RESUMO
BACKGROUND: Semantic dementia (SD), a subtype of frontotemporal dementia, manifests as verbal symptoms, including social and behavioural deficits, associated with focal atrophy of the frontotemporal lobes. This study aimed to clarify the experiences of individuals with early-onset SD receiving speech and language rehabilitation (hereafter referred to as 'rehabilitation'), with the intent of making it routine, as well as the experiences of their families. METHODS: Individual interviews were conducted with nine families with members who had adopted rehabilitation. Verbatim transcripts were used as data, and analyzed inductively according to the content analysis process. RESULTS: The family members realised the changes in the personality and behaviour of the individual with SD early, to the extent that they thought the individual with SD was different from before and were distressed by the loss of verbal communication. Nevertheless, the family members found a way to communicate by maintaining residual functions through rehabilitation and utilising their unique relationship with the individual with SD. CONCLUSIONS: It is important to carefully explain the characteristics of the disease and the long-term significance of rehabilitation to individuals with SD and their families in the early stages of the disease.
Assuntos
Demência Frontotemporal , Humanos , Demência Frontotemporal/diagnóstico , Testes Neuropsicológicos , Idioma , Família , Pesquisa QualitativaRESUMO
AIM: The aim of this study was to investigate initial symptoms of early-onset dementia (EOD) for each dementia subtype. METHOD: We conducted a nationwide, population-based EOD prevalence study in Japan. Data were collected through service providers for people with EOD. Initial symptoms were assessed in six domains: loss of memory, difficulty in word generation, irritability, loss of motivation, increased mistakes in the workplace or domestically, and unusual behaviours or attitudes other than those listed. RESULTS: Participants were 770 people with EOD. Characteristic initial symptoms were observed for each EOD subtype. Loss of memory was more common in early-onset Alzheimer's disease (75.7%, P < 0.001), difficulty in word generation was more common in early-onset vascular dementia (41.3%, P < 0.001), and loss of motivation, increased mistakes in the workplace or domestically, and unusual behaviours or attitudes other than those listed were more common in early-onset frontotemporal dementia (34.9%, P < 0.001; 49.4%, P < 0.001; 34.9%, P < 0.001, respectively). In addition, we observed gender differences whereby loss of memory was more common among women and irritability was more common among men. More than half of the participants were employed at symptom onset, and 57.2% of those who were employed at the onset had initial symptoms of increased mistakes in the workplace or domestically. CONCLUSION: This report reveals differences in the frequency of initial symptoms by EOD subtype. The results contribute to increasing public awareness of the initial symptoms of EOD, which will facilitate early diagnosis and social support.
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Demência , Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Adulto Jovem , Idade de Início , Demência/classificação , Demência/diagnóstico , Demência/epidemiologia , Inquéritos Epidemiológicos , Japão/epidemiologia , Avaliação de SintomasRESUMO
Frontotemporal dementia (FTD) is characterised by atrophy of the frontal and/or temporal lobes. People with FTD show language and emotional disturbances from onset, and communication problems usually affect people with FTD and their families even before diagnosis. These unique characteristics of FTD are not well understood and create substantial problems for people living with FTD and their families. This review explores the experiences of families of people living with FTD. Studies were selected and screened according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. We searched four bibliographic databases for articles up to February 2021 to identify qualitative data on the experiences of families. The Critical Appraisal Skills Programme checklist for qualitative studies was used to assess all included studies. Of 235 identified articles, we included six studies in the qualitative synthesis. Meta-ethnography was conducted to interpret families' experiences of people living with FTD. The emergent concepts were synthesised into five themes: Something is wrong with my loved one; No one fully understands; Existential pain of caring for a loved one with FTD; Increased burden owing to specific FTD symptoms; and Forced to adapt to new and unique ways of living with a loved one with FTD. This review highlighted families' confusion and suffering (which began in the early stages of the disease, and sometimes before diagnosis) and the difficulty of communicating with people with FTD. These findings have implications for future practice, as they demonstrate the positive effect on family life of appropriate support that is provided early, rather than after the disease has progressed.
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Demência Frontotemporal , Doença de Pick , Humanos , Dor , Pesquisa QualitativaRESUMO
AIM: People living with early-onset dementia (EOD) have specific social needs. Epidemiological studies are needed to obtain current information and provide appropriate service planning. This study aimed to clarify the current prevalence and subtype distribution of EOD, as well as the services frequently used by individuals with EOD. METHODS: A multisite, population-based, two-step study was conducted. Questionnaires were sent to 26 416 candidate facilities in 12 areas with a target population of 11 630 322 to inquire whether any individuals with EOD had sought services or stayed during the last 12 months (step 1). When "yes" responses were received, additional questionnaires were sent to the facilities both to complete and to distribute to the target individuals with EOD to obtain more detailed information, including the dementia subtype (step 2). RESULTS: In step 1, valid responses were obtained from 16 848 facilities (63.8%), and 4077 cases were identified. In step 2, detailed information was obtained for 1614 cases (39.6%) from the facilities and 530 cases (13.0%) from the individuals. The national EOD prevalence rate was estimated to be 50.9/100 000 population at risk (95% confidence interval: 43.9-57.9; age range, 18-64 years). The number of individuals with EOD was estimated to be 35 700 as of 2018. Alzheimer-type dementia (52.6%) was the most frequent subtype, followed by vascular dementia (17.1%), frontotemporal dementia (9.4%), dementia due to traumatic brain injury (4.2%), dementia with Lewy bodies/Parkinson's disease dementia (4.1%), and dementia due to alcohol-related disorders (2.8%). Individuals with EOD were most frequently identified at medical centers for dementia. CONCLUSION: The prevalence rate estimated in this study was comparable to those in previous studies in Japan. However, the subtype distribution differed, with Alzheimer-type dementia being the most prominent. Based on the case identification frequencies, medical centers for dementia are expected to continue to function as the primary special health service by providing quality diagnosis and post-diagnostic support for individuals with EOD.
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Demência/classificação , Demência/epidemiologia , Adolescente , Adulto , Idade de Início , Doença de Alzheimer/classificação , Doença de Alzheimer/epidemiologia , Demência Vascular/classificação , Demência Vascular/epidemiologia , Feminino , Inquéritos Epidemiológicos , Humanos , Japão/epidemiologia , Masculino , Pessoa de Meia-Idade , Prevalência , Adulto JovemRESUMO
BACKGROUND: Sleep disturbances in Alzheimer disease (AD) may affect behavioral and psychological symptoms of dementia (BPSD). Our aim was to elucidate the associations between sleep disturbances and other BPSD at different stages of AD. METHODS: This investigation was part of a multicenter-retrospective study in Japan (J-BIRD). Eligible for final analyses were 684 AD patients. Global severity of dementia was estimated using the Clinical Dementia Rating (CDR) scale. BPSD were assessed using the Neuropsychiatric Inventory (NPI). We analyzed the relationships between sleep disturbances and BPSD at different stages of AD according to the CDR score. RESULTS: Among the 684 AD patients, 146 (21.3%) had sleep disturbances. Patients with very early AD (CDR 0.5) and sleep disturbances had significantly more BPSD than those without sleep disturbances, as indicated by the higher prevalence of the following four NPI items: anxiety, euphoria, disinhibition, and aberrant motor behavior. In AD at CDR 2, (moderate AD) only one NPI item (irritability) was affected, while none was affected at CDR 1 (mild AD) and 3 (severe AD). Multiple regression analyses were performed in those with AD having various CDR scores. At CDR 0.5, the presence of sleep disturbances was associated with a high total NPI score (ß = 0.32, p < 0.001). However, other factors, including cognitive decline, age, gender, and years of education, were not significantly associated with the NPI score. At CDR 1 and 2, no factor was significantly related to BPSD. CONCLUSION: Sleep disturbances were strongly associated with other BPSD in the very early stage of AD. Copyright © 2016 John Wiley & Sons, Ltd.
Assuntos
Doença de Alzheimer/psicologia , Sintomas Comportamentais/psicologia , Transtornos Mentais/psicologia , Distúrbios do Início e da Manutenção do Sono/psicologia , Idoso , Idoso de 80 Anos ou mais , Doença de Alzheimer/complicações , Ansiedade/psicologia , Sintomas Comportamentais/epidemiologia , Estudos Transversais , Feminino , Humanos , Japão/epidemiologia , Masculino , Transtornos Mentais/epidemiologia , Transtornos Motores/psicologia , Testes Neuropsicológicos , Prevalência , Escalas de Graduação Psiquiátrica , Análise de Regressão , Estudos Retrospectivos , Índice de Gravidade de Doença , Distúrbios do Início e da Manutenção do Sono/epidemiologiaRESUMO
BACKGROUND: D-3-aminoisobutyrate, an intermediary product of thymine, is converted to 2-methyl-3-oxopropanoate using pyruvate as an amino acceptor by D-3-aminoisobutyrate-pyruvate aminotransferase (D-AIB AT; EC 2.6.1.40). A large amount of D-AIB AT is distributed in the kidney and liver; however, small amounts are found in the brain. Recently, D-AIB AT was reported to metabolize asymmetric dimethylarginine (ADMA) in vivo and was suggested to be an important enzyme for nitric oxide metabolism because ADMA is a competitive inhibitor for nitric oxide synthase. In this study, we examined the distribution of D-AIB AT in the rat brain further to understand its role. We measured D-AIB AT mRNA and protein expression using quantitative RT-PCR and Western blotting, and monitored its distribution using immunohistochemical staining. RESULTS: D-AIB AT was distributed throughout the brain, with high expression in the cortex and hippocampus. Immunohistochemical staining revealed that D-AIB AT was highly expressed in the retrosplenial cortex and in hippocampal neurons. CONCLUSION: Our results suggest that D-AIB AT is distributed in the examined- just the regions and may play an important role there.
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Córtex Cerebral/enzimologia , Hipocampo/enzimologia , Neurônios/enzimologia , Transaminases/metabolismo , Animais , Células Cultivadas , Masculino , Especificidade de Órgãos , Ratos , Ratos Wistar , Distribuição TecidualRESUMO
OBJECTIVE: Neuropsychiatric symptoms affect many patients with Alzheimer's disease (AD). ((11)C)Pittsburgh Compound-B (PIB) positron emission tomography (PET) has enabled the in vivo visualisation of brain amyloid-ß (Aß) deposition. This study exploratively investigated the correlation between brain Aß deposition measured by ((11)C)PIB PET and neuropsychiatric symptoms in AD. METHODS: Participants were 28 patients (15 women, 13 men) with PIB-positive AD. Clinical assessments included Mini-Mental State Examination, Clinical Dementia Rating scale, neuropsychiatry inventory (NPI) and frontal assessment battery. All patients underwent three-dimensional T1-weighted MRI and ((11)C)PIB PET. The distribution volume ratio (DVR), an index of ((11)C)PIB retention and, thus, Aß deposition, was estimated voxel by voxel from ((11)C)PIB PET data with partial volume correction. Voxel-based correlation analysis was performed to assess the relationships between DVR and each NPI subscale. Additionally, voxel-based analysis of covariance (ANCOVA) of the DVR images was performed between Patients with AD with and without each neuropsychiatric symptom. Voxel-based morphometry analysis of MRI was also performed. RESULTS: Apathy subscale was correlated with ((11)C)PIB retention in the bilateral frontal and right anterior cingulate. ((11)C)PIB retention was greater in the bilateral frontal cortex of patients with AD with apathy than those of without apathy. Overlapping areas between the two analyses were the bilateral orbitofrontal gyrus and left superior frontal gyrus. Other NPI subscales were not correlated with ((11)C)PIB retention. Voxel-based morphometry analysis of MRI showed no significant cluster of correlation between grey matter volume and NPI subscales. CONCLUSIONS: This study revealed that prefrontal Aß deposition correlates with apathy.
Assuntos
Doença de Alzheimer/metabolismo , Doença de Alzheimer/psicologia , Peptídeos beta-Amiloides/metabolismo , Apatia , Córtex Pré-Frontal/metabolismo , Idoso , Idoso de 80 Anos ou mais , Doença de Alzheimer/diagnóstico , Doença de Alzheimer/diagnóstico por imagem , Feminino , Neuroimagem Funcional , Humanos , Masculino , Pessoa de Meia-Idade , Córtex Pré-Frontal/diagnóstico por imagem , Escalas de Graduação Psiquiátrica , CintilografiaRESUMO
AIM: In Japan, the government and media have become aware of the issues of early onset dementia (EOD), but policies for EOD have not yet been established and support systems are inadequate. To provide practical data about EOD, a two-step postal survey was performed. METHODS: A questionnaire requesting information on EOD cases was sent to target institutions in five catchment areas in Japan. According to the answers from the institutions, we estimated the prevalence of EOD using census data and determined the illnesses causing EOD. As a quality control study, the authors reviewed every diagnosis in a quarter of the reported cases using the medical and psychiatric records and neuroimaging data. This study was conducted from 2006 to 2007. RESULTS: Information from 2469 patients was collected from 12,747 institutions, and 2059 subjects with EOD were identified. The estimated prevalence of EOD was 47.6 per 100,000 (95% confidence interval, 47.1-48.1) for all of Japan. Of the illnesses causing EOD, vascular dementia (VaD) was the most frequent (39.8%), followed by Alzheimer's disease. CONCLUSIONS: The prevalence of EOD in Japan appeared to be similar to that in Western countries. However, unlike previously reported international experience, VaD was the most frequent cause of EOD in all catchment areas in Japan.
Assuntos
Demência/epidemiologia , Adolescente , Adulto , Idade de Início , Demência Vascular/epidemiologia , Feminino , Humanos , Japão/epidemiologia , Masculino , Pessoa de Meia-Idade , Prevalência , Inquéritos e Questionários , Adulto JovemRESUMO
It is difficult to confirm a diagnosis of early-onset Alzheimer's disease (EOAD) because patients sometimes have non-specific cortical features, such as psychiatric symptoms, executive functional impairment, and pyramidal symptoms, along with typical symptoms, such as recent memory impairment and disorientation. We encountered a patient with multiple psychotic symptoms, finally diagnosed with EOAD on genetic testing. A right-handed sixty-year-old man, whose mother was suspected of having dementia, developed memory impairment at the age of fifty, disorientation at the age of fifty-six, and both visual hallucination and dressing apraxia at the age of fifty-nine. After admission to a psychiatric hospital for treatment, his symptoms disappeared with antipsychotic medication. However, his ADL were declining and so he was referred to our university hospital. He had frontal lobe symptoms, pyramidal signs, and extrapyramidal signs with severe dementia. Neuropsychological examinations were not possible because of sedation. On brain MRI, he showed diffuse atrophy of the cerebral cortex and hippocampus. HMPO-SPECT showed hypoperfusion of cerebral cortices diffusely. We decided to perform genetic testing because he had both family and alcohol abuse histories. He showed EOAD with V717I mutation of the amyloid precursor protein gene. After the discontinuation of antipsychotics, excessive sedation and extrapyramidal signs disappeared. A dose of 10 mg of donepezil was effective to improve motivation and activity, and his mini mental examination score was calculable after recovery. The case supports usefulness of applying genetic testing for Alzheimer's disease to patients with early onset dementia, even when they do not have a family history.
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Doença de Alzheimer/genética , Encéfalo/fisiopatologia , Mutação/genética , Doença de Alzheimer/tratamento farmacológico , Doença de Alzheimer/patologia , Doença de Alzheimer/psicologia , Donepezila , Testes Genéticos/métodos , Humanos , Indanos/uso terapêutico , Masculino , Pessoa de Meia-Idade , Piperidinas/uso terapêuticoRESUMO
OBJECTIVE: Although the relationship between cognitive function and plasma lipids has attracted attention, previous studies have shown conflicting results. One possible confounding factor is due to the influence of gene-related modulator. We investigated the relationship between cognitive function and lipid plasma levels of old age after controlling for apolipoprotein E (APOE) genotype. METHODS: One thousand three hundred ninety-five subjects without dementia age 65 and older participated in this study. They were divided into two groups, with and without APOE4 [E4 (+) and E4 (-)]. Plasma concentrations of high-density lipoprotein (HDL), low-density lipoprotein (LDL), triglyceride (TG), total cholesterol (TC) and apolipoprotein E (apoE) were measured. Associations between plasma concentrations of lipids and cognitive function were investigated for each group. RESULTS: We found a positive association between cognitive scores and plasma apoE level in both E4 (-) and E4 (+) groups. A positive relationship was also observed between cognitive score and HDL level in the E4 (-) group, but not in the E4 (+) group. No substantial association between cognitive score and LDL, TG, and TC levels was found in either of the groups. CONCLUSIONS: Our findings suggest that plasma apoE have a positive influence on cognitive function in both E4 (-) and E4 (+) groups, whereas the positive influence of plasma HDL was shown only in E4 (-) group. The identification of the influences of (APOE) genotype and the intracellular linkage among apoE and HDL metabolism is hoped for new preventive and therapeutic strategies for cognitive change of elderly.
Assuntos
Envelhecimento/psicologia , Apolipoproteína E4/genética , Apolipoproteínas E/genética , Colesterol/genética , Cognição/fisiologia , Lipoproteínas/sangue , Lipoproteínas/genética , Triglicerídeos/genética , Idoso , Envelhecimento/sangue , Envelhecimento/genética , Apolipoproteínas E/sangue , Colesterol/sangue , Feminino , Genótipo , Humanos , Masculino , Testes Neuropsicológicos , Triglicerídeos/sangueRESUMO
BACKGROUND: Alzheimer's disease (AD) is the most common form of dementia. Mutations in genes such as those encoding amyloid precursor protein (APP), presenilin 1 and presenilin 2, are responsible for early-onset familial AD. CASE PRESENTATION: In this study, we report a 275341 G > C (Val717Leu) mutation in the APP gene in a Japanese family with early onset AD by genetic screening. This mutation has previously been detected in European families. In the Japanese family we screened, the age at onset of AD was 47.1 ± 3.1 years old (n = 9; range, 42-52). The symptoms in the affected members included psychiatric vulnerability and focal signs such as pyramidal signs, epileptic seizures, and myoclonic discharges. An MR imaging study showed relatively mild atrophic changes in the bilateral hippocampus and cerebral cortices in all affected members compared with their clinical presentations. CONCLUSION: We conclude that the clinical features of Alzheimer's disease can be different even when caused by the same mutation in the APP gene. Further clinical and genetic studies are required to clarify the relationship between phenotypes and genotypes.
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Doença de Alzheimer/congênito , Doença de Alzheimer/genética , Precursor de Proteína beta-Amiloide/genética , Predisposição Genética para Doença/genética , Mutação/genética , Linhagem , Polimorfismo de Nucleotídeo Único/genética , Adulto , Feminino , Testes Genéticos , Humanos , Japão , Masculino , Pessoa de Meia-Idade , FenótipoRESUMO
BACKGROUND: The aim of the study was to estimate the prevalence of DSM-III-R major depressive episodes (MDEs), depressive symptoms cases (DSCs) (defined as a score of ≥6 on the Geriatric Depression Scale but falling short of MDE), and coexisting mild cognitive impairment (MCI) among Japanese community-dwelling older people. METHODS: Prevalence was estimated based on screening evaluation, individual interviews, and door-to-door visits. MDE and DSC were diagnosed, and the cognitive status of the participants was determined to be dementia, MCI, or normal. RESULTS: A total of 1888 subjects of 2698 candidates (70.0%) participated. The prevalence of MDE and DSC were estimated to be 4.5% (95% CI, 3.4-6.0) and 11.5% (95% CI, 4.2-28.0), respectively. MCI was more prevalent in subjects with depression (26.2%) than those with normal mood (17.9%). Although no prototypical profile of cognitive dysfunction was revealed, multiple MCI was more prevalent in subjects with depression (12.2%) than subjects with normal mood (3.8%). Conversely, subjects with MCI (26.3%) were more likely to develop depression compared with those with normal cognitive function (18.0%). CONCLUSIONS: The prevalence of depression in our subjects seems to be similar with that of previous studies. MCI was more prevalent in subjects with depression than those with normal mood. Individuals with depression showed no particular association with any of the four MCIs. Given that depression and MCI are often associated with each other and that MCI is a predictor for development of dementia, the risk of developing dementia in the depressed older people with coexisting MCI should be acknowledged.
Assuntos
Disfunção Cognitiva/epidemiologia , Transtorno Depressivo/epidemiologia , Atividades Cotidianas , Idoso , Idoso de 80 Anos ou mais , Comorbidade , Transtorno Depressivo Maior/epidemiologia , Feminino , Avaliação Geriátrica , Humanos , Japão/epidemiologia , Masculino , Testes Neuropsicológicos , PrevalênciaRESUMO
BACKGROUND: Memory impairment has been proposed as the most common early sign of Alzheimer's disease (AD). The aims of this work were to evaluate the risk of progression from mild memory impairment/no dementia (MMI/ND) to clinically diagnosable AD in a community-based prospective cohort and to establish the risk factors for progression from MMI/ND to AD in the elderly. METHODS: Elderly subjects aged over 65 years were selected from the participants in the first Nakayama study. MMI/ND was defined as memory deficit on objective memory assessment, without dementia, impairment of general cognitive function, or disability in activities of daily living. A total of 104 MMI/ND subjects selected from 1242 community-dwellers were followed longitudinally for five years. RESULTS: During the five-year follow-up, 11 (10.6%) subjects were diagnosed with AD, five (4.8%) with vascular dementia (VaD), and six (5.8%) with dementia of other etiology. Logistic regression analysis revealed that diabetes mellitus (DM) and a family history of dementia (within third-degree relatives) were positively associated with progression to AD, while no factor was significantly associated with progression to VaD or all types of dementia. CONCLUSIONS: DM and a family history of dementia were significant risk factors for progression from MMI/ND to clinically diagnosable AD in the elderly in a Japanese community.
Assuntos
Doença de Alzheimer , Disfunção Cognitiva , Progressão da Doença , Competência Mental , Rememoração Mental , Idoso , Idoso de 80 Anos ou mais , Doença de Alzheimer/diagnóstico , Doença de Alzheimer/epidemiologia , Doença de Alzheimer/etiologia , Doença de Alzheimer/psicologia , Disfunção Cognitiva/complicações , Disfunção Cognitiva/diagnóstico , Disfunção Cognitiva/psicologia , Função Executiva , Feminino , Humanos , Vida Independente , Japão/epidemiologia , Modelos Logísticos , Masculino , Testes Neuropsicológicos , Linhagem , Prognóstico , Estudos Prospectivos , Escalas de Graduação Psiquiátrica , Fatores de RiscoRESUMO
BACKGROUND: Semantic dementia (SD) has been recognized as a representative of dementia with presenile onset; however, recent epidemiological studies have shown that SD also occurs in the elderly. There have been few studies about the differences of clinical profiles between early-onset SD (EO-SD) and late-onset SD (LO-SD). Age-associated changes in the brain might cause some additional cognitive and behavioural profiles of LO-SD in contrast to the typical EO-SD cases. The aim of the present study was to clarify the characteristics of neuropsychological, and behavioural and psychological symptoms of dementia (BPSD) profiles of LO-SD patients observed in screening tests in comparison with EO-SD patients and late-onset Alzheimer's disease (LO-AD) patients as controls. METHODS: Study participants were LO-SD (n = 10), EO-SD (n = 15) and LO-AD (n = 47). We examined the Mini-Mental State Examination (MMSE), the Raven's Coloured Progressive Matrices (RCPM), the Short-Memory Questionnaire (SMQ), the Neuropsychiatric Inventory (NPI) and the Stereotypy Rating Inventory (SRI). RESULTS: Both SD groups scored significantly lower than the LO-AD patients in 'naming' of the MMSE. In the 'construction' score of the MMSE and the RCPM score, however, the LO-SD patients as well as the LO-AD patients were significantly lower than the EO-SD patients. In the SMQ score, 'euphoria' and 'disinhibition' scores of the NPI, the SRI total and subscale scores, both SD groups were significantly higher, whereas in the 'delusion' score of the NPI, both SD groups were significantly lower than the LO-AD patients. CONCLUSIONS: Visuospatial and constructive skills of LO-SD patients might be mildly deteriorated compared with EO-SD patients, whereas other cognitive and behavioural profiles of LO-SD are similar to EO-SD. Age-associated changes in the brain should be considered when we diagnose SD in elderly patients.
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Degeneração Lobar Frontotemporal/diagnóstico , Degeneração Lobar Frontotemporal/psicologia , Testes Neuropsicológicos , Idade de Início , Idoso , Doença de Alzheimer/diagnóstico , Doença de Alzheimer/psicologia , Sintomas Comportamentais , Estudos de Casos e Controles , Cognição , Diagnóstico Diferencial , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Comportamento EstereotipadoRESUMO
BACKGROUND/AIMS: The aim of this study is to examine the clinical symptoms in a number of semantic dementia (SD) patients and to reveal the longitudinal progression and clinical course of these distinctive symptoms of SD. METHODS: 19 consecutive SD patients were examined. Symptoms were classified into 23 distinct categories: behavioral symptoms, language and cognitive symptoms and symptoms concerning the impairment of activities of daily living (ADL). We divided patients into two subgroups, left- and right-dominant SD, and compared the onset of each symptom. RESULTS: Language impairments occurred as the initial symptom in 16 cases. At the first examination, all cases showed both anomia and impairment of word comprehension. By around 3 years after onset, almost all language impairments were observed. Approximately 3-5 years after onset, prosopagnosia and behavioral symptoms appeared. Around the period when the loss of the language faculty and apathy became remarkable, impairment of ADL appeared. Patients spent all day in bed at this stage. Moreover, prosopagnosia appeared significantly earlier in right-dominant SD. CONCLUSION: Our findings clarify the progression of distinctive symptoms of SD patients. It is necessary to create a treatment strategy for SD patients with such a disease-specific course of SD.
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Demência/psicologia , Atividades Cotidianas , Idoso , Idoso de 80 Anos ou mais , Envelhecimento/psicologia , Anomia/psicologia , Comportamento/fisiologia , Cognição/fisiologia , Educação , Feminino , Seguimentos , Lateralidade Funcional/fisiologia , Humanos , Entrevista Psicológica , Idioma , Estudos Longitudinais , Masculino , Pessoa de Meia-Idade , Testes Neuropsicológicos , Prosopagnosia/psicologia , Caracteres Sexuais , Comportamento Estereotipado , Comportamento VerbalRESUMO
BACKGROUND AND PURPOSE: Few studies are available that have addressed the prevalence of early-onset dementia (EOD), including early-onset Alzheimer disease and other forms of dementia in Japan. METHODS: A 2-step postal survey was sent to all of the 2475 institutions providing medical or care services for individuals with dementia in Japan's Ibaraki prefecture (population, 2 966 000) requesting information on EOD cases. Data were then reviewed and collated. RESULTS: We identified 617 subjects with EOD. The estimated prevalence of EOD in the target population was 42.3 per 100 000 (95% CI, 39.4 to 45.4). Of the illnesses that cause EOD, vascular dementia was the most frequent (42.5%) followed by Alzheimer disease (25.6%), head trauma (7.1%), dementia with Lewy bodies/Parkinson disease with dementia (6.2%), frontotemporal lobar degeneration (2.6%), and other causes (16.0%). CONCLUSIONS: The prevalence of EOD in Japan appeared to be similar to that in Western countries with the notable exception that vascular dementia was the most frequent cause of EOD in Japan.
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Demência/epidemiologia , Demência/etiologia , Adulto , Idade de Início , Doença de Alzheimer/complicações , Doença de Alzheimer/epidemiologia , Traumatismos Craniocerebrais/complicações , Traumatismos Craniocerebrais/epidemiologia , Demência Vascular/complicações , Demência Vascular/epidemiologia , Feminino , Inquéritos Epidemiológicos , Humanos , Japão/epidemiologia , Masculino , Pessoa de Meia-Idade , Prevalência , Adulto JovemRESUMO
BACKGROUND/AIM: Some recent studies mentioned that late-onset frontotemporal dementia (FTD) is more common than previously assumed. Although much research has been done in the field, there are no systematic studies which have compared clinical characteristics of early- and late-onset FTD. The aim of this study was to compare cognitive function and psychiatric symptoms in patients with early- and late-onset FTD. METHODS: Study participants were consecutive outpatients. There were 35 FTD patients; their mean age at onset was 63.0 years. We studied sex, education, duration from onset to consultation, Clinical Dementia Rating (CDR) scores, Mini-Mental State Examination (MMSE) scores, Raven's Coloured Progressive Matrices (RCPM) scores, and Neuropsychiatric Inventory (NPI) scores at first consultation of early- and late-onset FTD patients. RESULTS: There were no significant differences in sex ratio, education, CDR scores, and duration from onset to consultation. There were significant differences in the total MMSE scores, 'three-word recall task', 'construction task', and RCPM scores; late-onset groups scored significantly lower than early-onset groups. There were significant differences in the apathy domain of NPI and total NPI scores; late-onset groups scored significantly higher than early-onset groups. CONCLUSION: Late-onset FTD patients may have memory and visuospatial deficits in addition to their behavioural changes, even if they are clinically diagnosed according to consensus diagnostic criteria. They also present more apathy, and they may have a different histolopathological background.
Assuntos
Transtornos Cognitivos/diagnóstico , Transtornos Cognitivos/psicologia , Demência/diagnóstico , Demência/psicologia , Idade de Início , Idoso , Feminino , Humanos , Japão , Masculino , Entrevista Psiquiátrica Padronizada , Pessoa de Meia-Idade , Testes Neuropsicológicos , Pacientes AmbulatoriaisRESUMO
Brugada syndrome is an arrhythmogenetic disease characterized by electrocardiographic ST segment elevation in right precordial leads, which is called "coved type", and an increased risk of sudden death as the result of ventricular fibrillation. We presented a case of Brugada syndrome with a convulsive seizure, during administration of a tricyclic antidepressant for the treatment of a depressive state. A 43-year-old man with bipolar II disorder and obsessive-compulsive disorder was admitted to our hospital for the treatment of a depressive state. There was no medical history of heart failure. Nortriptyline was effective for his depressive as well as for obsessive symptoms. During the treatment, however, he presented a convulsive syncope. Electrocardiography (ECG) showed "coved type" ST segment elevation, and the patient consulted a cardiologist. Electrophysiological study revealed Brugada syndrome, and an implantable cardioverter defibrillator was placed. An overdose of antidepressants has been reported to produce a Brugada-type ECG because of its Na channel antagonism. However, in the present case, the abnormal ECG findings occurred following a usual dosage of nortriptyline. Thus, it is suggested that Brugada syndrome is related to a susceptibility to antidepressants in the present case. Every psychiatrist managing antidepressant therapy should be aware of Brugada syndrome and this ECG pattern, which may be a marker of sudden death.
Assuntos
Antidepressivos Tricíclicos/efeitos adversos , Síndrome de Brugada/induzido quimicamente , Nortriptilina/efeitos adversos , Síncope/induzido quimicamente , Adulto , Antidepressivos Tricíclicos/administração & dosagem , Arritmias Cardíacas/induzido quimicamente , Morte Súbita Cardíaca/etiologia , Humanos , Masculino , Nortriptilina/administração & dosagem , Transtorno Obsessivo-Compulsivo/tratamento farmacológicoRESUMO
BACKGROUND/AIMS: Behavioral and psychological symptoms of dementia (BPSDs) negatively impact the prognosis of dementia patients and increase caregiver distress. The aims of this study were to clarify the differences of trajectories of 12 kinds of BPSDs by disease severity in four major dementias and to develop charts showing the frequency, severity, and associated caregiver distress (ACD) of BPSDs using the data of a Japan multicenter study (J-BIRD). METHODS: We gathered Neuropsychiatric Inventory (NPI) data of patients with Alzheimer's disease (AD; n = 1091), dementia with Lewy bodies (DLB; n = 249), vascular dementia (VaD; n = 156), and frontotemporal lobar degeneration (FTLD; n = 102) collected during a 5-year period up to July 31, 2013 in seven centers for dementia in Japan. The NPI composite scores (frequency × severity) of 12 kinds of items were analyzed using a principal component analysis (PCA) in each dementia. The factor scores of the PCA were compared in each dementia by disease severity, which was determined with Clinical Dementia Rating (CDR). RESULTS: Significant increases with higher CDR scores were observed in 1) two of the three factor scores which were loaded for all items except euphoria in AD, 2) two of the four factor scores for apathy, aberrant motor behavior (AMB), sleep disturbances, agitation, irritability, disinhibition, and euphoria in DLB, and 3) one of the four factor scores for apathy, depression, anxiety, and sleep disturbances in VaD. However, no increases were observed in any of the five factor scores in FTLD. CONCLUSIONS: As dementia progresses, several BPSDs become more severe, including 1) apathy and sleep disturbances in AD, DLB, and VaD, 2) all of the BPSDs except euphoria in AD, 3) AMB, agitation, irritability, disinhibition, and euphoria in DLB, and 4) depression and anxiety in VaD. Trajectories of BPSDs in FTLD were unclear.