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Background High levels of physical conditioning are associated with improvements in cognitive performance. In this sense, electroencephalographic (ECG) correlates are used to investigate the enhancing role of physical exercise on executive functions. Oscillations in the β frequency range are proposed to be evident during sensorimotor activity.

Background Autoimmune encephalitis (AIE) is a group of inflammatory diseases characterized by the presence of antibodies against neuronal and glial antigens, leading to subacute psychiatric symptoms, memory complaints, and movement disorders. The patients are predominantly young, and delays in treatment are associated with worse prognosis.

In recent decades, there have been significant advances in the diagnosis of diffuse gliomas, driven by the integration of novel technologies. These advancements have deepened our understanding of tumor oncogenesis, enabling a more refined stratification of the biological behavior of these neoplasms. This progress culminated in the fifth edition of the WHO classification of central nervous system (CNS) tumors in 2021. This comprehensive review article aims to elucidate these advances within a multidisciplinary framework, contextualized within the backdrop of the new classification. This article will explore morphologic pathology and molecular/genetics techniques (immunohistochemistry, genetic sequencing, and methylation profiling), which are pivotal in diagnosis, besides the correlation of structural neuroimaging radiophenotypes to pathology and genetics. It briefly reviews the usefulness of tractography and functional neuroimaging in surgical planning. Additionally, the article addresses the value of other functional imaging techniques such as perfusion MRI, spectroscopy, and nuclear medicine in distinguishing tumor progression from treatment-related changes. Furthermore, it discusses the advantages of evolving diagnostic techniques in classifying these tumors, as well as their limitations in terms of availability and utilization. Moreover, the expanding domains of data processing, artificial intelligence, radiomics, and radiogenomics hold great promise and may soon exert a substantial influence on glioma diagnosis. These innovative technologies have the potential to revolutionize our approach to these tumors. Ultimately, this review underscores the fundamental importance of multidisciplinary collaboration in employing recent diagnostic advancements, thereby hoping to translate them into improved quality of life and extended survival for glioma patients.

Background The saccadic eye movement is responsible for providing focus to a visual object of interest to the retina. In sports like volleyball, identifying relevant targets quickly is essential to a masterful performance. The training improves cortical regions underlying saccadic action, enabling more automated processing in athletes.

Background Sjogren-Larsson syndrome (SLS) is a neurocutaneous disease with an autosomal recessive inheritance, caused by mutations in the gene that encodes fatty aldehyde dehydrogenase (ALDH3A2), clinically characterized by ichthyosis, spastic diplegia, and cognitive impairment. Brain imaging plays an essential role in the diagnosis, demonstrating a nonspecific leukoencephalopathy. Data regarding brain atrophy and grey matter involvement is scarce and discordant.

Background Infantile epileptic spasms syndrome (IESS) is a rare but severe condition affecting children early and is usually secondary to an identifiable brain disorder. It is related to psychomotor deterioration in childhood and epilepsy in adult life. Treatment is challenging as infantile spasms may not respond to most antiseizure medication, and relapse is frequent.

Background Phenylketonuria (PKU) is an inborn error of metabolism caused by deficient activity of phenylalanine hydroxylase. In Brazil, the National Neonatal Screening Program enables early treatment of patients with PKU, which prevents them from developing severe neurological damage and mental disabilities. However, between 20 and 30% of early-treated patients with PKU present focal cognitive deficits, including deficits in working memory, processing speed, and psychiatric symptoms such as anxiety, depression, and attention deficit hyperactivity disorder (ADHD). Therefore, age-specific neuropsychiatric and cognitive tests are important components of PKU patient care. To date, there are no officially approved guidelines or recommendations of tools in Portuguese validated for use in Brazil that could be applied to assess these parameters in patients with PKU.

Background Flexibility is crucial to the harmonious execution of joint movements. While skeletal muscle dysfunction in patients with HTLV-1 can interfere with mobility, it is unclear whether these patients experience reduced flexibility.

Plasma exchange (PLEX) is a therapeutic apheresis modality in which the plasma is separated from inflammatory factors such as circulating autoreactive immunoglobulins, the complement system, and cytokines, and its therapeutic effect is based on the removal of these mediators of pathological processes. Plasma exchange is well established for various neurological disorders, and it is applied successfully in central nervous system inflammatory demyelinating diseases (CNS-IDD). It mainly modulates the humoral immune system; thus, it has a greater theoretical effect in diseases with prominent humoral mechanisms, such as neuromyelitis optica (NMO). However, it also has a proven therapeutic effect in multiple sclerosis (MS) attacks. Several studies have suggested that patients with severe attacks of CNS-IDD have poor response to steroid therapy but show clinical improvement after the PLEX treatment. Currently, PLEX is generally established only as a rescue therapy for steroid unresponsive relapses. However, there are still research gaps in the literature regarding plasma volume, number of sessions, and how early the apheresis treatment needs to started. Thus, in the present article, we summarize the clinical studies and meta-analyses, especially about MS and NMO, outlining clinical data regarding the experience with therapeutic PLEX in severe attacks of CNS-IDD, the clinical improvement rates, the prognostic factors of a favorable response, and highlighting the likely role of the early apheresis treatment. Further, we have gathered this evidence and suggested a protocol for the treatment of CNS-IDD with PLEX in the routine clinical practice.

Background Implementing stroke care protocols has intended to provide better care quality, favor early functional recovery, and achieving long-term results for the rehabilitation of the patient. Objective To analyze the effect of implementing care protocols on the outcomes of acute ischemic stroke. Methods Primary studies published from 2011 to 2020 and which met the following criteria were included: population should be people with acute ischemic stroke; studies should present results on the outcomes of using protocols in the therapeutic approach to acute ischemic stroke. The bibliographic search was carried out in June 2020 in 7 databases. The article selection was conducted by two independent reviewers and the results were narratively synthesized. Results A total of 11,226 publications were retrieved in the databases, of which 30 were included in the study. After implementing the protocol, 70.8% of the publications found an increase in the rate of performing reperfusion therapy, such as thrombolysis and thrombectomy; 45.5% identified an improvement in the clinical prognosis of the patient; and 25.0% of the studies identified a decrease in the length of hospital stay. Out of 19 studies that addressed the rate of symptomatic intracranial hemorrhage, 2 (10.5%) identified a decrease. A decrease in mortality was mentioned in 3 (25.0%) articles out of 12 that evaluated this outcome. Conclusions We have identified the importance of implementing protocols in increasing the performance of reperfusion therapies, and a good functional outcome with improved prognosis after discharge. However, there is still a need to invest in reducing post-thrombolysis complications and mortality.

Background Levodopa is the most used and effective medication for motor symptoms of Parkinson disease (PD), its long-term use is associated with the appearance of levodopa-induced dyskinesia (LID). Uric acid (UA) is believed to play an important neuroprotective role in PD. Objective To investigate if serum UA levels are related with the presence of LIDs in PD patients. Also, we investigated the associations among UA levels and clinical features of PD. Methods We enrolled 81 PD patients (dyskinesia = 48; no dyskinesia = 33) in the present study. A blood sample was collected to evaluate serum UA levels, clinical evaluation included the following instruments: Montreal Cognitive Assessment (MoCA), Beck Depression Inventory II (BDI-II), MDS-Unified Parkinson's Disease Rating Scale (MDS-UPDRS), Hoehn and Yahr (HY), and the sub-item 4.1 of MDS-UPDRS IV (score ≥ 1). Additional relevant clinical information was obtained by a clinical questionnaire. Results Serum UA levels were lower in the dyskinesia group when compared with the no dyskinesia group. The same result was found in the UA levels of both men and women. The multivariate analysis showed lower uric acid levels were significantly associated with having dyskinesia (odds ratio [OR] = 0.424; 95% confidence interval [CI]: 0.221–0.746; p = 0.005). Additional analysis verified that serum UA levels are inversely correlated with depressive symptoms, disease duration, MDS-UPDRS IV and time spent with dyskinesia. A positive correlation was found with age at onset of PD symptoms. Conclusions The present study provides a possible role of serum UA levels in LID present in PD patients.

Background Depression is an important nonmotor symptom of Parkinson's disease (PD) and has been associated with the motor symptoms in these individuals. Objectives To determine whether there are relationships between depressive symptoms and abnormalities in axial postural alignment and axial motor deficits, especially postural instability, and trunk rigidity in PD. Methods In this cross-sectional study, 65 individuals were evaluated using the Beck Depression Inventory-II (BDI-II) for the analysis of depressive symptoms and underwent a postural assessment of head, trunk, and hip sagittal alignment through computerized photogrammetry. The MDS-UPDRS was used to assess clinical aspects of PD, the Trunk Mobility Scale was used to assess axial rigidity, and the MiniBESTest to assess balance. To determine the relationship between depressive symptoms and postural alignment, multiple linear regression analysis was performed. Results The participants with depressive symptoms had more severe motor deficits as well as greater trunk rigidity and worse postural instability (p < 0.05). When the postural angles were compared between men and women using Student's t-test, it was found that men had greater flexion angles of the head (p = 0.003) and trunk (p = 0.017). Using multiple linear regression analysis corrected for the age and sex of the participants, we verified that the anterior trunk inclination was significantly larger in the PD population with depressive symptoms (R2 = 0.453, β = 0.116, and p = 0.045). Conclusion PD individuals with depressive symptoms have more severe flexed trunk posture, mainly in older men. Additionally, more severe depressive symptoms are associated with worsening postural instability, trunk rigidity and motor deficits in this population.

Background Lacosamide (LCM) is a third-generation anti-seizure drug approved in Europe and the United States, either as a monotherapy or adjunctive therapy, to treat partial-onset seizures in adults, adolescents, and children. In Brazil, LCM is licensed for treatment only in patients older than 16 years of age. Objective To evaluate a cohort of children presenting with refractory epilepsy who received LCM as an add-on therapy and observe the response and tolerability to the LCM treatment. Methods A retrospective cohort study conducted in a tertiary health care facility, which included 26 children, aged up to 16 years, who presented with refractory epilepsy and received LCM as an add-on treatment. The follow-up visits were scheduled every 3 months until 9 months of treatment with LCM. Results After 3 months of LCM administration, in 73.1% of the children, there was a reduction of > 50% in the frequency of seizures, and this clinical improvement was maintained in most patients (73.9%) for the following 9 months. Mild (such as, somnolence and behavioral changes) or severe (seizure worsening) adverse effects were observed in two and three children respectively. Among responders to LCM, there was a higher prevalence of males, fewer concomitant anti-seizure drugs, and lower percentage of patients using sodium channel blockers. Conclusions Lacosamide should be considered as an early treatment option in pediatric patients with refractory epilepsy, mainly focal seizures.

Background Most of the Brazilian population relies on public healthcare and stroke is a major cause of disability in this country of continental dimensions. There is limited information about access to rehabilitation after stroke in Brazil. Objective To provide comprehensive information about Access to Rehabilitation After discharge from public hospitals in Brazil (AReA study), up to 6 months after stroke. Methods The present study intends to collect information from 17 public health centers in 16 Brazilian cities in the 5 macroregions of the country. Each center will include 36 participants (n = 612). The inclusion criteria are: age ≥ 18 years old; ischemic or hemorrhagic stroke, from 6 months to 1 year prior to the interview; admission to a public hospital in the acute phase after stroke; any neurological impairment poststroke; patient or caregiver able to provide informed consent and answer the survey. Patients can only be recruited in public neurology or internal medicine outpatient clinics. Outcomes will be assessed by a standard questionnaire about rehabilitation referrals, the rehabilitation program (current status, duration in months, number of sessions per week) and instructions received. In addition, patients will be asked about preferences for locations of rehabilitation (hospitals, clinics, or at home). Trial Status The study is ongoing. Recruitment started on January 31st, 2020 and is planned to continue until June 2022. Conclusion The AReA study will fill a gap in knowledge about access to stroke rehabilitation in the public health system in different Brazilian regions.

Background International studies performed during the periods of social isolation highlighted the potential loss of student's learning skills. The present study fills a gap in Brazilian research on this topic and focuses on the development of reading fluency. Objective To investigate the development of the reading fluency of students in the early years of elementary school during e-learning as a result of the social distancing measures put into effect due to the coronavirus disease 2019 (COVID-19) pandemic. Methods Students from grades 2 to 5 were recorded. The number of words read per minute and of those read correctly per minute were analyzed. Descriptive statistical analysis was performed, using analysis of variance (ANOVA) for repeated measures with Bonferroni correction in the longitudinal study, and the t-test in the cross-sectional study. Results In the cross-sectional study, 162 students participated. Only the comparison between the 2nd grade classes of 2020 and 2021 showed a statistically significant difference. In the prepandemic classes, the students had better results in reading accuracy than the students assessed during the pandemic. The longitudinal study included 75 students, who improved in fluency rate and accuracy as expected between March and December 2020. In March 2021, the results showed a drop, which may be related to school closures during the Brazilian summer vacation. Conclusions The present research demonstrates the results of Brazilian students in terms of the development of reading fluency during the pandemic. There was an expressive development in grades 2 and 3, with stability in the following grades. The 2nd grade class of 2021 suffered a major impact due to the pandemic.

Background Multiple sclerosis (MS) and neuromyelitis optica spectrum disorders (NMOSD) are the most common autoimmune diseases of the central nervous system (CNS). They present chronic relapsing courses that demand treatment with disease-modifying drugs (DMDs) to prevent inflammatory activity. Disease-modifying drugs lead to immunomodulation or immunosuppression through diverse mechanisms (e.g., shifting lymphocyte and cytokine profile, suppressing specific lymphocyte subpopulations). Thus, patients are more prone to infectious complications and associated worsening of disease. Objective To present feasible strategies for mitigating the infection risk of MS and NMOSD treated patients. Methods Targeted literature review concerning the management of infection risk with an emphasis on vaccination, therapy-specific measures, and particularities of the Brazilian endemic infectious diseases' scenario. Conclusion We propose a vaccination schedule, infectious screening routine, and prophylactic measures based on the current scientific evidence. Awareness of emergent tropical diseases is necessary due to evidence of demyelinating events and possible parainfectious cases of MS and NMOSD.

ABSTRACT Normal pressure hydrocephalus (NPH) has been a topic of debate since its introduction in publications. More frequent in the elderly population, it is characterized by gait disturbance, urinary urge incontinence and cognitive decline. Therefore, it is a clinical-radiological entity with relatively common findings for the age group, which together may have greater specificity. Therefore, its diagnosis must be careful for an adequate selection of patients for treatment with ventricular shunt, since the symptoms are potentially reversible. The tap test has a high positive predictive value as a predictor of therapeutic response, but a negative test does not exclude the possibility of treatment. Scientific efforts in recent years have been directed towards a better understanding of NPH and this narrative review aims to compile recent data from the literature in a didactic way for clinical practice.

ABSTRACT This article aims to expand the understanding of how it is possible to alleviate suffering and enable a dignified life trajectory for patients with progressive neurological diseases or with severe and permanent neurological impairment. The four most common disease trajectories described for people with chronic and progressive disease used to advance care planning, Brazilian normative ethical resolutions, evidence-based benefits of palliative care (PC), as well as particularities of PC in neurology, such as neurological symptom control, caring for existential and psychological suffering, care provider’s needs and particularities of pediatric neurologic PC are reviewed.

ABSTRACT Background: Acquired demyelinating disorders lead to overlapping visual, pyramidal, sensory, autonomic, and cerebellar deficits and may lead to severe disability. Early diagnosis and start of treatment are fundamental towards preventing further attacks and halting disability. Objective: In this paper we provide an updated overview of the differential diagnoses of acquired demyelinating disorders. Methods: We performed a critical targeted review of the diagnoses of the most prevalent demyelinating disorders: multiple sclerosis (MS), neuromyelitis optica spectrum disorders (NMOSD) and myelin oligodendrocyte glycoprotein antibody disease (MOGAD). Results: We discuss the workup, diagnostic criteria and new biomarkers currently being used for the diagnosis of these disease entities taking into account the particularities of the Brazilian population and healthcare system. Conclusion: A comprehensive analysis of medical history, physical examination, biomedical and imaging data should be performed to obtain differential diagnosis. Diagnostic criteria should be mindfully employed considering ethnic and environmental particularities of each patient.

ABSTRACT Alzheimer’s disease (AD) is the most common neurodegenerative disease. Biomarkers have demonstrated that AD pathology exists over the disease continuum from a stage preceding symptoms over 15-25 years to the progressively more impaired symptomatic states, mild cognitive impairment (MCI), and dementia. Biomarkers include: amyloid (Aß), phosphorylated tau, and neurodegeneration. The plasma assays for Aß and tau show great promise for clinical and research use. This review has aimed not only to present the ATN diagnostic classification and the preclinical AD concepts in addressing some possibilities of cognitive assessment instruments, but also to briefly summarize the main anti-amyloid monoclonal antibodies studied in clinical trials. In addition, this paper presents a critical analysis by experts in cognitive neurology while addressing the question as to whether we are prepared for the anti-amyloid therapy era or not.

Background Stroke is one of the most common causes of death and incapacity in the world. The benefits of reperfusion therapies and hospitalization in neurologic intensive care units (ICUs) are undeniable. However, these treatments are not widely available in a continental-sized country like Brazil.

ABSTRACT Background: The diagnosis of autism spectrum disorder (ASD) in Down syndrome (DS) is underestimated because it is necessary to understand which aspects of the behavioral phenotype are related to DS and which are related to ASD. Objective: To conduct a systematic review of the literature on early identification and diagnosis of ASD in patients with DS. Data source: The VHL, MEDLINE, Cochrane, CINAHL, Scopus, Web of Science and Embase databases were searched and data were evaluated using PRISMA. Data synthesis: Out of 1,729 articles evaluated, 15 were selected. Although well studied, identification of ASD in DS can be difficult because of the need to understand which aspects of the behavioral phenotype are related to Down syndrome and which to autism. In this review, the prevalence of ASD was found to range from 12% to 41%. Early identification of autism risk in individuals with Down syndrome is still poorly studied, even though there are screening instruments for infants. Several instruments for diagnosing autism in individuals with Down syndrome were found, but a developmental approach is fundamental for making a clear diagnosis. Conclusions: Screening procedures are important for detecting early signs of autism risk in the first year of life. Careful evaluation methods are needed to establish the diagnosis, which include choosing appropriate tools for evaluation of development and cognition, and analysis of qualitative aspects of social interaction, among others. It has been indicated in the literature that early detection and timely accurate diagnosis, in association with an intervention, may benefit development, quality of life and social inclusion.

ABSTRACT Background: Headache is a very common complaint and it is increasingly prevalent among university students. Tinnitus consists of subjectively perceived sounds that occur in the absence of an external auditory signal. Presence of headache and tinnitus in association has implications for therapy and prognosis, because this describes the temporality of the symptoms. Recognition of the epidemiological profile of symptomatic students might contribute to interventions. Objective: To investigate the prevalence of the association between headache and tinnitus, and to describe the epidemiological profile of the study population and the chronological order of appearance of these symptoms. Methods: Cross-sectional, observational and analytical study on a sample representative of an academic center. Data referring to the epidemiological and clinical profile of headache and tinnitus among medical students were collected through an online questionnaire built using the Google Forms tool. Results: Out of the 234 participants, 26.1% reported having tinnitus and headache (p < 0.001). The participants with headache were more likely to be women (p = 0.045), white (p = 0.009) and 21-25 years old (p = 0.356). Among right-sided, left-sided and non-unilateral headaches, tinnitus was present predominantly in the non-unilateral type, but without statistical significance. Regarding timing, 18.0% of the students said that tinnitus started before headache, 57.4% said that headache started before tinnitus and 24.6% said that they started simultaneously. Conclusions: An important association between headache and tinnitus regarding lateralization and temporality was demonstrated. Thus, these data match the presumption that headache and tinnitus have a physiopathological connection.

ABSTRACT The Scientific Department of Neuroimmunology of the Brazilian Academy of Neurology (DCNI/ABN) and Brazilian Committee for Treatment and Research in Multiple Sclerosis and Neuroimmunological Diseases (BCTRIMS) provide recommendations in this document for vaccination of the population with demyelinating diseases of the central nervous system (CNS) against infections in general and against the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), which causes COVID-19. We emphasize the seriousness of the current situation in view of the spread of COVID-19 in our country. Therefore, reference guides on vaccination for clinicians, patients, and public health authorities are particularly important to prevent some infectious diseases. The DCNI/ABN and BCTRIMS recommend that patients with CNS demyelinating diseases (e.g., MS and NMOSD) be continually monitored for updates to their vaccination schedule, especially at the beginning or before a change in treatment with a disease modifying drug (DMD). It is also important to note that vaccines are safe, and physicians should encourage their use in all patients. Clearly, special care should be taken when live attenuated viruses are involved. Finally, it is important for physicians to verify which DMD the patient is receiving and when the last dose was taken, as each drug may affect the induction of immune response differently.

The Brazilian Practice Guidelines for Stroke Rehabilitation – Part II, developed by the Scientific Department of Neurological Rehabilitation of the Brazilian Academy of Neurology (Academia Brasileira de Neurologia, in Portuguese), focuses on specific rehabilitation techniques to aid recovery from impairment and disability after stroke. As in Part I, Part II is also based on recently available evidence from randomized controlled trials, systematic reviews, meta-analyses, and other guidelines. Part II covers disorders of communication, dysphagia, postural control and balance, ataxias, spasticity, upper limb rehabilitation, gait, cognition, unilateral spatial neglect, sensory impairments, home rehabilitation, medication adherence, palliative care, cerebrovascular events related to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection, the future of stroke rehabilitation, and stroke websites to support patients and caregivers. Our goal is to provide health professionals with more recent knowledge and recommendations for better rehabilitation care after stroke.

Background The Parkinson disease (PD) is frequently associated with autonomic dysfunctions. However, data regarding the influence of PD on the autonomic responses to postural changes is limited. Objective To analyze and compare the autonomic responses, evaluated through linear and non-linear methods of heart rate variability, and cardiorespiratory parameters in two groups: Parkinson disease (PDG) and control (CG), at rest and during the active tilt test. Methods A total of 48 participants were analyzed (PDG: n = 25;73.40 ± 7.01 years / CG: n = 23;70.17 ± 8.20 years). The autonomic modulation and cardiorespiratory parameters were evaluated at rest and during the active tilt test. To assess the autonomic modulation the linear indices, at the time (rMSSD, SDNN) and frequency (LF, HF, LF/HF) domains, and the non-linear indices, obtained through the Poincaré plot (SD1, SD2, SD1/SD2), were calculated. The cardiorespiratory parameters evaluated were heart rate (HR), systolic (SBP), and diastolic blood pressure (DBP), peripheral oxygen saturation (SpO2), and respiratory rate. Results At rest, the PDG presented significantly lower values of rMSSD, SDNN, LF, HF, SD1, SD2, and DBP, and higher values of SpO2. During test, in the PD group, modifications were observed in HR, and SBP, besides a reduced parasympathetic response, and an increased global modulation. The qualitative analysis of the Poincaré plot showed that the PDG has a lower dispersion of the RR intervals during rest and the active tilt test. Conclusion Individuals with PD present reduced global variability and parasympathetic modulation at rest, and reduced parasympathetic response and damage in HR regulation when performing the active tilt test, compared with controls.

Background The treatment of acute ischemic stroke with cerebral reperfusion therapy requires rapid care and recognition of symptoms. Objective To analyze the effectiveness of implementing protocols for acute ischemic stroke in reducing care time. Methods Systematic review, which was performed with primary studies in Portuguese, English, and Spanish published between 2011 and 2020. Inclusion criteria: study population should comprise people with acute ischemic stroke and studies should present results on the effectiveness of using urgent care protocols in reducing care time. The bibliographic search was conducted in June 2020 in the LILACS, MEDLINE, Embase, Scopus, CINAHL, Academic Search Premier, and SocINDEX databases. The articles were selected, and data were extracted by two independent reviewers; the synthesis of the results was performed narratively. The methodological quality of articles was evaluated through specific instruments proposed by the Joanna Briggs Institute. Results A total of 11,226 publications were found, of which 35 were included in the study. Only one study reported improvement in the symptoms-onset-to-door time after protocol implementation. The effectiveness of the therapeutic approach protocols for ischemic stroke was identified in improving door-to-image, image-to-needle, door-to-needle and symptoms-onset-to-needle times. The main limitation found in the articles concerned the lack of clarity in relation to the study population. Conclusions Several advances have been identified in in-hospital care with protocol implementation; however, it is necessary to improve the recognition time of stroke symptoms among those who have the first contact with the person affected by the stroke and among the professionals involved with the prehospital care.

ABSTRACT Background: Simulations are becoming widely used in medical education, but there is little evidence of their effectiveness on neurocritical care. Because acute stroke is a neurological emergency demanding prompt attention, it is a promising candidate for simulation training. Objective: To assess the impact of a stroke realistic simulation course on clinicians’ self-perception of confidence in the management of acute stroke. Methods: We conducted a controlled, before-after study. For our intervention, 17 healthcare professionals participated in a stroke realistic simulation course. As controls, participants were chosen from a convenience sample of attendees to the courses Emergency Neurologic Life Support (ENLS) (18 participants) and Neurosonology (20 participants). All participants responded pre- and post-test questionnaires evaluating their self-perception of confidence in acute stroke care, ranging from 10 to 50 points. We evaluated the variation between pre- and post-test results to assess the change on trainees’ self-perception of confidence in the management of acute stroke. Multivariate analysis was performed to control for potential confounders. Results: Forty-six (83.63%) subjects completed both questionnaires. The post-test scores were higher than those from the pretests in the stroke realistic simulation course group [pretest median (interquartile range - IQR): 41.5 (36.7-46.5) and post-test median (IQR): 47 (44.7-48); p=0.033], but not in the neurosonology [pretest median (IQR): 46 (44-47) and post-test median (IQR): 46 (44-47); p=0.739] or the ENLS [pretest median (IQR): 46.5 (39-48.2), post-test median (IQR): 47 (40.2-49); p=0.317] groups. Results were maintained after adjustment for covariates. Conclusions: This stroke realistic simulation course was associated with an improvement on trainees’ self-perception of confidence in providing acute stroke care.

ABSTRACT Background: Williams-Beuren syndrome is a multisystemic disorder caused by a microdeletion of the 7q11.23 region. Although familial cases with autosomal dominant inheritance have been reported, the vast majority are sporadic. Objective: To investigate the main complaints and clinical findings of patients with Williams-Beuren syndrome. Methods: A total of 757 parents of patients registered in the Brazilian Association of Williams-Beuren Syndrome (ABSW) received a questionnaire via WhatsApp from March to July 2017. Results: In total, 229 parents answered the survey. Age of diagnosis ranged from 2 days to 34 years (median: 3 years). The main clinical findings reported by the parents were abdominal colic (83.3%), failure to thrive (71.5%), feeding difficulty in the first year (68.9%), otitis (56.6%), urinary tract infections (31.9%), precocious puberty (27.1%) and scoliosis (15.9%). Cardiac defects were present in 66% of patients, and the most frequent defect was supravalvular aortic stenosis (36%). Arterial hypertension was reported in 23%. Hypercalcemia was reported in 10.5% of patients, mainly during the first year of life. Hyperacusis and hypersociability were common complaints (both present in 89%). Other behavioral and neuropsychiatric symptoms reported by the parents included attention deficit (89%), anger crises (83%), excessive fear (66%), depression (64%), anxiety (67%) and hypersexuality (33%). The most common complaints were hypersensitivity to sounds, talkative personality, emotional dependence and learning difficulties. In 98.3%, the parents denied family history. Conclusions: Williams-Beuren syndrome requires close follow-up with different medical specialties due to their variable clinical comorbidities, including language and school learning difficulties, behavioral and psychiatric problems.

Abstract Background: Verticality misperception is relatively common among patients after stroke, and it may be evaluated in terms of (a) subjective visual vertical (SVV), (b) subjective haptic vertical (SHV) and (c) subjective postural vertical (SPV). To better understand these assessment methods, we conducted a systematic review of the methodological characteristics of different protocols for evaluating SVV, SHV and SPV among individuals after stroke. Objective: To standardize the methodological characteristics of protocols for evaluating verticality perception after stroke. Methods: We searched the following databases: PUBMED, regional BVS portal (MEDLINE, LILACS, IBECS, CUBMED, Psychology Index and LIS), CINAHL, SCOPUS, Web of Science, Science Direct, Cochrane Library and PEDro. Two review authors independently used the QUADAS method (Quality Assessment of Diagnostic Accuracy Studies) and extracted data. Results: We included 21 studies in the review: most (80.9%) used SVV, eight (38.1%) used SPV and four (19.0%) used SHV. We observed high variability in assessments of verticality perception, due to patient positions, devices used, numbers of repetitions and angle of inclination for starting the tests. Conclusion: This systematic review was one of the first to explore all the methods of assessing verticality perception after stroke, and it provides crucial information on how to perform the tests, in order to guide future researchers/clinicians.

ABSTRACT Background: Stroke is among the leading causes of death and disability worldwide. Interventions for stroke rehabilitation aim to minimize sequelae, promote individuals’ independence and potentially recover functional damage. The role of aerobic exercise as a facilitator of post-stroke neuroplasticity in humans is still questionable. Objective: To investigate the impact of aerobic exercise on neuroplasticity in patients with stroke sequelae. Methods: A systematic review of randomized clinical trials and crossover studies was performed, with searches for human studies in the following databases: PUBMED, EMBASE, LILACS and PeDRO, only in English, following the PRISMA protocol. The keywords used for selecting articles were defined based on the PICO strategy. Results: This systematic review evaluated the impacts of aerobic exercise on neuroplasticity through assessment of neural networks and neuronal excitability, neurotrophic factors, or cognitive and functional assessment. Studies that evaluated the effects of aerobic exercise on neuroplasticity after stroke measured through functional resonance (fMRI) or cortical excitability have shown divergent results, but aerobic exercise potentially can modify the neural network, as measured through fMRI. Additionally, aerobic exercise combined with cognitive training improves certain cognitive domains linked to motor learning. Studies that involved analysis of neurotrophic factors to assess neuroplasticity had conflicting results. Conclusions: Physical exercise is a therapeutic intervention in rehabilitation programs that, beyond the known benefits relating to physical conditioning, functionality, mood and cardiovascular health, may also potentiate the neuroplasticity process. Neuroplasticity responses seem more robust in moderate to high-intensity exercise training programs, but dose-response heterogeneity and non-uniform neuroplasticity assessments limit generalizability.

ABSTRACT Background: In 2019, the world witnessed the emergence of a new type of coronavirus - the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). The spectrum of coronavirus disease 2019 (COVID-19) is variable, and amongst its manifestations are neurological implications. Objective: This report aimed to describe electroencephalographic findings in COVID-19 patients from a general tertiary hospital in São Paulo, Brazil. Methods: It was a retrospective, observational, and non-interventional study. Data were collected anonymously, comprising inpatients from Mar 1 to Jun 30, 2020, either confirmed (positive RT-PCR) or probable cases (CO-RADS 4/5) who had performed EEG during hospitalization. Results: Twenty-eight patients were enrolled, 17 (60.7%) women and 11 men, with a median age of 58 (minimum and maximum: 18-86; IQR 23.5). COVID-19 diagnosis was confirmed in 22 (78.5%). Twenty-one patients (75%) had severe disease, requiring mechanical ventilation due to acute respiratory distress syndrome (ARDS); 16 (57.1%) patients developed adjunct sepsis throughout hospitalization. There was no specific pattern found for COVID-19 in EEG. No patients presented with status epilepticus or electrographic events; most patients developed an encephalopathic pattern, as seen in most studies, with a high prevalence of altered mental status as an indication for EEG. Adjunct sepsis was associated with higher mortality. Conclusions: EEG presents as a useful tool in the context of COVID-19, as in other conditions, to differentiate nonconvulsive status epilepticus (NCSE) from encephalopathy and other causes of mental status alterations. Further studies are required to analyze whether there might be a specific EEG pattern to the disease.

ABSTRACT Introduction: Coronavirus pandemic began in China in 2019 (COVID-19), causing not only public health problems but also great psychological distress, especially for physicians involved in coping with the virus or those of the risk group in social isolation, and this represents a challenge for the psychological resilience in the world population. Studies showed that health professionals had psychological symptoms such as depression, anxiety, insomnia, stress, among others. Objectives: To investigate the quality of sleep and the prevalence rate of sleeping disorders among physicians during COVID-19 pandemic, and identify the psychological and social factors associated with the condition. Methods: A cross-sectional study of an online questionnaire was applied for physicians in Brazil. Among the 332 participants included, 227 were women. Sociodemographic assessment was used in the questionnaire, as well as the scale of impact on the events of modifications caused by COVID-19, assessment on sleep quality (PSQI), presence and severity of insomnia (ISI), depressive symptoms (PHQ-9), and anxiety (GAD-7). Results: Most physicians (65.6%) had changes in sleep. Poor sleep quality was reported by 73.1%, depressive symptoms were present in 75.8%, and anxiety in 73.4%. Conclusion: Our study found that more than 70% of the physicians assessed had impaired sleep quality, characterizing insomnia symptoms during COVID-19 outbreak. Related factors included an environment of isolation, concerns about COVID-19 outbreak and symptoms of anxiety and depression. Special interventions are needed to promote health professionals’ mental well-being and implement changes in this scenario.

ABSTRACT Background: Azathioprine is a common first-line therapy for neuromyelitis optica spectrum disorder (NMOSD). Objective: The aim of this study was to determine whether long-term treatment (>10 years) with azathioprine is safe in NMOSD. Methods: We conducted a retrospective medical record review of all patients at the School of Medicine of the University of São Paulo (São Paulo, Brazil) who fulfilled the 2015 international consensus diagnostic criteria for NMOSD and were treated with azathioprine for at least 10 years. Results: Out of 375 patients assessed for eligibility, 19 were included in this analysis. These patients’ median age was 44 years (range=28-61); they were mostly female (17/19) and AQP4-IgG seropositive (18/19). The median disease duration was 15 years (range=10-39) and most patients presented a relapsing clinical course (84.2%). The median duration of treatment was 11.9 years (range=10.0-23.8). The median annualized relapse rates (ARR) pre- and post-treatment with azathioprine were 1 (range=0.1-2) and 0.1 (range=0-0.35); p=0.09. Three patients (15.7%) had records of adverse events during the follow-up, which consisted of chronic B12 vitamin deficiency, pulmonary tuberculosis and breast cancer. Conclusion: Azathioprine may be considered a safe agent for long-term treatment (>10 years) of NMOSD, but continuous vigilance for infections and malignancies is required.

Abstract Background: Spinocerebellar ataxia type 3 (SCA3) is the most common autosomal dominant spinocerebellar ataxia worldwide. Almost all patients with SCA3 exhibit nystagmus and/or saccades impairment. Objective: To investigate the presence of nystagmus as an early neurological manifestation, before ataxia, in some patients with SCA3 in the first six months of the disease. Methods: We evaluated a series of 155 patients with clinically and molecularly proven SCA3 between 2013 and 2020. Data regarding sex, age, age at onset, disease duration, CAG repeat expansion length, first symptom, presence of ataxia, scores on SARA and ICARS scales, and presence and characteristics of nystagmus were collected. Results: We identified seven patients with symptomatic SCA3 who presented with isolated nystagmus. In these seven individuals the age at onset ranged from 24 to 57 years, and disease duration from four to six months. Conclusions: Our study showed that nystagmus may be the first neurological sign in SCA3. This clinical observation reinforces the idea that the neurodegenerative process in SCA3 patients may start in vestibular system connections or in flocculonodular lobe. This study adds relevant information about pre-symptomatic features in SCA3 that may work as basis for a better understanding of brain degeneration and for future therapeutic clinical trials.

ABSTRACT Background: Migraine is one of the most common disorders in neurological clinical practice and is part of the group of primary headaches. It often affects individuals in the age group of 25-55 years, when people are at their peak of economic productivity. Many patients ultimately overuse analgesics. Overuse is defined by the use of analgesics for at least 15 times a month - within a minimal three-month period. Impulsivity and migraine cause losses in the lives of individuals suffering from them, as they can compromise these individuals’ social, emotional, and professional spheres. Regarding the professional sphere, it results in economic losses compared with the overall population. Objective: To investigate the presence of impulsive behavior in patients with migraine and with medication-overuse migraine. Methods: Cross-sectional study on 210 female and male patients; 140 were diagnosed with migraine according to criteria of the International Classification of Headache Disorders and were subdivided into two groups (70 patients each). One of these groups was composed of medication-overuse patients; the control group was composed of non-migraine patients (70 patients). All patients were evaluated with the Barratt Impulsivity Scale (BIS-11). Results: The group of migraine patients accounted for the highest impulsivity level, followed by the control group and, finally, by the medication-overuse group. However, these differences did not attain statistical significance. Conclusions: It was not possible to establish a clear connection between migraine and impulsive behavior. Association was higher among migraineurs without medication overuse.

ABSTRACT Background: More than one-third of COVID-19 patients present neurological symptoms ranging from anosmia to stroke and encephalopathy. Furthermore, pre-existing neurological conditions may require special treatment and may be associated with worse outcomes. Notwithstanding, the role of neurologists in COVID-19 is probably underrecognized. Objective: The aim of this study was to report the reasons for requesting neurological consultations by internists and intensivists in a COVID-19-dedicated hospital. Methods: This retrospective study was carried out at Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, Brazil, a 900-bed COVID-19 dedicated center (including 300 intensive care unit beds). COVID-19 diagnosis was confirmed by SARS-CoV-2-RT-PCR in nasal swabs. All inpatient neurology consultations between March 23rd and May 23rd, 2020 were analyzed. Neurologists performed the neurological exam, assessed all available data to diagnose the neurological condition, and requested additional tests deemed necessary. Difficult diagnoses were established in consensus meetings. After diagnosis, neurologists were involved in the treatment. Results: Neurological consultations were requested for 89 out of 1,208 (7.4%) inpatient COVID admissions during that period. Main neurological diagnoses included: encephalopathy (44.4%), stroke (16.7%), previous neurological diseases (9.0%), seizures (9.0%), neuromuscular disorders (5.6%), other acute brain lesions (3.4%), and other mild nonspecific symptoms (11.2%). Conclusions: Most neurological consultations in a COVID-19-dedicated hospital were requested for severe conditions that could have an impact on the outcome. First-line doctors should be able to recognize neurological symptoms; neurologists are important members of the medical team in COVID-19 hospital care.

ABSTRACT Background: The novel coronavirus disease 2019 (COVID-19) pandemic poses a potential threat to patients with autoimmune disorders, including multiple sclerosis (MS) and neuromyelitis optica spectrum disorder (NMOSD). Such patients are usually treated with immunomodulatory or immunosuppressive agents, which may tamper with the organism’s normal response to infections. Currently, no consensus has been reached on how to manage MS and NMOSD patients during the pandemic. Objective: To discuss strategies to manage those patients. Methods: We focus on how to 1) reduce COVID-19 infection risk, such as social distancing, telemedicine, and wider interval between laboratory testing/imaging; 2) manage relapses, such as avoiding treatment of mild relapse and using oral steroids; 3) manage disease-modifying therapies, such as preference for drugs associated with lower infection risk (interferons, glatiramer, teriflunomide, and natalizumab) and extended-interval dosing of natalizumab, when safe; 4) individualize the chosen MS induction-therapy (anti-CD20 monoclonal antibodies, alemtuzumab, and cladribine); 5) manage NMOSD preventive therapies, including initial therapy selection and current treatment maintenance; 6) manage MS/NMOSD patients infected with COVID-19. Conclusions: In the future, real-world case series of MS/NMOSD patients infected with COVID-19 will help us define the best management strategies. For the time being, we rely on expert experience and guidance.

Abstract Background: Motor Imagery (MI) represents the cognitive component of the movement and recruits dopaminergic systems. Objective: To investigate the role of dopaminergic system through the action of methylphenidate and risperidone over beta coherence during execution, action observation and motor imagery. Methods: Electroencephalography (EEG) data were recorded before and after the substance intake. For statistical analysis, a three-way ANOVA was used to identify changes in beta coherence induced by the group, task and the moment variables. Statistical significance was set at p≤0.007. Results: We found a main effect for group for C3/CZ, and a main effect for task for CZ/C4 pairs of electrodes. Furthermore, significant differences were found in the post-drug administration between groups for C3/CZ pair of electrodes, and between task for C4/CZ pair of electrodes. Conclusion: The administration of methylphenidate and risperidone was able to produce electrocortical changes of the cortical central regions, even when featuring antagonistic effects on the dopaminergic pathways. Moreover, the execution task allowed beta-band modulation increase.

Blood plasma specimens are the clinical standard for HIV-1 pol gene genotyping from viral populations; however, it is not always successful, often from low viral loads or the presence of polymerase chain reaction (PCR) inhibitors. Objective To describe the successful of HIV-1 genotyping in two samples of cerebrospinal fluid (CSF), after genotype procedures failed from blood. Method Two HIV-infected patients enrolled in a neurocognitive research study were evaluated when standard HIV-1 genotyping failed from blood plasma samples. Genotyping was performed using the commercial system TRUGENE HIV-1 Genotyping Kit and the OpenGene DNA Sequencing System (Siemens Healthcare Diagnostics, Tarrytown, NY, USA). Results CSF genotyping was performed via the same commercial platform and was successful in both cases. Conclusion This report demonstrates that CSF could be used as an alternate clinical specimen for HIV-1 genotyping when it fails from blood.

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Part 1 of this guideline addressed the differential diagnosis of the neurofibromatoses (NF): neurofibromatosis type 1 (NF1), neurofibromatosis type 2 (NF2) and schwannomatosis (SCH). NF shares some features such as the genetic origin of the neural tumors and cutaneous manifestations, and affects nearly 80 thousand Brazilians. Increasing scientific knowledge on NF has allowed better clinical management and reduced rate of complications and morbidity, resulting in higher quality of life for NF patients. Most medical doctors are able to perform NF diagnosis, but the wide range of clinical manifestations and the inability to predict the onset or severity of new features, consequences, or complications make NF management a real clinical challenge, requiring the support of different specialists for proper treatment and genetic counseling, especially in NF2 and SCH. The present text suggests guidelines for the clinical management of NF, with emphasis on NF1.