Functional status in behavioral variant frontotemporal dementia: a systematic review

The aim was to conduct a systematic review of studies that described the functional profile of patients with behavioral variant frontotemporal dementia (bvFTD), published between 2000 and 2013. The bibliographic search was conducted using the terms “frontotemporal dementia” and “frontotemporal lobar degeneration” in combination with “independence,” “functionality,” “basic activities of daily living,” “disability,” and scales that measure functional performance: “Disability Assessment for Dementia-DAD,” “Functional Activities Questionnaire (FAQ),” “Direct Assessment of Functional Status (DAFS).” To be included in the review, the study had to mention the characterization of the functional status of patients with bvFTD in the objectives of the study, using a previously validated instrument of functional assessment. Fourteen studies met this criterion. The reviewed studies suggested that individuals with bvFTD have greater functional impairment when compared to those with other subtypes of frontotemporal lobar degeneration or Alzheimer’s disease. The studies documented a significant association between cognitive impairment and measures of functionality in these patients. The cognitive profile of patients may predict faster functional decline.FAPESPID 83712

Apathy and functional disability in behavioral variant frontotemporal dementia

Background Behavioral variant frontotemporal dementia (bvFTD) has profound consequences on patients and their families. In this multicenter study, we investigated the contribution of cognitive and neuropsychiatric factors to everyday function at different levels of overall functional impairment. Methods In a retrospective cross-sectional study, 109 patients with bvFTD from 4 specialist frontotemporal dementia centers (Australia, England, India, and Brazil) were included. The measures administered evaluated everyday function (Disability Assessment for Dementia [DAD]), dementia staging (Clinical Dementia Rating [CDR]), general cognition (Addenbrooke’s Cognitive Examination–revised [ACE-R]), and neuropsychiatric symptoms (Neuropsychiatric Inventory [NPI]). Patients were then subdivided according to functional impairment on the DAD into mild, moderate, severe, and very severe subgroups. Three separate multiple linear regression analyses were run, where (1) total DAD, (2) basic activities of daily living (BADL), and (3) instrumental activities of daily living (IADL) scores were dependent variables; ACE-R total score and selected NPI domains (agitation/aggression, euphoria, apathy, disinhibition, irritability, aberrant motor behavior) were used as independent variables. Age, sex, education, and country of origin were controlled for in the analyses. Results Cognitive deficits were similar across the mild, moderate, and severe subgroups but significantly worse in the very severe subgroup. NPI domain scores (agitation/aggression, euphoria, apathy, disinhibition, irritability, aberrant motor behavior) did not differ across the DAD subgroups. In the multiple regression analyses, a model including ACE-R and NPI apathy explained 32.5% of the variance for total DAD scores. For IADL, 35.6% of the variance was explained by the ACE-R only. No model emerged for BADL scores. Conclusions Cognitive deficits and apathy are key contributors to functional disability in bvFTD but factors underlying impairment in BADLs remain unclear. Treatments targeting reduction of disability need to address apathy and cognitive impairment to ensure greater efficacy, especially in regards to IADLs

Epidural analgesia and cesarean delivery in multiple sclerosis post-partum relapses: the Italian cohort study

BACKGROUND: Few studies have systematically addressed the role of epidural analgesia and caesarean delivery in predicting the post-partum disease activity in women with Multiple Sclerosis (MS).The objective of this study was to assess the impact of epidural analgesia (EA) and caesarean delivery (CD) on the risk of post-partum relapses and disability in women with MS. METHODS: In the context of an Italian prospective study on the safety of immunomodulators in pregnancy, we included pregnancies occurred between 2002 and 2008 in women with MS regularly followed-up in 21 Italian MS centers. Data were gathered through a standardized, semi-structured interview, dealing with pregnancy outcomes, breastfeeding, type of delivery (vaginal or caesarean) and EA. The risk of post-partum relapses and disability progression (1 point on the Expanded Disability Status Sclae, EDSS, point, confirmed after six months) was assessed through a logistic multivariate regression analysis. RESULTS: We collected data on 423 pregnancies in 415 women. Among these, 349 pregnancies resulted in full term deliveries, with a post-partum follow-up of at least one year (mean follow-up period 5.5±3.1 years). One hundred and fifty-five patients (44.4%) underwent CD and 65 (18.5%) EA. In the multivariate analysis neither CD, nor EA were associated with a higher risk of post-partum relapses. Post-partum relapses were related to a higher EDSS score at conception (OR=1.42; 95% CI 1.11-1.82; p=0.005), a higher number of relapses in the year before pregnancy (OR=1.62; 95% CI 1.15-2.29; p=0.006) and during pregnancy (OR=3.07; 95% CI 1.40-6.72; p=0.005). Likewise, CD and EA were not associated with disability progression on the EDSS after delivery. The only significant predictor of disability progression was the occurrence of relapses in the year after delivery (disability progression in the year after delivery: OR= 4.00; 95% CI 2.0-8.2; p<0.001; disability progression over the whole follow-up period: OR= 2.0; 95% CI 1.2-3.3; p=0.005). CONCLUSIONS: Our findings, show no correlation between EA, CD and postpartum relapses and disability. Therefore these procedures can safely be applied in MS patients. On the other hand, post-partum relapses are significantly associated with increased disability, which calls for the need of preventive therapies after delivery

Desempenho de uma população brasileira no teste de alfabetização funcional para adultos na área de saúde

OBJECTIVE: To analyze the scoring obtained by an instrument, which evaluates the ability to read and understand items in the health care setting, according to education and age. METHODS: The short version of the Test of Functional Health Literacy in Adults was administered to 312 healthy participants of different ages and years of schooling. The study was conducted between 2006 and 2007, in the city of São Paulo, Southeastern Brazil. The test includes actual materials such as pill bottles and appointment slips and measures reading comprehension, assessing the ability to read and correctly pronounce a list of words and understand both prose passages and numerical information. Pearson partial correlations and a multiple regression model were used to verify the association between its scores and education and age. RESULTS: The mean age of the sample was 47.3 years(sd=16.8) and the mean education was 9.7 years(sd=5; range: 1 - 17). A total of 32.4% of the sample showed literacy/numeracy deficits, scoring in the inadequate and marginal functional health literacy ranges. Among the elderly (65 years or older) this rate increased to 51.6%. There was a positive correlation between schooling and scores (r=0.74; pOBJETIVO: Analizar los escores de instrumento que evalúa habilidad de lectura y comprensión de materiales del área de salud según escolaridad y edad. MÉTODOS: Fueron evaluados 312 participantes saludables de diferentes edades por medio de la versión reducida del instrumento Test of Functional Health Literacy in Adults. El estudio fue realizado entre 2006 y 2007 en la ciudad de Sao Paulo (Sureste de Brasil). El instrumento incluye materiales como frascos de medicamentos y cartones de para marcar consultas, evaluando la comprensión de lectura y de conceptos numéricos. Las pruebas de correlación parcial y de Pearson y un modelo de regresión múltiple fueron usados para verificar la asociación entre los escores en el instrumento, escolaridad y edad. RESULTADOS: Los promedios de edad y de escolaridad de la muestra fueron respectivamente 47,3 (dp=16,8) y 9,7 (dp=5; de uno a 17 años de estudio). El total de 32,4% de la muestra mostraron déficit de alfabetización funcional/uso de conceptos numéricos en el área de salud, con desempeño inadecuado o limítrofe en el instrumento. Entre ancianos (65 años o más) esta tasa afectó 51,6%. Se encontró correlación positiva entre años de estudio y escores en el instrumento (r=0,740; p< 0,01) y correlación negativa entre edad y escores en el instrumento (r=-0,259; p< 0,01).La correlación entre escores en el instrumento y edad no fue significativa cuando los efectos de la escolaridad fueron controlados (r=-0,031, p=0,584). Una asociación significativa (B=3,877, Beta=0,733; p< 0,001) fue encontrada entre años de estudio y escores en el instrumento. La edad no fue una variable predictiva en el modelo (B=-0,035, Beta=-0,22; p=0,584). CONCLUSIONES: El instrumento es adecuado para evaluar la alfabetización funcional en salud en la población brasilera. El elevado número de individuos clasificados como analfabetos funcionales indica la importancia de adopción de medidas especiales para ayudar estos individuos a comprender correctamente las orientaciones para cuidados de salud.OBJETIVO: Analisar os escores de instrumento que avalia habilidade de leitura e compreensão de materiais da área da saúde segundo escolaridade e idade. MÉTODOS: Foram avaliados 312 participantes saudáveis de diferentes idades por meio da versão reduzida do instrumento Test of Functional Health Literacy in Adults. O estudo foi realizado entre 2006 e 2007 na cidade de São Paulo (SP). O instrumento envolve materiais como frascos de medicamentos e cartões de agendamento de consultas, avaliando a compreensão de leitura e de conceitos numéricos. Os testes de correlação parcial e de Pearson e um modelo de regressão múltipla foram usados para verificar a associação entre os escores no instrumento, escolaridade e idade. RESULTADOS: As médias de idade e de escolaridade da amostra foram respectivamente 47,3 (dp=16,8 ) e 9,7 (dp=5; de um a 17 anos de estudo). O total de 32,4% da amostra mostraram déficits de alfabetização funcional/ uso de conceitos numéricos na área de saúde, com desempenho inadequado ou limítrofe no instrumento. Entre idosos (65 anos ou mais) esta taxa atingiu 51,6%. Encontrou-se correlação positiva entre anos de estudo e escores no instrumento (r=0,740;

Codon 129 polymorphism of prion protein gene in is not a risk factor for Alzheimer's disease

Interaction of prion protein and amyloid-b oligomers has been demonstrated recently. Homozygosity at prion protein gene (PRNP) codon 129 is associated with higher risk for Creutzfeldt-Jakob disease. This polymorphism has been addressed as a possible risk factor in Alzheimer disease (AD). Objective To describe the association between codon 129 polymorphisms and AD. Methods We investigated the association of codon 129 polymorphism of PRNP in 99 AD patients and 111 controls, and the association between this polymorphism and cognitive performance. Other polymorphisms of PRNP and additive effect of apolipoprotein E gene (ApoE) were evaluated. Results Codon 129 genotype distribution in AD 45.5% methionine (MM), 42.2% methionine valine (MV), 12.1% valine (VV); and 39.6% MM, 50.5% MV, 9.9% VV among controls (p>0.05). There were no differences of cognitive performance concerning codon 129. Stratification according to ApoE genotype did not reveal difference between groups. Conclusion Codon 129 polymorphism is not a risk factor for AD in Brazilian patients

Comments on the validity of Haemaphysalis cinnabarina Koch, 1844 (Acari : Ixodidae), a taxon known solely by the type specimens from northern Brazil

The types of Haemaphysalis cinnabarina Koch and its junior synonym H. sanguinolenta Koch, both from State of Para, Brazil, have been studied. Although H. cinnabarina has been considered a synonym of H. punctata Canestrini and Fanzago (a Palearctic species), they were compared to another closely related species H. chordedis (Packard) (a Nearctic species). Based on the morphology and geographical distance among of H. cinnabarina, H. chordedis and H. punctata, we are reasonably sure that all are valid taxa. The lack of additional reports of H. cinnabarina is more related to few investigations in South America, mainly in Northern Brazil, rather than suggesting that it does not exist

Comentários sobre a validade de Haemaphysalis cinnabarina Koch, 1844 (Acari: Ixodidae) um táxon conhecido somente pelos espécimes tipos do Norte do Brasil

Os tipos de Haemaphysalis cinnabarina Koch e da espécie sinônima H. sanguinolenta Koch, ambos do Estado do Pará, Brasil, foram estudados. Embora H. cinnabarina tenha sido considerada uma sinonímia de H. punctata Canestrini e Fanzago (uma espécie Paleártica), elas foram comparadas a uma outra espécie próxima, H. chordeilis (Packard) (uma espécie Neártica). Com base em morfologia e distância geográfica entre H. cinnabarina, H. chordeilis e H. punctata, consideramos válidos os três taxa. A ausência de relatos adicionais de H. cinnabarina está mais relacionada à ausência de investigações na América do Sul, principalmente no Norte do Brasil, do que a sugestão da sua não existênciaThe types of Haemaphysalis cinnabarina Koch and its junior synonym H. sanguinolenta Koch, both from State of Pará, Brazil, have been studied. Although H. cinnabarina has been considered a synonym of H. punctata Canestrini and Fanzago (a Palearctic species), they were compared to another closely related species H. chordeilis (Packard) (a Nearctic species). Based on the morphology and geographical distance among of H. cinnabarina, H. chordeilis and H. punctata, we are reasonably sure that all are valid taxa. The lack of additional reports of H. cinnabarina is more related to few investigations in South America, mainly in Northern Brazil, rather than suggesting that it does not exis

Neuropsychological differences between frontotemporal lobar degeneration and Alzheimer's disease

Abstract Memory impairment is the main clinical feature in Alzheimer disease (AD), whereas in frontotemporal lobar degeneration (FTLD) behavioral and language disorders predominate. Objectives: To investigate possible differences between the neuropsychological performance in FTLD and AD. Methods: Fifty-six AD patients (mean age=72.98±7.43; mean schooling=9.62±4.68; 35 women and 21 men), 17 FTLD patients (mean age=67.64±7.93; mean schooling=12.12±4.77; 9 women and 8 men), and 60 controls (mean age=68.90±7.48; mean schooling=10.72±4.74; 42 women and 18 men) were submitted to a Dementia Rating Scale (DRS) and a comprehensive neuropsychological evaluation composed of tasks assessing attention, visuoperceptual abilities, constructive abilities, executive functions, memory and language. Results: DRS total score and subscales were not able to differentiate FTLD from AD patients. However, FTLD and AD patients showed statistically significant differences in performance in tests of verbal (Logical Memory, Rey Auditory Verbal Learning Test) and visual (Visual Reproduction, recall of the Rey Complex Figure) episodic memory, verbal immediate memory (Logical Memory), attention with interference (Trail Making Test - Part B), verbal fluency (semantic and phonemic) and concept formation (WCST). Conclusion: Contrary to expectations, only a few tasks executive function tasks (Trail Making Test - Part B, F.A.S. and WCST) and two memory tests (verbal and visual episodic memory tests) were able to differentiate between FTLD and AD patients

Diagnoses of and illustrated key to the species of Ixodes Latreille, 1795 (Acari: Ixodidae) from Brazil

The current Brazilian Ixodes fauna is composed of the following eight species: I. amarali Fonseca, 1935; I. aragaoi Fonseca, 1935; I. auritulus Neumann, 1904; I. fuscipes Koch, 1844; I. loricatus Neumann, 1899; I. luciae S,nevet, 1940; I. paranaensis Barros-Battesti, Arzua, Pichorim & Keirans, 2003; and I. schulzei AragA o pound & Fonseca, 1951. Further studies are needed to establish the taxonomic status of I. serrafreirei Amorim, Gazeta, Bossi & Linhares, 2003, a recently proposed species based solely on the nymphal stage. We present an up-to-date key to adults of the currently valid Brazilian species of Ixodes based on scanning electron microscopy. The relationships between Brazilian and other Neotropical Ixodes are also discussed.Fundacao de Amparo a Pesquisa do Estado de Sao Paulo - FAPESP[98/11666-8]Fundacao de Amparo a Pesquisa do Estado de Sao Paulo - FAPESP[99/05446-8]Conselho Nacional de Desenvolvimento Cientifico e Tecnologico - CNPqAcademic career scholarshipCAPE