Pattern of cortical thinning in logopenic progressive aphasia patients in Thailand.

BACKGROUND: Logopenic progressive aphasia (LPA) is an uncommon neurodegenerative disorder primarily characterized by word-finding difficulties and sentence repetition impairment. Prominent cortical atrophy around left temporo-parietal junction (TPJ) is a classical imaging feature of LPA. This study investigated cortical thinning pattern in clinically diagnosed LPA patients using non-demented subjects as a control group. We also aimed to explore whether there was prominent thinning of other cortical area additional to the well-recognized left TPJ. METHODS: Thicknesses of all cortical regions were measured from brain magnetic resonance images using an automated command on Freesurfer software. Cortical thickness of the LPA and control groups were compared by two methods: 1) using a general linear model (GLM) in SPSS software; and 2) using a vertex-by-vertex GLM, performed with Freesurfer's QDEC interface. RESULTS: Besides the well-recognized left TPJ, cortical regions that were significantly thinner in the LPA group by both comparison methods included left caudal middle frontal gyrus (CMFG) (p = 0.006 by SPSS, p = 0.0003 by QDEC), left rostral middle frontal gyrus (p = 0.001 by SPSS, p = 0.0001 by QDEC), left parahippocampal gyrus (p = 0.008 by SPSS, p = 0.005 by QDEC) and right CMFG (p = 0.005 by SPSS, p = 0.0001 by QDEC). CONCLUSIONS: Our results demonstrated that thinning of middle frontal gyri may be an additional feature in clinically diagnosed LPA patients. Involvement of left parahippocampal gyrus may reflect the underlying neuropathology of Alzheimer's disease in majority of the LPA patients.

MEM&SO protocol: understanding the determinants of social learning in neurodegenerative diseases.

BACKGROUND: People with neurodegenerative diseases may have difficulty learning new information, owing to their cognitive impairments. Teaching them techniques for learning in social contexts could alleviate this difficulty. The present study will examine the performances of patients with Alzheimer's disease and patients with the semantic variant of primary progressive aphasia on a memory test administered in three social contexts. The protocol will make it possible to identify determinants of social interactions, social abilities, cognition, and personality that can explain the potentially beneficial effect of social context on learning in these patients. METHODS: Thirty dyads (patient with primary memory impairment who meets criteria for Alzheimer's disease paired with caregiver), 16 dyads (patient meeting criteria for semantic variant of primary progressive aphasia paired with caregiver), and 46 dyads (healthy controls with no cognitive complaints) will be recruited. A nonverbal memory test (social memory task) will be administered to each dyad in three different social contexts (presence-only, observation, collaboration). Patients and healthy controls will also undergo a neuropsychological assessment to measure social (interactions and abilities), cognitive and personality aspects. Patients will be compared with controls on differential social scores calculated between the presence-only and collaboration contexts, and between the presence-only and observation contexts. A multiple comparative case study will be conducted to identify social, cognitive and personality variables that potentially explain the differential scores in the collaboration and observation contexts. DISCUSSION: For the first time, memory will be assessed in patients with Alzheimer's disease and patients with the semantic variant of primary progressive aphasia in three different contexts (presence-only, observation, collaboration). The multiple comparative case study will make it possible to identify the determinants of memory performance in the social context, in order to create the most beneficial learning context for individual patients, according to their profile. TRIAL REGISTRATION: This study was approved by the Ile de France XI institutional review board (2022-A00198-35), and registered on ClinicalTrials.gov (no. NCT05800028), on April 27, 2023.

[Rare forms of dementia-Atypical variants of Alzheimer's dementia]. / Seltene Demenzformen ­ atypische Varianten der Alzheimer-Demenz.

In Germany, around 1.8 million people currently suffer from dementia and the numbers are increasing. The main cause of dementia is Alzheimer's disease. This is classically manifested in the form of an amnestic syndrome but also encompasses various atypical variants, especially in younger patients and in the clinical routine are not always easy to recognize. These are described in this narrative review with case studies. Posterior cortical atrophy (PCA) presents with visual disorders, in the logopenic variants of primary progressive aphasia (lvPPA) impaired word retrieval is the main symptom, in the frontal variant of Alzheimer's disease behavioral disorders are prominent and in corticobasal syndrome (CBS) an akinetic rigid Parkinson's syndrome with alien limb phenomenon. As the clinical presentation of these atypical variants shows an overlap with other dementia disorders, the differential diagnosis is often challenging. In this context amyloid biomarkers can provide valuable services.

Crossed aphasia in a left-handed patient with non-fluent variant of primary progressive aphasia with left asymmetric brain SPECT.

Primary progressive aphasia is a clinical syndrome caused by neurodegeneration of areas and neural networks involved in language, usually in the left hemisphere. The term "crossed aphasia" denotes an acquired language dysfunction caused by a lesion in the ipsilateral hemisphere to the dominant hand. Objective: To describe a case of crossed aphasia in a 60-year-old left-handed patient with a non-fluent variant of primary progressive aphasia diagnosis (age of onset=52), evidenced by a left asymmetry on brain SPECT scan. Methods: Clinical and family history, the Edinburgh Handedness Inventory, Measurement of Functional Activities in Older Adults in the Community, the "Mini-Mental State Examination", the Trail Making Test, the Tower of London, and the Neuropsychological assessment for dementia, and neuroimaging studies were carried out. Results: Neuropsychological assessment showed severe cognitive impairment, especially regarding language. The magnetic resonance imaging showed important signs of cortico-subcortical atrophy, with predominance in the frontal and temporal lobes. The single-photon emission computed tomography scan showed moderate to severe hypoperfusion in the left cerebral hemisphere, including the hippocampus. Conclusion: We described a clinical case of crossed aphasia in a left-handed woman with a non-fluent variant of primary progressive aphasia with asymmetry on brain SPECT, mainly on the left, followed up for seven years.

A afasia progressiva primária é uma síndrome clínica causada por uma neurodegeneração de áreas e redes neurais envolvidas na linguagem, geralmente no hemisfério esquerdo. O termo "afasia cruzada" denota uma disfunção adquirida de linguagem causada por uma lesão no hemisfério ipsilateral da mão dominante. Objetivo: Relatamos um caso de afasia cruzada em uma paciente de 60 anos, canhota, com um quadro clínico de afasia progressiva primária variante não fluente (idade de início=52), evidenciada por assimetria no SPECT cerebral à esquerda. Métodos: Foram realizados para o diagnóstico do caso: história clínica e familiar, o Inventário de Dominância de Edinburgh, a Escala de Atividades Funcionais de Pfeffer, o Miniexame do Estado Mental, o Teste das Trilhas, o Teste da Torre de Londres, a Avaliação Neuropsicológica Adequada às Demências e exames de neuroimagem. Resultados: A avaliação neuropsicológica mostrou comprometimento cognitivo severo, principalmente sobre a linguagem; a ressonância magnética do crânio mostrou sinais de involução córtico-subcortical, com predominância nos lobos frontal e temporal e a cintilografia cerebral por emissão de fóton único mostrou hipoperfusão moderada a severa no hemisfério cerebral esquerdo, incluindo o hipocampo. Conclusão: Registramos um caso clínico de afasia cruzada em uma paciente canhota com afasia progressiva primária variante não fluente com assimetria no SPECT cerebral, principalmente à esquerda, seguida há sete anos.

Who are the speech and language therapists who work with primary progressive aphasia in Brazil? An exploratory cross-sectional survey study.

Primary Progressive Aphasia (PPA) is a progressive language disorder associated with frontotemporal impairment and mainly affects the left hemisphere of the brain. In general, this condition compromises abilities related to comprehension and expression of language. The diagnosis of PPA depends on in-depth knowledge regarding functions of language, neurology, and neuropsychology. Speech and language therapists (SLTs) have a pivotal role in the diagnosis and rehabilitation of PPA. The absence of these professionals involved in the diagnosis and rehabilitation may reflect on the quality of care of people with PPA. Objective: To identify the sociodemographic, educational, and professional practice characteristics of SLTs who work with people with PPA in Brazil. Methods: An online questionnaire was disseminated to reach SLTs across Brazil. The questionnaire collected information regarding sociodemographics, training and education, practice (time, setting, service provision), and sources of referral. Results: The study included 71 participants (95.8% women). Specialization was the most frequent educational level followed by master's degree, and participants where mainly from the Southeast and South regions of Brazil. Neurologists were the professionals who most referred patients with PPA to SLTs. Finally, SLTs worked primarily in homecare settings and provided mainly individual therapy services. Conclusion: SLTs who work with PPA in Brazil can be characterized mainly as professionals with postgraduate degrees, relatively young, and from the South and Southeast regions of Brazil.

A afasia progressiva primária (APP) é um distúrbio progressivo da linguagem associado à atrofia de regiões frontotemporais predominantemente do hemisfério esquerdo do cérebro. De modo geral, a APP afeta as capacidades compreensivas e expressivas da linguagem. O diagnóstico depende de profissionais com profundo conhecimento das funções da linguagem, neurologia e neuropsicologia. A fonoaudiologia tem papel essencial no diagnóstico e reabilitação da APP, e a ausência de fonoaudiólogos nesses processos pode refletir na qualidade do cuidado das pessoas com APP. Objetivo: Identificar as características sociodemográficas, educacionais e de atuação profissional de fonoaudiólogos que atuam com APP no Brasil. Métodos: Foi distribuído um questionário em formato online para fonoaudiólogos de todo o Brasil. O questionário coletou informações sobre aspectos sociodemográficos, de formação, atuação profissional (tempo, local de atuação, tipo de serviço oferecido) e fontes de encaminhamento. Resultados: O estudo incluiu 71 participantes (95,8% mulheres). O nível educacional mais frequente foi a especialização, e as regiões demográficas com maior incidência de profissionais que atendiam APP foram as Regiões Sudeste e Sul do país. Os neurologistas foram os profissionais que mais encaminhavam pacientes com APP para os fonoaudiólogos. Por fim, os fonoaudiólogos atuavam, principalmente, em homecare e realizando, em sua maioria, terapia individual. Conclusão: Os fonoaudiólogos que atuam com APP no Brasil podem ser caracterizados principalmente como profissionais pós-graduados, relativamente jovens e das Regiões Sul e Sudeste do Brasil.

Diagnosis of frontotemporal dementia: recommendations of the Scientific Department of Cognitive Neurology and Aging of the Brazilian Academy of Neurology.

"Frontotemporal dementia" (FTD) is a clinical syndrome characterized by the focal involvement of the frontal and/or temporal lobes. FTD has three clinical phenotypes: the behavioral variant and two linguistic subtypes, namely, non-fluent/agrammatic primary progressive aphasia (PPA-NF/A) and semantic PPA (PPA-S). FTD is the second most common cause of dementia in individuals under the age of 65 years. This article presents recommendations for the diagnosis of FTD in the Brazilian scenario, considering the three levels of complexity of the health system: primary health care, secondary and tertiary levels. Diagnostic guidelines are proposed, including cognitive testing, behavioral and language assessments, laboratory tests, and neuroimaging.

A "demência frontotemporal" (DFT) é uma síndrome clínica, cujo denominador comum é o acometimento focal dos lobos frontais e/ou temporais. A DFT tem três fenótipos clínicos distintos: a variante comportamental e dois subtipos linguísticos, a saber, a afasia progressiva primária não-fluente/agramática (APP-NF/A) e a afasia progressiva primária semântica (APP-S). A DFT é a segunda causa mais comum de demência em indivíduos com idade inferior a 65 anos, após a doença de Alzheimer. O presente artigo apresenta recomendações para diagnóstico da DFT no cenário brasileiro, considerando os três níveis de complexidade do sistema de saúde: atenção primária à saúde e níveis secundários. São propostos protocolos de investigação diagnóstica abrangendo testagem cognitiva, avaliação comportamental, avaliação fonoaudiológica, exames laboratoriais e de neuroimagem.

Synaptic vesicle protein 2A positron emission tomography images of semantic variant primary progressive aphasia patient with GRN mutation: a case report / 中华神经科杂志

Synaptic dysfunction plays an important role in the early stage of frontotemporal dementia (FTD), and there are differences in the pattern of synaptic damage in different genotypes. GRN gene mutations are rare in the Chinese population, and there are no reports of synaptic damage patterns in GRN mutations or semantic variant primary progressive aphasia (svPPA). The synaptic injury characteristics of a patient with svPPA harboring GRN gene mutations, which was characterized by decreased synaptic density in the left frontal, temporal, parietal lobe and contralateral cerebellum were reported in this article. The underlying mechanism of synaptic dysfunction involved in the disease process, and potential targets for future clinical interventions were indicated.

Crossed aphasia in a left-handed patient with non-fluent variant of primary progressive aphasia with left asymmetric brain SPECT / Afasia cruzada em paciente canhota com afasia progressiva primária variante não fluente com SPECT cerebral assimétrico à esquerda

ABSTRACT Primary progressive aphasia is a clinical syndrome caused by neurodegeneration of areas and neural networks involved in language, usually in the left hemisphere. The term "crossed aphasia" denotes an acquired language dysfunction caused by a lesion in the ipsilateral hemisphere to the dominant hand. Objective: To describe a case of crossed aphasia in a 60-year-old left-handed patient with a non-fluent variant of primary progressive aphasia diagnosis (age of onset=52), evidenced by a left asymmetry on brain SPECT scan. Methods: Clinical and family history, the Edinburgh Handedness Inventory, Measurement of Functional Activities in Older Adults in the Community, the "Mini-Mental State Examination", the Trail Making Test, the Tower of London, and the Neuropsychological assessment for dementia, and neuroimaging studies were carried out. Results: Neuropsychological assessment showed severe cognitive impairment, especially regarding language. The magnetic resonance imaging showed important signs of cortico-subcortical atrophy, with predominance in the frontal and temporal lobes. The single-photon emission computed tomography scan showed moderate to severe hypoperfusion in the left cerebral hemisphere, including the hippocampus. Conclusion: We described a clinical case of crossed aphasia in a left-handed woman with a non-fluent variant of primary progressive aphasia with asymmetry on brain SPECT, mainly on the left, followed up for seven years.

RESUMO A afasia progressiva primária é uma síndrome clínica causada por uma neurodegeneração de áreas e redes neurais envolvidas na linguagem, geralmente no hemisfério esquerdo. O termo "afasia cruzada" denota uma disfunção adquirida de linguagem causada por uma lesão no hemisfério ipsilateral da mão dominante. Objetivo: Relatamos um caso de afasia cruzada em uma paciente de 60 anos, canhota, com um quadro clínico de afasia progressiva primária variante não fluente (idade de início=52), evidenciada por assimetria no SPECT cerebral à esquerda. Métodos: Foram realizados para o diagnóstico do caso: história clínica e familiar, o Inventário de Dominância de Edinburgh, a Escala de Atividades Funcionais de Pfeffer, o Miniexame do Estado Mental, o Teste das Trilhas, o Teste da Torre de Londres, a Avaliação Neuropsicológica Adequada às Demências e exames de neuroimagem. Resultados: A avaliação neuropsicológica mostrou comprometimento cognitivo severo, principalmente sobre a linguagem; a ressonância magnética do crânio mostrou sinais de involução córtico-subcortical, com predominância nos lobos frontal e temporal e a cintilografia cerebral por emissão de fóton único mostrou hipoperfusão moderada a severa no hemisfério cerebral esquerdo, incluindo o hipocampo. Conclusão: Registramos um caso clínico de afasia cruzada em uma paciente canhota com afasia progressiva primária variante não fluente com assimetria no SPECT cerebral, principalmente à esquerda, seguida há sete anos.

Who are the speech and language therapists who work with primary progressive aphasia in Brazil? An exploratory cross-sectional survey study / Quem são os fonoaudiólogos que trabalham com afasia progressiva primária no Brasil? Um estudo de inquérito transversal exploratório

ABSTRACT Primary Progressive Aphasia (PPA) is a progressive language disorder associated with frontotemporal impairment and mainly affects the left hemisphere of the brain. In general, this condition compromises abilities related to comprehension and expression of language. The diagnosis of PPA depends on in-depth knowledge regarding functions of language, neurology, and neuropsychology. Speech and language therapists (SLTs) have a pivotal role in the diagnosis and rehabilitation of PPA. The absence of these professionals involved in the diagnosis and rehabilitation may reflect on the quality of care of people with PPA. Objective: To identify the sociodemographic, educational, and professional practice characteristics of SLTs who work with people with PPA in Brazil. Methods: An online questionnaire was disseminated to reach SLTs across Brazil. The questionnaire collected information regarding sociodemographics, training and education, practice (time, setting, service provision), and sources of referral. Results: The study included 71 participants (95.8% women). Specialization was the most frequent educational level followed by master's degree, and participants where mainly from the Southeast and South regions of Brazil. Neurologists were the professionals who most referred patients with PPA to SLTs. Finally, SLTs worked primarily in homecare settings and provided mainly individual therapy services. Conclusion: SLTs who work with PPA in Brazil can be characterized mainly as professionals with postgraduate degrees, relatively young, and from the South and Southeast regions of Brazil.

RESUMO A afasia progressiva primária (APP) é um distúrbio progressivo da linguagem associado à atrofia de regiões frontotemporais predominantemente do hemisfério esquerdo do cérebro. De modo geral, a APP afeta as capacidades compreensivas e expressivas da linguagem. O diagnóstico depende de profissionais com profundo conhecimento das funções da linguagem, neurologia e neuropsicologia. A fonoaudiologia tem papel essencial no diagnóstico e reabilitação da APP, e a ausência de fonoaudiólogos nesses processos pode refletir na qualidade do cuidado das pessoas com APP. Objetivo: Identificar as características sociodemográficas, educacionais e de atuação profissional de fonoaudiólogos que atuam com APP no Brasil. Métodos: Foi distribuído um questionário em formato online para fonoaudiólogos de todo o Brasil. O questionário coletou informações sobre aspectos sociodemográficos, de formação, atuação profissional (tempo, local de atuação, tipo de serviço oferecido) e fontes de encaminhamento. Resultados: O estudo incluiu 71 participantes (95,8% mulheres). O nível educacional mais frequente foi a especialização, e as regiões demográficas com maior incidência de profissionais que atendiam APP foram as Regiões Sudeste e Sul do país. Os neurologistas foram os profissionais que mais encaminhavam pacientes com APP para os fonoaudiólogos. Por fim, os fonoaudiólogos atuavam, principalmente, em homecare e realizando, em sua maioria, terapia individual. Conclusão: Os fonoaudiólogos que atuam com APP no Brasil podem ser caracterizados principalmente como profissionais pós-graduados, relativamente jovens e das Regiões Sul e Sudeste do Brasil.

Analysis of a case of corticobasal degeneration started with primary progressive aphasia / 中华神经科杂志

Objective:Through a typical case of corticobasal degeneration (CBD) with primary progressive aphasia (PPA) to analyze the clinical characteristics of CBD and the special manifestations of aphasia with that disease.Methods:Retrospective analysis was performed on a patient with PPA based CBD who was admitted to the First Affiliated Hospital, Sun Yat-sen University in July 2020 to summarize the clinical features and diagnostic thinking of CBD.Results:The patient was a 59-year-old male, manifested rapidly progressive dysfunction of language and memory function. The aphasia was mainly featured as slow speech, reduced content and grammatical errors, and diagnosed as PPA, non-fluent grammatical variation. The imaging results showed the atrophy of the left frontal lobe, parietal lobe, basal ganglia and thalamus, coupled with the reduction in 18F-fluorodeoxyglucose radioactive uptake. The patient was finally diagnosed as possible CBD. Conclusions:PPA as the initial manifestation of CBD is very rare in clinical practice. The high non-specificity of clinical features and the lack of typical motor symptoms result in the difficulty of correct diagnosis of CBD. Timely functional imaging in nuclear medicine and reliable biomarkers help to facilitate early diagnosis of atypical CBD.

Diagnóstico da demência frontotemporal: recomendações do Departamento Científico de Neurologia Cognitiva e do Envelhecimento da Academia Brasileira de Neurologia / Diagnosis of frontotemporal dementia: recommendations of the Scientific Department of Cognitive Neurology and Aging of the Brazilian Academy of Neurology

RESUMO A "demência frontotemporal" (DFT) é uma síndrome clínica, cujo denominador comum é o acometimento focal dos lobos frontais e/ou temporais. A DFT tem três fenótipos clínicos distintos: a variante comportamental e dois subtipos linguísticos, a saber, a afasia progressiva primária não-fluente/agramática (APP-NF/A) e a afasia progressiva primária semântica (APP-S). A DFT é a segunda causa mais comum de demência em indivíduos com idade inferior a 65 anos, após a doença de Alzheimer. O presente artigo apresenta recomendações para diagnóstico da DFT no cenário brasileiro, considerando os três níveis de complexidade do sistema de saúde: atenção primária à saúde e níveis secundários. São propostos protocolos de investigação diagnóstica abrangendo testagem cognitiva, avaliação comportamental, avaliação fonoaudiológica, exames laboratoriais e de neuroimagem.

ABSTRACT "Frontotemporal dementia" (FTD) is a clinical syndrome characterized by the focal involvement of the frontal and/or temporal lobes. FTD has three clinical phenotypes: the behavioral variant and two linguistic subtypes, namely, non-fluent/agrammatic primary progressive aphasia (PPA-NF/A) and semantic PPA (PPA-S). FTD is the second most common cause of dementia in individuals under the age of 65 years. This article presents recommendations for the diagnosis of FTD in the Brazilian scenario, considering the three levels of complexity of the health system: primary health care, secondary and tertiary levels. Diagnostic guidelines are proposed, including cognitive testing, behavioral and language assessments, laboratory tests, and neuroimaging.

The relationship between primary progressive aphasia and neurodegenerative dementia.

OBJECTIVE: To examine the relationship between primary progressive aphasia (PPA) and neurodegenerative dementia. METHODS: Subjects were selected from 1723 consecutive patients who had undergone a medical examination at the Kumamoto University Hospital Dementia Clinic, Japan, from April 2007 to October 2012. First, patients with semantic dementia (SD) and patients with progressive non-fluent aphasia were diagnosed by clinical diagnostic criteria for frontotemporal lobar degeneration. Next, in the same cohort, patients with PPA were diagnosed according to the recent international consensus criteria. The relationship and clinical symptoms including language and psychiatric symptoms in each patient group were then compared. RESULTS: In all, 12 of 27 SD patients fulfilled both SD and semantic variant PPA criteria (SD+PPA+ group), whereas the other 15 who met the SD criteria could not be included in the semantic variant PPA group due to prominent behavioural disturbances (SD+PPA- group). No significant differences in clinical characteristics and language functions were found between these 2 groups. Neuropsychiatric symptoms were more severe in the SD+PPA- group. CONCLUSION: The results suggest the possibility that SD and semantic variant PPA may be identical, regardless of different severities of behavioural disturbance. When considering the language disorder of neurodegenerative dementia, it may be more important to diagnose the subtype of language disorder the patient has than to emphasise isolated language deficits.

The atrophy pattern in the subtypes of frontotemporal lobar degeneration and Alzheimer disease by structural MRI / 中华放射学杂志

Objective To analyze the patterns of cortical atrophy of the two subtypes of frontotemporal lobar degeneration (FTLD ),behavioural-vsriant frontotemporal dementia (bvFTD ) and primary progressive aphasia (PPA).And to compare them with that of Alzheimer disease (AD) to provide an objective basis for early diagnosis and differential diagnosis.MethodsA total of 83 patients were enrolled in this study and there were 30 patients with cognitively normal controls (CN),30 with AD and 23 with FTLD (10 with bvFTD,13 with PPA).Philips 3.0 T TX scanner and 8 channel head coil was employed.Three dimensional turbo fast echo(3D-TFE)T1WI sequence with high resolution was used to collect the volume data of gray matter.3D-TFE T1 WI images were normalized and segmented into gray matter map for statistical analysis by SPM 8 and VBM 8.The false discovery rate (FDR) was adopted in P value adjustment,P ＜ 0.001,and the cluster size was set at 5.The full width at half maximum (FWHM ) was set at 4 mm for the smoothing.Paired t test was used for statistics.ResultsIn bvFTD,PPA and AD groups,there were diffuse regions with reduced volume in cerebral cortex and subcortical structures (such as the hippocampus,the amygdala,the caudate nuclei,et al).The most obvious atrophic region in bvFTD and PPA group was found in the frontotemporal.Compared with AD,gray matter atrophy in bvFTD was found in brain regions including bilateral temporal lobes,bilateral superior temporal pole gyri,bilateral middle temporal pole gyri,right fusiform gyrus and bilateral frontal lobes.Among them,temporal and frontal lobes atrophy had obvious right partial lateralizing,with 14 301 voxels in right temporal lobe and 5105 in left (t =-5.03,P＜0.05).The number of atrophy voxels in right and left frontal lobe were 1344 and 125 (t =3.45,P ＜0.05).The left temporooccipital lobe atrophy was more obvious than the right in PPA,with 15 637 voxels in left and 10 723 in right ( t =- 2.65,P ＜ 0.05 ).ConclusionsThere are different brain gray matter atrophy patterns in bvFD,PPA and AD.Among them,bvFTD has asymmetric right frontal and temporal lobe atrophy,which may be related to characteristic personality changes.On the other hand,the asymmetric atrophy in left temporooccipital lobe may be responsible for the aphasis of patients with PPA.

Avaliação fonoaudiológica da deglutição na demência frontotemporal / Phonoaudiological swallowing evaluation in frontotemporal dementia

Introdução: A deglutição e suas características principais ainda são desconhecidas na demência frontotemporal. Objetivos: Caracterizar a deglutição e o comportamento alimentar de pacientes com diagnóstico de demência frontotemporal que apresentam a variante comportamental (DFTvc) e a afasia progressiva primária (APP). Caracterizar os pacientes com DFT e seus cuidadores. Descrever aspectos cognitivos e comportamentais, funcionalidade global, comunicação funcional, e a funcionalidade da deglutição na DFT. Descrever os problemas de deglutição e do comportamento alimentar na DFTvc e APP. Correlacionar os aspectos cognitivos e comportamentais, funcionalidade global e a comunicação com as características da deglutição. Identificar fatores preditivos da piora da funcionalidade da deglutição e do comportamento alimentar na DFT. Avaliar o comportamento dos instrumentos empregados. Desenvolver a versão reduzida do Questionário de Habilidades de Alimentação e Deglutição nas Demências e do Questionário de Comunicação Funcional na Afasia. Método: Este estudo incluiu 46 indivíduos com DFT nas fases leve, moderada e grave, e seus 46 cuidadores. O Mini exame do estado mental (MEEM) e o Mini exame do estado mental grave (MEEM-g) foram usados para avaliar os aspectos cognitivos. A Escala de estadiamento da demência (CDR-DLFT) foi usada para confirmar a fase da doença. O Inventário Neuropsiquiátrico (INP) foi aplicado para investigar os problemas comportamentais. A Bateria de Avaliação Frontal (BAF) investigou as funções executivas. O Índice das Atividades de Vida Diária (Katz), Questionário para Avaliação da Comunicação Funcional na Afasia (QACFA) e a Escala de funcionalidade da deglutição (EFD) avaliaram as habilidades funcionais. O Questionário de Habilidades de Alimentação e Deglutição nas Demências (QHADD) avaliou as dificuldades na deglutição e alimentação. Resultados: Os grupos DFTvc e APP não mostraram diferença estatisticamente significante no MEEM,...

Introduction: Swallowing and its main characteristics are still unknown in frontotemporal dementia. Objectives: To characterize swallowing and feeding behavior of patients with frontotemporal dementia who have behavioral variant (bvFTD) and primary progressive aphasia (PPA). To characterize patients with FTD and their caregivers.To describe cognitive and behavioral aspects, functionalstatus, functional communication, and swallowing function in FTD.To describe swallowing problems and feeding behavior in bvFTD and PPA. To correlate cognitive and behavioral aspects, functional status, and communication with swallowing. To identify predictive factors associated with worsening of functionality of swallowing and feeding behavior in FTD. To evaluate the instruments used. To develop reduced versions of: "Assessment of Feeding and Swallowing Difficulties in Dementia" and "Functional Outcome Questionnaire Aphasia". Method: This study included 46 individuals with FTD in mild, moderate and severe phases, and their 46 caregivers. The Mini mental state examination (MMSE) and the Severe Mini mental state examination (SMMSE) were used to assess the cognitive aspects. The FTLD-modified Clinical Dementia Rating scale (FTLD-CDR) was used to confirm the stage of the disease. The Neuropsychiatric Inventory (NPI) was applied to investigate the behavioral problems. The Frontal Assessment Battery (FAB) investigated executive functions. The Index of Activities of Daily Living (Katz), Functional Outcome Questionnaire- Aphasia and Swallowing rating scale (SRE) evaluated the functional abilities. The Assessment of Feeding and Swallowing Difficulties in Dementia (QHADD) evaluated the difficulties in swallowing and feeding. Results: bvFTD and PPA groups showed no statistically significant difference in MMSE, CDR and FAB. Caregivers of patients with bvFTD had more hours of care per day compared to patients with PPA (p < 0.05). The groups differed in SRE (p < 0.05). The behavioral...

Primary progressive aphasia:a case analysis / 中华神经科杂志

Objective Primary progressive aphasia (PPA) is a rare neurodegenerative disease barely reported in China. Methods Complete neuropsychological testing on memory, cognitive function and aphasia was carried out. The brain function and structure was also examined with MRI and PET imaging analysis. Results The characteristics of clinical manifestation, neuropsychological testing and brain imaging analysis were reported. Except anomic aphasia, no other abnormal neuropsychological testing as well as sign of the nervous system was found. However, a significant atrophy and decrease in glucose metabolism of the left temporal lobe was observed.Conclusion PPA is clinically characterized by progressive anomic aphasia without impairment of the cognitive function and other abnormal sign of central nervous system. Atrophy of the frontal and temporal lobe in the dominant hemisphere provides strong support for the diagnosis.