeneration (FTLD) pathology due to tau or TDP, clinical-pathological studies also demonstrate many cases have Alzheimer's disease (AD) pathology. The logopenic variant of PPA (lvPPA) is most often associated with AD pathology, but this has proven to be the least reliable PPA to diagnose using published clinical criteria. In this study, we used cerebrospinal fluid (CSF) analytes to identify patients with likely AD pathology, and relate phenotypic features of lvPPA to CSF.Methods: We studied 46 PPA patients who had available CSF analytes, including 26 with a clinical diagnosis of lvPPA, 9 with non-fluent/agrammatic variant (naPPA), and 11 with semantic variant (svPPA). We identified patients with likely AD pathology using amyloid-beta 1–42 (Aβ1−42) < 192 pg/ml and assessed MRI gray matter atrophy in these patients.Results: We found that 23 (49%) of 46 PPA patients have a low CSF Aβ1−42 level consistent with AD pathology. Twenty-one (91%) of 23 patients had a lvPPA phenotype, and 18 (79%) of 23 cases with an elevated CSF Aβ1−42 level did not have a lvPPA phenotype. Patients with a lvPPA phenotype demonstrated GM atrophy in the left lateral temporal lobe, and this was also seen in those with a CSF Aβ1−42 level < 192 pg/ml.Conclusion: The lvPPA clinical phenotype may be a useful screen for CSF analytes that are a surrogate for likely AD pathology, and may help establish eligibility of these patients for disease-modifying treatment trials. primary progressive aphasia (ppa) PPA lvPPA logopenic variant primary progressive aphasia CSF logopenic primary progressive aphasia Neurology. Diseases of the nervous system Molly Ungrady verfasserin aut Amy Halpin verfasserin aut Charles Jester verfasserin aut Corey T. McMillan verfasserin aut David J. Irwin verfasserin aut Katheryn A. Cousins verfasserin aut Murray Grossman verfasserin aut In Frontiers in Neurology Frontiers Media S.A., 2010 10(2019) (DE-627)631498753 (DE-600)2564214-5 16642295 nnns volume:10 year:2019 https://doi.org/10.3389/fneur.2019.00485 kostenfrei https://doaj.org/article/7a11e6d2907b4d87ba6a96ac2f098846 kostenfrei https://www.frontiersin.org/article/10.3389/fneur.2019.00485/full kostenfrei https://doaj.org/toc/1664-2295 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2003 GBV_ILN_2014 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 10 2019 |
allfieldsGer |
10.3389/fneur.2019.00485 doi (DE-627)DOAJ032613539 (DE-599)DOAJ7a11e6d2907b4d87ba6a96ac2f098846 DE-627 ger DE-627 rakwb eng RC346-429 Catherine Norise verfasserin aut Clinical Correlates of Alzheimer's Disease Cerebrospinal Fluid Analytes in Primary Progressive Aphasia 2019 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Background: While primary progressive aphasia (PPA) is associated with frontotemporal lobar degeneration (FTLD) pathology due to tau or TDP, clinical-pathological studies also demonstrate many cases have Alzheimer's disease (AD) pathology. The logopenic variant of PPA (lvPPA) is most often associated with AD pathology, but this has proven to be the least reliable PPA to diagnose using published clinical criteria. In this study, we used cerebrospinal fluid (CSF) analytes to identify patients with likely AD pathology, and relate phenotypic features of lvPPA to CSF.Methods: We studied 46 PPA patients who had available CSF analytes, including 26 with a clinical diagnosis of lvPPA, 9 with non-fluent/agrammatic variant (naPPA), and 11 with semantic variant (svPPA). We identified patients with likely AD pathology using amyloid-beta 1–42 (Aβ1−42) < 192 pg/ml and assessed MRI gray matter atrophy in these patients.Results: We found that 23 (49%) of 46 PPA patients have a low CSF Aβ1−42 level consistent with AD pathology. Twenty-one (91%) of 23 patients had a lvPPA phenotype, and 18 (79%) of 23 cases with an elevated CSF Aβ1−42 level did not have a lvPPA phenotype. Patients with a lvPPA phenotype demonstrated GM atrophy in the left lateral temporal lobe, and this was also seen in those with a CSF Aβ1−42 level < 192 pg/ml.Conclusion: The lvPPA clinical phenotype may be a useful screen for CSF analytes that are a surrogate for likely AD pathology, and may help establish eligibility of these patients for disease-modifying treatment trials. primary progressive aphasia (ppa) PPA lvPPA logopenic variant primary progressive aphasia CSF logopenic primary progressive aphasia Neurology. Diseases of the nervous system Molly Ungrady verfasserin aut Amy Halpin verfasserin aut Charles Jester verfasserin aut Corey T. McMillan verfasserin aut David J. Irwin verfasserin aut Katheryn A. Cousins verfasserin aut Murray Grossman verfasserin aut In Frontiers in Neurology Frontiers Media S.A., 2010 10(2019) (DE-627)631498753 (DE-600)2564214-5 16642295 nnns volume:10 year:2019 https://doi.org/10.3389/fneur.2019.00485 kostenfrei https://doaj.org/article/7a11e6d2907b4d87ba6a96ac2f098846 kostenfrei https://www.frontiersin.org/article/10.3389/fneur.2019.00485/full kostenfrei https://doaj.org/toc/1664-2295 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2003 GBV_ILN_2014 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 10 2019 |
allfieldsSound |
10.3389/fneur.2019.00485 doi (DE-627)DOAJ032613539 (DE-599)DOAJ7a11e6d2907b4d87ba6a96ac2f098846 DE-627 ger DE-627 rakwb eng RC346-429 Catherine Norise verfasserin aut Clinical Correlates of Alzheimer's Disease Cerebrospinal Fluid Analytes in Primary Progressive Aphasia 2019 Text txt rdacontent Computermedien c rdamedia Online-Ressource cr rdacarrier Background: While primary progressive aphasia (PPA) is associated with frontotemporal lobar degeneration (FTLD) pathology due to tau or TDP, clinical-pathological studies also demonstrate many cases have Alzheimer's disease (AD) pathology. The logopenic variant of PPA (lvPPA) is most often associated with AD pathology, but this has proven to be the least reliable PPA to diagnose using published clinical criteria. In this study, we used cerebrospinal fluid (CSF) analytes to identify patients with likely AD pathology, and relate phenotypic features of lvPPA to CSF.Methods: We studied 46 PPA patients who had available CSF analytes, including 26 with a clinical diagnosis of lvPPA, 9 with non-fluent/agrammatic variant (naPPA), and 11 with semantic variant (svPPA). We identified patients with likely AD pathology using amyloid-beta 1–42 (Aβ1−42) < 192 pg/ml and assessed MRI gray matter atrophy in these patients.Results: We found that 23 (49%) of 46 PPA patients have a low CSF Aβ1−42 level consistent with AD pathology. Twenty-one (91%) of 23 patients had a lvPPA phenotype, and 18 (79%) of 23 cases with an elevated CSF Aβ1−42 level did not have a lvPPA phenotype. Patients with a lvPPA phenotype demonstrated GM atrophy in the left lateral temporal lobe, and this was also seen in those with a CSF Aβ1−42 level < 192 pg/ml.Conclusion: The lvPPA clinical phenotype may be a useful screen for CSF analytes that are a surrogate for likely AD pathology, and may help establish eligibility of these patients for disease-modifying treatment trials. primary progressive aphasia (ppa) PPA lvPPA logopenic variant primary progressive aphasia CSF logopenic primary progressive aphasia Neurology. Diseases of the nervous system Molly Ungrady verfasserin aut Amy Halpin verfasserin aut Charles Jester verfasserin aut Corey T. McMillan verfasserin aut David J. Irwin verfasserin aut Katheryn A. Cousins verfasserin aut Murray Grossman verfasserin aut In Frontiers in Neurology Frontiers Media S.A., 2010 10(2019) (DE-627)631498753 (DE-600)2564214-5 16642295 nnns volume:10 year:2019 https://doi.org/10.3389/fneur.2019.00485 kostenfrei https://doaj.org/article/7a11e6d2907b4d87ba6a96ac2f098846 kostenfrei https://www.frontiersin.org/article/10.3389/fneur.2019.00485/full kostenfrei https://doaj.org/toc/1664-2295 Journal toc kostenfrei GBV_USEFLAG_A SYSFLAG_A GBV_DOAJ SSG-OLC-PHA GBV_ILN_11 GBV_ILN_20 GBV_ILN_22 GBV_ILN_23 GBV_ILN_24 GBV_ILN_31 GBV_ILN_39 GBV_ILN_40 GBV_ILN_60 GBV_ILN_62 GBV_ILN_63 GBV_ILN_65 GBV_ILN_69 GBV_ILN_73 GBV_ILN_74 GBV_ILN_95 GBV_ILN_105 GBV_ILN_110 GBV_ILN_151 GBV_ILN_161 GBV_ILN_170 GBV_ILN_206 GBV_ILN_213 GBV_ILN_230 GBV_ILN_285 GBV_ILN_293 GBV_ILN_602 GBV_ILN_2003 GBV_ILN_2014 GBV_ILN_4012 GBV_ILN_4037 GBV_ILN_4112 GBV_ILN_4125 GBV_ILN_4126 GBV_ILN_4249 GBV_ILN_4305 GBV_ILN_4306 GBV_ILN_4307 GBV_ILN_4313 GBV_ILN_4322 GBV_ILN_4323 GBV_ILN_4324 GBV_ILN_4325 GBV_ILN_4338 GBV_ILN_4367 GBV_ILN_4700 AR 10 2019 |
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RC346-429 Clinical Correlates of Alzheimer's Disease Cerebrospinal Fluid Analytes in Primary Progressive Aphasia primary progressive aphasia (ppa) PPA lvPPA logopenic variant primary progressive aphasia CSF logopenic primary progressive aphasia |
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clinical correlates of alzheimer's disease cerebrospinal fluid analytes in primary progressive aphasia |
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Clinical Correlates of Alzheimer's Disease Cerebrospinal Fluid Analytes in Primary Progressive Aphasia |
abstract |
Background: While primary progressive aphasia (PPA) is associated with frontotemporal lobar degeneration (FTLD) pathology due to tau or TDP, clinical-pathological studies also demonstrate many cases have Alzheimer's disease (AD) pathology. The logopenic variant of PPA (lvPPA) is most often associated with AD pathology, but this has proven to be the least reliable PPA to diagnose using published clinical criteria. In this study, we used cerebrospinal fluid (CSF) analytes to identify patients with likely AD pathology, and relate phenotypic features of lvPPA to CSF.Methods: We studied 46 PPA patients who had available CSF analytes, including 26 with a clinical diagnosis of lvPPA, 9 with non-fluent/agrammatic variant (naPPA), and 11 with semantic variant (svPPA). We identified patients with likely AD pathology using amyloid-beta 1–42 (Aβ1−42) < 192 pg/ml and assessed MRI gray matter atrophy in these patients.Results: We found that 23 (49%) of 46 PPA patients have a low CSF Aβ1−42 level consistent with AD pathology. Twenty-one (91%) of 23 patients had a lvPPA phenotype, and 18 (79%) of 23 cases with an elevated CSF Aβ1−42 level did not have a lvPPA phenotype. Patients with a lvPPA phenotype demonstrated GM atrophy in the left lateral temporal lobe, and this was also seen in those with a CSF Aβ1−42 level < 192 pg/ml.Conclusion: The lvPPA clinical phenotype may be a useful screen for CSF analytes that are a surrogate for likely AD pathology, and may help establish eligibility of these patients for disease-modifying treatment trials. |
abstractGer |
Background: While primary progressive aphasia (PPA) is associated with frontotemporal lobar degeneration (FTLD) pathology due to tau or TDP, clinical-pathological studies also demonstrate many cases have Alzheimer's disease (AD) pathology. The logopenic variant of PPA (lvPPA) is most often associated with AD pathology, but this has proven to be the least reliable PPA to diagnose using published clinical criteria. In this study, we used cerebrospinal fluid (CSF) analytes to identify patients with likely AD pathology, and relate phenotypic features of lvPPA to CSF.Methods: We studied 46 PPA patients who had available CSF analytes, including 26 with a clinical diagnosis of lvPPA, 9 with non-fluent/agrammatic variant (naPPA), and 11 with semantic variant (svPPA). We identified patients with likely AD pathology using amyloid-beta 1–42 (Aβ1−42) < 192 pg/ml and assessed MRI gray matter atrophy in these patients.Results: We found that 23 (49%) of 46 PPA patients have a low CSF Aβ1−42 level consistent with AD pathology. Twenty-one (91%) of 23 patients had a lvPPA phenotype, and 18 (79%) of 23 cases with an elevated CSF Aβ1−42 level did not have a lvPPA phenotype. Patients with a lvPPA phenotype demonstrated GM atrophy in the left lateral temporal lobe, and this was also seen in those with a CSF Aβ1−42 level < 192 pg/ml.Conclusion: The lvPPA clinical phenotype may be a useful screen for CSF analytes that are a surrogate for likely AD pathology, and may help establish eligibility of these patients for disease-modifying treatment trials. |
abstract_unstemmed |
Background: While primary progressive aphasia (PPA) is associated with frontotemporal lobar degeneration (FTLD) pathology due to tau or TDP, clinical-pathological studies also demonstrate many cases have Alzheimer's disease (AD) pathology. The logopenic variant of PPA (lvPPA) is most often associated with AD pathology, but this has proven to be the least reliable PPA to diagnose using published clinical criteria. In this study, we used cerebrospinal fluid (CSF) analytes to identify patients with likely AD pathology, and relate phenotypic features of lvPPA to CSF.Methods: We studied 46 PPA patients who had available CSF analytes, including 26 with a clinical diagnosis of lvPPA, 9 with non-fluent/agrammatic variant (naPPA), and 11 with semantic variant (svPPA). We identified patients with likely AD pathology using amyloid-beta 1–42 (Aβ1−42) < 192 pg/ml and assessed MRI gray matter atrophy in these patients.Results: We found that 23 (49%) of 46 PPA patients have a low CSF Aβ1−42 level consistent with AD pathology. Twenty-one (91%) of 23 patients had a lvPPA phenotype, and 18 (79%) of 23 cases with an elevated CSF Aβ1−42 level did not have a lvPPA phenotype. Patients with a lvPPA phenotype demonstrated GM atrophy in the left lateral temporal lobe, and this was also seen in those with a CSF Aβ1−42 level < 192 pg/ml.Conclusion: The lvPPA clinical phenotype may be a useful screen for CSF analytes that are a surrogate for likely AD pathology, and may help establish eligibility of these patients for disease-modifying treatment trials. |
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The logopenic variant of PPA (lvPPA) is most often associated with AD pathology, but this has proven to be the least reliable PPA to diagnose using published clinical criteria. In this study, we used cerebrospinal fluid (CSF) analytes to identify patients with likely AD pathology, and relate phenotypic features of lvPPA to CSF.Methods: We studied 46 PPA patients who had available CSF analytes, including 26 with a clinical diagnosis of lvPPA, 9 with non-fluent/agrammatic variant (naPPA), and 11 with semantic variant (svPPA). We identified patients with likely AD pathology using amyloid-beta 1–42 (Aβ1−42) &lt; 192 pg/ml and assessed MRI gray matter atrophy in these patients.Results: We found that 23 (49%) of 46 PPA patients have a low CSF Aβ1−42 level consistent with AD pathology. Twenty-one (91%) of 23 patients had a lvPPA phenotype, and 18 (79%) of 23 cases with an elevated CSF Aβ1−42 level did not have a lvPPA phenotype. Patients with a lvPPA phenotype demonstrated GM atrophy in the left lateral temporal lobe, and this was also seen in those with a CSF Aβ1−42 level &lt; 192 pg/ml.Conclusion: The lvPPA clinical phenotype may be a useful screen for CSF analytes that are a surrogate for likely AD pathology, and may help establish eligibility of these patients for disease-modifying treatment trials.</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">primary progressive aphasia (ppa)</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">PPA</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">lvPPA</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">logopenic variant primary progressive aphasia</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">CSF</subfield></datafield><datafield tag="650" ind1=" " ind2="4"><subfield code="a">logopenic primary progressive aphasia</subfield></datafield><datafield tag="653" ind1=" " ind2="0"><subfield code="a">Neurology. Diseases of the nervous system</subfield></datafield><datafield tag="700" ind1="0" ind2=" "><subfield code="a">Molly Ungrady</subfield><subfield code="e">verfasserin</subfield><subfield code="4">aut</subfield></datafield><datafield tag="700" ind1="0" ind2=" "><subfield code="a">Amy Halpin</subfield><subfield code="e">verfasserin</subfield><subfield code="4">aut</subfield></datafield><datafield tag="700" ind1="0" ind2=" "><subfield code="a">Charles Jester</subfield><subfield code="e">verfasserin</subfield><subfield code="4">aut</subfield></datafield><datafield tag="700" ind1="0" ind2=" "><subfield code="a">Corey T. McMillan</subfield><subfield code="e">verfasserin</subfield><subfield code="4">aut</subfield></datafield><datafield tag="700" ind1="0" ind2=" "><subfield code="a">David J. Irwin</subfield><subfield code="e">verfasserin</subfield><subfield code="4">aut</subfield></datafield><datafield tag="700" ind1="0" ind2=" "><subfield code="a">Katheryn A. 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