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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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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.

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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.

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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.

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El propósito de este estudio es establecer el perfil neuropsicológico no lingüístico de las variantes de la Afasia Progresiva Primaria, mediante una revisión sistemática de la literatura de los últimos 5 años. La búsqueda sistemática y manual avanzada se llevó a cabo en las bases de datos Scopus, PubMed, Web of Science y Google Académico para identificar los estudios que describieran el perfil neuropsicológico no lingüístico de las variantes de la Afasia Progresiva Primaria (APP), se establecieron criterios de inclusión y exclusión para la selección de los artículos, se evaluó su calidad y pertinencia. La revisión sistemática arrojó 7 084 artículos, de los cuales 131 fueron preseleccionados de acuerdo a lo informado en el resumen y 26 validados para lectura completa. Para la síntesis narrativa se utilizaron 19 artículos. Las variantes de APP cursan con déficits neurocognitivos fuera del dominio del lenguaje, par-ticulares a cada variante. Esto resalta la importancia de una evaluación neuropsicológica integral que aporte información adicional a los criterios de consenso ya establecidos para la evaluación de la APP.

The purpose of this study is to establish the non-linguistic neuropsychological profile of variants of Primary Progressive Aphasia,through a systematic review of the literature of the last 5 years. The systematic and advanced manual search was carried out in the databases Scopus, PubMed, Web of Science and Google Scholar to identify the studies that describe the non- linguistic neurop-sychological profile of the variants of Primary Progressive Aphasia (PPA), inclusion and exclusion criteria were established for the selection of articles, their quality and relevance were evaluated. The systematic review yielded 7 084 articles, of which 131 were pre-selected as reported in the summary and 26 validated for full reading. Nineteen articles were used for the narrative synthesis. PPA variants present with neurocognitive deficits outside the language domain, this underlines the importance of a comprehensive neuropsychological evaluation that provides additional information to the consensus criteria already established for the evaluationof the PPA.

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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.

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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.

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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.

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BACKGROUND: Rapid technological advances offer a possibility to develop cost-effective digital cognitive assessment tools. However, it is unclear whether these measures are suitable for application in populations from Low and middle-income countries (LMIC). OBJECTIVE: To examine the accuracy and validity of the Brain Health Assessment (BHA) in detecting cognitive impairment in a Cuban population. METHODS: In this cross-sectional study, 146 participants (cognitively healthy = 53, mild cognitive impairment (MCI) = 46, dementia = 47) were recruited at primary care and tertiary clinics. The main outcomes included: accuracy of the BHA and the Montreal Cognitive Assessment (MoCA) in discriminating between controls and cognitively impaired groups (MCI and dementia) and correlations between the BHA subtests of memory, executive functions, and visuospatial skills and criterion-standard paper-and-pencil tests in the same domains. RESULTS: The BHA had an AUC of 0.95 (95% CI: 0.91-0.98) in discriminating between controls and cognitively impaired groups (MCI and dementia, combined) with 0.91 sensitivity at 0.85 specificity. In discriminating between control and MCI groups only, the BHA tests had an AUC of 0.94 (95% CI: 0.90-0.99) with 0.71 sensitivity at 0.85 specificity. Performance was superior to the MoCA across all diagnostic groups. Concurrent and discriminant validity analyses showed moderate to strong correlations between the BHA tests and standard paper-and-pencil measures in the same domain and weak correlations with standard measures in unrelated domains. CONCLUSION: The BHA has excellent performance characteristics in detecting cognitive impairment including dementia and MCI in a Hispanic population in Cuba and outperformed the MoCA. These results support potential application of digital cognitive assessment for older adults in LMIC.

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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.

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Purpose Primary progressive aphasia (PPA) and the amnestic variant of Alzheimer's disease (AD) are neurodegenerative conditions characterized by a profound loss of functional communication abilities. Communicative impairment in AD and PPA is especially apparent in the domain of naming common objects and familiar faces. We evaluated the effectiveness of a language intervention targeting maintenance of an individualized core vocabulary in a longitudinal cohort of older adults experiencing either PPA or AD. Method PPA (n = 9) and AD (n = 1) patients were administered a semantically based language treatment for up to 2 years. Patients repeatedly named and generated semantic features for a personalized lexicon consisting of 100 words. We evaluated naming accuracy and off-line neuropsychological measures at four successive timepoints. Naming accuracy was assessed in patients (n = 7) who completed at least three recurrent evaluations. Off-line neuropsychological performance was assessed across timepoints in all patients. Results Patients demonstrated relative preservation of naming trained words relative to a steep decline for untrained (control) words. The greatest decrements were observed for naming people relative to objects. Conclusion These results suggest that consistent training of a finite set of words can protect a core lexicon composed of crucial target concepts (e.g., a spouse's name). We discuss potential benefits and clinical implications of maintenance-based approaches to promoting language functioning in the context of neurodegeneration.

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INTRODUCTION: Abnormal behavioural and physiological reactivity to emotional stimuli is a hallmark of frontotemporal dementia (FTD), particularly the behavioural variant (bvFTD). As part of this repertoire, altered phobic responses have been reported in some patients with FTD but are poorly characterised. METHODS: We collected data (based on caregiver reports) concerning the prevalence and nature of any behavioural changes related to specific phobias in a cohort of patients representing canonical syndromes of FTD and Alzheimer's disease (AD), relative to healthy older controls. Neuroanatomical correlates of altered phobic reactivity were assessed using voxel-based morphometry. RESULTS: 46 patients with bvFTD, 20 with semantic variant primary progressive aphasia, 25 with non-fluent variant primary progressive aphasia, 29 with AD and 55 healthy age-matched individuals participated. Changes in specific phobia were significantly more prevalent in the combined FTD cohort (15.4% of cases) and in the bvFTD group (17.4%) compared both to healthy controls (3.6%) and patients with AD (3.5%). Attenuation of phobic reactivity was reported for individuals in all participant groups, however new phobias developed only in the FTD cohort. Altered phobic reactivity was significantly associated with relative preservation of grey matter in left posterior middle temporal gyrus, right temporo-occipital junction and right anterior cingulate gyrus, brain regions previously implicated in contextual decoding, salience processing and reward valuation. CONCLUSION: Altered phobic reactivity is a relatively common issue in patients with FTD, particularly bvFTD. This novel paradigm of strong fear experience has broad implications: clinically, for diagnosis and patient well-being; and neurobiologically, for our understanding of the pathophysiology of aversive sensory signal processing in FTD and the neural mechanisms of fear more generally.

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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.

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OBJECTIVE: To measure postmortem burden of frontotemporal lobar degeneration (FTLD) with TDP-43 (FTLD-TDP) or tau (FTLD-Tau) proteinopathy across hemispheres in primary progressive aphasia (PPA) using digital histopathology and to identify clinicopathological correlates of these distinct proteinopathies. METHODS: In an autopsy cohort of PPA (FTLD-TDP = 13, FTLD-Tau = 14), we analyzed laterality and regional distribution of postmortem pathology, quantified using a validated digital histopathological approach, in available brain tissue from up to 8 cortical regions bilaterally. We related digital pathology to antemortem structural neuroimaging and specific clinical language features. RESULTS: Postmortem cortical pathology was left-lateralized in both FTLD-TDP (beta = -0.15, standard error [SE] = 0.05, p = 0.007) and FTLD-Tau (beta = -0.09, SE = 0.04, p = 0.015), but the degree of lateralization decreased with greater overall dementia severity before death (beta = -8.18, SE = 3.22, p = 0.015). Among 5 core pathology regions sampled, we found greatest pathology in left orbitofrontal cortex (OFC) in FTLD-TDP, which was greater than in FTLD-Tau (F = 47.07, df = 1,17, p < 0.001), and in left midfrontal cortex (MFC) in FTLD-Tau, which was greater than in FTLD-TDP (F = 19.34, df = 1,16, p < 0.001). Postmortem pathology was inversely associated with antemortem magnetic resonance imaging cortical thickness (beta = -0.04, SE = 0.01, p = 0.007) in regions matching autopsy sampling. Irrespective of PPA syndromic variant, single-word comprehension impairment was associated with greater left OFC pathology (t = -3.72, df = 10.72, p = 0.004) and nonfluent speech with greater left MFC pathology (t = -3.62, df = 12.00, p = 0.004) among the 5 core pathology regions. INTERPRETATION: In PPA, FTLD-TDP and FTLD-Tau have divergent anatomic distributions of left-lateralized postmortem pathology that relate to antemortem structural imaging and distinct language deficits. Although other brain regions may be implicated in neural networks supporting these complex language measures, our observations may eventually help to improve antemortem diagnosis of neuropathology in PPA. Ann Neurol 2019;85:630-643.

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La Afasia Progresiva Primaria (APP) es una patología neurodegenerativa que se presenta con afectación insidiosa y progresiva del lenguaje. Los criterios diagnósticos actuales diferencian tres subtipos de APP, cada una con perfiles neurolingüísticos específicos. Diversas investigaciones han propuesto que un síntoma característico de la APP variante semántica (APP-vs) es un mayor compromiso en el procesamiento de conceptos concretos que de abstractos (Efecto de Concretud Inverso - ECI). Para explicar este ECI se han propuesto diferentes explicaciones: (a). el patrón de compromiso neural, (b). el nivel educativo de los pacientes, (c). el estadio de la enfermedad. El objetivo del presente trabajo es estudiar en forma longitudinal la progresión en el procesamiento de conceptos concretos y abstractos en un paciente diagnosticado con APP-vs. Para ello se utilizó una tarea de juicios de sinonimia donde se debe identificar si dos palabras son sinónimos o no. La tarea cuenta con pares de conceptos concretos y abstractos. Se evaluó al paciente en tres momentos (2014, 2015 y 2016). Se observó un mejor desempeño de conceptos abstractos en la primera evaluación. El ECI desaparece en la segunda evaluación. El patrón se revierte en la tercera. Estos resultados apoyan la propuesta de que el ECI observado en pacientes con APP-vs es un síntoma de los estadios iniciales de la enfermedad. Este ECI se relacionaría con la afectación temprana de las porciones del Lóbulo Temporal Anterior que procesan rasgos visuales, que serían más relevantes para los conceptos concretos.

Primary Progressive Aphasia (PPA) is a neurodegenerative disease which appears with progressive and insidious affectation of language. Current diagnostic criteria establish three different subtypes of PPA, each showing specific neurolinguistic profiles. Several researches have proposed a Reverse Concreteness Effect (RCE) as a main symptom for the Semantic Variant of PPA (sv-PPA), that is, a better performance with abstract than concrete concepts. Different explanations for this effect include: (a). pattern of neural degeneration, (b). patients' educational level, (c). moment of disease progression. The aim of this work is to study the progression of concrete and abstract concepts processing in a patient diagnosed with sv-PPA. We used a synonyms judgement task where the subject has to indicate if two words are synonyms or not. The task include both concrete and abstract concepts. The patient was evaluated in three different moments (2014, 2015 and 2016). A better performance with abstract concepts was observed during the first evaluation. The RCE disappeared during the second assessment. The third time showed a reversed pattern. Our results support those proposing that the RCE can only be found at initial stages of vs-PPA. The RCE appears to be related to the early degeneration of some portions in the Anterior Temporal Lobe which process visual features. These would be much more relevant for processing concrete concepts.

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Frontotemporal dementia (FTD) affects behavior, language, and personality. This study aims to explore functional connectivity changes in three FTD variants: behavioral (bvFTD), semantic (svPPA), and nonfluent variant (nfvPPA). Seventy-six patients diagnosed with FTD by international criteria and thirty-two controls were investigated. Functional connectivity from resting functional magnetic resonance imaging (fMRI) was estimated for the whole brain. Two types of analysis were done: network basic statistic and topological measures by graph theory. Several hubs in the limbic system and basal ganglia were compromised in the behavioral variant apart from frontal networks. Nonfluent variants showed a major disconnection with respect to the behavioral variant in operculum and parietal inferior. The global efficiency had lower coefficients in nonfluent variants than behavioral variants and controls. Our results support an extensive disconnection among frontal, limbic, basal ganglia, and parietal hubs.

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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.

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INTRODUCTION: Few studies on instruments for staging frontotemporal dementia (FTD) have been conducted. OBJECTIVE: The objective of this study was to analyze the factor structure, internal consistency, reliability, and convergent validity of the Brazilian version of the Frontotemporal Dementia Rating Scale (FTD-FRS). METHODS: A total of 97 individuals aged 40 years and above with >2 years' education took part in the study, 31 patients diagnosed with behavioral variant FTD (bvFTD), 8 patients with primary progressive aphasia, 28 with Alzheimer disease, 8 with mild cognitive impairment, and a control group of 22 healthy subjects. The FTD-FRS was completed by family members or caregivers, and Neurologists completed the 8-item Clinical Dementia Rating for Frontotemporal Lobar Degeneration (CDR-FTLD) scale (6 original domains plus Language and Behavior). The Alzheimer disease and FTD patients had equivalent disease severity level. RESULTS: The internal consistency of the FTD-FRS, estimated by Cronbach α, was 0.975 whereas test-retest reliability was 0.977. Scree plot and exploratory factor (Varimax rotation) analyses revealed the existence of 4 factors, with eigenvalues >1, which together explained 77.13% of the total variance with values of 1.28 to 17.52. The domains of the Brazilian version of the FTD-FRS scale correlated with the domains of the CDR-FTLD. CONCLUSIONS: The present study is the first to document the factorial structure of the FTD-FRS and its convergent validity with the CDR-FTLD. These tools are key to determine dementia severity in FTD. The Brazilian FTD-FRS demonstrated adequate psychometric properties for use in Brazil. This instrument may contribute to disease staging in FTD and may help to document intervention-related changes.

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OBJECTIVE: To quantify the socioeconomic burden of frontotemporal degeneration (FTD) compared to previously published data for Alzheimer disease (AD). METHODS: A 250-item internet survey was administered to primary caregivers of patients with behavioral-variant FTD (bvFTD), primary progressive aphasia, FTD with motor neuron disease, corticobasal syndrome, or progressive supranuclear palsy. The survey included validated scales for disease staging, behavior, activities of daily living, caregiver burden, and health economics, as well as investigator-designed questions to capture patient and caregiver experience with FTD. RESULTS: The entire survey was completed by 674 of 956 respondents (70.5%). Direct costs (2016 US dollars) equaled $47,916 and indirect costs $71,737, for a total annual per-patient cost of $119,654, nearly 2 times higher than reported costs for AD. Patients ≥65 years of age, with later stages of disease, and with bvFTD correlated with higher direct costs, while patients <65 years of age and men were associated with higher indirect costs. An FTD diagnosis produced a mean decrease in household income from $75,000 to $99,000 12 months before diagnosis to $50,000 to $59,999 12 months after diagnosis, resulting from lost days of work and early departure from the workforce. CONCLUSIONS: The economic burden of FTD is substantial. Counting productivity-related costs, per-patient costs for FTD appear to be greater than per-patient costs reported for AD. There is a need for biomarkers for accurate and timely diagnosis, effective treatments, and services to reduce this socioeconomic burden.

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