Clinical marker for Alzheimer disease pathology in logopenic primary progressive aphasia

To determine whether logopenic features of phonologic loop dysfunction reflect Alzheimer disease (AD) neuropathology in primary progressive aphasia (PPA). We performed a retrospective case-control study of 34 patients with PPA with available autopsy tissue. We compared baseline and longitudinal clin...

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Veröffentlicht in:Neurology Jg. 88; H. 24; S. 2276
Hauptverfasser: Giannini, Lucia A A, Irwin, David J, McMillan, Corey T, Ash, Sharon, Rascovsky, Katya, Wolk, David A, Van Deerlin, Vivianna M, Lee, Edward B, Trojanowski, John Q, Grossman, Murray
Format: Journal Article
Sprache:Englisch
Veröffentlicht: United States 13.06.2017
Schlagworte:
Aged
Alzheimer Disease - diagnosis
Alzheimer Disease - pathology
Alzheimer Disease - psychology
Aphasia, Primary Progressive - diagnosis
Aphasia, Primary Progressive - pathology
Aphasia, Primary Progressive - psychology
Brain - diagnostic imaging
Brain - pathology
Cost of Illness
Female
Follow-Up Studies
Frontotemporal Lobar Degeneration - diagnosis
Frontotemporal Lobar Degeneration - pathology
Frontotemporal Lobar Degeneration - psychology
Humans
Language Tests
Longitudinal Studies
Magnetic Resonance Imaging
Male
Neuropsychological Tests
Phonetics
Retrospective Studies
ISSN:1526-632X, 1526-632X
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Zusammenfassung:To determine whether logopenic features of phonologic loop dysfunction reflect Alzheimer disease (AD) neuropathology in primary progressive aphasia (PPA). We performed a retrospective case-control study of 34 patients with PPA with available autopsy tissue. We compared baseline and longitudinal clinical features in patients with primary AD neuropathology to those with primary non-AD pathologies. We analyzed regional neuroanatomic disease burden in pathology-defined groups using postmortem neuropathologic data. A total of 19/34 patients had primary AD pathology and 15/34 had non-AD pathology (13 frontotemporal lobar degeneration, 2 Lewy body disease). A total of 16/19 (84%) patients with AD had a logopenic spectrum phenotype; 5 met published criteria for the logopenic variant (lvPPA), 8 had additional grammatical or semantic deficits (lvPPA+), and 3 had relatively preserved sentence repetition (lvPPA-). Sentence repetition was impaired in 68% of patients with PPA with AD pathology; forward digit span (DF) was impaired in 90%, substantially higher than in non-AD PPA (33%, < 0.01). Lexical retrieval difficulty was common in all patients with PPA and did not discriminate between groups. Compared to non-AD, PPA with AD pathology had elevated microscopic neurodegenerative pathology in the superior/midtemporal gyrus, angular gyrus, and midfrontal cortex ( < 0.01). Low DF scores correlated with high microscopic pathologic burden in superior/midtemporal and angular gyri ( ≤ 0.03). Phonologic loop dysfunction is a central feature of AD-associated PPA and specifically correlates with temporoparietal neurodegeneration. Quantitative measures of phonologic loop function, combined with modified clinical lvPPA criteria, may help discriminate AD-associated PPA.
Bibliographie:ObjectType-Article-1
SourceType-Scholarly Journals-1
ObjectType-Feature-2
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ISSN:1526-632X
1526-632X
DOI:10.1212/WNL.0000000000004034