Immune-mediated diseases were the most common cause of RPD in this series. Timely evaluation and diagnosis along with institution of appropriate therapy are required in RPD, especially in view of potentially reversible causes.

A BS TRACT: Background: Corticobasal syndrome (CBS) is an atypical parkinsonian syndrome related to multiple underlying pathologies. Objective: To investigate if individual brain [ 18 F]fluorodeoxyglucose-positron emission tomography (FDG-PET) patterns could distinguish CBS due to Alzheimer's disease (AD) from other pathologies based on [ 11 C]Pittsburgh Compound-B (PIB)-PET. Methods: Forty-five patients with probable CBS were prospectively evaluated regarding cognitive and movement disorders profile. They underwent FDG-PET and were distributed into groups: likely related to AD (CBS FDG-AD) or likely non-AD (CBS FDG-nonAD) pathology. Thirty patients underwent PIB-PET on a hybrid PETmagnetic resonance imaging equipment to assess their amyloid status. FDG and PIB-PET images were classified individually based on visual and semi-quantitative analysis, blinded to each other. Quantitative group analyses were also performed. Results: CBS FDG-AD group demonstrated worse cognitive performances, mostly concerning attention, memory, visuospatial domains, and displayed more myoclonus and hallucinations. The non-AD metabolic group presented more often limb dystonia, ocular motor dysfunction, motor perseveration, and dysarthria. All patients classified as CBS FDG-AD tested positive at PIB-PET compared to 3 of 20 in the non-AD group. The individual FDG-PET classification demonstrated 76.92% of sensitivity, 100% of specificity and positive predictive value and 88.5% of balanced accuracy to detect positive PIB-PET scans. Individuals with positive and negative PIB-PET showed hypometabolism in posterior temporoparietal areas and in thalamus and brainstem, respectively, mainly contralateral to most affected side, disclosing possible metabolic signatures of CBS variants. Conclusion: FDG-PET was useful to predict AD and non-AD CBS variants depicting their specific degeneration patterns, different clinical features, and brain amyloid deposition.

A cross-sectional study estimated the prevalence of work-related musculoskeletal disorders among nursing assistants in Salvador, Bahia. Three hundred and eight workers, randomly selected, answered a questionnaire applied by trained interviewers during working hours. The majority of respondents worked the day shift and did not usually work overtime. About 34% reported having another regular job. Average time in the formal or informal labor market was 19 years. There was high occupational exposure to repetitive hand movements, standing posture, walking, inadequate postures of the trunk and manual handling of loads. The prevalence of work-related musculoskeletal disorders in at least one body segment was 83.4%.The most affected body segments were: low back (53.9%), legs (51.9%), neck (36.4%), upper back (35.7%) and shoulders (33.8%). There was high prevalence of work-related musculoskeletal disorders in the studied population, which points out to the need for improvements in the working conditions of those professionals.

BackgroundAnemia is frequent among patients with traumatic brain injury (TBI) and is associated with an increased risk of poor outcome. The optimal hemoglobin concentration to trigger red blood cell (RBC) transfusion in patients with TBI is not clearly defined.MethodsAll eligible consecutive adult patients admitted to the intensive care unit (ICU) with moderate or severe TBI were randomized to a “restrictive” (hemoglobin transfusion threshold of 7 g/dL), or a “liberal” (threshold 9 g/dL) transfusion strategy. The transfusion strategy was continued for up to 14 days or until ICU discharge. The primary outcome was the mean difference in hemoglobin between groups. Secondary outcomes included transfusion requirements, intracranial pressure management, cerebral hemodynamics, length of stay, mortality and 6-month neurological outcome.ResultsA total of 44 patients were randomized, 21 patients to the liberal group and 23 to the restrictive group. There were no baseline differences between the groups. The mean hemoglobin concentrations during the 14-day period were 8.4 ± 1.0 and 9.3 ± 1.3 (p < 0.01) in the restrictive and liberal groups, respectively. Fewer RBC units were administered in the restrictive than in the liberal group (35 vs. 66, p = 0.02). There was negative correlation (r = − 0.265, p < 0.01) between hemoglobin concentration and middle cerebral artery flow velocity as evaluated by transcranial Doppler ultrasound and the incidence of post-traumatic vasospasm was significantly lower in the liberal strategy group (4/21, 3% vs. 15/23, 65%; p < 0.01). Hospital mortality was higher in the restrictive than in the liberal group (7/23 vs. 1/21; p = 0.048) and the liberal group tended to have a better neurological status at 6 months (p = 0.06).ConclusionsThe trial reached feasibility criteria. The restrictive group had lower hemoglobin concentrations and received fewer RBC transfusions. Hospital mortality was lower and neurological status at 6 months favored the liberal group.Trial registrationClinicalTrials.gov, NCT02203292. Registered on 29 July 2014.Electronic supplementary materialThe online version of this article (10.1186/s13054-018-2273-9) contains supplementary material, which is available to authorized users.

This article describes the development and operation of the Integrated Regional STEMI Network in Salvador, Bahia, Brazil, and defines its basic components. Moreover, we present a preliminary STEMI registry, including patients' demographics, clinical, interval times, and primary reperfusion © 2012 American Heart Association, Inc. Background-Regionalized integrated networks for ST-segment-elevation myocardial infarction (STEMI) care have been proposed as a step forward in overcoming real-world obstacles, but data are lacking on its performance in developing countries. We describe an integrated regional STEMI network in Salvador, Bahia, Brazil.Methods and Results-The network was created in 2009. It was coordinated by the prehospital emergency medical service and encompassed the public emergency system (prehospital mobile units, community-based emergency units, general hospitals, and cardiology reference centers). The 12-lead ECGs are interpreted via telemedicine. This network operates as follows: The Telemedicine Center sends each ECG suggestive of STEMI to a Regional STEMI Alert Team, which, together with emergency medical services, offers support for thrombolysis or immediate transfer for primary percutaneous coronary intervention. In 14 months, there were 433 suspected victims, of which in 287 (76.5%) the STEMI could be confirmed (age, 62.1±12.5 years; 63.4% men). Most of them were self-transported. The median pain-to-admission time was 180 minutes (interquartile range, 90-473 minutes), and the median admission-to-ECG time was 159.5 minutes (interquartile range, 83.5-340 minutes). The median interval time between the ECG and the telemedicine report was 31 minutes (interquartile range, 21-44 minutes). For those who sought medical attention and had an ECG performed within 12 hours after symptoms onset (n=119), the reperfusion rate was 75.6% (34.4% by thrombolysis and 65.6% by primary percutaneous coronary intervention).Conclusions-Regional STEMI networks may be feasible in developing countries. Preliminary results showed this network to be effective, achieving primary reperfusion rates comparable with those reported internationally despite the obstacles faced.(Circ Cardiovasc Qual Outcomes. 2013;6:9-17.)

Background Spinal muscular atrophy type 1 (SMA1) is a motor neuron disease associated with progressive muscle weakness, ventilatory failure, and reduced survival. Objective: To report the evaluation of the nusinersen, an antisense oligonucleotide, on the motor function of SMA1. Methods This was a longitudinal and observational study to assess the outcomes of nusinersen therapy in SMA1 patients using the HINE-2 and CHOP-INTEND scales. Results Twenty-one SMA1 patients (52.4% males) were included; the mean age at first symptoms was 2.7 months (SD =±1.5), and the mean disease duration at first dose was 34.1 (SD =±36.0) months. During posttreatment, the mean gain on the CHOP-INTEND was 4.9, 5.9, 6.6, and 14 points after 6, 12, 18, and 24 months, respectively. Starting medication with a disease duration of less than 12 months and/or without invasive ventilation were predictors of response on CHOP-INTEND. Of the patients, 28.6% acquired a motor milestone or gained at least three points on the HINE-2. The daily time for ventilatory support was reduced after treatment in most of the patients with noninvasive ventilation at baseline. No change in the daytime use of ventilation was observed in most of the patients using invasive ventilation at baseline. Conclusions Nusinersen produces improvements in motor and respiratory functions, even in long-term SMA1 patients. However, patients under invasive ventilation at the beginning of the treatment experience little benefit.

Background Spinal muscular atrophy (SMA) is a motor neuron disease associated with progressive muscle weakness and motor disability. Objective This study aims to report the evaluation of nusinersen, an antisense oligonucleotide, on motor function in patients with SMA types 2 and 3. Methods This single-center retrospective observational study assessed nusinersen therapy outcomes, measured by HSMFSE or CHOP-INTEND scales, in patients with SMA types 2 and 3, compared to untreated patients, for at least 24 months. Results In 30 patients with SMA types 2 and 3 (mean age: 10.6 years; 14 with SMA type 2) under nusinersen treatment, the mean change in HFMSE scores was +1.47 points (SD = 0.4) and +1.60 points (SD = 0.6) after 12 and 24 months of treatment, respectively. In contrast, the control group (N = 37) (mean age: 10.2 years; 20 with SMA type 2) presented a mean change of −1.71 points (SD = 0.02) and −3.93 points (SD = 0.55) after 12 and 24 months of follow-up, respectively. The most severe patients (N = 11) showed a change of +2.37 (SD = 1.13) on the CHOP-INTEND scale after 12 months of follow-up. Disease duration at the beginning of treatment was the main predictor of functional improvement. Despite functional gain and motor stabilization, treatment with nusinersen did not prevent the progression of scoliosis. Conclusions Our data provide evidence for the long-term safety and efficacy of nusinersen use in the treatment of later-onset SMA, and patients with shorter disease duration showed better response to treatment.

Background: We validated a new noninvasive tool (B4C) to assess intracranial pressure waveform (ICPW) morphology in a set of neurocritical patients, correlating the data with ICPW obtained from invasive catheter monitoring. Materials and Methods: Patients undergoing invasive intracranial pressure (ICP) monitoring were consecutively evaluated using the B4C sensor. Ultrasound-guided manual internal jugular vein (IJV) compression was performed to elevate ICP from the baseline. ICP values, amplitudes, and time intervals (P2/P1 ratio and time-to-peak [TTP]) between the ICP and B4C waveform peaks were analyzed. Results: Among 41 patients, the main causes for ICP monitoring included traumatic brain injury, subarachnoid hemorrhage, and stroke. Bland–Altman’s plot indicated agreement between the ICPW parameters obtained using both techniques. The strongest Pearson’s correlation for P2/P1 and TTP was observed among patients with no cranial damage (r = 0.72 and 0.85, respectively) to the detriment of those who have undergone craniotomies or craniectomies. P2/P1 values of 1 were equivalent between the two techniques (area under the receiver operator curve [AUROC], 0.9) whereas B4C cut-off 1.2 was predictive of intracranial hypertension (AUROC 0.9, p < 000.1 for ICP > 20 mmHg). Conclusion: B4C provided biometric amplitude ratios correlated with ICPW variation morphology and is useful for noninvasive critical care monitoring.