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1.
J Aging Soc Policy ; : 1-16, 2023 Feb 22.
Artigo em Inglês | MEDLINE | ID: mdl-36814064

RESUMO

The scholarly debate on advance directives (ADs) in the context of dementia is mainly built around ethical arguments. Empirical studies that shed light into the realities of ADs of persons living with dementia are few and far between and too little is known about the effect of national AD legislation on such realities. This paper offers insight into the preparation phase of ADs according to German legislation in the context of dementia. It presents results from a document analysis of 100 ADs and from 25 episodic interviews with family members. Findings show that drafting an AD involves family members and different professionals in addition to the signatory, whose cognitive impairment differed considerably at the time of preparing the AD. The involvement of family members and professionals is at times problematic, which prompts the question of how much and what kind of involvement of others turns an AD of a person living with dementia into an AD about a person living with dementia. The results invite policy makers to critically review legislation on ADs from the perspective of cognitively impaired persons, who might find it difficult to protect themselves from inappropriate involvement when completing an AD.

2.
Alzheimer Dis Assoc Disord ; 36(1): 44-51, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35001030

RESUMO

BACKGROUND: Primary progressive aphasia (PPA) may present with three distinct clinical sybtypes: semantic variant PPA (svPPA), nonfluent/agrammatic variant PPA (nfvPPA), and logopenic variant PPA (lvPPA). OBJECTIVE: The aim was to examine the utility of the German version of the Repeat and Point (R&P) Test for subtyping patients with PPA. METHOD: During the R&P Test, the examiner reads out aloud a noun and the participants are asked to repeat the word and subsequently point to the corresponding picture. Data from 204 patients (68 svPPA, 85 nfvPPA, and 51 lvPPA) and 33 healthy controls were analyzed. RESULTS: Controls completed both tasks with >90% accuracy. Patients with svPPA had high scores in repetition (mean=9.2±1.32) but low scores in pointing (mean=6±2.52). In contrast, patients with nfvPPA and lvPPA performed comparably in both tasks with lower scores in repetition (mean=7.4±2.7 for nfvPPA and 8.2±2.34 for lvPPA) but higher scores in pointing (mean=8.9±1.41 for nfvPPA and 8.6±1.62 for lvPPA). The R&P Test had high accuracy discriminating svPPA from nfvPPA (83% accuracy) and lvPPA (79% accuracy). However, there was low accuracy discriminating nfvPPA from lvPPA (<60%). CONCLUSION: The R&P Test helps to differentiate svPPA from 2 nonsemantic variants (nfvPPA and lvPPA). However, additional tests are required for the differentiation of nfvPPA and lvPPA.


Assuntos
Afasia Primária Progressiva , Afasia Primária Progressiva não Fluente , Afasia Primária Progressiva/diagnóstico , Humanos , Idioma
3.
BMC Geriatr ; 22(1): 214, 2022 03 16.
Artigo em Inglês | MEDLINE | ID: mdl-35296254

RESUMO

BACKGROUND: Polypharmacy is common in people with dementia. The use of psychotropic drugs (PDs) and other, potentially inappropriate medications is high. The aims of this cross-sectional study were 1) to investigate the use of drugs in people with advanced dementia (PWAD), living at home or in long term care (LTC); 2) to focus on PD use; and 3) to identify determinants of PD use. METHODS: The study was performed in the context of EPYLOGE (IssuEs in Palliative care for people in advanced and terminal stages of YOD and LOD in Germany). 191 PWAD were included. All drugs that were administered at the date of the examination were recorded. Multiple logistic regression analysis identified determinants of PD use. RESULTS: 96% of PWAD received medication with a median number of four drugs. 49.7% received five or more drugs. According to the Beers Criteria 39% of PWAD ≥ 65 years received at least one potentially inappropriate medication. 79% of PWAD were treated with PDs. Older PWAD and PWAD living in LTC facilities received significantly more drugs than younger PWAD, and PWAD living at home, respectively. Dementia etiology was significantly associated with the use of antipsychotics, antidepressants and sedative substances. Place of living was associated with the use of pain medication. Behavioral disturbances were associated with the use of antipsychotics and sedative substances. CONCLUSIONS: To mitigate the dangers of polypharmacy and medication related harm, critical examination is required, whether a drug is indicated or not. Also, the deprescribing of drugs should be considered on a regular basis. TRIAL REGISTRATION: Clinicaltrial.gov, NCT03364179 . Registered 6 December 2017.


Assuntos
Demência , Psicotrópicos , Antipsicóticos/uso terapêutico , Estudos Transversais , Demência/tratamento farmacológico , Humanos , Hipnóticos e Sedativos/uso terapêutico , Polimedicação , Psicotrópicos/efeitos adversos
4.
Nervenarzt ; 93(9): 912-920, 2022 Sep.
Artigo em Alemão | MEDLINE | ID: mdl-35771240

RESUMO

BACKGROUND: Guidelines for the treatment of behavioral and psychological symptoms of dementia (BPSD) recommend that if antipsychotics are necessary, these should be used only for a limited time and with the lowest possible dose. OBJECTIVE: In order to raise awareness of the risks of antipsychotic treatment in patients with dementia and the resulting need for time-limited and as low-dose antipsychotic treatment as possible, recommendations for deprescribing of antipsychotics in people with dementia have been developed. MATERIAL AND METHODS: The recommendations were developed in a multistage process: 1. Literature search with special appreciation of agreed guideline recommendations and results of an intervention study (READY study) 2. Discussion and consensus in a panel of experts, 3. Online survey of medical dementia experts working in Germany. RESULTS: From the 51 statements that were initially drafted 32 statements on antipsychotic deprescribing were formulated after the discussion in the multiprofessional expert panel. Of the 32 statements 25 were finally implemented after agreement by the experts in the online survey. DISCUSSION: In Germany, there are so far no specific recommendations for the reduction and discontinuation of antipsychotics used to treat BPSD in people with dementia. The article is intended as recommendations for general practitioners and specialists. The recommendations are not guidelines and do not replace personal responsibility. The individual approach always depends on the individual treatment situation.


Assuntos
Antipsicóticos , Demência , Desprescrições , Antipsicóticos/uso terapêutico , Demência/tratamento farmacológico , Demência/psicologia , Redução da Medicação , Alemanha , Humanos
5.
BMC Palliat Care ; 19(1): 30, 2020 Mar 12.
Artigo em Inglês | MEDLINE | ID: mdl-32164707

RESUMO

BACKGROUND: Since people with advanced dementia are usually not able to make complex decisions, it is usually the family caregivers, as proxies, who have to decide on treatments and their termination. However, these decisions are difficult for the caregivers to make, as they are often inadequately informed and cannot properly assess the consequences; moreover, they are concerned about harming the sick person. We aimed to first develop an informative booklet about palliative care issues for caregivers of people with advanced dementia. Secondly, we aimed to investigate a change in family caregivers' knowledge regarding palliative care issues and caregivers' involvement in medical and care decisions before and after studying this booklet. METHODS: A first version of the booklet was drafted by an experienced psychiatrist and palliative care specialist based on existing booklets and guidelines; necessary cultural adaptions were taken into consideration. A nominal group process was conducted to develop the informative guide. In order to investigate the acceptance of the booklet and the possibility to implement it, 38 patient-caregiver dyads were recruited, and caregivers were interviewed both before receiving the booklet and after 3 months of receiving the booklet. RESULTS: Experts from various disciplines collaborated on a German booklet for family caregivers of people with advanced dementia as an information aid regarding issues of palliative care. The subsequent test showed that all caregivers had experienced a personal benefit from the booklet. Caregivers had a significant gain of knowledge after provision of the booklet. A large proportion of caregivers who had not previously considered and/or discussed medical topics reported that they had done so within 3 months after obtaining the booklet, or planned to do so in the near future. CONCLUSIONS: The caregivers valued the comprehensible, concise and well-structured information guide on palliative care issues in advanced dementia. They agreed it increases knowledge and prompts decision making and therefore should be developed in many languages and disseminated among family caregivers of people with dementia. TRIAL REGISTRATION: clinicaltrial.gov, NCT03548142. Retrospectively registered 7 June 2018.


Assuntos
Cuidadores/psicologia , Demência/terapia , Cuidados Paliativos/métodos , Cuidados Paliativos/normas , Idoso , Cuidadores/estatística & dados numéricos , Demência/psicologia , Feminino , Alemanha , Humanos , Masculino , Pessoa de Meia-Idade , Cuidados Paliativos/estatística & dados numéricos , Estudos Retrospectivos
6.
BMC Psychiatry ; 18(1): 271, 2018 08 31.
Artigo em Inglês | MEDLINE | ID: mdl-30170575

RESUMO

BACKGROUND: Scientific research on palliative care in dementia is still underdeveloped. In particular, there are no research studies at all on palliative care issues in young onset dementia (YOD), although significant differences compared to late onset dementia (LOD) are expected. Most studies have focused on persons with dementia in long term care (LTC) facilities but have neglected persons that are cared for at home. We hypothesize that unmet care needs exist in advanced and terminal stages of YOD and LOD and that they differ between YOD and LOD. METHODS/DESIGN: The EPYLOGE-study (IssuEs in Palliative care for people in advanced and terminal stages of Young-onset and Late-Onset dementia in GErmany) aims to prospectively assess and survey 200 persons with YOD and LOD in advanced stages who are cared for in LTC facilities and at home. Furthermore, EPYLOGE aims to investigate the circumstances of death of 100 persons with YOD and LOD. This includes 1) describing symptoms and management, health care utilization, palliative care provision, quality of life and death, elements of advance care planning, family caregivers' needs and satisfaction; 2) comparing YOD and LOD regarding these factors; 3) developing expert-consensus recommendations derived from the study results for the improvement and implementation of strategies and interventions for palliative care provision; 4) and communicating the recommendations nationally and internationally in order to improve and adapt guidelines, to change current practice and to give a basis and perspectives for future research projects. The results will also be communicated to patients and their families in order to counsel and support them in their decision making processes and their dialogue with professional caregivers and physicians. DISCUSSION: EPYLOGE is the first study in Germany that assesses palliative care and end-of-life issues in dementia. Furthermore, it is the first study internationally that focuses on the specific palliative care situation of persons with YOD and their families. EPYLOGE serves as a basis for the improvement of palliative care in dementia. TRIAL REGISTRATION: The study is registered in ClinicalTrials.gov ( NCT03364179 ; Registered: 6. December 2017.


Assuntos
Cuidadores/psicologia , Demência/psicologia , Pesquisas sobre Atenção à Saúde/métodos , Cuidados Paliativos/psicologia , Aceitação pelo Paciente de Cuidados de Saúde/psicologia , Adulto , Idade de Início , Idoso , Tomada de Decisões , Demência/terapia , Feminino , Alemanha , Humanos , Masculino , Pessoa de Meia-Idade , Cuidados Paliativos/métodos , Estudos Prospectivos , Qualidade de Vida , Projetos de Pesquisa
7.
Int Psychogeriatr ; 27(12): 1939-44, 2015 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-26424328

RESUMO

BACKGROUND: It may be assumed that increased public awareness of dementia due to Alzheimer's disease (AD) together with the availability of efficacious treatment will result in diagnostic evaluation at earlier stages of cognitive decline and diagnosis of dementia due to AD at earlier stages. METHODS: All persons that were examined at a university based memory clinic, in Germany, between 1985 and 2009 were included. RESULTS: In the 3,951 persons identified, linear regression analysis revealed a positive association between Mini Mental State Examination (MMSE) score and year of initial examination (yearIE) (ß = 0.266; p < 0.001). In the 1,821 patients diagnosed with dementia due to AD, a positive association between MMSE score and yearIE (ß = 0.230; p < 0.001) was revealed. MMSE scores were higher (ß = 0.195; p < 0.001) after the introduction of cholinesterase inhibitors in Germany in 1997. CONCLUSIONS: Diagnostic evaluation of individuals occurred at progressively earlier stages of cognitive decline. Dementia due to AD was diagnosed at progressively earlier stages, and this trend was associated with the availability of efficacious treatment. This is the first study on changes in patient referral and diagnosis based on a continuous 25 years period.


Assuntos
Doença de Alzheimer/complicações , Transtornos Cognitivos/diagnóstico , Diagnóstico Precoce , Transtornos da Memória/diagnóstico , Encaminhamento e Consulta/tendências , Adulto , Idoso , Idoso de 80 Anos ou mais , Doença de Alzheimer/tratamento farmacológico , Inibidores da Colinesterase/uso terapêutico , Feminino , Previsões , Alemanha , Humanos , Modelos Lineares , Masculino , Pessoa de Meia-Idade , Testes Neuropsicológicos , Escalas de Graduação Psiquiátrica , Adulto Jovem
8.
Gerontologist ; 64(4)2024 Apr 01.
Artigo em Inglês | MEDLINE | ID: mdl-37650918

RESUMO

BACKGROUND AND OBJECTIVES: German legislation establishes advance directives (ADs) as legally binding instruments that all involved parties need to adhere to. This applies also to family members who have been authorized as official surrogates of the AD's author. As surrogates, they are expected to make sure that the AD is being implemented. Our study aims at uncovering how family members experience their legally assigned role as an advocate of an AD. RESEARCH DESIGN AND METHODS: We conducted 25 episodic interviews with family surrogates of persons living with dementia and used thematic analysis to make sense of our interview data. RESULTS: Family surrogates expressed scepticism toward ADs as instruments for planning end-of-life care. They did not necessarily follow the decisions expressed in the AD. We found cases in which family surrogates intentionally refrained from bringing the ADs to the attention of health care providers as well as cases in which the ADs conflicted with self-related interests of family surrogates. DISCUSSION AND IMPLICATIONS: Our findings lead us to critique the legal construction of family surrogates as advocates of the AD. Family members are directly and enduringly affected by the end-of-life care decisions that the AD sets out and are thus no neutral agents who have no stake in the matter. Expecting them to simply transmit decisions formulated in the AD means expecting them to render their own interests irrelevant-which, arguably, might be too much to ask.


Assuntos
Demência , Cuidados Paliativos na Terminalidade da Vida , Assistência Terminal , Humanos , Diretivas Antecipadas , Pesquisa Qualitativa
9.
J Alzheimers Dis ; 81(2): 843-852, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33843681

RESUMO

BACKGROUND: End of life symptoms and symptom management as well as the quality of dying (QoD) of persons with advanced dementia (PWAD) have not yet been systematically studied in Germany. OBJECTIVE: 1) To investigate symptoms, treatment and care at the end of life, advance care planning, and circumstances of death of recently deceased PWAD; 2) To determine whether there are differences between young and late onset dementia (YOD and LOD). METHODS: The study was performed in the context of the project EPYLOGE (IssuEs in Palliative care for persons in advanced and terminal stages of Young-onset and Late-Onset dementia in Germany). Closest relatives of recently deceased patients with advanced YOD (N = 46) and LOD (N = 54) living at home or in long term care were interviewed. RESULTS: Circumstances of death, symptoms, and treatment appeared to be similar between YOD and LOD, except that persons with LOD had significantly more somatic comorbidities and were admitted to hospital in the last three months of life more often than persons with LOD. At end of life, 60% of PWAD appeared to be "at peace". Difficulty swallowing, gurgling, shortness of breath, and discomfort were observed most frequently. Large interindividual differences in suffering and QoD were present. Determinants of QoD were not identified. CONCLUSION: Our findings suggest that low QoD was caused by inadequate recognition and/or insufficient treatment of burdensome physical and emotional symptoms. PWADs' needs should be assessed regularly, and strategies focusing on treatment and implementing support for both the patient and caregiver must be established.


Assuntos
Idade de Início , Cuidadores/psicologia , Demência/psicologia , Cuidados Paliativos/psicologia , Planejamento Antecipado de Cuidados , Idoso , Idoso de 80 Anos ou mais , Demência/reabilitação , Feminino , Humanos , Assistência de Longa Duração , Masculino , Qualidade de Vida
10.
Front Neurol ; 12: 647396, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34385968

RESUMO

Background and Objectives: Data on suicidal ideation, behavior and the risk factors in patients with dementia is scarce. To evaluate the prevalence of death wishes, suicidal ideation, and suicidal behavior of young (YOD) and late onset dementia (LOD) and to identify risk factors for suicidal ideation and behavior. Methods: We interviewed 157 family caregivers of patients with advanced dementia using questions from the Columbia-Suicide Severity Rating Scale to gather information about suicidal ideation and behavior before the onset of symptoms of dementia, after the onset of dementia and within 30 days prior to the interview. At the time of the interview, we also assessed disease severity, cognitive function, and other psychological, behavioral and physical symptoms of the patients as well as the caregivers' psychological well-being. Results: Forty four (28%) of the patients expressed suicidal ideation or behavior at some time after the onset of symptoms, and 14 (9%) of these within the month prior to the assessment. Two patients had attempted suicide after the onset of dementia. There were no statistically significant differences between patients with and without suicidal ideations or behavior with regards to demographics or age at onset of dementia. In patients with advanced dementia, Alzheimer's disease (rather than frontotemporal lobar degeneration), better cognitive function, more severe psychological, behavioral, and physical symptoms, and a reduced quality of life were associated with the expression of suicidal ideation. Conclusions: According to caregivers' reports, majority of patients with dementia did not express suicidal ideation or show suicidal behavior. Patients who expressed suicidal ideation during early stages of dementia often stopped expressing them in advanced stages. It remains unclear if this was due to reduced communication abilities, a reduction of disease awareness, and/ or an adjustment to their situation.

11.
J Alzheimers Dis ; 80(1): 283-297, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-33523011

RESUMO

BACKGROUND: Advanced stages of dementia are characterized by severe cognitive and physical impairment. It has not yet been investigated whether persons with young onset dementia (YOD) and late onset dementia (LOD) differ in advanced disease stages. OBJECTIVES: To compare quality of life (QoL) between persons with advanced YOD and LOD; to explore the determinants of QoL; to investigate whether YOD and LOD differ with regard to symptoms and care. METHODS: The study was performed in the context of EPYLOGE (IssuEs in Palliative care for persons in advanced and terminal stages of YOD and LOD in Germany). Persons with advanced dementia (PWAD) were assessed and caregivers were interviewed. QoL was measured with the proxy rating Quality of Life in Late Stage Dementia (QUALID) scale. RESULTS: 93 persons with YOD and 98 with LOD were included. No significant differences in QoL were detected. Determinants of QoL were similar in YOD and LOD. Behavioral and psychological symptoms of dementia (BPSD), suffering and other distressing symptoms were associated with a lower QoL. In YOD but not in LOD antipsychotic treatment was associated with low QoL. The group of persons who were younger than 65 years at the time of the study visit experienced significantly more distressing symptoms than older PWAD. CONCLUSION: Overall, persons with advanced YOD do not appear to be disadvantaged compared to old and oldest PWAD. Special attention, however, must be paid to the group of the very young persons who seem to be particularly vulnerable.


Assuntos
Idade de Início , Demência/psicologia , Qualidade de Vida , Adulto , Idoso , Idoso de 80 Anos ou mais , Antipsicóticos/uso terapêutico , Sintomas Comportamentais/etiologia , Sintomas Comportamentais/psicologia , Cuidadores , Demência/complicações , Demência/tratamento farmacológico , Progressão da Doença , Feminino , Alemanha , Serviços de Assistência Domiciliar , Humanos , Individualidade , Masculino , Pessoa de Meia-Idade , Dor/complicações , Dor/tratamento farmacológico , Dor/psicologia , Cuidados Paliativos , Psicotrópicos/uso terapêutico
12.
Cortex ; 140: 66-79, 2021 07.
Artigo em Inglês | MEDLINE | ID: mdl-33933931

RESUMO

OBJECTIVE: Motor speech disorders (MSDs) are characteristic for nonfluent primary progressive aphasia (nfvPPA). In primary progressive aphasia (PPA) of the semantic (svPPA) and of the logopenic type (lvPPA), speech motor function is considered typically intact. However, knowledge on the prevalence of MSDs in svPPA and lvPPA is mainly based on studies with a priori knowledge of PPA syndrome diagnosis. This fully blinded retrospective study aims to provide data on the prevalence of all types of MSDs in a large sample of German-speaking patients with different subtypes of PPA. METHOD: Two raters, blinded for PPA subtype, independently evaluated connected speech samples for MSD syndrome and severity from 161 patients diagnosed with nfvPPA, svPPA or lvPPA in the database of the German Consortium of Frontotemporal Lobar Degeneration (FTLDc). In case of disagreement, a third experienced rater re-evaluated the speech samples, followed by a consensus procedure. Consensus was reached for 160 patients (74 nfvPPA, 49 svPPA, 37 lvPPA). MAIN RESULTS: Across all PPA syndromes, 43.8% of the patients showed MSDs. Patients with nfvPPA demonstrated the highest proportion of MSDs (62.2%), but MSDs were also identified in svPPA (26.5%) and lvPPA (29.7%), respectively. Overall, dysarthria was the most common class of MSDs, followed by apraxia of speech. In addition, we identified speech abnormalities presenting as "syllabic speech", "dysfluent speech", and "adynamic speech". DISCUSSION: Our study confirmed MSDs as frequently occurring in PPA. The study also confirmed MSDs to be most common in patients with nfvPPA. However, MSDs were also found in substantial proportions of patients with svPPA and lvPPA. Furthermore, our study identified speech motor deficits that have not received attention in previous studies on PPA. The results are discussed against the background of the existing literature on MSDs in PPA, including theoretical considerations of the neuroanatomical conditions described for each of the different subtypes of PPA.


Assuntos
Afasia Primária Progressiva , Afasia Primária Progressiva não Fluente , Afasia Primária Progressiva/epidemiologia , Humanos , Afasia Primária Progressiva não Fluente/epidemiologia , Estudos Retrospectivos , Semântica , Fala
13.
Artigo em Inglês | MEDLINE | ID: mdl-32567375

RESUMO

Objective: The aims of our study were to describe the clinical phenotype and to characterize the cerebral glucose metabolism patterns as measured with fluordesoxyglucose-positron emission tomography (FDG-PET) in symptomatic FTLD-patients with different GRN variants. Methods: For this study, data were included from all patients (n = 10) of a single-center FTLD registry study who had a pathogenic GRN variant and who had undergone a cerebral FDG-PET scan. Results: An overt variability of clinical phenotypes was identified with half of the cases being not unambiguously classifiable into one of the clinical FTLD subtypes. Furthermore, GRN + patients showed a considerable inter-individual variability of FDG uptake pattern. In half of the GRN + patients, metabolic changes expanded from frontal and temporal brain regions to parietal brain regions including the posterior cingulate cortex. Striking asymmetry without a preference for either hemisphere was overt in half of GRN + cases. Conclusion: We conclude that GRN mutations cause variable patterns of neurodegeneration that often exceed the anatomical boundaries of the frontotemporal brain regions and produce clinical syndromes that cannot clearly be classified into one of the subtypes as defined by the diagnostic criteria.


Assuntos
Esclerose Lateral Amiotrófica , Degeneração Lobar Frontotemporal , Degeneração Lobar Frontotemporal/diagnóstico por imagem , Degeneração Lobar Frontotemporal/genética , Glucose , Humanos , Peptídeos e Proteínas de Sinalização Intercelular/genética , Mutação/genética , Progranulinas/genética , Síndrome
14.
J Neurol ; 266(2): 330-338, 2019 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30506397

RESUMO

The applause sign, i.e., the inability to execute the same amount of claps as performed by the examiner, was originally reported as a sign specific for progressive supranuclear palsy (PSP). Recent research, however, has provided evidence for the occurrence of the applause sign in various conditions. The aim of this study was to determine the prevalence of the applause sign and correlate its presence with neuropsychological and MRI volumetry findings in frontotemporal lobar degeneration and related conditions. The applause sign was elicited with the three clap test (TCT), with a higher score indicating poorer performance. Data were recorded from 272 patients from the cohort of the German consortium for frontotemporal lobar degeneration (FTLDc): 111 with behavioral variant frontotemporal dementia (bvFTD), 98 with primary progressive aphasia (PPA), 30 with progressive supranuclear palsy Richardson's syndrome, 17 with corticobasal syndrome (CBS) and 16 with amyotrophic lateral sclerosis with frontotemporal dementia (ALS/FTD). For comparison, 29 healthy elderly control subjects (HC) were enrolled in the study. All subjects underwent detailed language and neuropsychological assessment. In a subset of 156 subjects, atlas-based volumetry was performed. The applause sign occurred in all patient groups (40% in PSP, 29.5% in CBS, 25% in ALS/FTD, 13.3% in PPA and 9.0% in bvFTD) but not in healthy controls. The prevalence was highest in PSP patients. It was significantly more common in PSP as compared to bvFTD, PPA and HC. The comparison between the other groups failed to show a significant difference regarding the occurrence of the applause sign. The applause sign was highly correlated to a number of neuropsychological findings, especially to measures of executive, visuospatial, and language function as well as measures of disease severity. TCT scores showed an inverse correlation with the volume of the ventral diencephalon and the pallidum. Furthermore the volume of the ventral diencephalon and pallidum were significantly smaller in patients displaying the applause sign. Our study confirms the occurrence of the applause sign in bvFTD, PSP and CBS and adds PPA and ALS/FTD to these conditions. Although still suggestive of PSP, clinically it must be interpreted with caution. From the correlation with various cognitive measures we suggest the applause sign to be indicative of disease severity. Furthermore we suggest that the applause sign represents dysfunction of the pallidum and the subthalamic nucleus, structures which are known to play important roles in response inhibition.


Assuntos
Degeneração Lobar Frontotemporal/patologia , Degeneração Lobar Frontotemporal/fisiopatologia , Globo Pálido/patologia , Índice de Gravidade de Doença , Núcleo Subtalâmico/patologia , Paralisia Supranuclear Progressiva/patologia , Paralisia Supranuclear Progressiva/fisiopatologia , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Testes Neuropsicológicos
15.
Neurology ; 88(10): 961-969, 2017 Mar 07.
Artigo em Inglês | MEDLINE | ID: mdl-28179468

RESUMO

OBJECTIVE: To assess the utility of serum neurofilament for diagnosis and monitoring of primary progressive aphasia (PPA) variants. METHODS: We investigated neurofilament light chain (NF-L) levels in blood of 99 patients with PPA (40 with nonfluent variant PPA [nfvPPA], 38 with semantic variant PPA [svPPA], 21 with logopenic variant PPA [lvPPA]) and compared diagnostic performance with that reached by CSF NF-L, phosphorylated neurofilament heavy chain (pNF-H), ß-amyloid (Aß1-42), tau, and phosphorylated tau. The longitudinal change of blood NF-L levels was measured and analyzed for correlation with functional decline and brain atrophy. RESULTS: Serum NF-L is increased in PPA compared to controls and discriminates between nfvPPA/svPPA and lvPPA with 81% sensitivity and 67% specificity (cutoff 31 pg/mL). CSF NF-L, pNF-H, tau, phosphorylated tau, and Aß1-42 achieved similar performance, and pNF-H was the only marker for discrimination of nfvPPA from svPPA/lvPPA. In most patients with nfvPPA and svPPA, but not lvPPA, serum NF-L increased within follow-up. The increase correlated with functional decline and progression of atrophy of the left frontal lobe of all patients with PPAs and the right middle frontal gyrus of patients with nfvPPA and svPPA. CONCLUSIONS: Blood level of NF-L can aid the differential diagnosis of PPA variants, especially in combination with CSF pNF-H. Because serum NF-L correlates with functional decline and atrophy in the disease course, it qualifies as an objective disease status marker. Extended follow-up studies with cases of known neuropathology are imperative. CLASSIFICATION OF EVIDENCE: This study provides Class I evidence that in patients with PPA, blood levels of NF-L can distinguish the logopenic variant from the nonfluent/agrammatic and semantic variants.


Assuntos
Afasia Primária Progressiva/sangue , Proteínas de Neurofilamentos/sangue , Adulto , Idoso , Idoso de 80 Anos ou mais , Peptídeos beta-Amiloides/sangue , Peptídeos beta-Amiloides/líquido cefalorraquidiano , Afasia Primária Progressiva/diagnóstico por imagem , Atrofia/diagnóstico por imagem , Atrofia/patologia , Biomarcadores , Encéfalo/diagnóstico por imagem , Encéfalo/patologia , Feminino , Humanos , Estudos Longitudinais , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Proteínas de Neurofilamentos/líquido cefalorraquidiano , Fragmentos de Peptídeos/sangue , Fragmentos de Peptídeos/líquido cefalorraquidiano , Curva ROC , Proteínas tau/sangue , Proteínas tau/líquido cefalorraquidiano
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