scholarly journals Memory Resilience in Alzheimer Disease With Primary Progressive Aphasia

Neurology ◽  
2021 ◽  
Vol 96 (6) ◽  
pp. e916-e925
Author(s):  
M.-Marsel Mesulam ◽  
Christina Coventry ◽  
Alan Kuang ◽  
Eileen H. Bigio ◽  
Qinwen Mao ◽  
...  
Keyword(s):  
Alzheimer Disease ◽  
Verbal Memory ◽  
Incidental Finding ◽  
Primary Progressive Aphasia ◽  
Neurofibrillary Degeneration ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Language Functions ◽  
Link Type ◽  
Potential Factors

ObjectiveTo determine whether memory is preserved longitudinally in primary progressive aphasia (PPA) associated with Alzheimer disease (AD) and to identify potential factors that maintain memory despite underlying neurofibrillary degeneration of mediotemporal memory areas.MethodsLongitudinal memory assessment was done in 17 patients with PPA with autopsy or biomarker evidence of AD (PPA-AD) and 14 patients with amnestic dementia of the Alzheimer type with AD at autopsy (DAT-AD).ResultsIn PPA-AD, episodic memory, tested with nonverbal items, was preserved at the initial testing and showed no decline at retesting 2.35 ± 0.78 years later, at which time symptoms had been present for 6.26 ± 2.21 years. In contrast, language functions declined significantly during the same period. In DAT-AD, both verbal memory and language declined with equal severity. Although imaging showed asymmetric left-sided mediotemporal atrophy in PPA-AD, autopsy revealed bilateral hippocampo-entorhinal neurofibrillary degeneration at Braak stages V and VI. Compared to DAT-AD, however, the PPA-AD group had lower incidence of APOE ε4 and of mediotemporal TAR DNA-binding protein 43 (TDP-43) pathology.ConclusionsMemory preservation in PPA is not just an incidental finding at onset but a core feature that persists for years despite the hippocampo-entorhinal AD neuropathology that is as severe as that of DAT-AD. Asymmetry of mediotemporal atrophy and a lesser impact of APOE ε4 and of TDP-43 on the integrity of memory circuitry may constitute some of the factors underlying this resilience. Our results also suggest that current controversies on memory in PPA-AD reflect inconsistencies in the diagnosis of logopenic PPA, the clinical variant most frequently associated with AD.ClinicalTrials.gov IdentifierNCT00537004 and NCT03371706.

scholarly journals Stealing Cookies in the Twenty-First Century: Measures of Spoken Narrative in Healthy Versus Speakers With Aphasia

2019 ◽  
Vol 28 (1S) ◽  
pp. 321-329 ◽  
Author(s):  
Shauna Berube ◽  
Jodi Nonnemacher ◽  
Cornelia Demsky ◽  
Shenly Glenn ◽  
Sadhvi Saxena ◽  
...  
Keyword(s):  
Preliminary Data ◽  
Primary Progressive Aphasia ◽  
Total Content ◽  
First Century ◽  
Healthy Controls ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Link Type ◽  
The Future ◽  
Language Output

Purpose Our goal was to evaluate an updated version of the “Cookie Theft” picture by obtaining norms based on picture descriptions by healthy controls for total content units (CUs), syllables per CU, and the ratio of left–right CUs. In addition, we aimed to compare these measures from healthy controls to picture descriptions obtained from individuals with poststroke aphasia and primary progressive aphasia (PPA) to assess whether these measures can capture impairments in content and efficiency of communication. Method Using an updated version of this picture, we analyzed descriptions from 50 healthy controls to develop norms for numbers of syllables, total CUs, syllables per CU, and left–right CU. We provide preliminary data from 44 individuals with aphasia (19 with poststroke aphasia and 25 with PPA). Results A total of 96 CUs were established based on the written transcriptions of spoken picture descriptions of the 50 control participants. There was a significant effect of group on total CUs, syllables, syllables per CU, and left–right CUs. The poststroke participants produced significantly fewer total CU and syllables than those with PPA. Each aphasic group produced significantly fewer total CUs, fewer syllables, more syllables per CU, and lower left–right CUs (indicating a right-sided bias) compared to controls. Conclusions Results show that the measures of numbers of syllables, total CUs, syllables per CU, and left–right CUs can distinguish language output of individuals with aphasia from controls and capture impairments in content and efficiency of communication. A limitation of this study is that we evaluated only 44 individuals with aphasia. In the future, we will evaluate other measures, such as CUs per minute, lexical variability, grammaticality, and ratio of nouns to verbs. Supplemental Material https://doi.org/10.23641/asha.7015223

NAMING DISORDERS IN LOGOPENIC VARIANT OF PRIMARY PROGRESSIVE APHASIA

2019 ◽  
Vol 17 (1) ◽  
pp. 87-95
Author(s):  
Justyna Antczak-Kujawin
Keyword(s):  
Study Participant ◽  
Primary Progressive Aphasia ◽  
Semantic Knowledge ◽  
Speech Disorders ◽  
Speech Comprehension ◽  
Motor Speech Disorders ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Language Functions

Language functions, particularly disordered lexical skills were diagnosed in the examined woman based on selected diagnostic tests of the Boston Diagnostic Aphasia Examination (BDAE). Furthermore, an experimental version of the author's original test for assessing lexical-semantic performance in dementia was used. The author presents a case study of a 79-year-old woman diagnosed with logopenic variant primary progressive aphasia (lvPPA) secondary to Alzheimer’s disease. The author describes the symptoms of anomie manifested by the study participant and the supplementary strategies she applied in the case of lexical deficits. The analysis of the findings obtained in the course of language function assessment allowed the author to assess the fluency of speech, speech comprehension, repetition and naming. The study participant diagnosed with lvPPA was observed to manifest the following: an absence of motor speech disorders, absence of characteristics of evident agrammatism, preserved comprehension of individual words, preserved semantic knowledge of objects, disordered retrieval of words in spontaneous speech and in attempts to name, and disordered repetition of sentences and phrases. The analysis of the discussed case study allowed the author to discuss the progressive lexical deficits manifested by the lvPPA patient and to record those supplementary strategies that were most frequently applied in the lexical difficulties experienced by the female patient diagnosed with lvPPA.

scholarly journals Relationships between [18F]-THK5351 Retention and Language Functions in Primary Progressive Aphasia

2019 ◽  
Vol 15 (4) ◽  
pp. 527
Author(s):  
Hye Jin Jeong ◽  
Cindy W Yoon ◽  
Seongho Seo ◽  
Sang-Yoon Lee ◽  
Mee Kyung Suh ◽  
...  
Keyword(s):  
Primary Progressive Aphasia ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Language Functions

scholarly journals Profile of linguistic and cognitive deficits in persons with a semantic variant of a primary progressive aphasia

2020 ◽  
Vol 54 (3) ◽  
pp. 113-119
Author(s):  
Lana Jerkić ◽  
Dragan Pavlović ◽  
Mile Vuković ◽  
Jelena Todorović ◽  
Mirna Zelić
Keyword(s):  
Specific Treatment ◽  
Primary Progressive Aphasia ◽  
Semantic Knowledge ◽  
Cognitive Changes ◽  
Progressive Aphasia ◽  
Progressive Deterioration ◽  
Primary Progressive ◽  
Language Functions ◽  
Semantic Variant ◽  
Speech And Language Disorders

Primary progressive aphasia (PPA) includes a group of neurodegenerative disorders that are characterized by progressive deterioration of language functions, while other cognitive functions, at least at the onset of the disease, are relatively spared. There are three basic subtypes of PPA: the nonfluent progressive aphasia (nvPPA), the semantic variant of a primary progressive aphasia (svPPA), and the logopenic progressive aphasia (lvPPA). The semantic variant of a PPA can also be found in the literature under the term of semantic dementia. It is clinically manifested by progressive deterioration of semantic knowledge, fluent aphasia, impaired naming and comprehension, prosopagnosia and surface dyslexia and dysgraphia (in languages with irregular orthography). As the disease progresses, other cognitive changes can be observed. The main cause of the disorder is progressive bilateral atrophy of the anterior temporal lobes, which is more manifested in the left hemisphere. The literature is modest in terms of the use of specific treatment methods in the rehabilitation of these patients. Since speech and language disorders are the most conspicuous symptom, at least at the beginning of the disease, the role of speech therapists in the assessment and restitution of speechlanguage and communication skills is also indisputable.

P1-354: RELATIONSHIPS BETWEEN [18 F]-THK5351 RETENTION AND LANGUAGE FUNCTIONS IN PRIMARY PROGRESSIVE APHASIA

2019 ◽  
Vol 15 ◽  
pp. P386-P386
Author(s):  
Young Noh ◽  
Hye Jin Jeong ◽  
Cindy W. Yoon ◽  
Kee Hyung Park ◽  
Kyoung-Min Lee ◽  
...  
Keyword(s):  
Primary Progressive Aphasia ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Language Functions

scholarly journals Multimodal predictors for Alzheimer disease in nonfluent primary progressive aphasia

Neurology ◽  
2010 ◽  
Vol 75 (7) ◽  
pp. 595-602 ◽  
Author(s):  
W. T. Hu ◽  
C. McMillan ◽  
D. Libon ◽  
S. Leight ◽  
M. Forman ◽  
...  
Keyword(s):  
Alzheimer Disease ◽  
Primary Progressive Aphasia ◽  
Progressive Aphasia ◽  
Primary Progressive

scholarly journals Multidimensional Apathy in Behavioral Variant Frontotemporal Dementia, Primary Progressive Aphasia, and Alzheimer Disease

2020 ◽  
pp. 089198872092471
Author(s):  
Ratko Radakovic ◽  
Shuna Colville ◽  
Denise Cranley ◽  
John M. Starr ◽  
Suvankar Pal ◽  
...  
Keyword(s):  
Alzheimer Disease ◽  
Frontotemporal Dementia ◽  
Primary Progressive Aphasia ◽  
Future Research ◽  
Self Awareness ◽  
Behavioral Variant Frontotemporal Dementia ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Patient Groups ◽  
Rating Discrepancy

Apathy is prevalent in dementia, such as behavioral variant frontotemporal dementia (bvFTD), primary progressive aphasia (PPA), and Alzheimer disease (AD). As a multidimensional construct, it can be assessed and subsumed under a Dimensional Apathy Framework. A consistent apathy profile in bvFTD and PPA has yet to be established. The aim was to explore apathy profiles and awareness in bvFTD, PPA, and AD. A total of 12 patients with bvFTD, 12 patients with PPA, 28 patients with AD, and 20 matched controls, as well as their informants/carers, were recruited. All participants completed the Dimensional Apathy Scale (DAS), assessing executive, emotional, and initiation apathy subtypes, a 1-dimensional apathy measure, depression measure, and functional and cognitive screens. Apathy subtype awareness was determined through DAS informant/carer and self-rating discrepancy. Apathy profile comparison showed patients with bvFTD had significantly higher emotional apathy than patients with AD ( P < .01) and significantly higher apathy over all subtypes than patients with PPA ( Ps < .05). Additionally, patients with bvFTD had significantly lower awareness for emotional apathy ( P < .01) when compared to patients with AD and PPA. All patient groups had significant global apathy over all subtypes compared to controls. The emergent apathy profile for bvFTD seems to be emotional apathy (indifference or emotional/affective neutrality), with lower self-awareness in this subtype. Further, lower self-awareness for executive apathy (lack of motivation for planning, organization, or attention) differentiates bvFTD from PPA. Future research should investigate the cognitive and neural correlates as well as the practical impact of apathy subtypes.

scholarly journals TDP-43 pathology in primary progressive aphasia and frontotemporal dementia with pathologic Alzheimer disease

2010 ◽  
Vol 120 (1) ◽  
pp. 43-54 ◽  
Author(s):  
Eileen H. Bigio ◽  
Manjari Mishra ◽  
Kimmo J. Hatanpaa ◽  
Charles L. White ◽  
Nancy Johnson ◽  
...  
Keyword(s):  
Alzheimer Disease ◽  
Frontotemporal Dementia ◽  
Primary Progressive Aphasia ◽  
Progressive Aphasia ◽  
Primary Progressive

scholarly journals Clinical marker for Alzheimer disease pathology in logopenic primary progressive aphasia

Neurology ◽  
2017 ◽  
Vol 88 (24) ◽  
pp. 2276-2284 ◽  
Author(s):  
Lucia A.A. Giannini ◽  
David J. Irwin ◽  
Corey T. McMillan ◽  
Sharon Ash ◽  
Katya Rascovsky ◽  
...  
Keyword(s):  
Alzheimer Disease ◽  
Digit Span ◽  
Primary Progressive Aphasia ◽  
Angular Gyrus ◽  
Lexical Retrieval ◽  
Central Feature ◽  
Retrospective Case ◽  
Progressive Aphasia ◽  
Primary Progressive ◽  
Sentence Repetition

Objective:To determine whether logopenic features of phonologic loop dysfunction reflect Alzheimer disease (AD) neuropathology in primary progressive aphasia (PPA).Methods: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.Results: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%, p < 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 (p < 0.01). Low DF scores correlated with high microscopic pathologic burden in superior/midtemporal and angular gyri (p ≤ 0.03).Conclusions: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.

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