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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.

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.

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.