Search Results (22)

Background: Primary Progressive Aphasia (PPA) variants include the non-fluent agrammatic (nfvPPA), logopenic (lvPPA), and semantic (svPPA), which differ in their effects on speech production. However, their impact on modality (oral vs. written) and grammatical word class (nouns vs. verbs) remains controversial. A significant effect of these variables might assist in classification. Materials and Methods: This study used first-visit data from 300 participants with PPA who completed oral and written noun and verb naming (matched in surface word frequency across word class) to test the hypothesis that the three variants show differential impairment on word class or modality. Group differences were evaluated with rank-transformed repeated measures ANOVA. Within individual differences between nouns and verbs and between oral and written modalities were tested with Fisher’s exact tests. Results: A significant modality × variant interaction (p = 0.017) was observed. Participants with lvPPA and nfvPPA demonstrated greater oral than written naming, with nfvPPA also performing better on nouns than verbs. Those with svPPA showed no modality or word class effects but had an overall low accuracy. Three participants with svPPA (but no individuals with the other variants) demonstrated significantly (p = 0.003) more accurate verb than noun naming. Conclusions: Differing modality and word class patterns characterize PPA variants, with nfvPPA more accurate in nouns than verbs on average. Within individuals, only those with svPPA occasionally showed significantly more proficient verb than noun naming. Grammatical word class effects likely arise at distinct levels of cognitive processing underlying naming. Full article

Background and Clinical Significance: In our case study, the patient experienced approximately a year-long delay in her diagnosis, where her initial diagnosis was mistakenly a primary psychiatric disorder, resulting in undue stress on her family. The aim of this case study is to raise awareness of frontal variant Alzheimer’s dementia (fvAD) and to increase knowledge amongst clinicians about this disorder, its management and the need for long-term follow up in specialized clinics. Case Presentation: In January 2023, a 56-year-old woman first presented with a 4-month history of worsening cognitive symptoms with considerable overlapping mood symptoms. Her Mini-Mental State Examination (MMSE) score was 20/28, whereas her Frontal Assessment Battery (FAB) score was 6/18. Upon neuropsychological evaluation, she demonstrated multidomain cognitive deficits, where impairments were most prominent in executive dysfunction, learning, memory and semantic fluency. There was evidence of progressive neurodegenerative changes, with brain MRI (April 2024) showing predominant bilateral frontal and parietal volume loss, sparing the occipital and temporal lobes. Amyloid positron emission tomography (PET) was diffusely positive. A diagnosis of fvAD (frontal variant Alzheimer’s dementia) with BPSD was made. Other differential diagnoses included a major neurocognitive disorder due to multiple etiologies (AD and dementia with Lewy bodies (DLB)), frontotemporal dementia (bvFTD), primary progressive aphasia (PPA) and the psychiatric disorder of pseudodementia secondary to a mood disorder. Conclusions: This case presented significant challenges given the atypical neuropsychological profile and the complexity of the symptom presentation with significant neuropsychiatric overlay. The preliminary research findings underscore the complexity of fvAD, warranting future research using fundamental approaches. Full article

Background/Objectives: Oral word production (OWP) deficits are prominent in the logopenic variant of primary progressive aphasia (lvPPA); however, their functional origin remains unclear. Some studies suggest a lexical, post-lexical, or even a combined functional origin of these deficits. The aim of the present study was to synthesize and analyze the information on the functional origin of the OWP deficits in patients with lvPPA. Methods: A quantitative systematic literature review was carried out using four databases: CINAHL, PsycINFO, Linguistics and Language Behavior Abstracts, and PubMed. Fourteen studies, including a total of 243 patients with lvPPA, and reporting results on picture naming and/or word and/or pseudoword repetition, were selected. Results: The overall findings of this review highlighted that two main functional origins appear to explain the OWP deficits in lvPPA: a lexical impairment affecting lexical processing and a post-lexical impairment affecting phonological short-term memory. Interestingly, the possibility of a third functional origin, affecting the semantic processing level, was also suggested by some studies. Conclusions: We concluded that the presence of different functional origins of OWP in this population may be explained, at least partially, by the diversity of assessment tasks used in studies and the varied manipulation and control of psycholinguistic properties of words (e.g., frequency, length), as well as the various interpretations and analyses of the participants’ errors. Further studies are needed to substantiate these findings by examining all the components involved in OWP, carefully manipulating the psycholinguistic properties and qualitatively analyzing the errors made by lvPPA participants. Full article

Introduction. Primary progressive aphasia (PPA) is a clinical syndrome characterized by a progressive deterioration in language and speech. It is classified into three variants based on symptom patterns: logopenic, semantic, and non-fluent. Due to the lack of fully reliable and valid screening tests for diagnosing PPA and its variants, a Spanish version of the Mini Linguistic State Examination (MLSE) has recently been introduced. Materials and methods. This study aimed to describe the language impairments in a small sample of six patients with PPA and classify them into the three variants using the decision tree and syndrome guide proposed by the MLSE authors. Results. The findings demonstrate the test’s utility in classifying some PPA variants through a qualitative analysis of patient performance and error types. The study revealed a 50% accuracy rate for the decision tree and an 83.33% accuracy rate when using the syndrome guide. Discussion. This discrepancy arises because the decision tree often classified cases as logopenic variant PPA (lvPPA) when working memory was significantly impaired. Specifically, it tended to misclassify patients with semantic, motor, or speech impairments as having lvPPA due to its reliance on the sentence repetition task for assessing working memory. Full article

Syntactic deficits are not a core symptom of the semantic variant of primary progressive aphasia (svPPA). However, some studies indicate syntactic impairments that deteriorate with progress of the disease. The study addresses the presence of a syntactic deficit and its association with clinical, neuropsychological, neuroanatomical and demographic variables. Nine individuals with svPPA were tested with a cognitive screening test (Addenbrooke’s Cognitive Examination, ACE-R), a language screening test (Boston Diagnostic Aphasia Examination—BDAE) and a syntactic test (object question elicitation). Additionally, the regional cerebral flow was measured with brain single-photon emission computed tomography (SPECT). The results indicated significant impairment in the production of object questions for some patients. Performance on these clauses was not predicted by the ACE-R or by hypoperfusion in the language areas. While there was no evidence for an effect of disease duration on syntactic performance, the results reveal a potential effect of the age of symptom onset. Furthermore, the results reveal a significant association between the performance in BDAE and the years of education and production of object questions. Overall, these results indicate a significant specific deficit in syntax for some patients with svPPA and highlight the importance of the premorbid educational status for the progress of the disease. Full article

Background: The goal of this study was to investigate whether the combination of semantic feature analysis (SFA) and transcranial direct current stimulation (tDCS) is effective in treating word retrieval in the semantic variant of primary progressive aphasia (svPPA) and how long the potential effects last. Methods: A 56-year-old woman diagnosed with frontotemporal dementia (FTD) and svPPA participated in this longitudinal single-subject design. A total of four 2-week stimulation phases were conducted over a 14-month period, each of which was started depending on the participant’s language performance. Follow-up testing was conducted shortly after the stimulation period, approximately 2 weeks, and approximately 4 weeks thereafter. Results: Significant improvement in word retrieval occurred after SFA and tDCS therapy. Two weeks after the end of each stimulation phase, approx. 80% of the trained words could be named correctly. For the untrained words, also significantly more words were correctly named at follow-ups compared to the baseline. Furthermore, the Boston Naming Test (BNT) demonstrated a significant increase in naming performance and showed that phonological cues facilitated word retrieval compared to semantic cues. Conclusion: The combination of SFA and tDCS was able to counteract the expected language deterioration of a participant with svPPA. This effect increased until approximately 2 weeks after each intervention. In addition, a generalization of the effect to untrained words was shown. Full article

Naming decline is one of the most common symptoms of primary progressive aphasia (PPA). Most studies on anomia in PPA are performed without taking into account PPA variants, especially for action naming. Only limited data are available for the neuroanatomical basis of anomia considering differences in the pathogenesis of PPAs. The aim of our study is to investigate the associations between anomia severity for both noun and verb naming and gray matter (GM) atrophy, as well as accompanying functional connectivity (FC) changes in three PPA variants. A total of 17 patients with non-fluent (nfvPPA), 11 with semantic (svPPA), and 9 with logopenic (lvPPA) PPA variants were included in the study and underwent cognitive/naming assessments and brain MRIs. Voxel-based morphometry was performed to evaluate GM volume. A resting-state functional MRI was applied to investigate FC changes in the identified GM areas. The study shows that different brain regions are involved in naming decline in each PPA variant with a predominantly temporal lobe involvement in svPPA, parietal lobe involvement in lvPPA, and frontal lobe involvement in nfvPPA. Separate data for object and action naming in PPA variants are provided. The obtained results mainly correspond to the current understanding of language processing and indicate that the evaluation of language impairments is preferable for each PPA variant separately. A further analysis of larger cohorts of patients is necessary to confirm these preliminary results. Full article

Semantic Variant of Primary Progressive Aphasia (svPPA) has often been considered as a loss of knowledge stored in semantic memory, but might also be due to a general disruption of mechanisms allowing the acquisition, storage, and retrieval of semantic memories. In order to assess any parallelism in svPPA patients between loss of semantic knowledge and inability to acquire new semantic information, we administered a battery of semantic learning tasks to healthy individuals and svPPA patients, where they were requested to learn new conceptual representations and new word forms, and to associate the former with the latter. A strong relation was found between loss of semantic knowledge and disruption of semantic learning: (a) patients with severe svPPA had the lowest scores in the semantic learning tasks; (b) significant correlations were found between scores obtained in semantic learning tasks and scores obtained in semantic memory disorders in svPPA patients. Full article

The concreteness effect (CE), namely a better performance with concrete compared to abstract concepts, is a constant feature in healthy people, and it usually increases in persons with aphasia (PWA). However, a reversal of the CE has been reported in patients affected by the semantic variant of Primary Progressive Aphasia (svPPA), a neurodegenerative disease characterized by anterior temporal lobe (ATL) atrophy. The present scoping review aims at identifying the extent of evidence regarding the abstract/concrete contrast in Alzheimer’s disease (AD) and svPPA and associated brain atrophy. Five online databases were searched up to January 2023 to identify papers where both concrete and abstract concepts were investigated. Thirty-one papers were selected and showed that while in patients with AD, concrete words were better processes than abstract ones, in most svPPA patients, there was a reversal of the CE, with five studies correlating the size of this effect with ATL atrophy. Furthermore, the reversal of CE was associated with category-specific impairments (living things) and with a selective deficit of social words. Future work is needed to disentangle the role of specific portions of the ATL in concept representation. Full article

The logopenic variant of primary progressive aphasia (lvPPA) shows different features from the non-fluent (nfvPPA) and semantic (svPPA) variants of PPA. Although language impairments remain the core symptoms, studies have highlighted the presence of executive disorders at the onset of the disease. Nevertheless, the results are contradictory, particularly in lvPPA. The aim of this study was to explore the executive profile of lvPPA. We compared executive functioning in lvPPA with the other two variants of PPA, Alzheimer’s disease (AD) and a cognitively healthy group. In total, 70 patients with PPA, 32 patients with AD, and 41 healthy controls were included. They underwent a comprehensive executive battery assessing short-term and working memory, inhibition, flexibility, planning, and initiation. The analyses showed significant differences between the lvPPA group and the control group, except on visuospatial spans and the Stroop test, and between the lvPPA group and the other PPA groups and the AD group for several tasks. Thus, this research highlighted the existence of an executive dysfunction from the onset of the disease in lvPPA but also in the other two variants of PPA. Full article

The aim of this study was to characterize the oral discourse of CBS patients and to verify whether measures obtained during a semi-spontaneous speech production could differentiate CBS patients from controls. A second goal was to compare the performance of patients with CBS probably due to Alzheimer’s disease (CBS-AD) pathology and CBS not related to AD (CBS-non-AD) in the same measures, based on the brain metabolic status (FDG-PET) and in the presence of amyloid deposition (amyloid-PET). Results showed that CBS patients were significantly different from controls in speech rate, lexical level, informativeness, and syntactic complexity. Discursive measures did not differentiate CBS-AD from CBS-non-AD. However, CBS-AD displayed more lexical-semantic impairments than controls, a profile that is frequently reported in patients with clinical AD and the logopenic variant of primary progressive aphasia (lvPPA). CBS-non-AD presented mainly with impairments related to motor speech disorders and syntactic complexity, as seen in the non-fluent variant of PPA. Full article

Background: Social cognition helps people to understand their own and others’ behavior and to modulate the way of thinking and acting in different social situations. Rapid and accurate diagnoses of neurodegenerative diseases are essential, as social cognition is affected by these diseases. The Revised Self-Monitoring Scale (RSMS) is a scale that detects social–emotional cognition deficits. Aim: The aim of the current study is to examine how socioemotional parameters are affected by neurodegenerative diseases and whether the RSMS can discern these disorders based on the socioemotional parameters in the Greek population. Methods/Design: A total of 331 dementia subjects were included. Mini Mental State Examination (MMSE) and Addenbrooke’s Cognitive Examination (Revised, ACE-R) measurements were used in order to assess the cognitive deficits. The Neuropsychiatric Inventory (NPI) was used for the evaluation of the neuropsychiatric symptoms. The RSMS and its two subscales was used in order to detect the socioemotional deficits. Results: The RSMS and its two subscales (RSMS_EX and RSMS_SP) can effectively detect neurodegenerative diseases. The RSMS can detect bvFTD in Alzheimer’s Disease (AD), AD in a healthy cohort, behavioral variant Frontotemporal Dementia (bvFTD) in a healthy cohort, bvFTD in Parkinson’s Disease (PD) and Frontotemporal Semantic Dementia (FTD/SD) in a healthy cohort. It is a useful tool in order to detect frontotemporal dementias. RSMS correlated negatively with the NPI questionnaire total and the subcategories of apathy, disinhibition and eating disorders. The RSMS results are associated with the ACE-R score (specifically verbal fluency). Conclusions: The RSMS is a helpful tool in order to identify socioemotional deficits in neurodegenerative dementias. It is also a useful scale that can discern bvFTD and svPPA in AD patients. A worse RSMS score correlates with a worse ACE-R and NPI. It seems to be a useful scale that can reliably measure social behavior in non-reversible neurodegenerative disorders, such as AD, FTD (bvFTD, svPPA), PDD and PD. The results also apply to the Greek population. Full article

Primary progressive aphasia (PPA) damages the parts of the brain that control speech and language. There are three clinical PPA variants: nonfluent/agrammatic (nfvPPA), logopenic (lvPPA) and semantic (svPPA). The pathophysiology underlying PPA variants is not fully understood, including the role of micro (mi)RNAs which were previously shown to play a role in several neurodegenerative diseases. Using a two-step analysis (array and validation through real-time PCR), we investigated the miRNA expression pattern in serum from 54 PPA patients and 18 controls. In the svPPA cohort, we observed a generalized upregulation of miRNAs with miR-106b-5p and miR-133a-3p reaching statistical significance (miR-106b-5p: 2.69 ± 0.89 mean ± SD vs. 1.18 ± 0.28, p < 0.0001; miR-133a-3p: 2.09 ± 0.10 vs. 0.74 ± 0.11 mean ± SD, p = 0.0002). Conversely, in lvPPA, the majority of miRNAs were downregulated. GO enrichment and KEGG pathway analyses revealed that target genes of both miRNAs are involved in pathways potentially relevant for the pathogenesis of neurodegenerative diseases. This is the first study that investigates the expression profile of circulating miRNAs in PPA variant patients. We identified a specific miRNA expression profile in svPPA that could differentiate this pathological condition from other PPA variants. Nevertheless, these preliminary results need to be confirmed in a larger independent cohort. Full article

The semantic variant of a primary progressive aphasia (svPPA) is characterized by progressive disruption of semantic knowledge. This study aimed to compare the semantic features of words produced during a narrative speech in svPPA and the logopenic variant of PPA (lvPPA) and to explore their neuroanatomical correlates. Six patients with svPPA and sixteen with lvPPA underwent narrative speech tasks. For all the content words, a semantic depth index (SDI) was determined based on the taxonomic structure of a large lexical database. Study participants underwent an MRI examination. Cortical thickness measures were extracted according to the Desikan atlas. Correlations were computed between SDI and the thickness of cortical regions. Mean SDI was lower for svPPA than for lvPPA. Correlation analyses showed a positive association between the SDI and the cortical thickness of the bilateral temporal pole, parahippocampal and entorhinal cortices, and left middle and superior temporal cortices. Disruption of semantic knowledge observed in svPPA leads to the production of generic terms in narrative speech, and the SDI may be useful for quantifying the level of semantic impairment. The measure was associated with the cortical thickness of brain regions associated with semantic memory. Full article

We investigated the survival time of each clinical syndrome of frontotemporal dementia (FTD) and the impacts of behavioral and motor features on survival of FTD. A total of 216 patients with FTD [82 behavioral variant FTD (bvFTD), 78 semantic variant primary progressive aphasia (svPPA), 43 non-fluent/agrammatic variant PPA (nfvPPA), 13 FTD-motor neuron disease (MND)] were enrolled from 16 centers across Korea. Behaviors and parkinsonism were assessed using the Frontal Behavioral Inventory and Unified Parkinson’s Disease Rating Scale Part III, respectively. The Kaplan–Meier method was used for the survival analysis and the Cox proportional hazards model was applied for analysis of the effect of behavioral and motor symptoms on survival, after controlling vascular risk factors and cancer. An overall median survival of FTD was 12.1 years. The survival time from onset was shortest for FTD-MND and longest for svPPA. The median survival time of patients with bvFTD was unavailable but likely comparable to that of patients with nfvPPA. In the bvFTD group, negative behavioral symptoms and akinetic rigidity were significantly associated with survival. In the nfvPPA group, the presence of dysarthria had a negative impact on survival. These findings provide useful information to clinicians planning for care. Full article