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Sydenham's chorea is an autoimmune chorea emerging after a group A beta-hemolytic streptococcal (GABHS) infection, i.e. a rheumatic chorea with or without the presence of carditis or arthritis. The disorder, defined by the presence of chorea, is also associated with cognitive and behavioral symptoms, including emotional lability, anxiety, depressive and obsessive-compulsive symptoms. The authors review the pathophysiology, clinical characteristics, and available evidence on therapeutic strategies, the latter including the secondary prevention of GABHS infections, reduction of chorea, and immune modulation. Sydenham's chorea has been regarded as a model for pediatric autoimmune neuropsychiatric disorders, however, the field is marked by conflicting results and controversies. Regarding therapeutics, there are limited high-quality interventional studies and the selection of treatment strategy often relies on the clinician's experience. A serial treatment algorithm is presented based upon the severity of clinical presentation and response to symptomatic pharmacotherapy.

Context: Epilepsy affects more than 50 million people worldwide, which is an important cause of morbidity and functional limitations. Cortical development malformations represent about 8% of epilepsy cases, and are associated with cognitive deficits, that are frequently diagnosed in childhood. Case report: L.G.M, female, 35 years old, was attended in an emergency department with psychotic symptoms, aggressiveness and lowering of the sensorium. Computed Tomography (CT) scan of the brain evidenced hypodensity and loss of cortico-medullary differentation in the left temporo-parietal region. The neuroimaging findings motivated the referral to our service for investigation. Upon admission, has been shown a history of frequent focal motor and non-motor seizures associated with cognitive deficit since the age of 12. During hospitalization, a Magnetic Resonance Imaging (MRI) of the brain was performed, which showed subependymal heterotopy of gray matter (Figures 1 and 2). Since then, the monotherapy treatment with carbamazepine aiming at seizure control was chosen. Discussion and conclusion: Cortical development malformations can be classified into three groups of abnormalities, such as: 1) neuronal and glial proliferation and apoptosis; 2) neuronal migration; 3) cortical organization. A heterotopy of the gray matter is related to the migration disorder of the germinal matrix neurons on the wall of ventricle lateral to the cortex. It is the most frequent anomaly of cortical development. The perception of cognitive deficit associated with epileptic seizures should always awaken to the need for early investigation by image examination, in particular brain MRI, in order to diagnose possible malformations of cortical development.

Context: COVID19 has better known respiratory impacts than cardiovascular1 and high D-dimer as the most significant coagulation parameter2 . Otherwise, paradoxical embolism due to Patent Foramen Ovale (PFO) and Ischaemic Cerebral Vascular Accident (iCVA) mechanisms associated are poorly documented3, 4 . We aimed to report an associated case of iCVA and PFO and highlight COVID19 hypercoagulability triggering thromboembolisms. Case report: CTT, 76y, female, hypertensive, former-smoker was hospitalized with right-hemiplegia, transcortical aphasia, dysarthria by iCVA and evolved with headache, odynophagia, fever, chills. RT-PCR-SARS-Cov2: positive; chestCT: 25% bilateral pulmonary involvement, ground-glass opacities. 8days later, was transferred to ICU-COVID-HU-UFJF; D-dimer=827. In 30days, went to ward with O2-dependent pulmonary sequelae; CT-angiography excluded Pulmonary Thromboembolism. Searched iCVA mechanism, ECO detected PFO=2.8mm and Interatrial Septum aneurysm. Prescribed anticoagulants. Subsequently, presented right-clonus, further previous RankinScale=5. Requested brain-MRI pointed lobar hemorrhage with mass effect in recent iCVA’s territory (left-Middle Cerebral Artery). After 10days, a head-CT evidenced partial resorption and reduced mass effect. Patient was discharged taking rivaroxabana. Conclusions: Retrospectively, we suspected that COVID19 hypercoagulability triggered Deep Vein Thrombosis and the consequent PFO paradoxical embolism, which caused iCVA. Therefore, vascular pathologies in COVID19 deserve further studies. Treatment for secondary prevention in iCVA by PFO is uncertain3 .

Background: Alzheimer’s disease (AD) represents one of the main causes of cognitive and functional decline in the world. Concomitant with pharmacological treatment, the practice of aerobic exercises (AE) can help in the symptomatic control of the disease. Objectives: To evaluate the effects of AE on activities of daily living and cognition in patients with AD. Methods: A systematic review was undertaken. EMBASE, Pubmed and BVS databases were searched using the terms “Alzheimer disease”, “Alzheimer syndrome” and “Alzheimer dementia”; “aerobic” and “exercise”. The inclusion criteria were: randomized controlled trials from 2016 to 2021, English language studies and human studies. Among 854 studies found, six were included in the review. Results: The potential benefits of AE training in AD patients are: improvement of functioning, quality of life and cognitive performance; better control of neuropsychiatric symptoms and possible reduction of systemic inflammation. Conclusions: AEs are associated with cognitive and functional performance gain in AD, probably related to synaptic plasticity optimization and improvement of the feeling of well-being. Although AEs may improve cognitive and neuropsychiatric symptoms, the response to treatment is individual. Future longitudinal studies with larger cohorts and functional neuroimaging studies are required for a better understanding of the real benefit of AE in AD.

Background: Headache is the most frequent neurological complaint in the population and the group of tension-type headaches (TTH) is the most prevalent subtype. Nevertheless, more information about the clinical features of headaches in patients attended at specialized centers are demanded. Methods: Cross-sectional, descriptive study. Data from patients referred to an outpatient specialized headache center from 2018 to 2019 were analyzed and clinical and epidemiological information was collected. This study was authorized by the research ethics committee of the HUUFJF (CAAE 03530818.9.0000.5133). Results: Data from 153 patients were assessed. The mean age of patients was 45,6 years and most cases were women (80,4%). The most frequent diagnosis were migraine (49,7%), TTH (22,8%) and temporomandibular disorders (8,5%). The prevalence of chronic headaches was 46,6%. Analgesic abuse was identified in 32% of participants, with a higher prevalence in women (Fischer’s exact test, P=0,05). Prophylactic treatment was used by 84,3% of the subjects. Pain was self-reported mild in 21,6% of cases, moderate in 30,1% and severe in 47,1%. The pain severity was inversely proportional to age (P=0,012). The most frequent associated symptoms were photophobia (57,5%), phonophobia (56,9%), nausea/ vomiting (47,1%). Conclusions: The findings show important differences in the prevalence of headache cases in specialized centers compared to the general population. Given the high prevalence of analgesic abuse reported, the development of effective educational programs for patients and healthcare providers at primary and secondary health services, might reduce the social burden of chronic headaches and decrease the demand for consultations on specialized headache clinics.

ObjectiveIschaemic stroke is a severe complication of rheumatic heart disease (RHD), which may result in permanent disability and death. This study aimed to assess the incidence and predictors of stroke in patients with RHD in the current era of evidence-based recommendations for prevention.MethodsConsecutive patients with RHD diagnosed by clinical and echocardiographic criteria were selected. A structured clinical and neurological assessment was performed to determine the aetiology and classification of stroke at enrolment. The primary endpoint was an ischaemic cerebrovascular event, which included fatal or non-fatal stroke. Risk of stroke was estimated accounting for competing risks.ResultsA total of 515 patients were enrolled, 438 women (85%), 46±12 years of age. The most frequent valve lesion was mixed mitral (80%). At the time of enrolment, 92 patients (18%) had a prior stroke, with anterior circulation infarction being the most frequent topography (72%). During the mean follow-up of 3.9 years, 27 patients (5.2%) had stroke with the overall incidence of 1.47 strokes per 100 patient-years. Predictors of stroke by the Cox model were prior stroke (adjusted HR 5.395, 95% CI 2.272 to 12.811), age (HR 1.591, 95% CI 1.116 to 2.269) and atrial fibrillation (AF) at baseline (HR 2.945, 95% CI 1.083 to 8.007). By considering death as a competing risk, the effect of AF on stroke risk was attenuated (HR 2.287, 95% CI 0.962 to 5.441).ConclusionsIn this large cohort of patients with RHD, stroke occurred in 5.2% of the patients, which was predicted by age, AF and prior stroke. The effect of AF on stroke risk estimation was influenced by death as competing risk.

CNPq - Conselho Nacional de Desenvolvimento Científico e Tecnológico FAPEMIG - Fundação de Amparo à Pesquisa do Estado de Minas Gerais CAPES - Coordenação de Aperfeiçoamento de Pessoal de Nível Superior Introdução: A cardiopatia reumática crônica (CRC) representa a manifestação mais grave de febre reumática (FR) aguda, resultante de uma resposta imune anormal à faringite estreptocócica a qual desencadeia tanto o dano valvar quanto a potenciais danos ao sistema nervoso central. O presente estudo avaliou a prevalência de transtornos neurológicos e psiquiátricos em pacientes com CRC e apresenta as características associadas à disfunção motora dos núcleos da base nessa população. Métodos: Realizamos avaliação clínica, neurológica e psiquiátrica estruturada de pacientes com CRC recrutados de um centro especializado em tratamento de doenças valvares. Foram aplicados questionários validados para avaliação da cognição, depressão, ansiedade e sintomas obsessivo-compulsivos. A disfunção motora dos núcleos da base (DMNB) foi definida como a presença de disfunção clínica dos circuitos motores fronto-estriatais, tendo como base a detecção de distúrbios do movimento hipercinéticos ao exame neurológico. Resultados: Cinquenta pacientes foram incluídos com a média de idade de 43,2 ± 10,8 anos, nos quais 42 pacientes (84%) foram do sexo feminino. A valva mitral foi acometida em 47 pacientes (94%) e 21 deles (42%) também tiveram acometimento da valva aórtica. A prevalência de transtornos neurológicos e psiquiátricos foi alta representado por coreia (22%), tiques crônicos (18%), sintomas obsessivo-compulsivos (48%), transtorno depressivo maior (34%), transtorno de ansiedade generalizada (54%), queixas cognitivas (66%), migrânea (52%) e crises epilépticas (18%). Os fatores independentemente associados aos sinais clínicos de DMNB foram a idade (odds ratio ajustada [OR]: 0,916, intervalo de confiança de 95% [IC]: 0,853 - 0,985; p = 0,018), a depressão maior (OR ajustada: 7,534, IC 95%: 1,528 - 37,148; p = 0,013) e o escore de sintomas obsessivo-compulsivos Yale-Brown Obsessive Compulsive Scale (Y-BOCS) (OR ajustado: OR: 1,101, IC 95%: 1,022 - 1,187; p = 0,011). A gravidade da doença cardíaca não se associou à DMNB. Conclusões: As manifestações neurológicas e comportamentais são frequentes em pacientes com CRC, as quais podem persistir por até 3 décadas desde a FR aguda. A idade, o transtorno depressivo maior e o escore Y-BOCS foram associados à DMNB. Estas manifestações merecem uma atenção especial de clínicos e pesquisadores que lidam com pacientes adultos com CRC. Background: Rheumatic heart disease (RHD) is the most serious manifestation of rheumatic fever resulting from an abnormal immune response to a streptococcal pharyngitis, which may also affect the brain. The present study assessed the prevalence of neurological and psychiatric disorders in RHD patients and clinical features associated with basal ganglia motor dysfunction. Methods: We conducted structured neurologic and psychiatric assessment in randomly selected patients with RHD among those routinely referred to a tertiary center for heart valve diseases. Validated questionnaires for the evaluation of cognition, depression, anxiety, and obsessive-compulsive symptoms were applied. Basal ganglia motor dysfunction (BGMD) was defined as the presence of clinically-determined involvement of motor frontostriatal circuits based on the detection of hyperkinetic movement disorders. Echocardiography was performed to assess the pattern of valvular involvement and RHD severity. Results: Fifty patients with age of 43.2 ± 10.8 years, 84% female, were included. The mitral valve was affected in 47 patients (94%), and 21 of them (42%) had also aortic valve involvement. Neurological and psychiatric disorders were frequent, including chorea (22%), chronic tics (18%), obsessive-compulsive symptoms (48%), major depression (34%) and generalized anxiety disorder (54%), cognitive complaints (66%), migraine (52%) and seizures (18%). The factors associated with clinical signs of BGMD were age (adjusted odds ratio [OR]: 0.916, 95% confidence interval [CI]: 0.853 - 0.985; p = 0.018), major depression (adjusted OR: 7.534, 95% CI: 1.528 - 37.148; p = 0.013), and Yale-Brown Obsessive Compulsive (Y-BOCS) score (adjusted OR: 1.101, 95% CI: 1.022 - 1.187; p = 0.011). The severity of heart disease was not associated with clinically-defined BGMD. Conclusions: Neurological and behavioral manifestations are frequent in RHD patients, which may persist up to 3 decades after acute rheumatic fever. Age, major depression and Y-BOCS score were independently associated with BGMD. These manifestations deserve a close attention of clinicians and researchers dealing with adults patients with RHD.

Psychiatric disorders, notably mood and anxiety disorders, are frequently associated with migraine and chronic daily headaches. The obsessive-compulsive disorder (OCD) is included in the spectrum of anxiety disorders and may be a comorbid condition in headache patients. However, little information has been reported in the literature about this association. This is an important issue as OCD may contribute to the development or maintenance of treatment-resistant chronic headaches. In this paper, we describe a young female patient with refractory chronic migraine and OCD. Considerations on diagnosis, management and treatment of these comorbid conditions are presented.

Dear Editor,Hemiballismus, a term derived from a Greek wordmeaning ‘‘to throw’’, is characterized by involuntary,flinging, throwing and kicking large-amplitude movementsof the limbs of one side of the body. The area of the brainusually implicated in its pathophysiology is the contralat-eral subthalamic nucleus. However, hemiballismus has alsobeen reported to occur outside the subthalamic nucleus atpoints that involve afferent or efferent pathwaysconnecting the nucleus to its projection areas including thebasal ganglia, and motor and premotor cortex. It is believedthat hemiballismus is the overt clinical manifestation ofdisinhibited thalamic outflow when tonic subthalamicactivity is interrupted [1].The most common cause of hemiballismus is cerebro-vascular disease, but a range of diseases can occur(hemorrhage, vascular malformations, tuberculomas,demyelinating plaques, tuberous sclerosis, toxoplasmosis,non-ketotic hyperglycemia, lupus vasculitis, leukemicinfiltration of the brain, primary brain tumors and drug-induced disease) [2]. Herein, we report a patient withmetastatic breast cancer who presented with hemiballismus.A 73-year-old illiterate woman with a past medicalhistory of asthma and cardiac insufficiency was diagnosedwith bone and pulmonary metastatic breast cancer andunderwent mastectomy and chemotherapy in June 2012.She presented to a neurological consultation in February2013 with a 5-year history of progressive cognitiveimpairment that subsequently led to dementia withdependence for most activities of daily living and a1-month history of generalized involuntary flingingmovements of her proximal limbs and face. Examinationshowed a third nerve palsy (sequela from traumatic injury)combined with a lower-limb predominant right hemichorea(Video1). Brain magnetic resonance imaging revealedmultiple contrast-enhanced nodular lesions, one of them inthe left subthalamus (Fig. 1). Cerebrospinal fluid analysiswas normal. She received risperidone with partial recoveryof chorea and cranial radiotherapy.Hemiballismus secondary to metastatic tumors are dis-tinctly rare, but it has already been reported with carcino-mas of the breast, lung and gall bladder [3, 4]. The firstcase of a metastatic lesion affecting the subthalamus wasreported in 1973 [3] and it was followed by a French case

Studies using resources of a public family health program to estimate the prevalence of chronic daily headaches (CDH) are lacking.To estimate the 1-year prevalence of CDH, as well as the presence of associated psychiatric and temporomandibular disorders (TMD) comorbidities, on the entire population of a city representative of the rural area of Brazil.This was a cross-sectional, population-based, 2-phase study. In the first phase, health agents interviewed all individuals older than 10 years, in a rural area of Brazil. In the second stage, all individuals who reported headaches on 4 or more days per week were then evaluated by a multidisciplinary team. CDH were classified according to the second edition of the International Classification of Headache Disorders (ICHD-2). Medication overuse headache was diagnosed, as per the ICHD-2, after detoxification trials. Psychiatric comorbidities and TMD were diagnosed based on the DSM-IV and on the Research Diagnostic Criteria for Temporomandibular Disorders criteria, respectively.A total of 1631 subjects participated in the direct interviews. Of them, 57 (3.6%) had CDH. Chronic migraine was the most common of the CDH (21, 36.8%). Chronic tension-type headache (10, 17.5%), medication overuse headache (13, 22.8%) and probable medication overuse headache (10, 17.5%) were also common. Psychiatric disorders were observed in 38 (67.3%) of the CDH subjects. TMD were seen in 33 (58.1)% of them.The prevalence of CDH in the rural area of Brazil is similar to what has been reported in previous studies. A significant proportion of them have psychiatric comorbidities and/or TMD. In this sample, comorbidities were as frequent as reported in convenience samples from tertiary headache centers.

Objective.— To estimate the 1-year prevalence of headache, using face-to-face interviews of the entire population of a city in Brazil. Methods.— This was a cross-sectional, population-based study. We conducted face-to-face interviews of all individuals older than 10 years, in a town (Capela Nova) in Brazil. Prevalence of the headache was estimated using prevalence ratios, adjusted by gender, age, marital status, and level education. Results.— The estimated 1-year prevalence of any headache was 65.4%. Headache was 1.5 times more prevalent in women and individuals aged 20 and 29, but less prevalent in the elderly and divorced. There was not association with the level of education. Conclusions.— From a public healthcare perspective, headache should be seen as hypertension and diabetes, and actively screened and treated.

Ophthalmoplegic migraine (OM) is a rare disorder characterized by recurrent episodes of migraine-like headaches associated with extrinsic ocular musculature palsy. In this article, we report a patient with OM that presented recurrent palsy of the abducens nerve and other atypical features. Case reports of OM with abducens nerve palsy were also reviewed.

JUSTIFICATIVA E OBJETIVOS: A comparação entre as características da cefaleia encontradas na população geral e em centros de cuidados terciários pode elucidar fatores associados à procura de consulta médica e obstáculos ao atendimento. O objetivo deste estudo foi contrastar os achados demográficos e a frequência de migrânea e de cefaleia do tipo tensional (CTT) na população geral e em um centro de atendimento especializado. MÉTODO: Todos os habitantes de uma pequena cidade foram entrevistados quanto à presença de cefaleia. Em uma região, escolhida por sorteio, os moradores que responderam positivamente foram avaliados por uma equipe de neurologistas especialistas em cefaleia. Esses profissionais também avaliaram uma casuística de pacientes atendidos consecutivamente em um centro especializado. Os diagnósticos seguiram os critérios da Classificação Internacional das Cefaleias-2004. RESULTADOS: Foram entrevistados 1.605 moradores em toda cidade e 258 na região da amostra. Destes, os 76 que tinham cefaleia passaram por avaliação neurológica, bem como 289 pacientes do centro especializado. As mulheres representaram a maioria, tanto na comunidade quanto no ambulatório. Na população, a frequência de CTT foi de 77,6% e a de migrânea de 61,8%, havendo sobreposição diagnóstica em boa parcela dos casos. Já no ambulatório a vasta maioria dos pacientes tinham migrânea (79,8%), enquanto apenas 20,4% tinham CTT, sendo a associação diagnóstica bem menos comum. CONCLUSÃO: A CTT é mais comum na comunidade e a migrânea em centros especializados. Conhecer os contrastes destas cefaleias primárias nestes dois cenários pode auxiliar o planejamento de ações preventivas e utilização dos recursos assistenciais. BACKGROUND AND OBJECTIVES: The comparison of headache features in general population and in tertiary care centers may explain factors associated to the search for medical assistance and the obstacles to such assistance. This study aimed at comparing demographic findings and the frequency of migraine and tension headache (THA) in general population and in a specialized care center. METHOD: All inhabitants of a small village were interviewed about the presence of headache. In one randomly selected region, people who answered positively were evaluated by a team of neurologists specialized in headache. They have also evaluated a number of patients consecutively treated by a specialized center. Diagnoses have followed International Headaches Classification criteria (2004). RESULTS: Participated in this study 1605 inhabitants of the whole village and 258 inhabitants of the region selected as sample. From these, 76 people reporting headache went through a neurological evaluation, as well as 289 patients of the specialized center. THA was the most common headache among general population (77.6%), followed by migraine (61.8%) with diagnostic overlapping in a good percentage of cases. In the outpatient setting the vast majority of patients had migraine (79.8%), while only 20.4% had THA, being the diagnostic association far less common. CONCLUSION: THA is more common in the community and migraine prevails in specialized centers. Understanding the contrasts of both primary headaches within these two scenarios may help the planning of preventive actions and the use of health care resources.