RESUMO

BACKGROUND: Speech and language therapists (SLTs) play an important role in assessing and rehabilitating communication disorders in people with dementia, but there is evidence to suggest that they do not receive appropriate training to provide management and support during their training. AIM: To investigate the level of awareness and knowledge that practising SLTs from Brazil have about dementia and their role in the care of dementia through an online survey. METHODS & PROCEDURES: An online survey tool was developed to collect information from practising Brazilian SLTs regarding their knowledge about dementia, awareness about their role in the care of people with dementia, and opinions on how SLTs may be better prepared to work in the dementia field. The survey was disseminated via social media, websites, and e-mail lists of researchers and stakeholders. OUTCOMES & RESULTS: A total of 227 SLTs completed the survey. Participants showed good knowledge of dementia in general, while their answers were less accurate on primary progressive aphasia. Regarding the awareness by SLTs of their role in the care of people with dementia, most agreed or strongly agreed that SLTs could help people in the diagnosis, treatment and prevention of dementia (> 80%). However, fewer participants agreed or strongly agreed that they felt confident in contributing to the treatment and diagnosis process of dementia (about 50%). To improve the training of SLTs in Brazil, most participants believed that it would be necessary to improve the teaching of dementia at the undergraduate speech and language therapy curriculum level and to develop recommendations or guidelines about speech and language therapy practice in dementia. CONCLUSIONS & IMPLICATIONS: The results of this survey point to a need for improvement in the knowledge and confidence of Brazilian SLTs about dementia. To reach this goal, targeted training courses and applied practice opportunities should be embedded within university curricula and training programmes. WHAT THIS PAPER ADDS: What is already known on the subject Many studies confirm the importance of speech and language therapy in the non-pharmacological treatment of people with dementia. However, other evidence suggests to a possible lack of training for Brazilian SLTs, especially in the curriculum of undergraduate courses. What this paper adds to existing knowledge This study reveals that Brazilian SLTs have substantial knowledge of dementia and recognize the significance of their role in treating people with dementia. However, a minority expressed confidence in their ability to assess and treat people with dementia. What are the potential or actual clinical implications of this work? The findings of this research demonstrate that Brazilian SLTs have good knowledge of dementia and endorse their professional role in dementia care; however, they lack confidence in their own skills and expertise in diagnostic assessment and treatment of dementia. Interventions aimed at boosting the SLT's confidence level could lead to improved patients outcomes and overall quality of care within clinical settings.

RESUMO

RESUMEN INTRODUCCIÓN: Las demencias son un conjunto de trastornos neurocognitivos, en personas con edad menor a 65 años sobresale la demencia frontotemporal, síndrome neurodegenerativo heterogéneo que tiene dos grandes variantes: conductual y afasia primaria progresiva. En esta última se describen tres variantes: no fluente, semántica y logopénica, que exigen en la práctica conocimientos actualizados para su diferenciación y comprensión. El objetivo de este escrito es hacer una revisión narrativa sobre las tres variantes clínicas de la afasia primaria progresiva, profundizando en diagnóstico, evolución, características imagenológicas y manejo. MATERIALES Y MÉTODOS: Artículo de revisión narrativa a partir del estado del arte en literatura biomédica sobre demencia frontotemporal, afasia primaria progresiva y sus variantes. RESULTADOS: El compromiso del lenguaje y de otras funciones cognitivas, así como los hallazgos imagenológicos, son heterogéneos en las tres variantes. Semiológicamente, la afasia primaria progresiva no fluente se caracteriza por apraxia del habla, la variante logopénica por fallas en la nominación y la variante semántica por fallas en el significado del mensaje. El compromiso imagenológico en la afasia primaria progresiva no fluente es más frontoinsular y corticosubcortical; en la variante semántica es habitualmente temporal del lado dominante; y en la variante logopénica priman alteraciones temporoparietales. No hay tratamiento específico, pero se puede vincular algunas opciones farmacológicas con procesos/técnicas de rehabilitación del lenguaje. CONCLUSIÓN: Si bien se trata de una forma heterogénea de demencia, tiene características clínicas (síntomas, signos y evolución) e imagenológicas importantes a la hora de su detección y diagnóstico en ambientes clínicos.

ABSTRACT INTRODUCTION: Dementias are a group of neurocognitive disorders, and in people under 65 years of age, frontotemporal dementia stands out, a heterogeneous neurodegenerative syndrome that has two major variants: behavioral and primary progressive aphasia. In the latter, three variants are described: non-fluent, semantic and logopenic, which require up-to-date knowledge in practice for their differentiation and understanding. The objective is to carry out a narrative review on the three clinical variants of primary progressive aphasia, delving into diagnosis, evolution, imaging characteristics and management. MATERIALS AND METHODS: Narrative review article based on the state of the art in biomedical literature on frontotemporal dementia, primary progressive aphasia and its variants. RESULTS: The compromise of language and other cognitive functions, as well as the imaging findings, are heterogeneous in the three variants. Semiologically, non-fluent progressive primary aphasia is characterized by apraxia of speech, the logopenic variant by failures in the nomination and the semantic variant by failures in the meaning of the message. Imaging involvement in non-fluent progressive primary aphasia is mainly frontoinsular and cortico-subcortical; in the semantic variant it is usually temporary on the dominant side; and in the logopenic variant, temporo-parietal alterations prevail. There is no specific treatment, but some pharmacological options can be linked with language rehabilitation processes / techniques. CONCLUSION: although Frontotemporal dementia is an heterogenous disorder, there are important clinical and imagenologic features that are useful to the diagnostic approach in the clinical field.

Assuntos

RESUMO

Sentence-comprehension deficits have been described in patients with primary progressive aphasia (PPA). However, most instruments to address this domain in more detail and in a clinical context have not been adapted and translated into several languages, posing limitations to clinical practice and cross-language research. Objectives: The study aimed to (1) test the applicability of the Brazilian version of the Test for Reception of Grammar (TROG2-Br) to detect morphosyntactic deficits in patients with PPA; (2) investigate the association between performance in the test and sociodemographic and clinical variables (age, years of formal education, and disease duration); (3) characterize the performance of individuals presenting with the three more common variants of PPA (non-fluent, semantic, and logopenic) and mixed PPA (PPA-Mx) and analyze whether TROG-2 may assist in the distinction of these clinical profiles. Methods: A total of 74 cognitively healthy participants and 34 individuals diagnosed with PPA were assessed with TROG2-Br. Overall scores (correct items, passed blocks), types, and categories of errors were analyzed. Results: In controls, block scores were significantly correlated with years of formal education (Spearman's r = 0.33, p = 004) but not with age. In PPA, age, education, and disease duration were not significantly associated with performance in the test. Controls presented a significantly higher performance on TROG2-Br compared to PPA individuals and their errors pattern pointed to mild general cognitive processing difficulties (attention, working memory). PPA error types pointed to processing and morphosyntactic deficits in nonfluent or agrammatic PPA, (PPA-NF/A), logopenic PPA (PPA-L), and PPA-Mx. The semantic PPA (PPA-S) subgroup was qualitatively more similar to controls (processing difficulties and lower percentage of morphosyntactic errors). TROG2-Br presented good internal consistency and concurrent validity. Discussion: Our results corroborate findings with TROG-2 in other populations. The performance of typical older adults with heterogeneous levels of education is discussed along with recommendations for clinical use of the test and future directions of research.

RESUMO

Primary Progressive Aphasia (PPA) is a neurological syndrome characterized by impaired language due to neurodegeneration. It is subdivided into three variants: semantic, agrammatic or nonfluent, and logopenic. Pieces of evidence have suggested that learning disabilities in childhood, such as dyslexia, might be susceptibility factors in the occurrence of PPA in adulthood. The objective of this study was to verify the existence of the relationship between PPA and the history of learning disabilities of patients and their children, compared to a control group of individuals with Alzheimer's disease (AD). A questionnaire was applied to investigate the presence of indicators of learning disabilities and difficulties in individuals with PPA and AD and their children. Twenty subjects with PPA and 16 with AD participated in the study. Our findings are presented and discussed in light of the current scientific evidence and the social, educational, and economic Brazilian scenario. Despite the challenges of doing research with individuals with PPA in Brazil, we present the first evidence about the investigation of association between the history of learning disabilities and difficulties and PPA in native Brazilian Portuguese speakers.

RESUMO

BACKGROUND: Several subtypes of primary progressive aphasia (PPA) have been proposed. Most reports use small samples, and few have included Spanish-speaking participants. AIM: To analyze the language profile and nonlinguistic deficits in a large sample of PPA Spanish monolingual participants. METHOD: 177 individuals were diagnosed with PPA in a sample consisting of 69 men and 108 women (Mage = 66.40 years, SD = 9.30). The participants were assessed using the Spanish versions of the Western Aphasia Battery Revised (SWAB-R) and the Boston Diagnostic Aphasia Examination (SBDAE). Non-verbal reasoning was evaluated with the Raven's Colored Progressive Matrices. RESULTS: 41.8% of the sample met the criteria for the logopenic variant (lvPPA), while 28.2% met the criteria for semantic (svPPA), 15.3% for lexical (lxvPPA), and 14.7% for nonfluent/agrammatic (nfvPPA) variants. Language difficulties were similar in all variants except for lxvPPA. Scores on Spontaneous Language, Auditory Comprehension, Repetition, and Naming were significantly higher for the lxvPPA group. Raven's Colored Progressive Matrices scores were significantly lower in lvPPA. Years of education correlated with all test scores, while age was negatively associated with naming. When the PPA variants were classified according to the traditional aphasia classification, discrepancies were evident. Furthermore, the most frequent type of aphasia was Amnesic, while the least frequent was Wernicke's aphasia. CONCLUSION: The SWAB-R is useful in describing the clinical characteristics of aphasia for each variant of PPA, but quantitative scores from this battery are not capable of distinguishing between variants of PPA, with the exception of lxvPPA.

Assuntos

RESUMO

BACKGROUND: The relatively isolated atrophy of the temporal lobes leads to a clinical radiological pattern, referred to as the temporal variant of frontotemporal dementia. While semantic dementia and behavioral variant frontotemporal dementia are classically related to this syndrome, the logopenic variant of primary progressive aphasia has been less commonly reported. This case report aims to give a pictorial description of a case in which a patient with asymmetric temporal lobe atrophy presented with the logopenic variant of primary progressive aphasia and complex rituals of cleanliness. CASE PRESENTATION: We report on the case of a 68-year-old, right-handed White woman with complex rituals and progressive speech impairment. The obsessive-compulsive rituals represented an exacerbation of lifelong preoccupations with cleanliness and orderliness that were praised by her relatives. Neuropsychological assessment revealed a striking impairment of language and memory, with relative sparing of tool-use praxis and visuospatial skills. Magnetic resonance imaging and 18fluorodeoxyglucose-positron emission tomography scans showed bilateral asymmetrical temporal lobe atrophy and hypometabolism. A year later, she was still able to entertain conversation for a short while, but her vocabulary and fluency had further declined. Praxis and visuospatial skills remained intact. She did not experience pathological elation, delusions, or hallucinations. The disease followed a relentless progression into a partial Klüver-Bucy syndrome, abulia, and terminal dementia. She died from acute myocardial infarction 8 years after the onset of aphasia. The symptoms and their temporal course supported a diagnosis of logopenic variant of primary progressive aphasia due to asymmetric temporal variant frontotemporal lobar degeneration. CONCLUSIONS: This report gives a pictorial description of a temporal variant of frontotemporal dementia in a patient who presented with worsening of a lifelong obsessive-compulsive disorder and logopenic variant of primary progressive aphasia.

Assuntos

RESUMO

Frontotemporal lobar degeneration describes a group of progressive brain disorders that primarily are associated with atrophy of the prefrontal and anterior temporal lobes. Frontotemporal lobar degeneration is considered to be equivalent to frontotemporal dementia. Frontotemporal dementia is characterized by progressive impairments in behavior, executive function, and language. There are two main clinical subtypes: behavioral-variant frontotemporal dementia and primary progressive aphasia. The early diagnosis of frontotemporal dementia is critical for developing management strategies and interventions for these patients. Without validated biomarkers, the clinical diagnosis depends on recognizing all the core or necessary neuropsychiatric features, but misdiagnosis often occurs due to overlap with a range of neurologic and psychiatric disorders. In the studies reviewed a very large number of microRNAs were found to be dysregulated but with limited overlap between individual studies. Measurement of specific miRNAs singly or in combination, or as miRNA pairs (as a ratio) in blood plasma, serum, or cerebrospinal fluid enabled frontotemporal dementia to be discriminated from healthy controls, Alzheimer's disease, and amyotrophic lateral sclerosis. Furthermore, upregulation of miR-223-3p and downregulation of miR-15a-5p, which occurred both in blood serum and cerebrospinal fluid, distinguished behavioral-variant frontotemporal dementia from healthy controls. Downregulation of miR-132-3p in frontal and temporal cortical tissue distinguished frontotemporal lobar degeneration and frontotemporal dementia, respectively, from healthy controls. Possible strong miRNA biofluid biomarker contenders for behavioral-variant frontotemporal dementia are miR-223-3p, miR-15a-5p, miR-22-3p in blood serum and cerebrospinal fluid, and miR-124 in cerebrospinal fluid. No miRNAs were identified able to distinguish between behavioral-variant frontotemporal dementia and primary progressive aphasia subtypes. Further studies are warranted on investigating miRNA expression in biofluids and frontal/temporal cortical tissue to validate and extend these findings.

RESUMO

BACKGROUND: Practice-based evidence can inform and support clinical decision making. Case-report series about the implementation of programs in real-world clinical settings may contribute to verifying the effectiveness of interventions for treating PPA in specific contexts, as well as illustrating challenges that need to be overcome. OBJECTIVE: To describe and provide practice-based evidence on the effectiveness of four cognitive rehabilitation programs designed for individuals with PPA and directed to speech and language impairments, which were implemented in a specialized outpatient clinic. METHODS: Multiple single-case study. Eighteen individuals with different subtypes of PPA were each assigned to one out of four training programs based on comprehensive speech and language assessments. The treatments targeted naming deficits, sentence production, speech apraxia, and phonological deficits. Pre- and post-treatment assessments were undertaken to compare trained and untrained items. Gains were generalized to a different task in the first two types of intervention (naming and sentence production). A follow-up assessment was conducted 1-8 months after treatment among 7 participants. RESULTS: All individuals presented better performance in the trained items at the post-test for each rehabilitation program accomplished, demonstrating that learning of the trained strategies was achieved during the active phase of treatment. For 13 individuals, statistical significance was reached; while for five, the results were maintained. Results about untrained items, generalization to other tasks, and follow-up assessments are presented. CONCLUSIONS: The positive results found in our sample bring some practice-based evidence for the benefits of speech and language treatment strategies for clinical management of individuals with PPA.

RESUMO

The differential diagnosis of primary progressive aphasia (PPA) is challenging due to overlapping clinical manifestations of the different variants of the disease. This is particularly true for the logopenic variant of PPA (lvPPA), in which such overlap was reported with regard to impairments in repetition abilities. In this study, four individuals with lvPPA underwent standard neuropsychological and language assessments. The influence of psycholinguistic variables on their performance of in word, nonword and sentence repetition tasks was also specifically explored. Some level of heterogeneity was found in cognitive functions and in language. The four participants showed impairment in sentence repetition in which their performance was negatively affected by semantic reversibility and syntactic complexity. This study supports the heterogeneity of lvPPA with respect to the cognitive and linguistic status of participants. It also shows that sentence repetition is influenced not only by length, but also by semantic reversibility and syntactic complexity, two psycholinguistic variables known to place additional demands on phonological working memory.

O diagnóstico diferencial da afasia progressiva primária (APP) é desafiador devido às sobreposições das manifestações clínicas das diferentes variantes da doença. Isso é particularmente verdadeiro para a variante logopênica do APP (APPlg), em que tal sobreposição foi relatada em relação à deficiências nas habilidades de repetição. No presente estudo, quatro indivíduos com APPlg foram submetidos à avaliações neuropsicológica e de linguagem. A influência de variáveis psicolinguísticas em seu desempenho de palavras, não-palavras e tarefas de repetição de frases também foi especificamente explorada. Certo nível de heterogeneidade foi encontrado nas funções cognitivas e na linguagem. Os quatro participantes apresentaram prejuízo na repetição de frases em que seu desempenho foi afetado negativamente pela reversibilidade semântica e complexidade sintática. O estudo apoia a heterogeneidade do APPlg no que diz respeito ao status cognitivo e linguístico dos participantes. Mostra também que a repetição de sentenças é influenciada não apenas pelo comprimento, mas também pela reversibilidade semântica e complexidade sintática, duas variáveis psicolinguísticas conhecidas por colocarem demandas adicionais na memória operacional fonológica.

RESUMO

Introduction: Corticobasal syndrome (CBS) is a progressive neurological disorder related to multiple underlying pathologies, including four-repeat tauopathies, such as corticobasal degeneration and progressive supranuclear palsy, and Alzheimer's disease (AD). Speech and language are commonly impaired, encompassing a broad spectrum of deficits. We aimed to investigate CBS speech and language impairment patterns in light of a multimodal imaging approach. Materials and Methods: Thirty-one patients with probable CBS were prospectively evaluated concerning their speech-language, cognitive, and motor profiles. They underwent positron emission tomography with [18F]fluorodeoxyglucose (FDG-PET) and [11C]Pittsburgh Compound-B (PIB-PET) on a hybrid PET-MRI machine to assess their amyloid status. PIB-PET images were classified based on visual and semi-quantitative analyses. Quantitative group analyses were performed on FDG-PET data, and atrophy patterns on MRI were investigated using voxel-based morphometry (VBM). Thirty healthy participants were recruited as imaging controls. Results: Aphasia was the second most prominent cognitive impairment, presented in 67.7% of the cases, following apraxia (96.8%). We identified a wide linguistic profile, ranging from nonfluent variant-primary progressive aphasia to lexical-semantic deficits, mostly with impaired verbal fluency. PIB-PET was classified as negative (CBS-A- group) in 18/31 (58%) and positive (CBS-A+ group) in 13/31 (42%) patients. The frequency of dysarthria was significantly higher in the CBS-A- group than in the CBS-A+ group (55.6 vs. 7.7%, p = 0.008). CBS patients with dysarthria had a left-sided hypometabolism at frontal regions, with a major cluster at the left inferior frontal gyrus and premotor cortex. They showed brain atrophy mainly at the opercular frontal gyrus and putamen. There was a positive correlation between [18F]FDG uptake and semantic verbal fluency at the left inferior (p = 0.006, R 2 = 0.2326), middle (0.0054, R 2 = 0.2376), and superior temporal gyri (p = 0.0066, R 2 = 0.2276). Relative to the phonemic verbal fluency, we found a positive correlation at the left frontal opercular gyrus (p = 0.0003, R 2 = 0.3685), the inferior (p = 0.0004, R 2 = 0.3537), and the middle temporal gyri (p = 0.0001, R 2 = 0.3993). Discussion: In the spectrum of language impairment profile, dysarthria might be helpful to distinguish CBS patients not related to AD. Metabolic and structural signatures depicted from this feature provide further insights into the motor speech production network and are also helpful to differentiate CBS variants.

RESUMO

ABSTRACT. The differential diagnosis of primary progressive aphasia (PPA) is challenging due to overlapping clinical manifestations of the different variants of the disease. This is particularly true for the logopenic variant of PPA (lvPPA), in which such overlap was reported with regard to impairments in repetition abilities. In this study, four individuals with lvPPA underwent standard neuropsychological and language assessments. The influence of psycholinguistic variables on their performance of in word, nonword and sentence repetition tasks was also specifically explored. Some level of heterogeneity was found in cognitive functions and in language. The four participants showed impairment in sentence repetition in which their performance was negatively affected by semantic reversibility and syntactic complexity. This study supports the heterogeneity of lvPPA with respect to the cognitive and linguistic status of participants. It also shows that sentence repetition is influenced not only by length, but also by semantic reversibility and syntactic complexity, two psycholinguistic variables known to place additional demands on phonological working memory.

RESUMO. O diagnóstico diferencial da afasia progressiva primária (APP) é desafiador devido às sobreposições das manifestações clínicas das diferentes variantes da doença. Isso é particularmente verdadeiro para a variante logopênica do APP (APPlg), em que tal sobreposição foi relatada em relação à deficiências nas habilidades de repetição. No presente estudo, quatro indivíduos com APPlg foram submetidos à avaliações neuropsicológica e de linguagem. A influência de variáveis psicolinguísticas em seu desempenho de palavras, não-palavras e tarefas de repetição de frases também foi especificamente explorada. Certo nível de heterogeneidade foi encontrado nas funções cognitivas e na linguagem. Os quatro participantes apresentaram prejuízo na repetição de frases em que seu desempenho foi afetado negativamente pela reversibilidade semântica e complexidade sintática. O estudo apoia a heterogeneidade do APPlg no que diz respeito ao status cognitivo e linguístico dos participantes. Mostra também que a repetição de sentenças é influenciada não apenas pelo comprimento, mas também pela reversibilidade semântica e complexidade sintática, duas variáveis psicolinguísticas conhecidas por colocarem demandas adicionais na memória operacional fonológica.

Assuntos

RESUMO

The differential diagnosis among the behavioral variant of frontotemporal dementia FTD (bvFTD) and the linguist one primary progressive aphasia (PPA) is challenging. Presentations of dementia type or variants dominated by personality change or aphasia are frequently misinterpreted as psychiatric illness, stroke, or other conditions. Therefore, it is important to identify cognitive tests that can distinguish the distinct FTD variants to reduce misdiagnosis and best tailor interventions. We aim to examine the discriminative capacity of the most frequently used cognitive tests in their Spanish version for the context of dementia evaluation as well as the qualitative aspects of the neuropsychological performance such as the frequency and type of errors, perseverations, and false positives that can best discriminate between bvFTD and PPA. We also described mood and behavioral profiles of participants with mild to moderate probable bvFTD and PPA. A total of 55 subjects were included in this cross-sectional study: 20 with PPA and 35 with bvFTD. All participants underwent standard dementia screening that included a medical history and physical examination, brain MRI, a semistructured caregiver interview, and neuropsychological testing. We found that bvFTD patients had worse performance in executive function tests, and the PPA presented with the lower performance in language tests and the global score of Mini-Mental State Examination (MMSE). After running the linear discriminant model, we found three functions of cognitive test and subtests combination and three functions made by the Montreal Cognitive Assessment (MoCA) language subtest and performance errors that predicted group belonging. Those functions were more capable to classify bvFTD cases rather than PPA. In conclusion, our study supports that the combination of an individual test of executive function and language, MoCA's subtest, and performance errors as well have good accuracy to discriminate between bvFTD and PPA.

RESUMO

Background: Primary Progressive Aphasia (PPA) is characterized by progressive language impairment due to focal degeneration of brain areas related to linguistic processing. The detection and differential diagnosis of PPA can be difficult with clinical features that may overlap with features of other neurological conditions, such as Alzheimer's disease (AD). The scientific production on PPA in Latin American patients is still scarce. This study investigated the first symptoms in a Brazilian sample of patients with PPA in comparison with AD patients. Method: We compared the first symptoms reported by caregivers of people with PPA (n = 20; semantic variant n = 8, non-fluent variant n = 7, logopenic variant n = 3, and unclassified cases n = 2) and AD (n = 16). Data were collected through the application of a structured questionnaire that was presented in an interview format to the caregiver who knew the patient best. Results: Anomia, paraphasias and motor speech difficulties were the first symptoms capable of differentiating patients with PPA from those with AD, while memory was exclusive of AD. Among the PPA variants, anomia was the initial symptom associated with the semantic variant, while motor speech difficulties were associated with the non-fluent variant. The results are discussed considering the unique cultural and sociodemographic characteristics of this studied population. Conclusion: This study demonstrated that some of the initial symptoms of PPA patients may be unique to clinical variants of PPA and of AD, and their investigation may be useful for the early and differential diagnosis of this population.

RESUMO

Resumen El trastorno neurocognitivo frontotemporal es una enfermedad neurodegenerativa que incluye manifestaciones clínicas de subtipo comportamental y lingüística. La afasia progresiva primaria (APP) es un síndrome en el que aparecen alteraciones del lenguaje que comprende tres tipos de variantes: no fluente, semántica y logopénica. Este estudio describe la evolución clínica y las características neuropsicológicas de una mujer de 63 años que presenta un deterioro progresivo del lenguaje. Se evalúan las funciones de atención, memoria, lenguaje y funciones ejecutivas. La paciente obtuvo un bajo rendimiento en memoria, velocidad de procesamiento y funciones ejecutivas. Su lenguaje se caracteriza por presentar baja fluidez, agramatismo, parafasias verbales y dificultades en denominación. Se concluye que la paciente presenta características de la APP no fluente, que varía a través del tiempo y afecta su funcionamiento; características de un curso clínico de un trastorno neurocognitivo mayor posible debido a una degeneración del lóbulo frontotemporal.

Abstract Mild cognitive impairment, frontotemporal dementia (FTD) is a neurodegenerative disease characterized by clinical manifestations of behavior and linguistic subtypes. Primary Progressive Aphasia (APP) is a syndrome in which language alterations appear that include three types of variations: Non - fluent, Semantic and Logopenic. This study describes the clinical evolution and the neurophysiological characteristics of a 63 years old woman that started with a progressive language impairment. The functions which are evaluated are attention, memory, language and executive functions. The patient obtained a low performance in memory, processing speed and executive functions. The language is characterized by low fluency, agramatism, paraphasias and denomination difficulties. It is concluded, that the patient has characteristics of APP non-fluent which varies throughout the time and it affects her performance; characteristics of a clinical course of a greater neurocognitive disorder might be due to a lobe frontotemporal degeneration.

Assuntos

RESUMO

INTRODUCTION: There is a shortage of validated instruments to estimate disease progression in frontotemporal dementia (FTD). OBJECTIVES: To evaluate the ability of the FTD Rating Scale (FTD-FRS) to detect functional and behavioral changes in patients diagnosed with the behavioral variant of FTD (bvFTD), primary progressive aphasia (PPA), and Alzheimer disease (AD) after 12 months of the initial evaluation, compared to the Clinical Dementia Rating scale-frontotemporal lobar degeneration (CDR-FTLD) and the original Clinical Dementia Rating scale (CDR). METHODS: The sample consisted of 70 individuals, aged 40+ years, with at least 2 years of schooling, 31 with the diagnosis of bvFTD, 12 with PPA (8 with semantic variant and 4 with non-fluent variant), and 27 with AD. The FTD-FRS, the CDR, and the 2 additional CDR-FTLD items were completed by a clinician, based on the information provided by the caregiver with frequent contact with the patient. The Addenbrooke Cognitive Examination-Revised was completed by patients. After 12 months, the same protocol was applied. RESULTS: The FTD-FRS, CDR-FTLD, and CDR detected significant decline after 12 months in the 3 clinical groups (exception: FTD-FRS for PPA). The CDR was less sensitive to severe disease stages. CONCLUSIONS: The FTD-FRS and the CDR-FTLD are especially useful tools for dementia staging in AD and in the FTD spectrum.

Assuntos

RESUMO

Existen 3 variantes de afasia progresiva primaria (APP), que se distinguen según el dominio lingüístico predominantemente alterado: no fluente, logopénica y semántica. Una herramienta que ayuda a su clasificación es el Sydney Language Battery (Sydbat), el cual no se encuentra validado en Chile. El objetivo del presente trabajo es analizar la posible relación entre edad, años de escolaridad y rendimiento obtenido en las subpruebas del Sydbat en adultos sanos. Participaron 38 adultos sanos a losque se les aplicó el test.El promedio de edad fue 56,8 años (DE = 8,3) y tenían una media de 13,8 años de escolaridad (DE = 3,9). Todos provenían de la Región Metropolitana. Se realizaron correlaciones de Pearson y Spearman, para explorar la correlación entre el rendimiento en las subpruebas, la edad y la escolaridad. Considerando la variabilidad en el rendimiento en las subpruebas, se realizaron modelos uni y multivariados. Se encontraron correlaciones significativas entre edad y la subprueba comprensión (p<0,05) y los años de escolaridad con las subpruebas asociación semántica (p<0,001) y nominación (p<0,01). En personas con más de 12 años de escolaridad, el puntaje en la subprueba asociación semántica varió aproximadamente 2 puntos, con respecto a personas con menos de 12 años de escolaridad (p<0,01). Se concluye que las variaciones en el rendimiento del test, dependen la edad y la escolaridad. A mayor edad, menor es el rendimiento en la subprueba de comprensión. A mayor escolaridad, mayor es el rendimiento en las subpruebas de asociación semántica y nominación. El efecto mencionado debe ser considerado en el proceso de validación de Sydbat.

There are 3 variants of primary progressive aphasia (APP), which are distinguished according to the predominantly altered linguistic domain: non-fluent, logopenic, and semantic. A tool that helps in its classification is the Sydney Language Battery (Sydbat), which is not validated in Chile. The objective of this work is to analyze the possible relationship between age, years of schooling and performance obtained in the Sydbat subtests in healthy adults. 38 healthy adults participated in the test. The average age was 56.8 years (SD = 8.3) and they had a mean of 13.8 years of schooling (SD = 3.9). They all came from the Metropolitan Region. Pearson and Spearman correlations were performed to explore the correlation between subtest performance, age, and schooling. Considering the variability in performance in the subtests, univariate and multivariate models were performed. Significant correlations were found between age and the comprehension subtest (p <0.05) and the years of schooling with the semantic association (p <0.001) and nomination (p <0.01) subtests. In participants with more than 12 years of schooling, the score in the semantic association subtest varied approximately 2 points, with respect to people with less than 12 years of schooling (p <0.01). It is concluded that the variations in test performance depend on age andeducation. The older participants are, the lower their performance on the comprehension subtest. The higher the schooling, the higher the performance on the semantic association and nomination subtests. The mentioned effect must be considered in the Sydbat validation process.

Assuntos

RESUMO

The non-fluent/agrammatic variant of primary progressive aphasia (nfvPPA) and semantic variant (svPPA) of frontotemporal dementia (FTD) are neurodegenerative diseases. Previous works have shown alterations of fractional anisotropy (FA) and mean diffusivity (MD) from diffusion tensor images (DTIs). This manuscript is aimed at using DTI images to build a global tractography and to identify atrophy patterns of white matter in each variant. Twenty patients with svPPA, 20 patients with nfvPPA, 26 patients with behavioral variant of FTD (bvFTD) and, 33 controls were included. An analysis based on the connectivity of structural networks showed changes in FA and MD in svPPA and nfvPPA with respect to bvFTD. Much damage in the internal networks of the left temporal lobe was found in svPPA patients; in contrast, patients with nfvPPA showed atrophy in networks from the basal ganglia to motor and premotor areas. Those findings support the dual stream model of speech and language.

RESUMO

Although primary progressive aphasia (PPA) is clinically typified by linguistic impairments, emerging evidence highlights the presence of early deficits in social cognition. This review systematically describes the latter patterns, specifying their relation to the characteristic linguistic dysfunctions and atrophy patterns of non-fluent, semantic, and logopenic variants of the disease (nfvPPA, svPPA, and lvPPA, respectively), relative to closely related dementia types. Whereas the evidence on lvPPA proves scant, studies on nfvPPA and svPPA patients show consistent deficits in emotion recognition, theory of mind, and empathy. Notably, these seem to be intertwined with language impairments in nfvPPA, but they prove primary and independent of language disturbances in svPPA. Also, only the profile of svPPA resembles that of behavioral variant frontotemporal dementia, probably reflecting the overlap of fronto-temporal disruptions in both conditions. In short, the neurocognitive relationship between linguistic and socio-cognitive deficits cannot be precisely predicated for PPA as a whole; instead, specific links must be acknowledged in each variant. These emergent patterns pave the way for fruitful dimensional research in the field.

Assuntos

RESUMO

The nonfluent/agrammatic variant of primary progressive aphasia is characterized by apraxia of speech and agrammatism. Apraxia of speech limits patients' communication due to slow speaking rate, sound substitutions, articulatory groping, false starts and restarts, segmentation of syllables, and increased difficulty with increasing utterance length. Speech and language therapy is known to benefit individuals with apraxia of speech due to stroke, but little is known about its effects in primary progressive aphasia. This is a case report of a 72-year-old, illiterate housewife, who was diagnosed with nonfluent primary progressive aphasia and received speech and language therapy for apraxia of speech. Rate and rhythm control strategies for apraxia of speech were trained to improve initiation of speech. We discuss the importance of these strategies to alleviate apraxia of speech in this condition and the future perspectives in the area.

A variante não-fluente/agramática da afasia progressiva primária caracteriza-se por apraxia de fala e agramatismo. A apraxia de fala limita a comunicação devido a redução no fluxo de fala, substituição de sons, tentativas articulatórias, falsos inícios de fala e reinícios, segmentação de sílabas, e aumento da dificuldade conforme o aumento do enunciado. A terapia fonoaudiológica é benéfica em casos de apraxia de fala decorrentes de acidente vascular cerebral, porém pouco se sabe sobre seus efeitos na afasia progressiva primária. Este artigo é um relato de caso de uma mulher de 72 anos, analfabeta e trabalhadora do lar, que foi diagnosticada com afasia progressiva não-fluente e recebeu terapia fonoaudiológica para apraxia de fala. Estratégias de controle do ritmo e da taxa de fala foram utilizadas a fim de melhorar a iniciação da fala. A importância de estratégias para minimizar a apraxia de fala e as perspectivas futuras na área são discutidas neste artigo.

RESUMO

ABSTRACT The nonfluent/agrammatic variant of primary progressive aphasia is characterized by apraxia of speech and agrammatism. Apraxia of speech limits patients' communication due to slow speaking rate, sound substitutions, articulatory groping, false starts and restarts, segmentation of syllables, and increased difficulty with increasing utterance length. Speech and language therapy is known to benefit individuals with apraxia of speech due to stroke, but little is known about its effects in primary progressive aphasia. This is a case report of a 72-year-old, illiterate housewife, who was diagnosed with nonfluent primary progressive aphasia and received speech and language therapy for apraxia of speech. Rate and rhythm control strategies for apraxia of speech were trained to improve initiation of speech. We discuss the importance of these strategies to alleviate apraxia of speech in this condition and the future perspectives in the area.

RESUMO A variante não-fluente/agramática da afasia progressiva primária caracteriza-se por apraxia de fala e agramatismo. A apraxia de fala limita a comunicação devido a redução no fluxo de fala, substituição de sons, tentativas articulatórias, falsos inícios de fala e reinícios, segmentação de sílabas, e aumento da dificuldade conforme o aumento do enunciado. A terapia fonoaudiológica é benéfica em casos de apraxia de fala decorrentes de acidente vascular cerebral, porém pouco se sabe sobre seus efeitos na afasia progressiva primária. Este artigo é um relato de caso de uma mulher de 72 anos, analfabeta e trabalhadora do lar, que foi diagnosticada com afasia progressiva não-fluente e recebeu terapia fonoaudiológica para apraxia de fala. Estratégias de controle do ritmo e da taxa de fala foram utilizadas a fim de melhorar a iniciação da fala. A importância de estratégias para minimizar a apraxia de fala e as perspectivas futuras na área são discutidas neste artigo.

Assuntos