Whipple's Disease With Neurological Manifestations: Case Report.

Whipple's disease (WD) is an uncommon multisystem condition caused by the bacillus Tropheryma whipplei. Central nervous system involvement is a classical feature of the disease observed in 20 to 40% of the patients. We report the case of a 62 year old man with WD that developed neurological manifestations during its course, and discuss the most usual signs and symptoms focusing on recent diagnostic criteria and novel treatment regimens.62342-

Validity and Reliability of the Frontotemporal Dementia Rating Scale (FTD-FRS) for the Progression and Staging of Dementia in Brazilian Patients

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

Disease progression in frontotemporal dementia and Alzheimer disease: the contribution of staging scales

Introduction: There is a shortage of validated instruments to estimate disease progression in frontotemporal dementia (FTD). Objectives: To evaluate the ability of the FTD Rating Scale (FTD-FRS) to detect functional and behavioral changes in patients diagnosed with the behavioral variant of FTD (bvFTD), primary progressive aphasia (PPA) and Alzheimer disease (AD) after 12 months of the initial evaluation, compared to the Clinical Dementia Rating scale - frontotemporal lobar degeneration (CDR-FTLD) and the original Clinical Dementia Rating scale (CDR). Methods: The sample consisted of 70 individuals, aged 40+ years, with at least two years of schooling, 31 with the diagnosis of bvFTD, 12 with PPA (8 with semantic variant and 4 with non-fluent variant) and 27 with AD. The FTD-FRS, the CDR and the two additional CDR-FTLD items were completed by a clinician, based on the information provided by the caregiver with frequent contact with the patient. The Addenbrooke’s Cognitive Examination-Revised (ACE-R) was completed by patients. After 12 months, the same protocol was applied. Results: The FTD-FRS, CDR-FTLD and CDR detected significant decline after 12 months in the three clinical groups (exception: FTD-FRS for PPA). The CDR was less sensitive to severe disease stages. Conclusions: The FTD-FRS and the CDR-FTLD are especially useful tools for dementia staging in AD and in the FTD spectrum

Perfil funcional de pacientes com demência frontotemporal variante comportamental (bvDFT) em comparação com pacientes com doença de Alzheimer e controles normais

There are few studies describing the functional changes in behavioral variant frontotemporal dementia (bvFTD) and it is not clear which aspects of functionality are affected by the disease. Objective: The aim of the present investigation was to characterize the functional profile of patients previously diagnosed with bvFTD. Methods: The sample consisted of 31 patients diagnosed with bvFTD, who were compared to patients with Alzheimer’s disease (AD) (n=31) and to healthy control subjects (NC) (n=34), matched for schooling and age. bvFTD and AD patients were matched by severity of dementia. The protocol included the Mini-Mental State Examination (MMSE), Geriatric Depression Scale (GDS), Direct Assessment of Functional Status (DAFS-BR), Functional Activities Questionnaire (PFAQ), Disability Assessment for Dementia (DAD) and the Clinical Dementia Rating scale (CDR). Results: The group with bvFTD showed worse performance on Initiation and Planning/Organization in the DAD and on ability to feed oneself in the DAFS-BR, as well as higher scores on the PFAQ, \ud suggesting greater dependence in the bvFTD group. Conclusion: The results suggest that individuals with bvFTD display greater functional impairment compared to AD patients with a similar degree of dementia severity and to healthy controls. Direct assessment of functionality proved unable to clearly differentiate between the dementia subtypes.Existem poucos estudos sobre alterações funcionais na variante comportamental da demência frontotemporal (DFTvc). Objetivo: Caracterizar o desempenho funcional de pacientes com diagnóstico prévio de DFTvc. Métodos: Trinta e um pacientes com DFTvc foram comparados a pacientes com doença de Alzheimer (DA) (n=31) e adultos saudáveis (NC) (n=34), pareados para idade e escolaridade. Os pacientes com DFTvc e DA foram pareados pela gravidade da demência. O protocolo incluiu o Mini Exame do Estado Mental, Escala de Depressão Geriátrica (GDS), Direct Assessment of Functional \ud Status (DAFS-BR), Disability Assessment for Dementia (DAD), Functional Activities Questionnaire (PFAQ) e Clinical Dementia Rating scale (CDR). Resultados: O grupo com DFTvc apresentou pior desempenho em Iniciação e Planejamento/Organização na DAD, em Alimentação na DAFS-BR e pontuação mais elevada na PFAQ, sugerindo que a dependência na DFTvc é mais acentuada. Conclusão: Os resultados apresentados sugerem que indivíduos com DFTvc apresentam maior prejuízo funcional, quando comparados com participantes com DA com grau semelhante de gravidade e com adultos saudáveis. A avaliação direta da funcionalidade não ajudou a diferenciar os subtipos de demência de modo significativo

Tradução, adaptação transcultural e aplicabilidade da escala de estadiamento e progressão da degeneração lobar frontotemporal

Background: Staging scales for dementia have been devised for grading Alzheimer’s disease (AD) but do not include the specific symptoms of frontotemporal lobar degeneration (FTLD). Objective: To translate and adapt the Frontotemporal Dementia Rating Scale (FTD-FRS) to Brazilian Portuguese. Methods: The cross-cultural adaptation \ud process consisted of the following steps: translation, back-translation (prepared by independent translators), discussion with specialists, and development of a final version after minor adjustments. A pilot application was carried out with 12 patients diagnosed with bvFTD and 11 with AD, matched for disease severity (CDR=1.0). The evaluation protocol included: Addenbrooke’s Cognitive Examination-Revised (ACE-R), Mini-Mental State Examination (MMSE), Executive Interview (EXIT-25), Neuropsychiatric Inventory (NPI), Frontotemporal Dementia Rating Scale (FTD-FRS) and Clinical Dementia Rating scale (CDR). Results: The Brazilian version of the FTD-FRS seemed appropriate for use in this country. Preliminary results revealed greater levels of disability in bvFTD than in AD patients (bvFTD: 25% mild, 50% moderate and 25% severe; AD: 36.36% mild, 63.64% moderate). It appears that the CDR underrates disease severity in bvFTD since a relevant proportion of patients rated as having mild dementia (CDR=1.0) in fact had moderate or severe levels of disability according to the FTD-FRS. Conclusion: The Brazilian version of the FTD-FRS seems suitable to aid staging and determining disease progression.Introdução: As escalas de estadiamento das demências, como a Clinical Dementia Rating (CDR), foram elaboradas para graduar a doença de Alzheimer (DA) e não incluem os sintomas específicos da degeneração lobar frontotemporal (DLFT). Objetivo: Realizar a tradução e adaptação cultural da Frontotemporal Dementia Rating Scale (FTD-FRS) para o contexto brasileiro e apresentar dados preliminares da sua aplicabilidade. Métodos: O processo de adaptação transcultural consistiu em: tradução, retrotradução (realizadas por tradutores independentes), discussão com especialistas sobre a versão em português e equivalência com a versão original, desenvolvimento da versão final com pequenos ajustes. Foi feita uma aplicação piloto em 12 pacientes com diagnóstico de demência frontotemporal variante comportamental (DFTvc) e 11 com DA, pareados quanto à gravidade da demência (CDR=1). O protocolo de avaliação incluiu a Addenbrooke’s Cognitive Examination-Revised (ACE-R), Mini Exame do Estado Mental (MEEM), Executive Interview (EXIT-25), Inventário Neuropsiquiátrico (INP) e a Escala de Avaliação Clínica da Demência (CDR). Resultados: A FTD-FRS na versão brasileira \ud pareceu apropriada. Resultados preliminares revelaram maiores níveis de incapacidade na DFTvc do que em pacientes com DA (DFTvc: 25% leve, 50% moderado, 25% grave; AD: 36.36% leve, 63.64% moderado). A CDR parece subestimar a gravidade da demência na DFTvc, uma vez que uma relevante proporção dos pacientes classificados com leves (CDR=1) de fato apresentaram nível moderado ou grave de comprometimento na FTD-FRS. Conclusão: A versão brasileira da FTD-FRS pode se mostrar adequada para auxiliar no estadiamento e determinar a progressão da DLFT

On the existence and stability of periodic orbits in non ideal problems: General results

In this work, motivated by non-ideal mechanical systems, we investigate the following O.D.E. ẋ = f (x) + εg (x, t) + ε2g (x, t, ε), where x ∈ Ω ⊂ ℝn, g, g are T periodic functions of t and there is a 0 ∈ Ω such that f (a 0) = 0 and f′ (a0) is a nilpotent matrix. When n = 3 and f (x) = (0, q (x 3) , 0) we get results on existence and stability of periodic orbits. We apply these results in a non ideal mechanical system: the Centrifugal Vibrator. We make a stability analysis of this dynamical system and get a characterization of the Sommerfeld Effect as a bifurcation of periodic orbits. © 2007 Birkhäuser Verlag, Basel

Category verbal fluency performance may be impaired in amnestic mild cognitive impairment

Abstract To study category verbal fluency (VF) for animals in patients with amnestic mild cognitive impairment (aMCI), mild Alzheimer disease (AD) and normal controls. Method: Fifteen mild AD, 15 aMCI, and 15 normal control subjects were included. Diagnosis of AD was based on DSM-IV and NINCDS-ADRDA criteria, while aMCI was based on the criteria of the International Working Group on Mild Cognitive Impairment, using CDR 0.5 for aMCI and CDR 1 for mild AD. All subjects underwent testing of category VF for animals, lexical semantic function (Boston Naming-BNT, CAMCOG Similarities item), WAIS-R forward and backward digit span, Rey Auditory Verbal Learning (RAVLT), Mini-Mental Status Examination (MMSE), and other task relevant functions such as visual perception, attention, and mood state (with Cornell Scale for Depression in Dementia). Data analysis used ANOVA and a post-hoc Tukey test for intergroup comparisons, and Pearson's coefficient for correlations of memory and FV tests with other task relevant functions (statistical significance level was p<0.05). Results: aMCI patients had lower performance than controls on category VF for animals and on the backward digit span subtest of WAIS-R but higher scores compared with mild AD patients. Mild AD patients scored significantly worse than aMCI and controls across all tests. Conclusion: aMCI patients may have poor performance in some non-memory tests, specifically category VF for animals in our study, where this could be attributable to the influence of working memory