Longitudinal naming and repetition relates to AD pathology and burden in autopsy‐confirmed primary progressive aphasia

Abstract

Introduction In primary progressive aphasia (PPA) patients with autopsy‐confirmed Alzheimer's disease (AD) or frontotemporal lobar degeneration (FLTD), we tested how the core clinical features of logopenic PPA—naming and repetition—change over time and relate to pathologic burden. Methods In PPA with AD (n = 13) or FTLD (n = 16) pathology, Boston Naming Test and Forward Digit Span measured longitudinal naming and repetition; as reference, Mini‐Mental State Examination (MMSE) measured global cognition. Pathologic burden in left peri‐Sylvian regions was related to longitudinal cognitive decline. Results PPA with AD showed greater decline in naming (P = 0.021) and repetition (P = 0.020), compared to FTLD; there was no difference in MMSE decline (P = 0.99). Across all PPA, declining naming (P = 0.0084) and repetition (P = 0.011) were associated with angular, superior‐middle temporal (naming P = 0.014; repetition P = 0.011) and middle frontal (naming P = 0.041; repetition P = 0.030) pathologic burden. Discussion Unique longitudinal profiles of naming and repetition performance in PPA with AD are related to left peri‐Sylvian pathology.

Event-based modeling of T1-weighted MRI is related to pathology in frontotemporal lobar degeneration due to tau and TDP

“…This relationship provides converging evidence with past studies examining associations between structural imaging measures and pathologic burden ( Burke et al, 2022 , Giannini et al, 2019a , Irwin et al, 2016a , Whitwell et al, 2009a ). Though MMSE is regularly shown to be impaired in patients with FTLD and worsens over time ( Bian et al, 2008 , Cousins et al, 2021 , Tan et al, 2013 ), we did not find such an association. This may be due in part to the fact that the MMSE was originally developed to monitor overall clinical change in AD patients with memory difficulty, and future work should perhaps examine EBM-estimated stages measures that are potentially more specific for FTLD, such as automated speech measures or the Philadelphia Brief Assessment of Cognition (PBAC) ( Cho et al, 2021 , Cousins et al, 2021 , Libon et al, 2011 , Nevler et al, 2019 ).…”

Case report: Non-Alzheimer's disease tauopathy with logopenic variant primary progressive aphasia diagnosed using amyloid and tau PET

“…In particular, tau PET tracer accumulations in the posterior temporal lobe and inferior parietal lobe (supramarginal/angular gyrus) may be the underlying neural basis for the “logopenic” status, which is explained by the dysfunction of the “phonological loop,” a component of short-term memory that includes a store in which phonological memory traces are held over a period of a few seconds, and an articulatory rehearsal process that refreshes them ( 3 ). The impairment of the “phonological loop,” which is generally well correlated with AD pathology ( 6 , 14 ), seemed to have manifested in our patient as syllabic errors in naming and reading aloud, incomplete sentence repetition, and impaired auditory comprehension of sentences. For example, as also described in the Results section, she was able to repeat the first two or three words/morphemes in the sentence repetition task of the MMSE correctly, but she failed to complete subsequent parts because of simplifications or substitutions; in the SLTA, errors were observed in sentence-level auditory comprehension, despite spared word-level auditory comprehension and sentence-level reading comprehension; in the JART, she answered with gestures or a roundabout explanation for some kanji words with highly irregular readings, suggesting that she knew the meaning of the words, but could not find the proper words and/or phonological representation (i.e., how to read the words aloud).…”

“…While the clinicopathological relationships in lv-PPA have remained somewhat unclear, recent studies have described the clinical characteristics of cases with atypical heterogeneous lv-PPA, as well as those of autopsy-confirmed cases of AD with lv-PPA, which have promoted a better understanding of the syndrome ( 5 – 7 , 14 – 18 ). For example, approximately one-third of patients with lv-PPA may have cerebral microbleeds and superficial siderosis ( 16 , 17 ); in rare instances, patients with lv-PPA may have GRN mutations ( 18 ).…”

A neuropsychological profile and its correlation with neuroimaging markers in patients with subcortical ischaemic vascular dementia

“…Although deficits in language and visuospatial function were also observed in SIVD patients, the impairments in naming and orientation judgement were milder than those in AD patients. This distinction is reasonable because the peri-Sylvian language regions 38 and the temporal lobe, 39 which mainly contribute to naming and spatial processing, were the regions with early involvement in AD pathology.…”

“…periventricular white matter using the Fazekas scale. 29 PVS was scored as 0 (none), 1 (1-10), 2 (11)(12)(13)(14)(15)(16)(17)(18)(19)(20), 3 (21)(22)(23)(24)(25)(26)(27)(28)(29)(30)(31)(32)(33)(34)(35)(36)(37)(38)(39)(40), and 4 (>40) according to the numbers in basal ganglia (BG) and centrum semiovale (CSO), respectively. 30 The higher score of the left or right hemisphere was used in the analysis for both WMH and PVS.…”