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COVID-19 induces acute and persistent neurological symptoms in mild and severe cases. Proposed concomitant mechanisms include direct viral infection and strain, coagulopathy, hypoxia, and neuroinflammation. However, underlying molecular alterations associated with multiple neurological outcomes in both mild and severe cases are majorly unexplored. To illuminate possible mechanisms leading to COVID-19 neurological disease, we retrospectively investigated in detail a cohort of 35 COVID-19 mild and severe hospitalized patients presenting neurological alterations subject to clinically indicated cerebrospinal fluid (CSF) sampling. Clinical and neurological investigation, brain imaging, viral sequencing, and cerebrospinal CSF analyses were carried out. We found that COVID-19 patients presented heterogeneous neurological symptoms dissociated from lung burden. Nasal swab viral sequencing revealed a dominant strain at the time of the study, and we could not detect traces of SARS-CoV-2's spike protein in patients' CSF by multiple reaction monitoring analysis. Patients presented ubiquitous systemic hyper-inflammation and broad alterations in CSF proteomics related to inflammation, innate immunity, and hemostasis, irrespective of COVID-19 severity or neuroimaging alterations. Elevated CSF interleukin-6 (IL6) correlated with disease severity (sex-, age-, and comorbidity-adjusted mean Severe 24.5 pg/ml, 95% confidence interval (CI) 9.62–62.23 vs. Mild 3.91 pg/mL CI 1.5–10.3 patients, p = 0.019). CSF tumor necrosis factor-alpha (TNFα) and IL6 levels were higher in patients presenting pronounced neuroimaging alterations compared to those who did not (sex-, age-, and comorbidity-adjusted mean TNFα Pronounced 3.4, CI 2.4–4.4 vs. Non-Pronounced 2.0, CI 1.4–2.5, p = 0.022; IL6 Pronounced 33.11, CI 8.89–123.31 vs Non-Pronounced 6.22, CI 2.9–13.34, p = 0.046). Collectively, our findings put neuroinflammation as a possible driver of COVID-19 acute neurological disease in mild and severe cases.

Over the last three decades, stroke care has undergone significant transformations mainly driven by the introduction of reperfusion therapy and the organization of systems of care. Patients receiving treatment through a well-structured stroke service have a much higher chance of favorable outcomes, thereby decreasing both disability and mortality. In this article, we reviewed the scientific evidence for stroke reperfusion therapy, including thrombolysis and thrombectomy, and its implementation in the public health system in Brazil.

Migraine and ischemic strokes are two of the most prevalent diseases worldwide. Besides having a coincident symptomatology, for long researchers have been searching for a possible causal relation between these diseases. Current evidence based on data suggest that patients with aura migraine could have a doubled risk of developing an ischemic stroke, when compared to the rest of the population. At the same time, migraine sufferers apparently have higher incidences of risk factors for cardiovascular events. The aim of this review was not only to dissect some of the more compelling evidence based on data regarding this association, but also to discuss the possible clinical and therapeutic implications.

Although the prevalence of neuropsychiatric disorders among patients with cerebrovascular illness is relatively high, there are only few case reports describing post-stroke psychotic symptoms. In general, post-stroke psychoses have been reported to emerge few days after the vascular event and to vanish soon afterwards. In this report, we describe delayed-onset post-stroke delusional disorder, persecutory type. A middle-aged female patient developed a persistent delusional disorder with homicidal behavior about one year after a cerebrovascular accident affecting the right fronto-temporo-parietal region and a long period of chronic post-stroke mixed anxiety and depressive symptoms. Our case suggests that there might be long intervals between stroke and the appearance of psychotic symptoms.

Neurosonological studies, specifically transcranial Doppler (TCD) and transcranial color-coded duplex (TCCD), have high level of specificity and sensitivity and they are used as complementary tests for the diagnosis of brain death (BD). A group of experts, from the Neurosonology Department of the Brazilian Academy of Neurology, created a task force to determine the criteria for the following aspects of diagnosing BD in Brazil: the reliability of TCD methodology; the reliability of TCCD methodology; neurosonology training and skills; the diagnosis of encephalic circulatory arrest; and exam documentation for BD. The results of this meeting are presented in the current paper.

Patent foramen ovale (PFO), a relatively common abnormality in adults, has been associated with migraine. Few studies also linked PFO with cluster headache (CH). To verify whether right-to-left shunt (RLS) is related to headaches other than migraine and CH, we used transcranial Doppler following microbubbles injection to detect shunts in 24 CH, 7 paroxysmal hemicrania (PH), one SUNCT, two hemicrania continua (HC) patients; and 34 matched controls. RLS was significantly more frequent in CH than in controls (54% vs. 25%, p=0.03), particularly above the age of 50. In the HC+PH+SUNCT group, RLS was found in 6 patients and in 2 controls (p=0.08). Smoking as well as the Epworth Sleepiness Scale correlated significantly with CH, smoking being more frequent in patients with RLS. PFO may be non-specifically related to trigeminal autonomic cephalalgias and HC. The headache phenotype in PFO patients probably depends on individual susceptibility to circulating trigger factors.O forame oval patente (FOP), uma anormalidade cardíaca relativamente comum em adultos, tem sido associado à enxaqueca, mas raramente às cefaléias trigêmino-autonômicas (TACs). Utilizamos o Doppler transcraniano (DTC) para detecção de shunt direito-esquerdo (SDE) em 24 pacientes com cefaléia em salvas (CS), sete com hemicrania paroxística (HP), dois com hemicrania continua (HC) e um com SUNCT; alem de 34 controles. O SDE foi mais frequente nos pacientes com CS do que nos controles (54% vs. 25% p=0,03), particularmente acima de 50 anos. No grupo HP+HC+SUNCT, o SDE foi encontrado em seis pacientes e dois controles (p=0,08). O hábito de fumar, bem como sonolência excessiva diurna foram mais frequentes em paciente com CS. O FOP pode ter importância inespecífica na fisiopatologia das TACs e HC, na dependência da susceptibilidade individual a fatores desencadeantes.

Monomelic amyotrophy (MA) is a rare condition in which neurogenic amyotrophy is restricted to an upper or lower limb. Usually sporadic, it usually has an insidious onset with a mean evolution of 2 to 4 years following first clinical manifestations, which is, in turned, followed by stabilization. We report a case of 20-years-old man who presented slowly progressive amyotrophy associated with proximal paresis of the right upper limb, which was followed by clinical stabilization 4 years later. Eletroneuromyography revealed denervation along with myofasciculations in various muscle groups of the right upper limb. We call atention to this rare location of MA, as well as describe some theories concerning its pathophysiology .A amiotrofia monomélica é condição rara em que a amiotrofia neurogênica é restrita somente a um membro superior ou inferior. Usualmente esporádica, possui um início insidioso e evolução lenta de 2-4 anos após as primeiras manifestações, seguida por estabilização. Relatamos o caso de um homem que aos 20 anos apresentou lentamente amiotrofia e paresia proximal no membro superior direito, estabilizando-se em quatro anos. A eletroneuromiografia identificou a presença de desnervação e fasciculações em diversos músculos proximais do membro superior direito. Chamamos a atenção para esta rara localização desta entidade, assim como descrevemos algumas das hipóteses da fisiopatologia.

O acidente vascular cerebral (AVC) é a terceira causa de óbito e a principal causa de incapacidade em indivíduos adultos. Embora a mortalidade do AVC esteja diminuindo em alguns países, a morbidade tem aumentado em razão do envelhecimento da população e do aumento da sobrevida dos pacientes¹. O tratamento com ativador do plasminogênio tissular recombinante (rt-PA) é eficaz quando instituído em até 3 horas após o início dos sintomas², porém seu uso está limitado a cerca de 5% dos pacientes na fase aguda do AVC isquêmico. Além disso, nenhum agente para neuroproteção teve sua eficácia comprovada em estudos clínicos em humanos. Portanto, outras estratégias terapêuticas precisam ser desenvolvidas. Em modelos animais, o uso de células-tronco correlacionou-se com melhora funcional após o AVC³. Publicações recentes têm demonstrado a segurança do tratamento com células mononucleares da medula óssea (CMMO) injetadas via intracoronária em pacientes portadores de cardiopatia isquêmica aguda ou crônica4,5. Baseado nesses dados iniciais, há crescente interesse no estudo do transplante com CMMO na fase aguda do AVC. Relatamos o primeiro caso de transplante autólogo de CMMO via intra-arterial na fase aguda do AVC isquêmico.Stroke is the third cause of death and the leading cause of disability in adult subjects. Although stroke mortality has been declining in some countries, stroke morbidity has been increasing due to the aging of population and patients improved survival.¹ Treatment with recombinant tissue plasminogen activator (rtPA) is successful provided it is administered within 3 hours of symptoms onset,² but its use is limited to about 5% of the patients with acute ischemic stroke. Furthermore, no neuroprotective agent has yet been proven effective in human clinical trials. The development of other therapeutic strategies is, therefore, warranted. The use of stem cells in animal models has led to functional improvement following stroke.³ Recent publications have shown that bone marrow mononuclear cells (BM-MNC) therapy through intracoronary injection is a safe procedure in patients with acute or chronic ischemic heart disease.4,5 Based on these preliminary data, there has been growing interest in the study of BM-MNC transplantation for acute ischemic stroke. We report the first case of intra-arterial autologous BM-MNC transplantation for acute ischemic stroke.

The authors report the case of a 29 years old male patient presenting classical Guillain-Barré syndrome following head trauma. Only one other similar case is described in the literature. Head trauma as a precipitating event of the disease is discussed.Os autores relatam o caso de um paciente de 29 anos que apresentou síndrome de Guillain-Barré clássica após traumatismo crânio-encefálico. Citam o único caso semelhante descrito na literatura e discutem o trauma como fator desencadeante desta polirradiculoneuropatia.

Com finalidade de orientar e oferecer subsídios para a conduta diagnóstica e terapêutica em diferentes situações dentro das doenças cerebrovasculares, a Sociedade Brasileira de Doenças Cerebrovasculares (SBDCV) constituiu um comitê composto por neurologistas de diferentes áreas do Brasil que emitiram um parecer, denominado "Opinião Nacional", redigido nos moldes dos consensos. O presente artigo analisa a "neuroproteção no acidente vascular cerebral" discutindo o nível de evidência para o uso de drogas de potencial ação neuroprotetora e ensaios clínicos em andamento.The Brazilian Stroke Society constituted a committee composed by specialists from different areas of Brazil that emitted a viewpoint called "National Opinion", written similar to the consensus pattern. The study purpose is to guide and offer subsidies for diagnosis and therapeutical plans for different situations in cerebrovascular diseases. The current article analyses "neuroprotective agents in stroke", discussing the level of evidence for the use of potential neuroprotective drugs and ongoing clinical trials.

Foram estudados 12 pacientes com distrofia muscular miotônica com o objetivo de verificar o comprometimento do sistema nervoso periférico. Todos apresentavam os sinais e sintomas principais da doença. Nenhum tinha outras causas que pudessem justificar uma polineuropatia. Todos foram submetidos a biópsia do nervo sural com contagem de fibras mielínicas e realização de histograma. Dois não apresentavam histograma bimodal. Os pacientes mostraram redução do número de fibras mielínicas. Concluímos que a polineuropatia pode fazer parte do quadro clínico polimorfo da distrofia muscular miotônica.Twelve patients with myotonic dystrophy were studied to look for the involvement of the peripheral nervous system in this disease. All of them showed the main signs and symptoms of the disease. They did not have another causes to justify a polineuropathy. They were submitted to sural nerve biopsy with counting of myelinated fibers and histogram. Patients showed a reduction in the number of myelinated fibers and in two patients the histogram was unimodal. We concluded that polineuropathy may be another multisystemic manifestation of myotonic dystrophy.

A doença de Charcot-Marie-Tooth (CMT) é uma das moléstias mais frequentes do sistema nervoso periférico e ocupa o primeiro lugar dentre as afecções neuromusculares hereditárias. Estudamos 45 pacientes com doença de CMT atendidos no Hospital Universitário Antônio Pedro da Universidade Federal Fluminense. Foram classificados de acordo com a neurocondução em: tipo I (desmielinizante), 11 casos; e tipo II (anoxal), 34 casos. Vinte e três tinham herança autossômica dominante, 7 casos eram do tipo autossômico recessivo e 15 eram esporádicos. A maioria teve o início dos sintomas na 1ª ou 2ª décadas. Todos tiveram paresia distal dos membros inferiores. Nos membros superiores a paresia foi vista em 38,2%. A amiotrofia distal ocorreu em 80% nos membros inferiores e em 50% nos superiores. A arreflexia patelar e aquiliana foi elevada. Abolição dos reflexos profundos nos membros superiores ocorreu em 28%. As alterações sensitivas limitaram-se a discreta hipoestesia nos membros inferiores. Em 7 enfermos encontramos hipertrofia dos troncos nervosos. Os pés cavos e a escoliose foram vistos em 21 casos e 7 casos respectivamente. Tremor nas mãos foi encontrado em 6 pacientes. Outros sinais mais raros, como oligofrenia e atrofia óptica, foram vistos excepcionalmente. A evolução da doença foi quase sempre benigna. Somente 7 casos evoluíram rapidamente.Charcot-Marie-Tooth (CMT) disease is the commonest inherited peripheral neuropathy. The clinical study of 45 patients with CMT is presented. They were derived from Antonio Pedro Hospital of Universidade Federal Fluminense in Niteroi, RJ, Brazil. Such patients could be divided by the motor conduction velocity in two types: a demyelinating form or type I (11 cases) and an axonal form or type II (34 cases). The disease was inherited as an autosomal dominant trait in 23 patients and as an autosomal recessive trait in 7 cases. In 15 patients the disorder was sporadic. The age of onset was in most of our cases before the 20 years. All of them had distal weakness in lower limbs. 38.2% had also distal weakness in upper limbs. 80% had distal wasting of the lower limbs and 50% had distal wasting of upper limbs. The tendon reflexes were absent in 64% in lower limbs and in 28% in upper limbs. The sensitive impairment in the distal regions of the extremities was mild in most patients. We found enlargement of peripheral nerves in 7 patients of type I. Pes cavus was present in 21 cases and scoliosis in 7. We found postural tremor of hands in 6 patients. In 9 cases there were rare features as mental retardation, trigeminal nevralgia, optic atrophy, deafness and calf enlargement. In most of our cases the clinical course was very slow progressive. A greater severity was seen in our sporadic cases.

São apresentados os resultados da biópsia do nervo sural à microscopia óptica e eletrônica (ME) em 41 pacientes com doença de Charcot-Marie-Tooth (CMT) Por estudos de neurocondução prévios nove eram do tipo I e 32 do tipo II. No tipo I, todos tinham grande diminuição do número de fibras, sendo os histogramas do tipo unimodal. Encontramos imagens de desmielinização, remielinização, formação de bulbos de cebola e de regeneração. Um paciente apresentava espessamento da bainha de mielina (atrofia axonal). No tipo II, sete pacientes não apresentavam anomalia à microscopia, com histograma normal. Nos restantes havia discreta a intensa perda de fibras mielínicas principalmente as de grande calibre. Em cinco enfermos havia aumento do número de fibras devido a grande regeneração. Alguns pacientes do tipo II apresentavam à ME imagens de pequenos bulbos de cebola e em um havia também atrofia axonal. Comparando com os dados clínicos e de neurocondução motora, no tipo I não encontramos relação entre a intensidade do quadro clínico e a perda de fibras mielínicas porém houve paralelismo da queda da neurocondução motora e a diminuição do número de fibras. No tipo II não houve relação entre o quadro clínico, a neurocondução e os achados da biópsia nervosa.We studied the pathological findings of sural nerve biopsy in 41 patients with Charcot-Marie-Tooth (CMT) disease. They were previously classified by the median motor conduction velocity (MCV) in two types. Type I (demyelinating) with 9 patients and type II (axonal) with 32 cases. In type I we found loss of myelinated fibers (unimodal histogram), demyelinated and remyelinated axons with numerous onion bulb formations. In one case there was thickness of myelin with thin axons (axonal atrophy). In type II there were seven patients with normal sural nerve biopsy. In 25 cases there were mild to severe loss of myelinated fibers. In 5 patients the number of myelinated fibers was increased due to the great regeneration of the axons. The electron microscopic studies in type II showed in a few cases small onion bulbs and in one case axonal atrophy. In type I there was no correlation between clinical severity and the loss of myelinated fibers, but there was relationship between the low MCV and the intensity of myelinated fibers. In type II we did not found any correlation between clinical course, MCV and pathological findings.

Transplantation of autologous bone marrow mononuclear cells (BMMCs) has been proven safe in animal and human studies. However, there are very few studies in stroke patients. In this study, intra-arterial autologous BMMCs were infused in patients with moderate to severe acute middle cerebral artery infarcts. The subjects of this study included 20 patients with early or late spontaneous recanalization but with persistent deficits, in whom treatment could be initiated between 3 and 7 days after stroke onset. Mononuclear cells were isolated from bone marrow aspirates and infused at the proximal middle cerebral artery of the affected hemisphere. Safety analysis (primary endpoint) during the 6-month follow-up assessed death, any serious clinical events, neurological worsening with ≥ 4-point increase in National Institutes of Health Stroke Scale (NIHSS) scores, seizures, epileptogenic activity on electroencephalogram, and neuroimaging complications including new ischemic, hemorrhagic, or neoplastic lesions. Satisfactory clinical improvement (secondary endpoint) at 90 days was defined according to the pretreatment NIHSS scores as follows: modified Rankin Scale score of 0 in patients with NIHSS 14. Good clinical outcome was defined as mRS ≤2 at 90 days. Serial clinical, laboratory, electroencephalogram, and imaging evaluations showed no procedure-related adverse events. Satisfactory clinical improvement occurred in 6/20 (30%) patients at 90 days. Eight patients (40%) showed a good clinical outcome. Infusion of intra-arterial autologous BMMCs appears to be safe in patients with moderate to severe acute middle cerebral artery strokes. No cases of intrahospital mortality were seen in this pilot trial. Larger prospective randomized trials are warranted to assess the efficacy of this treatment approach.

Chédiak-Higashi syndrome is a rare hematological disease characterized by increased fusion of cytoplasmatic granules. Neurological symptoms occur in approximately half of the patients. We describe the clinical, eletrophysiological, hematological and radiological findings in a girl who had Chédiak-Higashi syndrome and seizures.A síndrome de Chédiak-Higashi é uma doença hematológica rara caracterizada pelo aumento da fusão dos grânulos citoplasmáticos. Manifestações neurológicas ocorrem em cerca de metade dos pacientes. Relatamos o caso de uma criança com síndrome de Chédiak-Higashi e crises convulsivas.

ABSTRACT These guidelines are the result of a joint effort from writing groups of the Brazilian Stroke Society, the Scientific Department of Cerebrovascular Diseases of the Brazilian Academy of Neurology, the Brazilian Stroke Network and the Brazilian Society of Diagnostic and Therapeutic Neuroradiology. Members from these groups participated in web-based discussion forums with predefined themes, followed by videoconference meetings in which controversies and position statements were discussed, leading to a consensus. This guidelines focuses on the implications of the recent clinical trials on endovascular therapy for acute ischemic stroke due to proximal arterial occlusions, and the final text aims to guide health care providers, health care managers and public health authorities in managing patients with this condition in Brazil.