Radiation-induced cerebral cavernous malformation: is it just a childhood disease?

Cerebral cavernous malformations (CCM) are clusters of dilated sinusoidal channels, lined by a single layer of endothelium. Acquired form of these lesions was related to previous radiation-therapy, and might be related to its pathophysiology, because the vascular endothelial growth factor has higher expression in the immature brain of the child. Consequently, although it occurs mainly in the paediatric population, it can happen in adults. We report a case of radiation-induced CCM in an adult patient presenting with aggressive behaviour and cerebral haemorrhage.

Chemical Angioplasty with Nitroglycerin for Vasospasm after Subarachnoid Hemorrhage: Case Series and Review

Introduction Vasospasm is a common and potentially devastating complication in patients with subarachnoid hemorrhage, causing high morbidity and mortality. There is no effective and consistent way to prevent or treat cerebral vasospasm capable of altering the morbidity and mortality of this complication. Animal and human studies have attempted to show improvement in aneurysmal vasospasm. Some sought their prevention; others, the treatment of already installed vasospasm. Some achieved only angiographic improvement without clinical correlation, others achieved both, but with ephemeral duration or at the expense of very harmful associated effects. Endovascular techniques allow immediate and aggressive treatment of cerebral vasospasm and include methods such as mechanical and chemical angioplasty. These methods have risks and benefits. Objectives To analyze the results of chemical angioplasty using nitroglycerin (GTN). In addition, to performa comprehensive review and analysis of aneurysmal vasospasm. Methods We describe our series of 77 patients treated for 8 years with angioplasty for vasospasm, either mechanical (with balloon), chemical (with GTN) or both. Results Eleven patients received only balloon; 37 received only GTN; 29 received both. Forty-four patients (70.1%) evolved with delayed cerebral ischemia and 19 died (mortality of 24.7%). Two deaths were causally related to the rupture of the vessel by the balloon. The only predictors of poor outcome were the need for external ventricular drainage in the first hours of admission, and isolated mechanical angioplasty. Conclusions Balloon angioplasty has excellent results, but it is restricted to proximal vessels and is not without complications. Chemical angioplasty using nitroglycerin has reasonable but short-lived results and further research is needed about it. It is restricted to vasospasm angioplasties only in hospitals, like ours, where better and more potent vasodilator agents are not available.

Endovascular Treatment in the Subclavian Steal Syndrome: Series of 29 Patients

Subclavian steal syndrome is a group of symptoms resulting fromretrograde flow in the vertebral artery, "stealing" blood from the posterior intracranial circulation and other territories, caused by stenosis or occlusion of the subclavian artery proximal to the origin of the same vertebral artery, or even of the brachiocephalic trunk. Most of the time, it is an incidental finding in patients with other conditions or cerebrovascular risk factors. We report a series of 29 patients with an angiographic diagnosis, in which 7 received treatment (all endovascular), all with symptoms directly related to this condition. Advanced age, systemic arterial hypertension, diabetes mellitus, smoking and stroke were comorbidities frequently related. Six patients improved completely after the procedure and one remained with vertigo.

Origin of the Posterior Inferior Cerebellar Artery over the C1 Posterior Arch.

A artéria cerebelar inferoposterior geralmente nasce do segmento intracraniano (V4) da artéria vertebral. Apesar de ter diâmetro médio de 2 mm, usualmente irriga áreas eloquentes do encéfalo. Quando ocluída, seja por trauma ou cirurgia, pode causar infarto no tronco encefálico e no cerebelo. Apresentamos um caso de artéria cerebelar inferoposterior com origem anômala no segmento cervical (V3) da artéria vertebral, demonstrado por angiografia. Os resultados foram registrados e comparados com os de publicações anteriores. Foram fornecidas breves explicações sobre a anatomia, anomalias vasculares e embriologia. A revisão da literatura mostrou que os ramos anômalos do segmento cervical da artéria vertebral são infrequentes e devem ser conhecidos. Uma melhor compreensão da anatomia e suas variações permite fazer um diagnóstico topográfico preciso, bem como planejar a abordagem e a terapia cirúrgicas ideais. O conhecimento dessa variação anatômica é essencial, pois, se confundida com um ramo muscular e coagulada, pode causar isquemia com sequelas incapacitantes.

Origin of the Posterior Inferior Cerebellar Artery over the C1 Posterior Arch / Origem da artéria cerebelar inferoposterior sobre o arco posterior de C1

Resumo The posterior inferior cerebellar artery usually arises from the intracranial segment (V4) of the vertebral artery. Despite its mean diameter of 2 mm, it usually irrigates important areas of the brain. When occluded, whether due to trauma or surgery, it may cause infarction in the brain stem and cerebellum. The present report describes a case of incidental finding of a posterior inferior cerebellar artery arising from the cervical segment (V3) of the vertebral artery, demonstrated by angiography. The findings were recorded and compared to those of earlier publications. Brief explanations regarding anatomy, vascular anomalies and embryology were provided. A literature review showed that anomalous branches of the cervical segment of the vertebral artery are infrequent andmust be known. A better understanding of anatomy and its variations enables an accurate topographic diagnosis, as well as the planning of the optimal surgical approach and therapy. Knowledge of this anatomical variation is essential because, if it is mistaken for a muscle branch and coagulated, this can cause ischemia and disabling sequelae.

Resumo A artéria cerebelar inferoposterior geralmente nasce do segmento intracraniano (V4) da artéria vertebral. Apesar de ter diâmetro médio de 2 mm, usualmente irriga áreas eloquentes do encéfalo. Quando ocluída, seja por trauma ou cirurgia, pode causar infarto no tronco encefálico e no cerebelo. Apresentamos um caso de artéria cerebelar inferoposterior com origem anômala no segmento cervical (V3) da artéria vertebral, demonstrado por angiografia. Os resultados foram registrados e comparados com os de publicações anteriores. Foram fornecidas breves explicações sobre a anatomia, anomalias vasculares e embriologia. A revisão da literatura mostrou que os ramos anômalos do segmento cervical da artéria vertebral são infrequentes e devem ser conhecidos. Uma melhor compreensão da anatomia e suas variações permite fazer um diagnóstico topográfico preciso, bem como planejar a abordagem e a terapia cirúrgicas ideais. O conhecimento dessa variação anatômica é essencial, pois, se confundida com um ramo muscular e coagulada, pode causar isquemia com sequelas incapacitantes.

Endovascular Treatment in the Subclavian Steal Syndrome

When the proximal occlusion or stenosis of the subclavian or of the brachiocephalic artery may require distal arterial filling through reversal flow from the vertebral artery, causing clinically significant blood supply reduction to the brainstem, it is called subclavian steal syndrome (SSS). We report a 54-year-old male patient who presented with multiple episodes of syncopes and vascular claudication due to right SSS. He underwent an angioplasty, evolving with complete improvement of the symptoms. We review the clinical presentation, the diagnosticmethods, and the treatment options of the disease.

Scalp Cirsoid Aneurysm: An Updated Systematic Literature Review and an Illustrative Case Report.

Scalp cirsoid aneurysm is an arteriovenous fistula of the scalp that is unconnected by intracranial or cerebral vessels. Variceal dilatation of draining veins can produce cosmetic concerns, masses, local pain, palpable thrills, and audible bruits, headache, tinnitus, and hemorrhage. Its etiopathogenesis is not well understood. Treatment includes surgery (fistula repair by simple surgical ligation until gross total resection), embolization (whether percutaneous or endovascular), or a combination of the two. An updated systematic review of the last 10 years publications was performed. Also, we report an illustrative case of a young boy with posttraumatic cirsoid aneurysm and good documentation on head vascular examinations, treated by multiple routes (percutaneous embolization with coils and endovascular embolization with cyanoacrylate) with a decrease of the pulsatile mass. A plastic surgery team performed gross total resection, and the final esthetic result was extremely satisfactory.

Rescue therapy with stent retrievers for thromboembolism during endovascular treatment of intracranial aneurysms.

Thromboembolism is the most frequent complication in endovascular treatment of intracranial aneurysms, causing disability and death. As stent retrievers have achieved high rates of arterial recanalization in the management of ischemic stroke, these devices were tested as rescue therapy of thromboembolism during aneurysm embolization. We retrospectively analyzed 10 consecutive patients with transprocedural arterial occlusion, treated with mechanical thrombectomy at a single center. Good angiographic recanalization was achieved in eight cases, mTICI 3, 2b and 2a in five, three and two patients, respectively, without additional complications or any deaths. Five patients showed complete recovery (mRS 0) and all patients showed improvement of disability (average mRS 1.1) over a mean follow-up period of 31 months. Eight patients had good clinical recovery, while two remained with deficits (mRS 3 and 4). The study found that the stent retriever is a valuable, rapid and effective tool for restoring blood flow, improving the safety of endovascular treatment.

Cognitive and psychiatric changes as first clinical presentation in Sneddon Syndrome / Alterações cognitivas e psiquiátricas como primeira apresentação clínica na Síndrome de Sneddon

ABSTRACT Sneddon syndrome (SS) is a rare progressive non-inflammatory thrombotic vasculopathy affecting small/medium-sized blood vessels of unknown origin. It is strongly associated with the presence of antiphospholipid antibodies (AA). The presence of livedo reticularis and cerebrovascular disease are hallmark features. The condition is far more common in young women. We report a case of SS in a 43 year-old male with a two-year history of progressive cognitive impairment consistent with dementia syndrome, and major personality changes, besides livedo reticularis and cerebral angiographic pattern of vasculitis. AA were borderline. The recognition of skin blemishes that precede strokes should raise the hypothesis of SS. AA are elevated in more than half of cases, but their role in the pathogenesis or association of positive antibodies and SS remains unclear. Dementia syndrome in young patients should be extensively investigated to rule out reversible situations. Typical skin findings, MRI and angiography may aid diagnosis.

RESUMO A síndrome de Sneddon (SS) é uma vasculopatia trombótica não inflamatória progressiva rara que afeta os vasos sanguíneos de pequeno e médio tamanho com origem desconhecida. Está fortemente associada à presença de anticorpos antifosfolipídios (AA). A presença de livedo reticularis e doença cerebrovascular são a marca registrada. É muito mais comum em mulheres jovens. Relatamos um caso de SS em um homem de 43 anos de idade com dois anos de história de comprometimento cognitivo progressivo compatível com síndrome demencial e mudanças graves de personalidade, além de livedo reticular e padrão angiográfico cerebral de vasculite. AA eram limítrofes. O reconhecimento das manchas da pele que precedem eventos isquêmicos cerebrovasculares deve reforçar a hipótese de SS. Os AA são elevados em mais da metade dos casos, mas seu papel na patogênese ou associação de anticorpos positivos e SS permanece obscuro. A síndrome demencial em pacientes jovens deve ser amplamente investigada para se descartarem situações reversíveis. Achados típicos da pele, ressonância magnética e angiografia podem ajudar no diagnóstico.

Rescue therapy with stent retrievers for thromboembolism during endovascular treatment of intracranial aneurysms / Terapia de resgate com stent para tromboembolismo durante tratamento endovascular de aneurismas intracranianos

ABSTRACT Thromboembolism is the most frequent complication in endovascular treatment of intracranial aneurysms, causing disability and death. As stent retrievers have achieved high rates of arterial recanalization in the management of ischemic stroke, these devices were tested as rescue therapy of thromboembolism during aneurysm embolization. We retrospectively analyzed 10 consecutive patients with transprocedural arterial occlusion, treated with mechanical thrombectomy at a single center. Good angiographic recanalization was achieved in eight cases, mTICI 3, 2b and 2a in five, three and two patients, respectively, without additional complications or any deaths. Five patients showed complete recovery (mRS 0) and all patients showed improvement of disability (average mRS 1.1) over a mean follow-up period of 31 months. Eight patients had good clinical recovery, while two remained with deficits (mRS 3 and 4). The study found that the stent retriever is a valuable, rapid and effective tool for restoring blood flow, improving the safety of endovascular treatment.

RESUMO Tromboembolismo é a complicação mais frequente no tratamento endovascular de aneurismas cerebrais, podendo causar morte ou sequelas. Como os stent retrievers alcançaram altas taxas de recanalização arterial no tratamento do acidente vascular encefálico isquêmico, testamos esses dispositivos para tratar eventos tromboembólicos ocorridos durante a embolização de aneurismas. Foram analisados retrospectivamente 10 pacientes apresentando oclusão arterial transoperatória, tratados com trombectomia mecânica em um único centro. Obtivemos recanalização angiográfica em oito casos, mTICI 3, 2b e 2a em cinco, três e dois pacientes, respectivamente, sem complicações adicionais ou óbito. Cinco casos apresentaram recuperação completa (mRS 0) e todos os pacientes apresentaram melhora dos déficits (mRS médio 1.1) durante acompanhamento médio de 31 meses. Oito pacientes apresentaram boa recuperação clínica, enquanto dois permaneceram com déficits (mRS 3 e 4). O estudo concluiu que stents são uma ferramenta valiosa, rápida e eficaz para restaurar o fluxo sanguíneo, aumentando a segurança do tratamento endovascular.

Intra-arterial Thrombectomy with Stent Retriever for Acute Ischemic Stroke ­ a Retrospective, Single-centered Study from Brazil

Objective To report our initial experience with intra-arterial thrombectomy (IAT) with stent retriever for acute ischemic stroke. Methods We conducted a retrospective review of patients with acute ischemic stroke who underwent IAT from September 2010 to August 2016. Results Forty-one patients were included; mean age was 57 years (range: 29­85), and 54% were women. There were 32 anterior circulation occlusions, and 11 posterior circulation occlusions. The mean value of the National Institutes of Health Stroke Scale (NIHSS) upon admission (available in 9/41 patients) was 14 (range: 6­20). Nineteen patients had favorable outcomes (modified Rankin Scale [mRS]: 0­2 at 6 months), and 22 had unfavorable outcomes (mRS: 3­6 at 6 months). The mortality rate was 37% (15/41). Favorable outcomes were associated with revascularization within the first 360 minutes of the onset of symptoms (p » 0.000001), and satisfactory revascularization (thrombolysis in cerebral infarction [TICI] scale: 2b or 3) (p » 0.0018). Conclusion It is of paramount importance to educate stroke teams on the benefits of IAT for acute ischemic stroke and the population on identifying stroke and seeking immediate care following symptom onset.

Objetivo Relatar nossa experiência inicial com trombectomia intra-arterial (TIA) com uso de stent retriever em acidente vascular encefálico isquêmico (Avei) agudo. Métodos Análise retrospectiva de pacientes com Avei submetidos a TIA de setembro de 2010 a agosto de 2016. Resultados Foram incluídos 41 pacientes; a idade média foi 57 anos (intervalo: 29­85), e 54% dos pacientes eram mulheres. Trombos ocluindo vasos da circulação anterior foram encontrados em 32 casos, e 11 na circulação posterior. A média do valor da Escala de Acidente Vascular Encefálico do National Institutes of Health (NIH) na admissão (disponível para 9 dos 41 pacientes) foi 14 (intervalo: 6­20). Um total de 19 pacientes teve resultados favoráveis (Escala de Rankin modificada [ERm]: 0­2 em 6 meses), e 22 tiveram resultados não favoráveis (ERm: 3­6 em 6 meses). A mortalidade foi de 37% (15/41). Resultados favoráveis foram associados com revascularização dentro dos primeiros 360 minutos de instalação dos sintomas (p » 0.000001) e revascularização satisfatória (escala de trombólise em infarto cerebral [TEIC]: 2b ou 3) (p » 0.0018). Conclusão É de suma importância educar as equipes de acidente vascular encefálico sobre os benefícios da TIA para Avei agudo, e a população, na identificação do Avei, para que seja buscado atendimento imediato após o início dos sintomas.

Physicians are not well informed about the new guidelines for the treatment of acute stroke / Os médicos não estão bem informados sobre as novas diretrizes para o tratamento de AVC agudo

ABSTRACT Treatment of cerebrovascular disease has advanced rapidly in the last two decades. Recent data has added challenges to the treatment of ischemic stroke in the acute phase. Objective: To evaluate the knowledge of physicians about the treatment of ischemic stroke in the acute phase. Methods: An online questionnaire was submitted to all physicians enrolled in the Regional Council of Medicine in Brazil. Results: 456 physicians from different specialties answered the questions. Most of them did not know that mechanical endovascular thrombectomy is often considered as the gold standard treatment in cases of ischemic stroke in the acute phase; and 85% of them did not realize that thrombectomy together with intravenous thrombolysis was possible. The maximum time to act in an acute event also presented many divergences, even with regard to the infusion of rtPA. The lack of structure, medication and absence of a neurologist were considered the main barriers to treatment. Conclusion: Physicians are not well informed about the new guidelines for the treatment of acute stroke. Most physicians incorrectly answered most of the questions on the questionnaire.

RESUMO O tratamento da doença cerebrovascular tem avançado rapidamente nas últimas duas décadas. Dados recentes acrescentaram desafios ao tratamento do AVC isquêmico na fase aguda (AIFA). Objetivo: Avaliar o conhecimento médico sobre o tratamento do AIFA. Métodos: Um questionário on-line foi submetido a todos os médicos inscritos no Conselho Regional de Medicina. Resultados: 456 médicos de diferentes especialidades responderam às perguntas. A maioria deles não sabia que a trombectomia endovascular mecânica é freqüentemente considerada como tratamento padrão-ouro nos casos de AIFA. 85% não realizariam trombectomia junto com a trombólise intravenosa. O tempo máximo para atuar no evento agudo também apresentou muitas divergências, mesmo em relação à infusão de rtPA. A falta de estrutura, medicação e neurologista foram consideradas as principais barreiras ao tratamento. Conclusão: Os médicos não estão bem informados sobre as novas diretrizes para o tratamento do AIFA. A maioria dos médicos errou a maioria das perguntas desse questionário.

The role of vagus nerve stimulation in refractory epilepsy / O papel da estimulação do nervo vago na epilepsia refratária

ABSTRACT Vagus nerve stimulation is an adjunctive therapy used to treat patients with refractory epilepsy who are not candidates for resective surgery or had poor results after surgical procedures. Its mechanism of action is not yet fully comprehended but it possibly involves modulation of the locus coeruleus, thalamus and limbic circuit through noradrenergic and serotonergic projections. There is sufficient evidence to support its use in patients with focal epilepsy and other seizure types. However, it should be recognized that improvement is not immediate and increases over time. The majority of adverse events is stimulation-related, temporary and decreases after adjustment of settings. Future perspectives to improve efficacy and reduce side effects, such as different approaches to increase battery life, transcutaneous stimulation and identification of prognostic factors, should be further investigated.

RESUMO A estimulação vagal é uma terapia paliativa utilizada no tratamento de pacientes com epilepsia refratária que não são candidatos à cirurgia ressectiva ou naqueles com evolução insatisfatória após o procedimento cirúrgico. Seu mecanismo de ação ainda não foi completamente elucidado mas possivelmente envolve a modulação do locus coeruleus, tálamo e circuito límbico através de projeções noradrenérgicas e serotoninérgicas. Atualmente há evidência suficiente para corroborar o uso desta terapia em pacientes com epilepsia focal e outros tipos de crise, com resultados que, apesar de não imediatos, melhoram progressivamente no longo prazo. Os eventos adversos são, em sua maioria, relacionados à estimulação e auto-limitados. Perspectivas futuras para aumentar a eficácia e reduzir os efeitos colaterais como a utilização de baterias com maior durabilidade, estimulação transcutânea e identificação de fatores prognósticos devem ser investigadas.

Tratamiento endovascular plurimodal de aneurismas múltiples de la circulación anterior. Reporte de un caso / Multimodalities endovascular treatment of multiple aneurysms of the anterior circulation. Case report

Aneurismas cerebrales son una enfermedad grave, a pesar del alto grado de conocimiento respecto su fisiopatología y patogenia. La letalidad de ruptura de un aneurisma cerebral es todavía alrededor de 50%, allá de altas tasas de morbilidad. El tratamiento endovascular ha evolucionado en las últimas décadas para mejorar los resultados. Se presenta el caso de un paciente con síntomas neurológicos graves secundarios a la hemorragia subaracnoidea, cuya investigación mostró múltiples aneurismas. Varios dispositivos fueron utilizados en modalidades terapéuticas endovasculares consecutivas, incluyendo la embolización con remodelación, colocación de stents y desviador de flujo, con buen resultado clínico y angiográfico.

Cerebral aneurysms are a serious illness, despite the high degree of knowledge about its pathophysiology and pathogenesis. The lethality of cerebral aneurysm´s rupture is still about 50% beyond high morbidity. Endovascular treatment has evolved in recent decades to improve outcomes. It is reported the case of a patient with severe neurological symptoms secondary to subarachnoid hemorrhage, whose examination showed multiple aneurysms. Several devices were used on consecutive endovascular therapeutic modalities, including remodeling embolization, stenting and flow diverter, with good clinical and angiographic outcome.

Aneurisma dissecante da artéria vertebral e doença de behçet / Disseting aneurysm of the vertebral artery and behçets disease

A doença de Behcet é caracterizada por uma condição inflamatóriacronica, multissistemica e recorrente, que podeapresentar manifestações vasculares e neurológicas duranteseu curso. Raramente encontramos associação desta afecçãocom aneurismas cerebrais e quando presente, o predomínio éna circulação anterior. Descrevemos um caso raro de doençade Behcet associado a aneurisma roto da artéria vertebralsubmetido a tratamento endovascular.

Angioarchitecture and clinical presentation of brain arteriovenous malformations.

The purpose of this study was to correlate the angioarchitecture of brain arteriovenous malformations (AVM) with their clinical presentation. A total of 170 patients with AVM 78 males and 92 females, were studied. Univariate and multivariate analyses were conducted in order to test the associations between morphological features and clinical presentation. The most frequent clinical presentations at diagnosis were hemorrhage in 89 (52%) patients, headache in 79 (46%), focal neurological deficit in 54 (32%), and seizure in 52 (31%). According to the Spetzler-Martin classification, grade I was found in 15 patients, II in 49, III in 55, IV in 41, and grade V in 10 patients. AVM with small nidus size, single feeding artery and single draining vein were associated with hemorrhage. Hemorrhage was positively associated with Spetzler-Martin grade I and negatively with grade V. The association between seizure and large nidus size was positive, however negative with small nidus size.

Angioarchitecture and clinical presentation of brain arteriovenous malformations / Angioarquitetura e apresentação clínica de malformações arteriovenosas encefálicas

The purpose of this study was to correlate the angioarchitecture of brain arteriovenous malformations (AVM) with their clinical presentation. A total of 170 patients with AVM 78 males and 92 females, were studied. Univariate and multivariate analyses were conducted in order to test the associations between morphological features and clinical presentation. The most frequent clinical presentations at diagnosis were hemorrhage in 89 (52 percent) patients, headache in 79 (46 percent), focal neurological deficit in 54 (32 percent), and seizure in 52 (31 percent). According to the Spetzler-Martin classification, grade I was found in 15 patients, II in 49, III in 55, IV in 41, and grade V in 10 patients. AVM with small nidus size, single feeding artery and single draining vein were associated with hemorrhage. Hemorrhage was positively associated with Spetzler-Martin grade I and negatively with grade V. The association between seizure and large nidus size was positive, however negative with small nidus size.

O objetivo deste estudo foi correlacionar a angioarquitetura de malformações arteriovenosas encefálicas (MAV) com sua apresentação clínica. Foram estudados 170 pacientes portadores de MAV, sendo 78 do sexo feminino e 92 do masculino. Análises univariada e multivariada foram efetuadas para testar associações entre características morfológicas e quadro clínico. As principais formas de apresentação clínica no momento do diagnóstico incluíram hemorragia em 89 (52 por cento) pacientes, cefaléia em 79 (46 por cento), déficit focal em 54 (32 por cento) e convulsão em 52 (31 por cento). De acordo com a classificação de Spetzler e Martin, 15 pacientes tinham MAV grau I, 49 grau II, 55 grau III, 41 grau IV, e 10 grau V. MAV com nidus de tamanho pequeno, aferência e eferência únicas foram associados à hemorragia. Hemorragia foi positivamente associada com grau I e negativamente com grau V. A associação entre convulsão e nidus de tamanho grande foi positiva, porém negativa com nidus de tamanho pequeno.