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BACKGROUND AND PURPOSE: Dysphagia is an important feature of neurodegenerative diseases and potentially life-threatening in primary progressive aphasia (PPA) but remains poorly characterized in these syndromes. We hypothesized that dysphagia would be more prevalent in nonfluent/agrammatic variant (nfv)PPA than other PPA syndromes, predicted by accompanying motor features, and associated with atrophy affecting regions implicated in swallowing control. METHODS: In a retrospective case-control study at our tertiary referral centre, we recruited 56 patients with PPA (21 nfvPPA, 22 semantic variant [sv]PPA, 13 logopenic variant [lv]PPA). Using a pro forma based on caregiver surveys and clinical records, we documented dysphagia (present/absent) and associated, potentially predictive clinical, cognitive, and behavioural features. These were used to train a machine learning model. Patients' brain magnetic resonance imaging scans were assessed using voxel-based morphometry and region-of-interest analyses comparing differential atrophy profiles associated with dysphagia presence/absence. RESULTS: Dysphagia was significantly more prevalent in nfvPPA (43% vs. 5% svPPA and no lvPPA). The machine learning model revealed a hierarchy of features predicting dysphagia in the nfvPPA group, with excellent classification accuracy (90.5%, 95% confidence interval = 77.9-100); the strongest predictor was orofacial apraxia, followed by older age, parkinsonism, more severe behavioural disturbance, and more severe cognitive impairment. Significant grey matter atrophy correlates of dysphagia in nfvPPA were identified in left middle frontal, right superior frontal, and right supramarginal gyri and right caudate. CONCLUSIONS: Dysphagia is a common feature of nfvPPA, linked to underlying corticosubcortical network dysfunction. Clinicians should anticipate this symptom particularly in the context of other motor features and more severe disease.
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Afasia Primária Progressiva , Transtornos de Deglutição , Imageamento por Ressonância Magnética , Humanos , Transtornos de Deglutição/etiologia , Transtornos de Deglutição/patologia , Afasia Primária Progressiva/patologia , Afasia Primária Progressiva/diagnóstico por imagem , Afasia Primária Progressiva/complicações , Masculino , Feminino , Idoso , Pessoa de Meia-Idade , Estudos Retrospectivos , Estudos de Casos e Controles , Atrofia/patologiaRESUMO
OBJECTIVES: To examine the clinical trajectories and neural correlates of cognitive and emotion processing changes in the non-fluent/agrammatic (nfvPPA) and the logopenic (lvPPA) variants of primary progressive aphasia (PPA). DESIGN: Observational case-control longitudinal cohort study. SETTING: Research clinic of frontotemporal dementia. PARTICIPANTS: This study recruited 29 non-semantic PPA patients (15 nfvPPA and 14 lvPPA) and compared them with 15 Alzheimer's disease (AD) patients and 14 healthy controls. MEASUREMENTS: Participants completed an annual assessment (median = 2 years; range = 1-5 years) of general cognition, emotion processing and structural MRI. Linear mixed effects models investigated clinical and imaging trajectories between groups. RESULTS: Over time, lvPPA showed the greatest cognitive deterioration. In contrast, nfvPPA showed significant decline in emotion recognition, whereas AD showed preserved emotion recognition, even with disease progression. Importantly, lvPPA also developed emotion processing impairments, with disease progression. Both nfvPPA and lvPPA showed continuing cortical atrophy in hallmark language-processing regions associated with these syndromes, together with progressive involvement of the right hemisphere regions, mirroring left hemisphere atrophy patterns at presentation. Decline in emotion processing was associated with bilateral frontal atrophy in nfvPPA and right temporal atrophy in lvPPA. CONCLUSIONS: Our results show divergent clinical courses in nfvPPA and lvPPA, with rapid cognitive and neural deterioration in lvPPA and emotion processing decline in both groups and support the concurrent assessment of cognition and emotion processing in the clinic to inform diagnosis and monitoring in the non-semantic variants of PPA.
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Doença de Alzheimer , Afasia Primária Progressiva , Afasia Primária Progressiva não Fluente , Humanos , Doença de Alzheimer/psicologia , Afasia Primária Progressiva/diagnóstico por imagem , Afasia Primária Progressiva/complicações , Afasia Primária Progressiva/psicologia , Atrofia , Progressão da Doença , Emoções , Estudos Longitudinais , Afasia Primária Progressiva não Fluente/complicações , Estudos de Casos e ControlesRESUMO
The patient was a 66-year-old man brought to the emergency room with impaired consciousness due to hypercarbonemia, managed on a respirator, and diagnosed with amyotrophic lateral sclerosis (ALS). MRI showed atrophy of the anterior and medial surfaces of the bilateral temporal lobes that was more severe in the right side. The patient had dysgraphia in both kana and kanji. Detailed examinations of the language function revealed impaired single-word comprehension, impaired naming, and surface dysgraphia, leading to the diagnosis of semantic variant primary progressive aphasia (svPPA). ALS patients with atrophy of the anterior temporal lobe and surface dysgraphia of kanji may have svPPA as a complication. (Received April 14, 2023; Accepted June 21, 2023; Published October 1, 2023).
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Agrafia , Esclerose Lateral Amiotrófica , Afasia Primária Progressiva , Masculino , Humanos , Idoso , Agrafia/etiologia , Semântica , Esclerose Lateral Amiotrófica/complicações , Idioma , Imageamento por Ressonância Magnética/efeitos adversos , Afasia Primária Progressiva/diagnóstico por imagem , Afasia Primária Progressiva/complicações , Atrofia/complicaçõesRESUMO
Language disorders can occur as a consequence of stroke or neurodegenerative disorders, among other causes. Poststroke aphasia (PSA) and primary progressive aphasia (PPA) are syndromes that, despite having common features, differ in the brain mechanisms that cause their symptoms. These differences in the underlying functional neuroanatomical changes may influence the way they are addressed by different noninvasive brain stimulation techniques and, in particular, by repetitive transcranial magnetic stimulation (rTMS). The aim of this systematic review is to evaluate the efficacy of rTMS in the treatment of PSA and PPA, as well as the differences in the approach to these disorders using rTMS. To this end, a total of 36 articles were found in the Web of Science, Scopus, and PubMed. The results obtained suggest that whereas in PSA, the selection of the stimulation paradigm is based on bihemispheric functional reorganisation models with a tendency towards the application of inhibitory rTMS in the contralateral right hemisphere, in PPA, the application of excitatory rTMS in functionally compromised areas seems to show promising changes. It is concluded that rTMS is a potential treatment in the therapy of both disorders, although differences in the underlying brain mechanisms differentiate the rTMS approach in each case.
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Afasia Primária Progressiva , Afasia , Acidente Vascular Cerebral , Humanos , Estimulação Magnética Transcraniana/métodos , Resultado do Tratamento , Acidente Vascular Cerebral/complicações , Acidente Vascular Cerebral/terapia , Afasia/etiologia , Afasia/terapia , Afasia Primária Progressiva/terapia , Afasia Primária Progressiva/complicaçõesRESUMO
Successful communication in daily life depends on accurate decoding of speech signals that are acoustically degraded by challenging listening conditions. This process presents the brain with a demanding computational task that is vulnerable to neurodegenerative pathologies. However, despite recent intense interest in the link between hearing impairment and dementia, comprehension of acoustically degraded speech in these diseases has been little studied. Here we addressed this issue in a cohort of 19 patients with typical Alzheimer's disease and 30 patients representing the three canonical syndromes of primary progressive aphasia (non-fluent/agrammatic variant primary progressive aphasia; semantic variant primary progressive aphasia; logopenic variant primary progressive aphasia), compared to 25 healthy age-matched controls. As a paradigm for the acoustically degraded speech signals of daily life, we used noise-vocoding: synthetic division of the speech signal into frequency channels constituted from amplitude-modulated white noise, such that fewer channels convey less spectrotemporal detail thereby reducing intelligibility. We investigated the impact of noise-vocoding on recognition of spoken three-digit numbers and used psychometric modelling to ascertain the threshold number of noise-vocoding channels required for 50% intelligibility by each participant. Associations of noise-vocoded speech intelligibility threshold with general demographic, clinical and neuropsychological characteristics and regional grey matter volume (defined by voxel-based morphometry of patients' brain images) were also assessed. Mean noise-vocoded speech intelligibility threshold was significantly higher in all patient groups than healthy controls, and significantly higher in Alzheimer's disease and logopenic variant primary progressive aphasia than semantic variant primary progressive aphasia (all P < 0.05). In a receiver operating characteristic analysis, vocoded intelligibility threshold discriminated Alzheimer's disease, non-fluent variant and logopenic variant primary progressive aphasia patients very well from healthy controls. Further, this central hearing measure correlated with overall disease severity but not with peripheral hearing or clear speech perception. Neuroanatomically, after correcting for multiple voxel-wise comparisons in predefined regions of interest, impaired noise-vocoded speech comprehension across syndromes was significantly associated (P < 0.05) with atrophy of left planum temporale, angular gyrus and anterior cingulate gyrus: a cortical network that has previously been widely implicated in processing degraded speech signals. Our findings suggest that the comprehension of acoustically altered speech captures an auditory brain process relevant to daily hearing and communication in major dementia syndromes, with novel diagnostic and therapeutic implications.
Assuntos
Doença de Alzheimer , Afasia Primária Progressiva , Afasia , Humanos , Doença de Alzheimer/complicações , Doença de Alzheimer/diagnóstico por imagem , Doença de Alzheimer/metabolismo , Compreensão , Fala , Encéfalo/patologia , Afasia/patologia , Afasia Primária Progressiva/complicações , Testes NeuropsicológicosRESUMO
BACKGROUND: The Boston Naming Test (BNT) is the most widely used measure to assess anomia. However, it has been criticized for failing to differentiate the underlying cognitive process of anomia. OBJECTIVE: We validated the color-picture version of BNT (CP-BNT) in a sample with diverse neurodegenerative dementia diseases (NDDs). We also verified the differential ability of the composite indices of CP-BNT across NDDs groups. METHODS: The present study included Alzheimer's disease (nâ=â132), semantic variant primary progressive aphasia (svPPA, nâ=â53), non-svPPA (nâ=â33), posterior cortical atrophy (PCA, nâ=â35), and normal controls (nâ=â110). We evaluated psychometric properties of CP-BNT for the spontaneous naming (SN), the percentage of correct responses on semantic cuing and word recognition cuing (% SC, % WR). Receiver operating characteristic analysis was used to examine the discriminatory power of SN alone and the composite indices (SN, % SC, and % WR). RESULTS: The CP-BNT had sufficient internal consistency, good convergent, divergent validity, and criterion validity. Different indices of CP-BNT demonstrated distinct cognitive underpinnings. Category fluency was the strongest predictor of SN (ß=â0.46, pâ<â0.001). Auditory comprehension tests highly associated with % WR (Sentence comprehension: ß=â0.22, pâ=â0.001; Word comprehension: ß=â0.20, pâ=â0.001), whereas a lower visuospatial score predicted % SC (ß=â-0.2, pâ=â0.001). Composite indices had better predictability than the SN alone when differentiating between NDDs, especially for PCA versus non-svPPA (area under the curve increased from 63.9% to 81.2%). CONCLUSION: The CP-BNT is a highly linguistically relevant test with sufficient reliability and validity. Composite indices could provide more differential information beyond SN and should be used in clinical practice.
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Anomia , Testes de Linguagem , Doenças Neurodegenerativas , Humanos , Anomia/diagnóstico , Anomia/etiologia , População do Leste Asiático , Doenças Neurodegenerativas/complicações , Testes Neuropsicológicos , Reprodutibilidade dos Testes , Afasia Primária Progressiva/complicações , Doença de Alzheimer/complicações , Demência/complicaçõesRESUMO
We report a case of non-fluent/agrammatic variant of primary progressive aphasia in a 79-year-old right-handed man who was admitted with a 5-year history of non-fluent speech and apraxia of speech. He also presented with agrammatism and logoclonia (the meaningless repetition of the middle or final syllable of a word). Furthermore, brain MRI revealed atrophy of the bilateral frontal and temporal lobes, while N-isopropyl-p-123I-iodoamphetamine single-photon emission computed tomography (SPECT) revealed relative hypoperfusion in the right basal ganglia. In addition, dopamine transporter SPECT revealed a decrease in specific binding ratio values, indicating neural dopamine dysfunction, which led to his diagnosis of progressive non-fluent aphasia with logoclonia. Logoclonia is a severe linguistic dysfunction usually observed in the advanced stages of Alzheimer's disease. However, based on the clinical course and cerebrospinal fluid evaluation results, our patient did not show any features of Alzheimer's disease. Thus, logoclonia might be associated with lesions involving the basal ganglia, right hemisphere, and left frontotemporal lobe.
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Doença de Alzheimer , Afasia Primária Progressiva , Masculino , Humanos , Idoso , Tomografia Computadorizada de Emissão de Fóton Único , Neuroimagem , Imageamento por Ressonância Magnética , Afasia Primária Progressiva/diagnóstico por imagem , Afasia Primária Progressiva/complicações , Afasia Primária Progressiva/patologiaRESUMO
Semantic variant primary progressive aphasia (svPPA) is a neurodegenerative disorder characterized by a loss of semantic knowledge in the context of anterior temporal lobe atrophy (left > right). Core features of svPPA include anomia and single-word comprehension impairment. Despite growing evidence supporting treatment for anomia in svPPA, there is a paucity of research investigating neural mechanisms supporting treatment-induced gains and generalization to untrained items. In the current study, we examined the relation between the structural integrity of brain parenchyma (tissue inclusive of gray and white matter) at pre-treatment and treatment outcomes for trained and untrained items in a group of 19 individuals with svPPA who completed lexical retrieval treatment. Two structural neuroimaging approaches were used: an exploratory, whole-brain, voxel-wise approach and an a priori region of interest (ROI) approach. Based on previous research, bilateral temporal (inferior, middle, and superior temporal gyri), parietal (supramarginal and angular gyri), frontal (inferior and middle frontal gyri) and medial temporal (hippocampus and parahippocampal gyri) ROIs were selected from the Automated Anatomical Labeling (AAL) atlas. Analyses revealed improved naming of trained items and generalization to untrained items following treatment, providing converging evidence that individuals with svPPA can benefit from treatment for anomia. Better post-treatment naming accuracy was associated with the structural integrity of inferior parietal cortex and the hippocampus. Specifically, improved naming of trained items was related to the left supramarginal (phonological processing) and angular gyri (phonological and semantic processing), and improved naming of trained and untrained items was related to the left hippocampus (episodic, context-based memory). Future research should examine treatment outcomes in relation to pre-treatment functional and structural connectivity as well as changes in network dynamics following speech-language intervention to further elucidate the neural mechanisms underlying treatment response in svPPA and related disorders.
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Afasia Primária Progressiva , Semântica , Humanos , Afasia Primária Progressiva/diagnóstico por imagem , Afasia Primária Progressiva/terapia , Afasia Primária Progressiva/complicações , Anomia/diagnóstico por imagem , Anomia/terapia , Imageamento por Ressonância Magnética/métodos , Resultado do TratamentoRESUMO
INTRODUCTION: Three Words-Three Shapes (3W3S) is a bedside test that assesses verbal and non-verbal memory and has proven useful in staging memory decline in amnestic disorders and primary progressive aphasia. Given its simple structure, the 3W3S can be easily adapted to other languages maintaining the original shapes and only modifying the words. We aim to validate a Spanish version of the 3W3S test and establish whether memory loss patterns present in amnesic disorders associated with Alzheimer's etiology and PPA were correctly characterized. METHOD: The translation and adaptation of the 3W3S were performed according to standardized guidelines and applied to a cohort of patients with Dementia of Alzheimer's type (DAT = 20), mild cognitive impairment (aMCI= 20), primary progressive aphasia (PPA = 20), and healthy controls (HC = 20). RESULTS: In verbal memory performance, PPA patients' score was lower than that of MCI and HC and similar to DAT's in the effortless encoding (p < 0.001), delayed recall (p < 0.001), and recognition (p < 0.012). For non-verbal performance, PPA patients performed better than DAT and similar to HC and MCI subjects (p < 0.001). CONCLUSIONS: Results show good applicability of 3W3S to determine memory function in PPA patients, independently from language ability. Visual and verbal components of memory are dissociated in PPA.
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Doença de Alzheimer , Afasia Primária Progressiva , Humanos , Doença de Alzheimer/complicações , Doença de Alzheimer/diagnóstico , Doença de Alzheimer/psicologia , Afasia Primária Progressiva/complicações , Afasia Primária Progressiva/diagnóstico , Afasia Primária Progressiva/psicologia , Testes Neuropsicológicos , Transtornos da Memória/etiologia , Transtornos da Memória/complicações , IdiomaRESUMO
Primary progressive aphasia (PPA) classification relies on profile characterization of quantitatively impaired/spared performance in language tasks. In this study, we coextracted 8 qualitative types of errors in 67 PPA patients submitted to a comprehensive language assessment. Canonical correlation analysis was applied to simultaneously correlate qualitative errors and brain metabolism, collected with FDG-PET. Results showed the contribution of semantic, syntactic and working memory errors associated with specific correlates of regional metabolic changes. Reduced metabolism in the left fusiform gyrus, anterior-middle and inferior-temporal gyri and middle-temporal pole correlated with an increase of semantic errors. Hypometabolism in the left inferior, middle, and superior frontal gyri, insula and right middle-occipital gyrus was related to syntactic errors. Higher metabolism in the bilateral pallidum, putamen, and left thalamus, as well as hypometabolism in the left angular and supramarginal gyri, inferior-parietal lobule, posterior-middle and inferior-temporal gyri and posterior cingulum predicted the increase of working memory errors. A relevant role of working memory subcomponents was associated with distinct neural systems. Patients' profiles are easily represented in a qualitative multidimensional space, in which mixed PPA overlapped with different phenotypes.
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Afasia Primária Progressiva , Humanos , Afasia Primária Progressiva/diagnóstico por imagem , Afasia Primária Progressiva/complicações , Fluordesoxiglucose F18 , Tomografia por Emissão de Pósitrons/métodos , Idioma , Semântica , Imageamento por Ressonância Magnética/métodos , Encéfalo/diagnóstico por imagemRESUMO
Progressive apraxia of speech (AOS) is a motor speech disorder affecting the ability to produce phonetically or prosodically normal speech. Progressive AOS can present in isolation or co-occur with agrammatic aphasia and is associated with degeneration of the supplementary motor area. We aimed to assess breakdowns in structural connectivity from the supplementary motor area in patients with any combination of progressive AOS and/or agrammatic aphasia to determine which supplementary motor area tracts are specifically related to these clinical symptoms. Eighty-four patients with progressive AOS or progressive agrammatic aphasia were recruited by the Neurodegenerative Research Group and underwent neurological, speech/language, and neuropsychological testing, as well as 3 T diffusion magnetic resonance imaging. Of the 84 patients, 36 had apraxia of speech in isolation (primary progressive apraxia of speech, PPAOS), 40 had apraxia of speech and agrammatic aphasia (AOS-PAA), and eight had agrammatic aphasia in isolation (progressive agrammatic aphasia, PAA). Tractography was performed to identify 5 distinct tracts connecting to the supplementary motor area. Fractional anisotropy and mean diffusivity were assessed at 10 positions along the length of the tracts to construct tract profiles, and median profiles were calculated for each tract. In a case-control comparison, decreased fractional anisotropy and increased mean diffusivity were observed along the supplementary motor area commissural fibers in all three groups compared to controls. PPAOS also had abnormal diffusion in tracts from the supplementary motor area to the putamen, prefrontal cortex, Broca's area (frontal aslant tract) and motor cortex, with greatest abnormalities observed closest to the supplementary motor area. The AOS-PAA group showed abnormalities in the same set of tracts, but with greater involvement of the supplementary motor area to prefrontal tract compared to PPAOS. PAA showed abnormalities in the left prefrontal and frontal aslant tracts compared to both other groups, with PAA showing greatest abnormalities furthest from the supplementary motor area. Severity of AOS correlated with tract metrics in the supplementary motor area commissural and motor cortex tracts. Severity of aphasia correlated with the frontal aslant and prefrontal tracts. These findings provide insight into how AOS and agrammatism are differentially related to disrupted diffusivity, with progressive AOS associated with abnormalities close to the supplementary motor area, and the frontal aslant and prefrontal tracts being particularly associated with agrammatic aphasia.
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Afasia Primária Progressiva , Afasia , Apraxias , Córtex Motor , Afasia/complicações , Afasia Primária Progressiva/complicações , Apraxias/diagnóstico por imagem , Humanos , Córtex Motor/diagnóstico por imagem , Córtex Motor/patologia , Testes Neuropsicológicos , FalaRESUMO
BACKGROUND: Although language impairment is the most salient feature of cognitive impairment in both primary progressive aphasia (PPA) and stroke aphasia (SA), memory can also be impaired in both patient populations. OBJECTIVE: To identify distinctive features of verbal and nonverbal memory processing in individuals with PPA and those with SA. METHOD: We gave individuals with PPA (n = 14), those with SA (n = 8), and healthy controls (HC; n = 13) a comprehensive neuropsychological test battery and the Turkish version of the Three Words Three Shapes Test (3W3S-Turkish). The 3W3S-Turkish Test includes five subtests: Copy, Incidental Recall, Acquisition, Delayed Recall, and Recognition. High-resolution brain scans were performed in a subset of individuals with PPA and those with SA. Lesion distribution was limited to the dorsal language areas in the SA group, whereas peak atrophy areas in the PPA group extended beyond the language network, including the medial temporal lobe, precuneus, and posterior/medial portions of the cingulate cortex. RESULTS: Both the PPA and SA groups showed impairment in incidental recall, and the PPA group showed additional impairment in delayed recall. Greater impairment for verbal stimuli suggestive of material-specific memory impairment was evident in the PPA group's scores on the Incidental Recall and Delayed Recall subtests. Both aphasia groups retained the acquired information regardless of material type. CONCLUSION: Although both aphasia groups shared similarities in the involvement of the dorsal prefrontal working memory/attention network, the PPA group showed greater impairment in delayed recall compared with the SA group.
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Afasia Primária Progressiva , Afasia , Acidente Vascular Cerebral , Afasia Primária Progressiva/complicações , Afasia Primária Progressiva/patologia , Humanos , Transtornos da Memória/complicações , Testes Neuropsicológicos , Acidente Vascular Cerebral/complicaçõesRESUMO
Peripheral errors in writing, that is errors produced download the spelling, have been occasionally described in primary progressive aphasia (PPA), but the possibility that these errors might be a marker of parkinsonism associated to some subtypes of PPA has not been explored. We investigated whether errors of peripheral nature characterize the writing disorder in PPA when associated with parkinsonian signs (PSs). Subgroups of PPA without PSs and with PSs were studied. The proportion of the central and peripheral errors in writing words and pseudowords was calculated in each group. In writing words, central errors significantly exceeded peripheral errors in subgroups without PSs. The higher the number of peripheral errors, the higher the probability of presenting PSs. No relation emerged between any error and the Unified Parkinson's Disease Rating Scale, but both types of errors correlated with measures of cognitive ability. Peripheral errors emerge when PSs are associated with PPA and may be linked to a decay of the cognitive control on movement, possibly involving the right hemisphere. Peripheral errors have clinical relevance in PPA, to the extent that they may assume the significance of a marker of specific subtypes and can help to outline the specific clinical picture of individual patients.
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Afasia Primária Progressiva , Afasia Primária Progressiva/complicações , Afasia Primária Progressiva/psicologia , Humanos , RedaçãoRESUMO
BACKGROUND: The role of semantic knowledge in emotion recognition remains poorly understood. The semantic variant of primary progressive aphasia (svPPA) is a degenerative disorder characterized by progressive loss of semantic knowledge, while other cognitive abilities remain spared, at least in the early stages of the disease. The syndrome is therefore a reliable clinical model of semantic impairment allowing for testing the propositions made in theoretical models of emotion recognition. OBJECTIVE: The main goal of this study was to investigate the role of semantic memory in the recognition of basic emotions conveyed by music in individuals with svPPA. METHODS: The performance of 9 individuals with svPPA was compared to that of 32 control participants in tasks designed to investigate the ability: a) to differentiate between familiar and non-familiar musical excerpts, b) to associate semantic concepts to musical excerpts, and c) to recognize basic emotions conveyed by music. RESULTS: Results revealed that individuals with svPPA showed preserved abilities to recognize familiar musical excerpts but impaired performance on the two other tasks. Moreover, recognition of basic emotions and association of musical excerpts with semantic concepts was significantly better for familiar than non-familiar musical excerpts in participants with svPPA. CONCLUSION: Results of this study have important implications for theoretical models of emotion recognition and music processing. They suggest that impairment of semantic memory in svPPA affects both the activation of emotions and factual knowledge from music and that this impairment is modulated by familiarity with musical tunes.
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Afasia Primária Progressiva/complicações , Emoções/fisiologia , Transtornos da Memória/etiologia , Música , Semântica , Idoso , Afasia Primária Progressiva/diagnóstico por imagem , Atrofia/etiologia , Atrofia/patologia , Estudos de Casos e Controles , Feminino , Humanos , Conhecimento , Testes de Linguagem , Masculino , Entrevista Psiquiátrica Padronizada , Pessoa de Meia-Idade , Modelos Teóricos , Reconhecimento PsicológicoRESUMO
OBJECTIVE: The aims of this study are to describe the chronology of functional disabilities in primary progressive aphasia (PPA), and to examine associations between psychiatric comorbidities and functional disabilities. METHODS: We conducted a retrospective data analysis using subjects enrolled at Alzheimer's Disease Research Centers between 2005 and 2019. Data were obtained from the National Alzheimer's Coordinating Center database. We included subjects whose primary diagnosis was PPA. Functional status was coded as a binary variable for the following functions: ambulation, transaction skills, verbal communication, meal preparation, and self-care. Behavioral data derived from the Neuropsychiatric Inventory Questionnaire. Descriptive statistics and cox proportional hazard analyses were used to characterize the emergence of disabilities and their association with psychiatric comorbidities. RESULTS: Data included 91 subjects with a clinical dementia rating scale of zero at baseline. At the initial visit, no individuals had impairments in self-care, while 7% had impairments in transactions, 3% in ambulation, and 2% in meal preparation. Ninety-three percent had language impairments at the onset of the study, and all by visit 4. By visit 5, 41% of patients had impairments in ambulation and in self-care, 49% were impaired in meal preparation and 70% had impairment in transactions. The presence of anxiety, depression, sleep disturbance and psychosis were all significantly associated with an increased risk for multiple functional disabilities. CONCLUSION: These findings provide clinicians with guidance for forecasting disabilities and targeting interventions in PPA.
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Doença de Alzheimer , Afasia Primária Progressiva , Doença de Alzheimer/complicações , Afasia Primária Progressiva/complicações , Afasia Primária Progressiva/diagnóstico , Afasia Primária Progressiva/epidemiologia , Humanos , Testes Neuropsicológicos , Estudos RetrospectivosRESUMO
OBJECTIVE: To characterize and compare the neuropsychological profiles of patients with primary progressive apraxia of speech (PPAOS) and apraxia of speech with progressive agrammatic aphasia (AOS-PAA). METHOD: Thirty-nine patients with PPAOS and 49 patients with AOS-PAA underwent formal neurological, speech, language, and neuropsychological evaluations. Cognitive domains assessed included immediate and delayed episodic memory (Wechsler Memory Scale-Third edition; Logical Memory; Visual Reproduction; Rey Auditory Verbal Learning Test), processing speed (Trail Making Test A), executive functioning (Trail Making Test B; Delis-Kaplan Executive Functioning Scale - Sorting), and visuospatial ability (Rey-Osterrieth Complex Figure copy). RESULTS: The PPAOS patients were cognitively average or higher in the domains of immediate and delayed episodic memory, processing speed, executive functioning, and visuospatial ability. Patients with AOS-PAA performed more poorly on tests of immediate and delayed episodic memory and executive functioning compared to those with PPAOS. For every 1 unit increase in aphasia severity (e.g. mild to moderate), performance declined by 1/3 to 1/2 a standard deviation depending on cognitive domain. The degree of decline was stronger within the more verbally mediated domains, but was also notable in less verbally mediated domains. CONCLUSION: The study provides neuropsychological evidence further supporting the distinction of PPAOS from primary progressive aphasia and should be used to inform future diagnostic criteria. More immediately, it informs prognostication and treatment planning.
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Afasia Primária Progressiva , Afasia , Apraxias , Afasia Primária Progressiva/complicações , Afasia Primária Progressiva/diagnóstico , Apraxias/etiologia , Humanos , Idioma , Testes Neuropsicológicos , FalaRESUMO
Speech-language pathology caseloads often include individuals with hearing loss and a coexisting neurogenic communication disorder. However, specific treatment techniques and modifications designed to accommodate this population are understudied. Using a single-case experimental design, the current study investigated the utility of modified Video Implemented Script Training for Aphasia (VISTA) for an individual with nonfluent/agrammatic variant primary progressive aphasia and severe-to-profound, bilateral hearing loss. We analyzed the impact of this intervention, which incorporates orthographic input and rehearsal, on script production accuracy, speech intelligibility, grammatical complexity, mean length of utterance, and speech rate. Treatment resulted in comparable positive outcomes relative to a previous study evaluating script training in nonfluent/agrammatic primary progressive aphasia patients with functional hearing. Follow-up data obtained at three months, six months, and one year post-treatment confirmed maintenance of treatment effects for trained scripts. To our knowledge, this is the first study to investigate a modified speech-language intervention tailored to the needs of an individual with PPA and hearing loss, with findings confirming that simple treatment modifications may serve to broaden the range of treatment options available to those with concomitant sensory and communication impairments.
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Afasia Primária Progressiva , Afasia , Perda Auditiva , Afasia Primária Progressiva/complicações , Perda Auditiva/complicações , Humanos , Projetos de Pesquisa , FalaRESUMO
We report four cases depicting the heterogeneity of Alzheimer's disease (AD) associated with pure AD pathology. Case 1 was a 77-year-old man with a false positive diagnosis of dementia with Lewy bodies with reduced dopamine transporter uptake activity of the striatum but no Lewy body pathology. There were tau deposits in the large neurons in the putamen, which may be related to the development of parkinsonism. Case 2 was an AD patient in his early 30s who presented with a psychotic episode and a cognitive decline, and later developed myoclonus and seizures. He demonstrated considerable amyloid-beta deposits in the cerebral cortex, including cotton wool plaques, basal ganglia, and cerebellum. Tau deposits were also abundant in the cerebral neocortex, hippocampus, basal ganglia, and brain stem. Case 3 was a 60-year-old woman who exhibited typical symptoms characteristic of the logopenic variant of primary progressive aphasia (lvPPA). Case 4 was a 68-year-old man who exhibited the semantic variant of primary progressive aphasia (svPPA) plus repetition impairment, a rare case associated with AD pathology. In addition to tau pathology, astrocytic pathology was prominent in the white matter and cortical layers of the left temporoparietal cortices. While the main AD lesion in case 4 was evaluated by tau accumulation and astrogliosis in the left temporal lobe, that in case 3 in was evaluated by the same points in the left parietal lobe. Within the spectrum of lvPPA, case 4 may be regarded as a temporal variant of lvPPA presenting svPPA. The pathology of PPA associated with AD may have broader clinical manifestations than that in previously described cases. Case 4 also showed pathological features characteristic of cerebral amyloid angiopathy throughout the cerebral cortex. The distribution of tau and astrocytic pathologies in the cerebral cortex, basal ganglia, brain stem, and cerebellum may explain the various symptoms of atypical pure AD patients.
Assuntos
Doença de Alzheimer , Afasia Primária Progressiva , Disfunção Cognitiva , Doença por Corpos de Lewy , Idoso , Doença de Alzheimer/complicações , Afasia Primária Progressiva/complicações , Feminino , Humanos , Doença por Corpos de Lewy/complicações , Masculino , Pessoa de Meia-Idade , Placa AmiloideRESUMO
Transactive response DNA-binding protein 43 kDa (TDP-43) is aberrantly aggregated and phosphorylated in frontotemporal lobar degeneration of the TDP-43 type (FTLD-TDP), and in limbic-predominant age-related TDP-43 encephalopathy neuropathologic change (LATE-NC). We examined data from the National Alzheimer's Coordinating Center to compare clinical features of autopsy-confirmed LATE-NC and FTLD-TDP. A total of 265 LATE-NC and 92 FTLD-TDP participants were included. Cognitive and behavioral symptoms were compared, stratified by level of impairment based on global clinical dementia rating (CDR) score. LATE-NC participants were older at death, more likely to carry APOE ε4, more likely to have Alzheimer disease neuropathology, and had lower (i.e. less severe) final CDR global scores than those with FTLD-TDP. Participants with FTLD-TDP were more likely to present with primary progressive aphasia, or behavior problems such as apathy, disinhibition, and personality changes. Among participants with final CDR score of 2-3, those with LATE-NC were more likely to have visuospatial impairment, delusions, and/or visual hallucinations. These differences were robust after sensitivity analyses excluding older (≥80 years at death), LATE-NC stage 3, or severe Alzheimer cases. Overall, FTLD-TDP was more globally severe, and affected younger participants, whereas psychoses were more common in LATE-NC.
Assuntos
Cognição , Degeneração Lobar Frontotemporal/diagnóstico , Degeneração Lobar Frontotemporal/psicologia , Sistema Límbico/patologia , Proteinopatias TDP-43/diagnóstico , Proteinopatias TDP-43/psicologia , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Doença de Alzheimer/patologia , Afasia Primária Progressiva/complicações , Afasia Primária Progressiva/patologia , Apolipoproteína E4/genética , Delusões/etiologia , Delusões/psicologia , Feminino , Degeneração Lobar Frontotemporal/genética , Alucinações/etiologia , Alucinações/psicologia , Heterozigoto , Humanos , Masculino , Pessoa de Meia-Idade , Emaranhados Neurofibrilares/patologia , Testes Neuropsicológicos , Desempenho Psicomotor , Proteinopatias TDP-43/genéticaRESUMO
BACKGROUND: Alzheimer's disease is a neurodegenerative disease involving the deposition of pathologic amyloid-ß and tau protein in the cerebral cortex. Alzheimer's disease is commonly characterized by progressive impairment of recent memory. Primary progressive aphasia is also often observed in patients with Alzheimer's disease. Moreover, language-associated symptoms, such as primary progressive aphasia, are diverse and varied in Alzheimer's disease. However, nonfluent/agrammatic variant primary progressive aphasia is not generally considered a symptom of Alzheimer's disease. To date, there has been no longitudinal study of primary progressive aphasia in Japanese-speaking patients or in patients speaking other languages with pathologically diagnosed Alzheimer's disease. Here we present a longitudinal study of primary progressive aphasia in a Japanese patient pathologically diagnosed with Alzheimer's disease. CASE PRESENTATION: A 75-year-old Japanese man, whose wife reported that his memory was impaired, also suffered from suspected aphasia. He was pathologically diagnosed with Alzheimer's disease using 11C-Pittsburgh compound-B positron emission tomography and 18F-THK5351 positron emission tomography. Based on clinical observation and the results of the Japanese standard language test of aphasia, he was also diagnosed with nonfluent/agrammatic variant primary progressive aphasia. During the subsequent 2 years, his cognitive impairment, aphasia, and behavioral and psychological symptoms of dementia progressed. Furthermore, progression of pathologic amyloid-ß and tau protein deposition was revealed through 11C-Pittsburgh compound-B positron emission tomography and 18F-THK5351 positron emission tomography. Although the results of [123I] iodoamphetamine single-photon emission computed tomography suggested corticobasal degeneration, this was not observed on the [123I] FP-CIT single-photon emission computed tomography (SPECT) (DaTscan). A previous study had reported that Alzheimer's disease with a nonfluent/agrammatic variant primary progressive aphasia was accompanied by corticobasal degeneration; however, this was not true in our case. CONCLUSIONS: This is possibly the first longitudinal study of nonfluent/agrammatic variant primary progressive aphasia in a Japanese-speaking patient with pathologically diagnosed Alzheimer's disease, but without corticobasal degeneration.