Estudo longitudinal de um programa de reabilitação neuropsicológica dirigido a pacientes com doença de Alzheimer

Our aim was to study the duration of benefits derived from a neuropsychological rehabilitation program (NRP) for dementia patients. METHOD: The participants in this study were three patients diagnosed as probable Alzheimer's disease in the initial-to-moderate phase; the three were taking anticholinesterases. They were submitted to a neuropsychological evaluation (NE) before the NRP and then revaluated after 12 and 24 months of treatment. The aim of our intervention was to do practical work with implicit and explicit residual memory by training them in everyday life activities, and using compensatory strategies and their intact cognitive abilities. RESULTS: Analysis of quantitative NE data (descriptive measures) after the first year of NRP showed cognitive improvement, functional stabilization and fewer behavioral problems. However, this improvement did not continue in the second year, and the disease maintained its characteristic progression.OBJETIVO: Estudar a duração do beneficio de um programa de reabilitação neuropsicológica (PRN) dirigido a pacientes demenciados. MÉTODO: Participaram deste estudo, três pacientes com diagnóstico de provável doença de Alzheimer em fase inicial a moderada. Todos faziam uso de anti-colinesterásicos e passaram por uma avaliação neuropsicológica (AN) antes de começar o PRN e reavaliação após 12 e 24 meses do tratamento. O alvo de nossa intervenção foi trabalhar de forma prática a memória explicita residual e implícita, através do treino das atividades da vida diária, uso de estratégias compensatórias e habilidades cognitivas ainda preservadas. RESULTADOS: A análise dos dados quantitativos (medidas descritivas) da AN mostrou, que após o primeiro ano do PRN houve uma melhora cognitiva, estabilização funcional e redução dos problemas comportamentais nos pacientes. No entanto, observamos que essa melhora não se estendeu para o segundo ano, mostrando a doença sua característica progressiva.Universidade Federal de São Paulo (UNIFESP) Escola Paulista de Medicina Centro Paulista de NeuropsicologiaUNIFESP, EPM, Centro Paulista de NeuropsicologiaSciEL

Withdrawal of mechanical ventilation in amyotrophic lateral sclerosis patients: a multicenter Italian survey

Background: Law 219/2017 was approved in Italy in December 2017, after a years-long debate on the autonomy of healthcare choices. This Law, for the first time in Italian legislation, guarantees the patient's right to request for withdrawal of life-sustaining treatments, including mechanical ventilation (MV). Objective: To investigate the current status of MV withdrawal in amyotrophic lateral sclerosis (ALS) patients in Italy and to assess the impact of Law 219/2017 on this practice. Methods: We conducted a Web-based survey, addressed to Italian neurologists with expertise in ALS care, and members of the Motor Neuron Disease Study Group of the Italian Society of Neurology. Results: Out of 40 ALS Italian centers, 34 (85.0%) responded to the survey. Law 219/2017 was followed by an increasing trend in MV withdrawals, and a significant increase of neurologists involved in this procedure (p 0.004). However, variations across Italian ALS centers were observed, regarding the inconsistent involvement of community health services and palliative care (PC) services, and the intervention and composition of the multidisciplinary team. Conclusions: Law 219/2017 has had a positive impact on the practice of MV withdrawal in ALS patients in Italy. The recent growing public attention on end-of-life care choices, along with the cultural and social changes in Italy, requires further regulatory frameworks that strengthen tools for self-determination, increased investment of resources in community and PC health services, and practical recommendations and guidelines for health workers involved

Behavioural assessment of the dysexecutive syndrome (BADS) in healthy elders and Alzheimer´s disease patients: preliminary study

Abstract Although the main initial deficit is considered to be in the memory domain, an early impairment of executive functions is also found in AD where these deficits are correlated to functional impairment. Ecological tests are more indicated to evaluate executive impairment, and are better able to assist in treating AD patients than more commonly used tests. Objectives: The aim of this preliminary study is to verify the performance in executive functions using the Behavioural Assessment of the Dysexecutive Syndrome (BADS) in elder controls and mild AD patients, and to analyze its applicability in our environment. Methods: The BADS was performed by 17 healthy elders and 17 early AD patients matched for age, schooling and gender. Results: There were significant differences among controls and AD patients on MMSE scores, and in measures of executive functions, memory, and motor speed. Some sub items of BADS (rule shift cards, modified six elements, total score, standard, standard by age and overall classification by age) were also different between groups. Differences were also significant on the Dysexecutive Questionnaire (DEX) of BADS self-ratings and other-ratings. Conclusion: BADS was efficacious in detecting executive deficits in this sample, as confirmed by other executive functions tests applied

Retrospective observational study on the use of acetyl-L-carnitine in ALS

: ALCAR (Acetyl-L-carnitine) is a donor of acetyl groups and increases the intracellular levels of carnitine, the primary transporter of fatty acids across the mitochondrial membranes. In vivo studies showed that ALCAR decrease oxidative stress markers and pro-inflammatory cytokines. In a previous double-blind placebo-controlled phase II trial showed positive effects on self-sufficiency (defined as a score of 3+ on the ALSFRS-R items for swallowing, cutting food and handling utensils, and walking) ALSFRS-R total score and FVC. We conducted an observational, retrospective, multicentre, case-control study to provide additional data on the effects of ALCAR in subjects with ALS in Italy. Subjects treated with ALCAR 1.5 g/day or 3 g/day were included and matched with not treated subjects by sex, age at diagnosis, site of onset, and time from diagnosis to baseline, (45 subjects per group). ALCAR 3 g/day vs not treated: 22 not treated subjects (48.9%) were still alive at 24 months after baseline, compared to 23 (51.1%) treated subjects (adj. OR 1.18, 95% CI 0.46-3.02). No statistically significant differences were detected in ALSFRS nor FVC nor self-sufficiency. ALCAR 1.5 g/day vs not treated: 22 not treated subjects (48.9%) were still alive at 24 months after baseline, compared to 32 (71.1%) treated subjects (adj. OR 0.27, 95% CI 0.10-0.71). For ALSFRS-R, a mean slope of - 1.0 was observed in treated subjects compared to - 1.4 in those not treated (p = 0.0575). No statistically significant difference was detected in the FVC nor self-sufficiency. Additional evidence should be provided to confirm the efficacy of the drug and provide a rationale for the dosage