Dravet syndrome and Dravet syndrome-like phenotype: a systematic review of the SCN1A and PCDH19 variants.

Dravet syndrome (DS) is a rare and severe epileptic syndrome of childhood with prevalence between 1/22,000 and 1/49,900 of live births. Approximately 80% of patients with this syndrome present SCN1A pathogenic variants, which encodes an alpha subunit of a neural voltage-dependent sodium channel. There is a correlation between PCDH19 pathogenic variants, encodes the protocadherin 19, and a similar disease to DS known as DS-like phenotype. The present review aims to clarify the differences between DS and DS-like phenotype according to the SCN1A and PCDH19 variants. A systematic review was conducted in PubMed and Virtual Health Library (VHL) databases, using "Dravet Syndrome" and "Severe Myoclonic Epilepsy in Infancy (SMEI)" search words, selecting cohort of studies published in journal with impact factor of two or greater. The systematic review was according to the Preferred Reporting Items for Systematic Review and Meta-Analysis recommendations. Nineteen studies were included in the present review, and a significant proportion of patients with DS-carrying SCN1A was greater than patients with DS-like phenotype-harboring PCDH19 variants (76.6% versus 23.4%). When clinical and genetic data were correlated, autism was predominantly observed in patients with DS-like-carrying PCDH19 variants compared to SCN1A variant carriers (62.5% versus 37.5%, respectively, P-value = 0.044, P-value corrected = 0.198). In addition, it was noticed a significant predisposition to hyperthermia during epilepsy crisis in individuals carrying PCDH19 variants (P-value = 0.003; P-value corrected = 0.027). The present review is the first to point out differences between the DS and DS-like phenotype according to the SCN1A and PCDH19 variants.

Spinal Cord Stimulation in Pregnant Patients: Current Perspectives of Indications, Complications, and Results in Pain Control: A Systematic Review.

Spinal cord stimulation (SCS) has been described as a valuable neuromodulator procedure in the management of chronic medically untreated neuropathic pain. Although the use of this technique has been published in many papers, a question still remains regarding its applicability in pregnant patients. The goal of this paper is to discuss the risks, complications, and results as well as the prognosis of SCS in pregnant patients. We performed a systematic review from 1967 to 2018 using the databases MEDLINE, LILACS, SciELO, PubMed, and BIREME, utilizing language as selection criteria. Eighteen studies that met our criteria were found and tabulated. SCS is a reversible and adjustable surgical procedure, which results in patients that demonstrated a significant effect in the reduction of pain intensity in pregnant patients. The etiologies most frequent were complex regional pain and failed back pain syndromes, which together represented 94% of analyzed cases. The technical complications most frequent were lead migration (3%, n = 1). Regarding the risks, the authors did not show significative factors among the categorical variables that can suggest a teratogenicity, while the maternal risks have been associated to the consequences of technical complications due to, among other factors, improvement of abdominal pressure during pregnancy and delivery. Finally, although there are not significative cohorts of pregnant patients, the procedure is still an effective surgical approach of neuropathic pain associated to lower rates of complications and significative improvement in the quality of life of patients during pregnancy.

Brazilian Consensus for the Treatment of Multiple Sclerosis: Brazilian Academy of Neurology and Brazilian Committee on Treatment and Research in Multiple Sclerosis.

The expanding therapeutic arsenal in multiple sclerosis (MS) has allowed for more effective and personalized treatment, but the choice and management of disease-modifying therapies (DMTs) is becoming increasingly complex. In this context, experts from the Brazilian Committee on Treatment and Research in Multiple Sclerosis and the Neuroimmunology Scientific Department of the Brazilian Academy of Neurology have convened to establish this Brazilian Consensus for the Treatment of MS, based on their understanding that neurologists should be able to prescribe MS DMTs according to what is better for each patient, based on up-to-date evidence and practice. We herein propose practical recommendations for the treatment of MS, with the main focus on the choice and management of DMTs, as well as present a review of the scientific rationale supporting therapeutic strategies in MS.

Brazilian Consensus for the Treatment of Multiple Sclerosis: Brazilian Academy of Neurology and Brazilian Committee on Treatment and Research in Multiple Sclerosis / Consenso Brasileiro para o Tratamento da Esclerose Múltipla: Academia Brasileira de Neurologia e Comitê Brasileiro de Tratamento e Pesquisa em Esclerose Múltipla

ABSTRACT The expanding therapeutic arsenal in multiple sclerosis (MS) has allowed for more effective and personalized treatment, but the choice and management of disease-modifying therapies (DMTs) is becoming increasingly complex. In this context, experts from the Brazilian Committee on Treatment and Research in Multiple Sclerosis and the Neuroimmunology Scientific Department of the Brazilian Academy of Neurology have convened to establish this Brazilian Consensus for the Treatment of MS, based on their understanding that neurologists should be able to prescribe MS DMTs according to what is better for each patient, based on up-to-date evidence and practice. We herein propose practical recommendations for the treatment of MS, with the main focus on the choice and management of DMTs, as well as present a review of the scientific rationale supporting therapeutic strategies in MS.

RESUMO O crescent arsenal terapêutico na esclerose múltipla (EM) tem permitido tratamentos mais efetivos e personalizados, mas a escolha e o manejo das terapias modificadoras da doença (TMDs) tem se tornado cada vez mais complexos. Neste contexto, especialistas do Comitê Brasileiro de Tratamento e Pesquisa em Esclerose Múltipla e do Departamento Científico de Neuroimunologia da Academia Brasileira de Neurologia reuniram-se para estabelecer este Consenso Brasileiro para o Tratamento da EM, baseados no entendimento de que neurologistas devem ter a possibilidade de prescrever TMDs para EM de acordo com o que é melhor para cada paciente, com base em evidências e práticas atualizadas. Por meio deste documento, propomos recomendações práticas para o tratamento da EM, com foco principal na escolha e no manejo das TMDs, e revisamos os argumentos que embasam as estratégias de tratamento na EM.

Targets for treatment of dystonia caused by several etiologies. Meta analysis

Introducción: Para utilizar un meta-análisis de todos los casos reportados de la estimulación cerebral profunda (DBS) para ladistonía para determinar cuáles son los factores significativos resultados influencia relacionada con el destino. La escala demovimiento Burke-Fahn-Marsden (BFM), la medida más informado, fue elegida como la principal medida de resultado paraeste análisis. Material y Métodos: Una búsqueda en MEDLINE identificaron 137 pacientes que se sometieron a DBS para ladistonía en 24 estudios que tenían puntuaciones individuales BFM. Datos de los pacientes individuales, incluyendo la edad deinicio de la distonía, la edad de la cirugía, el género, la distribución de la distonía, la etiología de la distonía, la presencia decaracterísticas asociadas, anormalidad de las imágenes preoperatorias, cirugías estereotáxica anteriores, el núcleo estimulado,el tipo de anestesia que se utiliza, el tiempo de respuesta a la estimulación, y el momento de la evaluación de resultadosse introdujeron en una base de datos de SPSS para el análisis estadístico. Resultados: La media BFM cambio porcentual(mejora en la puntuación postoperatoria de la línea de base) fue 51,8% (rango - 34% a 100%). Significativamente se lograronmejores resultados con la estimulación del globo pálido interno (GPI) que con la estimulación de la parte posterior del núcleolateral ventral (VLP) del tálamo (p = 0,0001)...

Introduction: To use a meta-analysis on all reported cases of deep brain stimulation (DBS) for dystonia to reevaluate the good effect using the GPi as a target, which factors significant influence outcome related to the target. The Burke-Fahn-Marsden (BFM) movement scale, the most reported measure, was chosen as the primary outcome measure for this analysis. Material and Methods: Computerized MEDLINE searches on English literature search identified 137 patients who underwent BBS for dystonia in 24 studies that had individual BFM scores. The study was done with statistical analysis by intention to treat. Statistical analysis was made with a significant p- value of 0.05. For the comparison of pre- and postoperative scores, a test Wilcoxon signed was used. Results: The mean BFM percentage change (improvement in postoperative score from baseline) was 46.3 percent(range - 34 percent to 100 percent)...

Complex regional pain syndrome: new concepts regarding diagnosis and treatment

Antecedentes: Los autores presentan una revisión crítica sobre el cuadro clínico, el diagnóstico, clasificación y tratamientodel síndrome de dolor regional complejo, discutiendo todos los métodos de tratamiento y haciendo hincapié en que la reabilitación debe ser empleada con el fin de obtener un mejor resultado. Aspecto psicológico debe ser discutido en el tratamiento y también se anima equipo multidisciplinario para participar en él.

Background: The authors presented a critical review about the clinical picture, diagnosis, classification and treatment ofcomplex regional pain syndrome, discussing all methods of treatment and emphasizing that the reabiltation must be employed in order to obtain a better result. Psychological aspect must be involved in the treatment and also multidisciplinary team is encouraged to take part on it.

Hemispherectomy in treatment of seizures: current perspectives of the indications, techniques and complications: literature review

Introducción: A hemisferectomía es un procedimiento valioso en el tratamiento de trastornos convulsivos causados por desordenes hemisféricos unilaterales. El hemisferectomía anatómica se ha utilizado para este fin desde 1938, sin embargo, se abandonó este procedimiento después de informes de complicaciones postoperatorias causadas por hemosiderosis superficial, ependimitis e hidrocefalia obstructiva. Así que, se ha mostrado en la literatura modificaciones en las indicaciones y técnicas de hemisferectomía anatómica cuya finalidad es la de reducir la incidencia de esta complicación sin dejar de lograr control de las convulsiones. Sobre la base de la literatura, la hemisferectomía mejora la calidad de vida de los pacientes que tiene la indicación para realizar este procedimiento, ya que permite reducir la frecuencia de las convulsiones, si tónica o átona, tónico-clónicas Objetivo: El objetivo de esta revisión de la literatura es discutir los detalles técnicos, modalidades, riesgos, complicaciones, resultados y de pronóstico de hemisferectomía basado en la revisión crítica de la literatura. Casuística y Métodos: Se realizó la consulta bibliográfica, utilizando la base de datos MEDLINE, LILACS, SciELO, que utiliza el lenguaje como criterios de selección, la elección de los artículos recientes preferiblemente en portugués, español o inglés. Conclusión: Según las referencias, hemisferectomía es un procedimiento con buen resultado para las personas con convulsiones derivadas cuando está indicado para casos seleccionados y la tasa de éxito no es proporcional a la extensión de la resección del tejido neuronal. A mayor resección puede o no reducir la frecuencia de las crisis, sin embargo, la incidencia de la morbilidad puede ser mayor.

Background: The hemispherectomy is a valuable procedure in the management of seizure disorders caused by unilateral hemispheric disease. The anatomical hemispherectomy has been used for this purpose since 1938, however, it was abandoned after reports of postoperative complications caused by superficial hemosiderosis, ependymitis and obstructive hydrocephalus. So that, it has been showed modifications in the techniques of hemispherectomy whose the purpose is reduce the incidence of this complications while still achieving seizure control. Based on literature, the hemispherectomy improves the quality of life of patients that has the indication to perform this procedure because it allows reducing the frequency of seizures, whether tonic or atonic, tonic-clonic. Aim: The aim of this literature review is discuss the indications, technical details, modalities, risks, complications, results as well de prognosis of callosotomy based on critical literature review and the authors experience. Casuistry and Methods: It was performed bibliographical consultation, using the databases MEDLINE, LILACS, SciELO, utilizing language as selection criteria, choosing preferably recent articles in Portuguese, Spanish or English. Conclusion: According to references, the functional hemispherectomy has a good outocome for those with seizures arisin when indicated to selected cases and the success rate is not proportional to the extent of neuronal tissue resection. So that, a greater resection cannot necessarily reduce the seizure frequency, however the morbidity may also be larger.

Callosotomía: técnicas, resultados y complicaciones: revisión de la literatura / Callosotomy: techniques, results and complications: literature review

Background: Patients with intractable seizures who are not candidates for focal resective surgery are indicated for a palliative surgical procedure, the callosotomy. This procedure is based on the hypothesis that the corpus callosum is an important pathway for interhemispheric spread of epileptic activity and, for drug resistant epilepsy. It presents relatively low permanent morbidity and an efficacy in the control of seizures. Based on literature, the corpus callosotomy improves the quality of life of patients that has the indication to perform this procedure because it allows reducing the frequency of seizures, whether tonic or atonic, tonic-clonic, absence or frontal lobe complex partial seizures. Aim: The aim of this literature review is discuss the technical details, modalities, risks, complications, results as well de prognosis of callosotomy based on critical literature review and the authors experience. Casuistry and Methods: It was performed bibliographical consultation, using the databases MEDLINE, LILACS, SciELO, utilizing language as selection criteria, choosing preferably recent articles in Portuguese, Spanish or English, with publication year higher than 2000. Conclusion: According to author’s experience and references, callosotomy is a safe procedure when indicated to selected cases and the success rate is proportional to the extent of callosal resection. A greater resection can reduce the seizure frequency, however the morbidity may also be larger. There is no important study comparing VNS versus Callosotomy versus VNS plus callososotomy, what would be for future necessary for an important source of data about this topic.

Introducción: Los pacientes con convulsiones intratables que no son candidatos para la cirugía de resección focal están indicados para un procedimiento quirúrgico paliativo, la callosotomía. Este procedimiento se basa en la hipótesis de que el cuerpo calloso es una importante vía para la propagación interhemisférica de la actividad epiléptica y, para la epilepsia resistente a fármacos. Presenta relativamente baja morbilidad permanente y una eficacia en el control de las convulsiones. Sobre la base de la literatura, la callostomía mejora la calidad de vida de los pacientes que tiene la indicación para realizar este procedimiento, ya que permite reducir la frecuencia de las crisis, ya sean tónica o átona, tónico-clónicas, ausencia o lóbulo frontal crisis parciales complejas. Objetivo: El objetivo de esta revisión de la literatura es discutir los detalles técnicos, modalidades, riesgos, complicaciones, resultados y de pronóstico de callosotomía basado en la revisión crítica de la literatura y la experiencia de los autores. Casuística y Métodos: Se realizó la consulta bibliográfica, utilizando la base de datos MEDLINE, LILACS, SciELO, que utiliza el lenguaje como criterios de selección, la elección de los artículos recientes preferiblemente en portugués, español o Inglés, con el año de publicación superior a 2000. Conclusión: De acuerdo con la experiencia y las referencias del autor, callosotomía es un procedimiento seguro cuando indicado para casos seleccionados y la tasa de éxito es proporcional a la extensión de la resección del cuerpo calloso. A mayor resección puede reducir la frecuencia de las crisis, sin embargo, la morbilidad puede ser también mayor. No hay ningún estudio que compara la estimulación del nervio vago frente a frente callosotomía VNS más callososotomy, lo que sería la futura necesaria para una importante fuente de datos sobre este tema.

La Insulectomía podría ser un abordaje ideal para crisis insulares y peri-insulares? / Insulectomy for insular and peri-insular seizures could be an ideal and safe approach?

La insulectomía es una técnica microquirúrgica establecida para el tratamiento de la epilepsia refractaria al tratamiento farmacológico. El origen insular de la epilepsia es inusual, sin embargo, con la investigación a través de electrodos híbridos este tipo de epilepsia ha incrementado su diagnóstico. Los autores hacen hincapié en las funciones insulares, así como los puntos de referencia anatómicos para la cirugía. Se discuten las principales complicaciones y las bases fisiológicas para las indicaciones de cirugía.

Insulectomy is an established microsurgical technique for treatment of insular epilepsy refractory to clinical management. The insular origin of epilepsy is unusual, however with depth investigation through hybrids electrodes such kind of epilepsy is increasing its diagnosis. The authors emphasizes the insular functions as well as the anatomical landmarks for surgery. The main complications are discussed and physiological basis for indications.

A disturbed processing of graviceptive pathways may be involved in the pathophysiology of balance disorders in patients with multiple sclerosis.

The purpose of this study was to determine the relationship between perception of verticality and balance disorders in multiple sclerosis patients. We evaluated patients and healthy controls. Patients were divided into two groups according to their risk of fall, with or without risk of fall, measured by a Dynamic Gait Index scale. Graviceptive perception was assessed using the subjective visual vertical test. Patients with risk of fall showed worse perception than those without risk of fall, p < 0.001. Misperception of verticality was correlated with the dynamic gait index scores (p < 0.001), suggesting that the larger the error for verticality judgment, the greater risk for falling. Considering that the perception of verticality is essential for postural control, our results suggested that the disturbed processing of graviceptive pathways may be involved in the pathophysiology of balance disorders in these patients.

A disturbed processing of graviceptive pathways may be involved in the pathophysiology of balance disorders in patients with multiple sclerosis / O processamento incorreto das informações graviceptivas pode estar envolvido na fisiopatologia dos distúrbios do equilíbrio em pacientes com esclerose múltipla

ABSTRACT The purpose of this study was to determine the relationship between perception of verticality and balance disorders in multiple sclerosis patients. We evaluated patients and healthy controls. Patients were divided into two groups according to their risk of fall, with or without risk of fall, measured by a Dynamic Gait Index scale. Graviceptive perception was assessed using the subjective visual vertical test. Patients with risk of fall showed worse perception than those without risk of fall, p < 0.001. Misperception of verticality was correlated with the dynamic gait index scores (p < 0.001), suggesting that the larger the error for verticality judgment, the greater risk for falling. Considering that the perception of verticality is essential for postural control, our results suggested that the disturbed processing of graviceptive pathways may be involved in the pathophysiology of balance disorders in these patients.

RESUMO Nosso objetivo foi determinar a relação entre percepção de verticalidade e alterações do equilíbrio em pacientes com esclerose múltipla (EM). Foram avaliados pacientes e sujeitos saudáveis. Pacientes foram divididos em dois grupos de acordo com o risco de queda, mensurado pelo Índice de marcha dinâmica, formando os grupos com risco e sem risco de quedas. A percepção da verticalidade foi medida através do teste vertical visual subjetiva (VVS). Pacientes com risco de queda apresentaram pior percepção da verticalidade quando comparados aos sem risco, p < 0,001. O desempenho no teste da VVS foi pior em pacientes quando comparado aos controles (p < 0,001). O erro no julgamento da verticalidade foi correlacionado aos índices de risco de queda (p < 0,001), sugerindo que quanto maior o erro no julgamento da verticalidade, maior o risco de queda dos pacientes. Nossos resultados sugerem que alterações das informações em vias graviceptivas podem estar envolvidas nas alterações de equilíbrio dessa população.

Alternatives for reducing relapse rate when switching from natalizumab to fingolimod in multiple sclerosis.

Natalizumab is a therapeutic option for treating multiple sclerosis (MS) and is particularly efficacious for patients with highly active disease. A long washout period has been recommended between withdrawal of natalizumab and start of fingolimod (another option for treating MS). This long washout period has been associated with a significant increase in MS activity. In the present study, a group of 96 patients who were switched from natalizumab to fingolimod had short washout periods between drugs, or monthly corticosteroid pulse therapy if longer washout periods were recommended. This therapeutic approach led to the lowest reported relapse rate so far, among patients with MS switching from natalizumab to fingolimod (8.3%). No complications from short withdrawal were observed in this group of patients.

Natalizumab treatment in multiple sclerosis: the experience from two Brazilian MS centers.

Objective Analyze the demographics, clinical characteristics, efficacy and safety of natalizumab treatment in Brazilian patients with multiple sclerosis (MS) followed up for at least 12 months, in two tertiary MS care centers in São Paulo.Method We evaluated the effect of natalizumab treatment on annualized relapse rate and disability progression in 75 patients with MS treated with natalizumab for at least 12 months. A subgroup analysis was performed to evaluate efficacy of natalizumab treatment in patients with Expanded Disability Status Scale (EDSS) ≤ 3.0 vs patients with EDSS > 3.Results Patients treated for at least one year with natalizumab showed a 91% reduction in aRR, as well and an improvement in neurological disability. The impact of natalizumab treatment was greater in patients with EDSS < 3.0. Overall, natalizumab was safe but one patient developed progressive multifocal leukoencephalopathy.Conclusion Natalizumab as a third line therapy is safe and efficacious, especially in patients with mild neurological disability.

Natalizumab treatment in multiple sclerosis: the experience from two Brazilian MS centers / Natalizumabe no tratamento da esclerose múltipla: a experiência de dois centros brasileiros

Objective Analyze the demographics, clinical characteristics, efficacy and safety of natalizumab treatment in Brazilian patients with multiple sclerosis (MS) followed up for at least 12 months, in two tertiary MS care centers in São Paulo.Method We evaluated the effect of natalizumab treatment on annualized relapse rate and disability progression in 75 patients with MS treated with natalizumab for at least 12 months. A subgroup analysis was performed to evaluate efficacy of natalizumab treatment in patients with Expanded Disability Status Scale (EDSS) ≤ 3.0 vs patients with EDSS > 3.Results Patients treated for at least one year with natalizumab showed a 91% reduction in aRR, as well and an improvement in neurological disability. The impact of natalizumab treatment was greater in patients with EDSS < 3.0. Overall, natalizumab was safe but one patient developed progressive multifocal leukoencephalopathy.Conclusion Natalizumab as a third line therapy is safe and efficacious, especially in patients with mild neurological disability.

Objetivo Analisar as características clínicas e demográficas, assim como a eficácia e segurança do tratamento com natalizumabe (usado em terceira linha), por no mínimo 12 meses, em pacientes brasileiros acompanhados em dois centros de tratamento de esclerose múltipla, na cidade de São Paulo.Método Avaliamos o efeito do tratamento com natalizumabe na taxa anualizada de surto (aRR) e progressão de incapacidade (medida por Expanded Disability Status Scale (EDSS)) em 75 pacientes tratados por, no mínimo 12 meses. Realizamos uma análise de subgrupo em pacientes com EDSS ≤ 3,0 e com EDSS > 3, para avaliar o impacto no tratamento, considerando-se o grau de incapacidade neurológica.Resultados O tratamento com natalizumabe, por pelo menos um ano, reduziu a aRR em 91%, assim como melhorou a incapacidade neurológica. Em pacientes com EDSS ≤ 3,0 observamos um impacto maior do tratamento na incapacidade neurológica, reduzindo sua progressão em 51%, durante o período do estudo. O tratamento com natalizumabe é seguro, porém um paciente desenvolveu leucoencefalopatia multifocal progressiva.Conclusão O tratamento com natalizumabe, em terceira linha terapêutica é seguro e eficaz especialmente, em pacientes com incapacidade neurológica leve (EDSS ≤ 3.0).

Transcranial Doppler as a routine in the treatment of vasospasm following subarachanoid hemorrhage (SAH).

BACKGROUND: Vasospasm is an important complication observed after subarachnoid hemorrhage (SAH) and is a frequent cause of mortality and morbidity. We present our routine management of vasospasm after SAH and emphasize the importance of transcranial Doppler (TCD) ultrasonography in this management. METHOD: Historical records and images were sampled from June 2005 to September 2011 for 110 patients with SAH due to ruptured aneurysm in the anterior circulation. All surviving patients were followed after discharge. Vasospasm was defined as mild (Lindegaard index 3-4), moderate (Lindegaard index 4-5), and severe (Lindegaard index greater than 5). We excluded patients treated after 72 h of symptom onset. TCD was performed twice per day. FINDINGS: Ninety-nine patients had surgical clipping of the aneurysm, and 11 had endovascular treatment. Seventy patients treated by clipping and six treated by endovascular procedure had vasospasm. Of the 70 clipped patients with vasospasm, 40 had mild vasospasm, 13 had moderate vasospasm, and 17 had severe vasospasm. All six patients treated by coils had moderate vasospasm. The average duration of vasospasm was 9 days (from 7 to 32 days). CONCLUSIONS: TCD was crucial for monitoring patients with SAH, and to identify which patients will have a higher risk of developing vasospasm.

Removal of clots in subarachnoid space could reduce the vasospasm after subarachnoid hemorrhage.

BACKGROUND: Cerebral vasospasm after subarachnoid hemorrhage (SAH) is a major cause of morbidity and mortality. We studied the effects of clot removal on multiple outcome variables following the clipping of ruptured anterior communicating aneurysms. METHODS: From 2007 to 2011, 30 patients with Fisher grade III aneurysmal SAH underwent clipping of an anterior communicating artery aneurysm before SAH day 3. There were 20 women and 10 men, mean age 53.4, range 28-80 years. Seventeen underwent fenestration of lamina terminalis and cisternal removal of clots (group A), and 13 did not (Group B). We compared clinical grades, presence of hydrocephalus at admission, treatment modality, occurrence of clinical vasospasm, the need for interventional vasospasm therapy, and need for ventriculoperitoneal shunting. FINDINGS: Vasospasm affected 5 of 17 (29%) in group A and 8 of 13 (61.5%) in group B (p < 0.05). Endovascular treatment for vasospasm was required in one patient in group A (5.8% of 17, 20% of 5) and in five from group B (38.4% of 13, 62.5% of 8) (p < 0.05). Mortality was observed in one case in group A (5.8% of 17, 20% of 5) and in two cases in group B (15.3% of 13, 25% of 8) and was related to vasospasm after SAH. Ventriculoperitonal shunt (VPS) was required in one case in group A (5.8%) and in five cases in group B (38.4%). CONCLUSIONS: Fenestration of the lamina terminalis and removal of cisternal clots significantly decreased the incidence of post-SAH hydrocephalus and was associated with better outcomes in our series.

Use of temporary arterial occlusion durinng anterior cerebral artery aneurym repair: evaluation of prospective factors / Uso de oclusão arterial temporária durante clipagem de aneurisma de comunicante anterior: avaliação preditivos

O estudo visa determinar os fatores prognósticos para sequelas e complicações perioperatórias nas cirurgias de aneurismas da artéria comunicante anterior (ACoA), secundários ou relacionados à clipagem temporária. Num universo de 92 pacientes operados de aneurisma da ACoA entre 2000 e 2013, 32 foram operados nos últimos sete anos. Destes, 21 foram submetidos à clipagem temporária durante a cirurgia para reparo do aneurisma. Aneurismas maiores que 7mm tiveram uma tempo estatisticamente maior de clipagem temporária que aqueles menores (22±5.7 vs 11.3±4.1, Teste-t, p <0.0001). Não se evidenciou correlação estatística entre o tempo de oclusão e o prognóstico (r=0.92, Pearson, p>0.08). Idade, Escala de Coma de Glasgow (ECG) da primeira avaliação, e Escala de Fisher da Tomografia de Crânio da entrada foram fatores de pior prognóstico (Glasgow Outcome Scale GOS ≤ 3) (regressão-cox, p<0.001). Idade maior que 50 anos, ECG menor que 13, e Fisher III ou IV resultaram em pior prognóstico. Por sua vez gênero, tabagismo, alcoolismo, obesidade, hipertensão arterial sistêmica, dislipidemia, localização da oclusão temporária, rotura trans-operatória do aneurisma e tamanho do aneurisma não foram identificados como fatores independentes de prognóstico. Durante seguimento pós-operatório, dois terços dos pacientes tiveram um prognóstico favorável (GOS≥ 4), retomando suas atividades de vida diária sem maiores dificuldades. Cinquenta e dois por cento dos pacientes evoluíram com hidrocefalia, a despeito da fenestração rotineira da lâmina terminalis, realizada em 71,4% dos procedimentos. A maioria dos pacientes desenvolveu vasoespasmo (66%), sendo 19% vasoespasmo severo. Sequela neurológica por isquemia tardia foi observada em 28,5% dos pacientes, secundária ao vasoespasmo grave, sem correlação estatística com o tempo de oclusão ou com ruptura aneurismática intraoperatória.

Características morfológicas angiográficas de aneurismas de artéria cerebral media: classificação e efeitos sobre os riscos de ruptura / Angiographic morphological characteristics of middle cerebral Artery aneurysms: classification and effects on the risk of rupture

O tratamento de aneurismas de arteria cerebral media (AACM) e a relação de sua morfologia com a probabilidade de rupturasão tópicos importantes na neurocirurgia vascular. Objetivo: Avaliar a correlação entre a morfologia desses aneurismas e 1)probabilidade de ruptura do aneurisma e 2) sua mobimortalidade. Métodos: Vinte e nove pacientes portadores de AACM nosegmento M1 (4 pacientes com aneurismas múltiplos) foram acompanhados / tratados por nossa equipe em uma única instituiçãopelos últimos 5 anos. Catorze aneurismas sofreram ruptura no momento da admissão e 15 foram diagnosticados acidentalmente.Através do formato e geometria, os aneurismas foram classificados e correlacionados com a taxa de ruptura e morbimortalidade.Resultados: Os aneurismas apresentaram medidas entre 7 e 10 mm de diâmetro (90%), sem diferença no tamanho entre os rotos enão-rotos. Pacientes cujos AACMs se romperam na admissão eram o triplo daqueles não-rotos com formato elíptico transverso ede pera invertida (21% vs 9%, p<0,05). Por outro lado, pacientes com AACMs não-rotos possuem aneurisma em formato de pera(36,3% vx 5,2%, p<0,001). Aneurismas com formato arredondado eram os mais frequentes, embora não fosse significativamentepropensos à ruptura. Conclusão: Embora a amostra seja pequena, concluímos que aneurismas com formato elíptico transversoe de pera invertida estavam mais associados com a ruptura que aqueles redondos ou em forma de pera.

Hipotermia no tratamento de hipertensão intracraniana refratária em pacientes com HSA grave, casos associados a vasoespasmo secundário / Hypothermia in the treatment of refractory intracranial hypertension in patients with severe SAH, cases associated with secondary vasospasms

A hemorragia subaracnóidea (HAS) é uma doença grave na qual 10% dos pacientes podem apresentar morte súbita antes mesmode chegar ao hospital. Os pacientes apresentam um risco elevado de complicações secundárias, incluindo o vasoespasmo . Paravasoespasmo o refratário e, essencialmente, para o controle de PIC, a hipotermia pode ser utilizada de forma terapêutica.Nosso objetivo foi avaliar a influência da hipotermia terapêutica nas taxas de mortalidade de pacientes com HSA grave ecom pressão intracraniana elevada, apesar de melhor tratamento clínico implementado previamente. As temperaturas foramreduzidas utilizando colchão térmico para uma temperatura alvo de 33 -35o C.Cinco pacientes foram incluídos. Dois pacientes foram admitidos com um Hunt- Hess de 2, dois com Hunt Hess de 3 e um comHunt Hess de 4. O tempo médio de hipotermia terapêutica foi de 7,2 dias. Dois pacientes sobreviveram com uma escala de Rankinde 0, e três faleceram.Em nosso estudo observacional foi observada uma taxa de mortalidade de 180 dias de 40% , que aumentou para 60% emanálise de seis meses. Estudos recentes descrevem uma taxa de mortalidade de 90% em pacientes com HSA com Hipertensãointracraniana refratária. Acreditamos que a hipotermia pode seja o melhor método para ser utilizado na presente situação clínica.