Responses of benthic diatoms to waters affected by post-fire contamination.

Wildfire effects go beyond direct impact in terrestrial ecosystems. Specifically, the periphytic communities of aquatic ecosystems standing within and downstream the burnt areas are relevant ecological receptors of post-fire runoff contamination. Nevertheless, the off-site impacts of wildfires in these communities are limitedly studied so far. The present study aimed to assess the effects of river water contaminated with ash-loaded runoff in the growth benthic diatom Navicula libonensis (Schoeman 1970). Four surface water samples were collected approximately one year after the wildfire for laboratory testing with the diatom: one was collected from a site upstream the burnt area, within the Unhais river (UU); three were collected from sites standing within the burnt area, one in the Unhais river (UB) and two in the Zêzere river (Z1 and Z2), reflecting different hydrological regimes. N. libonensis was proven able to discriminate among river sites affected and unaffected by wildfire runoff, reflecting, in general, the expected trends considering the physico-chemical characterization of the water samples. The water samples from the sites standing within the burnt area inhibited the biomass yield and growth rate of the tested diatom, ranking the samples regarding toxicity as follows: Z1 > UB > Z2 > UU. However, UB rather than Z1 presented the highest contaminant burden, namely metal elements, and some were found above widely accepted safety benchmarks (polycyclic aromatic hydrocarbons were not detected). This inconsistency can be linked to unknown interactions among metals within each water sample, to differential nutrient enrichment of samples, as well as hydrological factors. Overall, our results suggest that monospecific laboratory assays with sensitive diatoms can be valuable as cost-effective screening tools to prioritize sites affected by wildfires runoff requiring in-depth monitoring of negative effects in benthic producer communities.

[Portuguese Consensus on the Diagnosis and Management of Lewy Body Dementia (PORTUCALE)]. / Consenso Português para o Diagnóstico e Gestão Clínica da Demência com Corpos de Lewy (PORTUCALE).

Lewy body dementia is a common cause of dementia leading to the progressive deterioration of cognitive function and motor skills, behavioral changes, and loss of autonomy, impairing the quality of life of patients and their families. Even though it is the second leading cause of neurodegenerative dementia, diagnosis is still challenging, due to its heterogenous clinical presentation, especially in the early stages of the disease. Accordingly, Lewy body dementia is often misdiagnosed and clinically mismanaged. The lack of diagnostic accuracy has important implications for patients, given their increased susceptibility to the adverse effects of certain drugs, such as antipsychotics, which may worsen some symptoms associated with Lewy body dementia. Therefore, a specialist consensus based on the analysis of the most updated and relevant literature, and on clinical experience, is useful to all professionals involved in the care of these patients. This work aims to inform and provide recommendations about the best diagnostic and therapeutic approaches in Lewy body dementia in Portugal. Moreover, we suggest some strategies in order to raise the awareness of physicians, policy makers, and the society at large regarding this disease.

A demência com corpos de Lewy é uma causa comum de demência, provocando a perda progressiva de funções cognitivas e capacidades motoras, alterações comportamentais, e perda de autonomia, com compromisso da qualidade de vida dos doentes e seus familiares. Apesar de ser a segunda causa mais frequente de demência neurodegenerativa, o diagnóstico mantém-se um desafio, devido à sua apresentação clínica heterogénea, sobretudo nas fases iniciais da doença. Por conseguinte, a demência com corpos de Lewy é frequentemente mal diagnosticada e clinicamente gerida de forma insuficiente. A falta de acuidade diagnóstica tem implicações significativas para os doentes, dada a maior suscetibilidade aos efeitos adversos de determinados fármacos, tais como os antipsicóticos, que podem agravar alguns sintomas associados à demência com corpos de Lewy. Por conseguinte, um consenso de especialistas, baseado na análise da literatura mais atual e relevante, e na experiência clínica, é útil para todos os profissionais envolvidos no cuidado destes doentes. O objetivo deste trabalho é informar e gerar recomendações acerca das melhores abordagens diagnóstica e terapêutica da demência com corpos de Lewy em Portugal. Além disso, sugerimos estratégias para aumentar a sensibilização dos médicos, dos decisores políticos e da sociedade em geral em relação a esta doença.

Tracking Cognitive Performance in the General Population and in Patients with Mild Cognitive Impairment with a Self-Applied Computerized Test (Brain on Track).

Repeated measurements could be helpful to identify patients with early cognitive decline. We compare the variation of cognitive performance over one year in patients with mild cognitive impairment (MCI) and healthy individuals using the Brain on Track self-applied computerized test (BoT). The study was initiated 30 patients with probable MCI and 377 controls from a population-based cohort, who performed the BoT test from home every three months for one year. The scores were compared using a linear mixed-effects model. All participants increased their scores in the first tests, after 120 days MCI patients started to decline, with a statistically significant higher rate. The area under the curve to detect MCI was 0.94. We identified a significant decline in cognitive performance over one year in patients with MCI using BoT and the test presented a high discriminative ability.

Prevalence and Causes of Cognitive Impairment and Dementia in a Population-Based Cohort From Northern Portugal.

BACKGROUND: Vascular disease may play an important role in the epidemiology of dementia in countries with high stroke incidence, such as Portugal. OBJECTIVE: To assess the prevalence and etiology of cognitive impairment in a population-based cohort from Portugal. METHODS: Individuals ≥55 years (n = 730) from the EPIPorto cohort were assessed using the Mini-Mental State Examination and the Montreal Cognitive Assessment. Those scoring below the age-/education-adjusted cutoff points were further evaluated to identify dementia or mild cognitive impairment (MCI) and to define its most common causes. RESULTS: Thirty-six cases of MCI/dementia were identified, corresponding to adjusted prevalences of 4.1% for MCI and 1.3% for dementia. The most common cause of MCI/dementia was vascular (52.8%), followed by Alzheimer's disease (36.1%). CONCLUSION: These findings highlight the importance of vascular cognitive impairment in the epidemiology of dementia in Portugal and carry an important public health message regarding its prevention and management, possibly extending to other countries with a high-stroke burden.

Development of a self-administered web-based test for longitudinal cognitive assessment.

Sequential testing with brief cognitive tools has been recommended to improve cognitive screening and monitoring, however the few available tools still depend on an external evaluator and periodic visits. We developed a self-administered computerized test intended for longitudinal cognitive testing (Brain on Track). The test can be performed from a home computer and is composed of several subtests, expected to evaluate different cognitive domains, all including random elements to minimize learning effects. An initial (A) and a refined version of the test (B) were applied to patients with mild cognitive impairment or early dementia (n = 88) and age and education-matched controls. A subsample of a population-based cohort (n = 113) performed the test at home every three months to evaluate test-retest reliability. The test's final version Cronbach's alpha was 0.90, test scores were significantly different between patients and controls (p = 0.001), the area under the receiver operating characteristic curve was 0.75 and the smallest real difference (43.04) was lower than the clinical relevant difference (56.82). In the test-retest reliability analysis 9/10 subtests showed two-way mixed single intraclass consistency correlation coefficient >0.70. These results imply good internal consistency, discriminative ability and reliability when performed at home, encouraging further longitudinal clinical and population-based studies.

InMS: Chronic insomnia disorder in multiple sclerosis - a Portuguese multicentre study on prevalence, subtypes, associated factors and impact on quality of life.

BACKGROUND: Sleep may be disrupted in Multiple Sclerosis (MS), but the prevalence of chronic insomnia disorder (CID) using standard diagnostic criteria is unknown. OBJECTIVES: To determine the prevalence of CID in an MS population, the frequency of CID subtypes, associated factors and impact on quality of life (QoL). METHODS: Multicentre, hospital-based cross-sectional study. An adapted version of the Brief Insomnia Questionnaire was applied to a consecutively recruited MS population. The influence of demographic, MS-related features, fatigue, medical and psychiatric comorbidities, nocturnal symptoms, other sleep disorders, dysfunctional beliefs about sleep in CID was evaluated. The relation between CID and QoL was analysed. RESULTS: Of 206 MS patients, 22.3% fulfilled criteria for CID, with initial insomnia in 11.7%, maintenance insomnia in 11.2% and terminal insomnia in 10.2% of patients. CID was more frequent in female patients, those with nocturnal symptoms, medical comorbidities, higher levels of anxiety, depression and fatigue. Multivariable analysis identified female sex, medical comorbidities, anxiety and fatigue as independent factors for CID. CID patients had a significantly lower self-reported QoL. CONCLUSIONS: CID is prevalent in MS patients and associated with psychiatric and medical comorbidities, as well as fatigue. It has a negative impact on QoL.

Motor task performance under vibratory feedback early poststroke: single center, randomized, cross-over, controlled clinical trial.

Stroke rehabilitation is far from meeting patient needs in terms of timing, intensity and quality. This study evaluates the efficacy and safety of an innovative technological tool, combining 3D motion analysis with targeted vibratory feedback, on upper-limb task performance early poststroke (<4 weeks). The study design was a two-sequence, two-period, randomized, crossover trial (NCT01967290) in 44 patients with upper-limb motor deficit (non-plegic) after medial cerebral artery ischemia. Participants were randomly assigned to receive either the experimental session (repetitive motor task under vibratory feedback and 3D motor characterization) or the active comparator (3D motor characterization only). The primary outcome was the number of correct movements per minute on a hand-to-mouth task measured independently. Vibratory feedback was able to modulate motor training, increasing the number of correct movements by an average of 7.2/min (95%CI [4.9;9.4]; P < 0.001) and reducing the probability of performing an error from 1:3 to 1:9. This strategy may improve the efficacy of training on motor re-learning processes after stroke, and its clinical relevance deserves further study in longer duration trials.

Web-based cognitive training: patient adherence and intensity of treatment in an outpatient memory clinic.

BACKGROUND: Cognitive training has been playing an increasing role in the treatment of patients with cognitive deficits. This type of intervention, namely its intensity, can be optimized by incorporating information technology-based systems. OBJECTIVE: The intent of the study was to determine the treatment intensity and patient adherence to home-based cognitive training strategies (Web-based cognitive training). METHODS: A cohort of 45 patients with neurologic and psychiatric diseases attending an outpatient memory clinic (average age 50.7 years, SD 17.0; average education 7.8 years, SD 4.9) was followed over 18 months. Participants were challenged to use a Web-based cognitive training system, "COGWEB", on a daily basis, and fulfilled at least four weeks of training supervised remotely. Additionally, 11 patients attended face-to-face sessions. RESULTS: The average duration of continuous cognitive training was 18.8 weeks (SD 18.9). Each patient performed on average 363.5 minutes/week (SD 136.6). At 6-month follow-up, 82.8% complied with their treatment plan. The average proportion of complete weeks was 0.75 (SD 0.22). Patients with dementia trained more intensively (444.6 minutes/week), followed by patients with static brain lesion (414.5 minutes/week; P=.01). The group that held face-to-face sessions performed more training overall (481.4 vs 366.9 minutes/week), achieving a stronger expression and statistical significance in the last week of training (652.6 versus 354.9 minutes/week, P=.027). CONCLUSIONS: Overall, the weekly training intensity was high. Patients with dementia and static lesions performed more cognitive training. Face-to-face sessions were associated with higher intensities. The combination of classical methods with information technology systems seems to ensure greater training intensity.

Implementation and Outcomes of a Collaborative Multi-Center Network Aimed at Web-Based Cognitive Training - COGWEB Network.

BACKGROUND: Cognitive care for the most prevalent neurologic and psychiatric conditions will only improve through the implementation of new sustainable approaches. Innovative cognitive training methodologies and collaborative professional networks are necessary evolutions in the mental health sector. OBJECTIVE: The objective of the study was to describe the implementation process and early outcomes of a nationwide multi-organizational network supported on a Web-based cognitive training system (COGWEB). METHODS: The setting for network implementation was the Portuguese mental health system and the hospital-, academic-, community-based institutions and professionals providing cognitive training. The network started in August 2012, with 16 centers, and was monitored until September 2013 (inclusions were open). After onsite training, all were allowed to use COGWEB in their clinical or research activities. For supervision and maintenance were implemented newsletters, questionnaires, visits and webinars. The following outcomes were prospectively measured: (1) number, (2) type, (3) time to start, and (4) activity state of centers; age, gender, level of education, and medical diagnosis of patients enrolled. RESULTS: The network included 68 professionals from 41 centers, (33/41) 80% clinical, (8/41) 19% nonclinical. A total of 298 patients received cognitive training; 45.3% (n=135) female, mean age 54.4 years (SD 18.7), mean educational level 9.8 years (SD 4.8). The number enrolled each month increased significantly (r=0.6; P=.031). At 12 months, 205 remained on treatment. The major causes of cognitive impairment were: (1) neurodegenerative (115/298, 38.6%), (2) structural brain lesions (63/298, 21.1%), (3) autoimmune (40/298, 13.4%), (4) schizophrenia (30/298, 10.1%), and (5) others (50/298, 16.8%). The comparison of the patient profiles, promoter versus all other clinical centers, showed significant increases in the diversity of causes and spectrums of ages and education. CONCLUSIONS: Over its first year, there was a major increase in the number of new centers and professionals, as well as of the clinical diversity of patients treated. The consolidation of such a national collaborative network represents an innovative step in mental health care evolution. Furthermore, it may contribute to translational processes in the field of cognitive training and reduce disease burden.

Monozygotic twin sisters discordant for familial hemiplegic migraine.

BACKGROUND: The high concordance rate of migraine in monozygotic twin pairs has long been recognised. In the current study, we present a monozygotic twin pair discordant for familial hemiplegic migraine (FHM). CASE PRESENTATIONS: We evaluated 12 adult family members in 2012. The twin pair was separately examined by neurologists at different time points. Mutation screening was performed for known FHM-related genes. The monozygosity of the twins was verified. Eleven individuals had a history of migraine or paroxysmal neurological symptoms, including four patients with motor aura. No mutations were detected in the CACNA1A, ATP1A2, SCN1A, PRRT2 or NOTCH3 genes. The monozygotic twin sisters, aged 52, were discordant for age of onset, motor aura and neuropsychological aura (forced thinking). Overall, the family members presented a wide range of phenotypical features. CONCLUSIONS: Familial hemiplegic migraine is a monogenic disorder that is distinct from migraine with typical aura. However, in certain families with motor aura, such as this one, it is possible that the most severe phenotype is caused by an unlikely combination of polygenic traits and non-genetic factors. In these kindreds, we propose that hemiplegic aura is only a severe and complex form of typical aura.

Ischemic vagus nuclei lesions and hyperglycemia: a study in 26 patients with lateral medullary infarction and matched controls.

BACKGROUND: Hyperglycemia is common after stroke in diabetic and nondiabetic patients. Furthermore, it has been associated with infarct expansion, worse functional outcomes and higher mortality. In a previous study, infarction of the insular region was related to higher poststroke glucose levels than infarcts in other cortical areas. Experimental studies in animal models suggested that the lower brainstem nuclei of the vagus nerve modulate insulin secretion. These nuclei are usually affected in lateral medullary infarction (LMI). We evaluated whether patients with lateral medullary stroke have worse poststroke glycemic control than other stroke patients. METHODS: A hospital-based stroke registry was used to identify 26 patients from the years 2000 to 2010 who fulfilled the following inclusion criteria: (1) a first-ever stroke; (2) neurological deficits compatible with LMI; (3) MRI confirmation of an ischemic lesion of the lateral medulla involving the vagus nerve nuclei, and (4) no simultaneous infarcts. Patients were excluded if they were admitted to the hospital more than 24 h after stroke onset or died in the first 24 h after hospital admission. A control group of other stroke patients was randomly selected from the same stroke registry and over the same time period, matching for the age and gender of the LMI group. The average glycemia was compared between the two groups using a linear regression model adjusted for confounders. Glycated hemoglobin at admission was used to estimate prestroke glycemic control. Prestroke glycemic averages were then compared with poststroke glycemia for the two groups using the Wilcoxon signed test for related samples. RESULTS: The average glycemia of the LMI patients in the first 24 h after stroke was 9.4 mmol/l (SD 3.2), and from 24 to 72 h it was 7.6 mmol/l (SD 2.8). In the comparison group, these values were 7.7 (SD 2.8) and 7.1 mmol/l (SD 2.7), respectively. As expected, diabetic patients had a significantly higher glycemia than nondiabetic patients (p < 0.0001). The adjusted linear regression model showed the average glycemia differences to be significant for the first 24 h (p = 0.001; R(2) = 55.6%) but not for the 24- 72 h period. The frequency of previous diabetes mellitus was similar in both groups. As compared to prestroke glycemic estimates, glycemia in lateral medullary stroke patients increased significantly more than in controls during the first 24 h after stroke (p = 0.01), but again there were no significant differences for the 24-72 h period. CONCLUSIONS: This study suggests that ischemic lesions of the vagus nerve nuclei are associated with worse early poststroke glycemic control than stroke in other locations. Confirmation of this hypothesis and the long-term implications of glucose control impairment warrant further prospective studies.

Superficial siderosis and anticoagulation therapy: different presentations, different outcomes.

Superficial siderosis is a potentially manageable neurodegenerative disorder, caused by chronic subarachnoid haemorrhage and iron deposition along the central nervous system surfaces. Association with oral anticoagulant therapy is well known, but its definite role as a causative agent is yet to be clarified. Two Caucasian women, both under long-term oral anticoagulation: a 74 year old woman with slowly progressive hearing loss and mild cerebellar ataxia; a 72 year old woman suffering from behavioural changes, rapidly progressive cognitive decline and latter developing paraparesis. Magnetic resonance imaging showed striking hypointensities along the surfaces of cerebellum, brainstem, frontotemporal cortices, spinal cord, and lumbar arachnoid therefore suggesting superficial siderosis. No specific bleeding source was found in any of the patients. Anticoagulation could not be stopped in the first patient due to a mechanic valve and slowly progressive worsening occurred. In contrast, for the second patient anticoagulation withdrawal was feasible and marked motor and cognitive improvement ensued. Superficial siderosis is associated with unvarying progression, mostly when no direct source of bleeding is identified. Nonetheless, we verified striking motor and cognitive improvement after anticoagulants withdrawal in one of the patients. This may reinforce the need to consider such modifiable factor in future patient management.