Measuring quality and safety of epilepsy monitoring units in Brazil: Adoption of quality indicators.

PURPOSE: Drug-resistant epilepsy affects a substantial proportion (30-40 %) of patients with epilepsy, often necessitating video-electroencephalography (video-EEG) monitoring. In 2016, Sauro et al. introduced a set of measures aimed at improving the quality and safety indicators reported in video-EEG evaluations. This study aims to report our experience with the implementation of these measures. METHODS: We analyzed video-EEG data regarding quality and safty from a period spanning January 2016 to January 2018, involving a total of 101 patients monitored in our video-EEG unit. RESULTS: Among the patients included in the study, a definitive diagnosis was attainable for 92.1 %, with 36.6 % experiencing a change in diagnosis and 65.3 % undergoing a change in treatment as a result of the video-EEG evaluation. Additionally, the referral question was fully addressed in 60.4 % of admissions, and video-EEG was considered to be very useful or extremely useful in 66.4 % of cases. Adverse events were observed in 26.7 % of patients, with the most common being the progression of focal seizures to bilateral tonic-clonic seizures (11.9 %) and the occurrence of seizure clusters (5.9 %). CONCLUSION: Our findings support the implementation of Sauro et al.'s set of measures, as they provide valuable criteria for improving the reporting of video-EEG quality and safety indicators. However, challenges may arise due to variations in terminology across studies and the lack of standardized criteria for defining essential questions in video-EEG evaluations. Further research utilizing these measures is necessary to enhance their effectiveness and encourage consistent reporting of results from epilepsy monitoring units.

Methylation of BDNF and SLC6A4 Gene Promoters in Brazilian Patients With Temporal Lobe Epilepsy Presenting or Not Psychiatric Comorbidities.

The relationship between epilepsy and psychiatric comorbidities has been recognized for centuries, but its pathophysiological mechanisms are still misunderstood. It is biologically plausible that genetic or epigenetic variations in genes that codify important neurotransmitters involved in epilepsy as well as in psychiatric disorders may influence the development of the latter in patients with epilepsy. However, this possibility remains poorly investigated. The aim of this study was to evaluate the methylation profile of the BDNF and SLC6A4, two genes importantly involved in neuroplasticity, in patients with temporal lobe epilepsy (TLE) regarding the development or not of psychiatric comorbidities. One hundred and thirty-nine patients with TLE, 90 females and 45 males, were included in the study. The mean age of patients was 44.0 (+12.0) years, and mean duration of epilepsy was 25.7 (+13.3) years. The Structured Clinical Interview for DSM-IV shows that 83 patients (59.7%) had neuropsychiatric disorders and 56 (40.3%) showed no psychiatric comorbidity. Mood disorders were the most common psychiatric disorder observed, being present in 64 (46.0%) of all 139 patients. Thirty-three (23.7%) patients showed anxiety disorders, 10 (7.2%) patients showed history of psychosis and 8 (5.8%) patients showed history of alcohol//drug abuse. Considering all 139 patients, 18 (12.9%) demonstrated methylation of the promoter region of both BDNF and SLC6A4 genes. A significant decreased methylation profile was observed only in TLE patients with mood disorders when compared with TLE patients without a history of mood disorders (O.R. = 3.45; 95% C.I. = 1.08-11.11; p = 0.04). A sub-analysis showed that TLE patients with major depressive disorder mostly account for this result (O.R. = 7.20; 95% C.I. = 1.01-56.16; p = 0.042). A logistic regression analysis showed that the independent factors associated with a history of depression in our TLE patients was female sex (O.R. = 2.30; 95% C.I. = 1.02-5.18; p = 0.044), not controlled seizures (O.R. = 2.51; 95% C.I. = 1.16-5.41; p = 0.019) and decreased methylation in BDNF and SLC6A4 genes (O.R. = 5.32; 95% C.I. = 1.14-25.00; p = 0.033). Our results suggest that BDNF or SLC6A4 genes profile methylation is independently associated with depressive disorders in patients with epilepsy. Further studies are necessary to clarify these matters.

Intradural Spinal Arachnoid Cyst in a Pediatric Patient: A Case Report

The present case reports a 13-year-old patient with an intradural arachnoid cyst, which manifested itself with a sudden loss of strength and sensitivity in the lower and upper limbs and a severe pain in the cervical and thoracic region. On examination, a lesion displayed as an intradural hematoma; however, a laminotomy was performed and it was realized that the lesion was an arachnoid spinal cyst of the cervical-dorsal spine.

Evaluating the Association of Calcified Neurocysticercosis and Mesial Temporal Lobe Epilepsy With Hippocampal Sclerosis in a Large Cohort of Patients With Epilepsy.

BACKGROUND: Neurocysticercosis (NCC) is a parasitic infection of the central nervous system that has been associated with mesial temporal lobe epilepsy with hippocampal sclerosis (MTLE-HS). However, this association has not been completely established. OBJECTIVE: To evaluate the prevalence of calcified NCC (cNCC), its characteristics and a possible association between cNCC and MTLE-HS in a cohort of 731 patients with epilepsy. METHODS: We review clinical, EEG and neuroimaging findings of 731 patients with epilepsy. From these, 659 had CT-scans and 441 patients had complete neuroimaging with CT-scans and MRI. In these patients, we review the prevalence and characteristic of epilepsy in cNCC and in MTLE-HS patients. RESULTS: Forty-two (6.4%) of the 659 patients studied with CT-scans had cNCC. cNCC lesions were more frequent in women than in men (n = 33-78.6% vs. n = 09-21.4%, respectively; OR = 3.64;(95%CI = 1.71-7.69); p < 0.001). cNCC was more often in patients who developed epilepsy later in life, in older patients, in patients who had a longer history of epilepsy, and in those with a lower educational level. MTLE-HS was observed in 93 (21.1%) of 441 patients that had complete neuroimaging, and 25 (26.9%) of these 93 patients also had cNCC. Calcified NCC was observed in only 17 (4.9%) of the remaining 348 patients that had other types of epilepsy rather than MTLE-HS. Thus, in our cohort, cNCC was more frequently associated with MTLE-HS than with other forms of epilepsy, O.R. = 11.90;(95%CI = 6.10-23.26); p < 0.0001). CONCLUSIONS: As expected, in some patients the epilepsy was directly related to cNCC lesional zone, although this was observed in a surprisingly lower number of patients. Also, cNCC lesions were observed in other forms of epilepsy, a finding that could occur only by chance, with epilepsy probably being not related to cNCC at all. In this cohort, cNCC was very commonly associated with MTLE-HS, an observation in agreement with the hypothesis that NCC can contribute to or directly cause MTLE-HS in many patients. Given the broad world prevalence of NCC and the relatively few studies in this field, our findings add more data suggesting a possible and intriguing frequent interplay between NCC and MTLE-HS, two of the most common causes of focal epilepsy worldwide.

Survival and prognostic factors in childhood medulloblastoma: A Brazilian single center experience from 1995 to 2016.

BACKGROUND: Medulloblastoma is the most common malignant brain tumor in the pediatric population. Despite prognosis improvement in the past two decades, one-third of the patients still remain incurable. New evidence suggests that medulloblastoma comprises four distinct entities; therefore, treatment de-escalation is required. The aim of this article is to evaluate epidemiological data from patients treated at our institution. The primary objective is to analyze overall survival (OS) and event-free survival (EFS) and the secondary objective is to identify prognostic factor from this cohort. METHODS: We retrospectively analyzed 69 patients who underwent surgical resection for medulloblastoma among 423 children from the tumor registry data bank of Santo Antônio Children's Hospital from 1995 to 2016. Kaplan-Meier method and Cox regression analysis were used to identify OS, EFS, and prognostic factors. RESULTS: The 5-year OS and EFS rates found were 44.5% and 36.4%, respectively. The extent of resection and radiotherapy as adjuvant treatments was positively correlated to outcome while metastatic disease at diagnosis was negatively related to OS. Age younger than 3 years old did not have a worse outcome in our cohort. CONCLUSION: Similar results to population-based studies were found, but we still face difficulties due to living in a developing country. In the near future, we look forward to new diagnostic techniques that will enable us to classify medulloblastomas according to molecular subgroups.

Stereotactic radiosurgery for movement disorders.

Initially designed for the treatment of functional brain targets, stereotactic radiosurgery (SRS) has achieved an important role in the management of a wide range of neurosurgical pathologies. The interest in the application of the technique for the treatment of pain, and psychiatric and movement disorders has returned in the beginning of the 1990s, stimulated by the advances in neuroimaging, computerized dosimetry, treatment planning software systems, and the outstanding results of radiosurgery in other brain diseases. Since SRS is a neuroimaging-guided procedure, without the possibility of neurophysiological confirmation of the target, deep brain stimulation (DBS) and radiofrequency procedures are considered the best treatment options for movement-related disorders. Therefore, SRS is an option for patients who are not suitable for an open neurosurgical procedure. SRS thalamotomy provided results in tremor control, comparable to radiofrequency and DBS. The occurrence of unpredictable larger lesions than expected with permanent neurological deficits is a limitation of the procedure. Improvements in SRS technique with dose reduction, use of a single isocenter, and smaller collimators were made to reduce the incidence of this serious complication. Pallidotomies performed with radiosurgery did not achieve the same good results. Even though the development of DBS has supplanted lesioning as the first alternative in movement disorder surgery; SRS might still be the only treatment option for selected patients.

Mielomeningocele: conceitos básicos e avanços recentes / Meningomielocele: basic concepts and recent advances

A mielomeningocele (MMC) é uma malformação embrionária do sistema nervoso central que ocorre nas primeiras quatro semanas de gestação decorrente de uma falha no processo de neurulação primária que é processo normal de fechamento do tubo neural. A MMC é a malformação decorrente do defeito de fechamento do tubo neural (DFTN) mais frequente. Tem etiologia multifatorial com participação de fatores genéticos e ambientais que afetam o metabolismo do ácido fólico. Os DFTN podem ter sua incidência reduzida drasticamente com a suplementação de 0,4 a 1 mg de ácido fólico antes da gestação e com políticas governamentais de fortificação de farinhas com o ácido fólico, como já é feito no Brasil.O diagnóstico pré-natal pode ser feito com segurança pela ecografia obstétrica, embora possa ser feito também com métodos mais sofisticados com a ressonância magnética.Um grande número de anomalias podem estar associadas à MMC, sendo a ocorrência de hidrocefalia e malformação de Chiari tipo II quase constantes. O exame neurológico pode mostrar alterações funcionais da medula espinhal em graus variados, na dependência do nível medular em que ocorre, lesões localizadas em segmentos medulares mais craniais determinam maiores prejuízos neurológicos. O tratamento cirúrgico deve ocorrer idealmente nas primeiras 24 horas após o nascimento para diminuir o risco de infecção. Os objetivos fundamentais do reparo cirúrgico da MMC são a preservação de todo tecido nervoso viável, reconstituição anatômica e a minimização ou prevenção do risco de infecção do sistema nervoso central. Mais recentemente foi concluído o longo estudo sobre o reparo intraútero (MOMS,2011) da MMC que demonstrou amplo benefício desta abordagem em casos selecionados.Os autores fazem uma ampla revisão dos conceitos básicos e os recentes avanços relacionados à MMC.

Uso de Stem Cells no tratamento do trauda raquimedular / Use of Stem Cells

O trauma raquimedular (TRM) é uma importante causa de morte, além de ser uma grande causa de morbidez nos sobreviventes. Uma vez ocorrida a lesão medular são limitadas as medidas terapêuticas, sendo o prognóstico reservado. Atualmente, através de técnicas inovadoras, têm sido pesquisadas novas opções de tratamento. A utilização de remanescentes de células-tronco do sistema nervoso central está sendo estudada. As técnicas empregadas são as mais variadas, embora todas utilizem modelos animais experimentais, tendo em mente um único objetivo, que é a recuperação da função após o trauma. Este artigo é uma revisão da literatura sobre o estado atual das pesquisas a respeito da utilização de células-tronco no TRM.

[Treatment of intracranial hypertension]. / Tratamento da hipertensão intracraniana.

OBJECTIVES: To review the current therapeutic approach of intracranial hypertension in pediatric patients admitted to intensive care unit. SOURCES OF DATA: Bibliographic review of the subject based on Medline. SUMMARY OF THE FINDINGS: The authors noticed that some measures to control intracranial hypertension are consensual, and others remain controversial. CONCLUSION: The goals of management of pediatric patients with intracranial hypertension include: normalizing the intracranial pressure, optimizing cerebral blood flow and cerebral perfusion pressure, preventing second insults that exacerbate secondary injury, and avoiding complications associated with the various treatment modalities employed.

Tratamento da hipertensäo intracraniana / Treatment of intracranial hypertension

OBJETIVO: revisar a abordagem terapêutica atual nos pacientes pediátricos com hipertensäo intracraniana, internados em unidade de terapia intensiva. FONTE DE DADOS: revisäo bibliográfica sobre o tema, utilizando como base de dados o Medline. SíNTESE DOS DADOS: a partir da literatura levantada pode-se observar a existência de medidas de monitorizaçäo e tratamento da hipertensäo intracraniana aceitas como consenso pelos diferentes autores, assim como abordagens que ainda motivam controvérsias. CONCLUSÕES: os objetivos no manejo do paciente pediátrico com hipertensäo intracraniana incluem a normalizaçäo da pressäo intracraniana, a otimizaçäo do fluxo sangüíneo cerebral e pressäo de perfusäo cerebral, prevenindo o segundo insulto que exacerba a lesäo secundária, evitando as complicações associadas com as várias modalidades de tratamento empregadas.

Hipertensao intracraniana em pediatria revisao sobre fisiopatologia, monitorizacao e tratamento / Intracranial hypertension in pediatrics review about physiopathology, monitoring and treatment

Os autores revisam os mecanismos fisiopatológicos da hipertensäo intracraniana e as indicações de monitorizaçäo e tratamento em diversas situações clínicas

Ganglioglioma desmoplásico infantil: relato de dois casos / Desmoplastic infantile ganglioglioma: report of two cases

O ganglioglioma desmoplásico infantil (GDI) é um tumor raro, que pertence ao grupo dos tumores mistos neurogliais, descrito pela primeira vez por VandenBerg, em 1987: São tumores supratentoriais, volumosos, apresentando frequentemente envolvimento leptomeníngeo. O exame anatomopatológico evidencia intensa desmoplasia, com diferenciação astrocítica e ganglionar; bem como um teste imunoistoquímico apresenta resultado positivo para "glial fibrillary acidic protein" (GFAP). São relatados dois casos de GDI e discutidos os aspectos clínicos e terapêuticos dos referidos casos.

Opióides endógenos e epilepsia: geradores ou controladores de crises?; uma revisäo crítica da literatura / Endogenous opioids and epilepsy: are thy generating or limiting crises?; a critical revien of the literature

Desde a descoberta dos opióides endógenos, muita pesquisa tem sido feita no sentido de investigar sua possível participaçäo nos fenômenos epilépticos humanos. Uma revisäo sobre o assunto é realizada e, a partir de dados da literatura e dos dados experimentais dos autores nesta área, as hipóteses atuais sobre o papel dos peptídios endógenos na epilepsia, bem como possíveis implicaçöes clínicas, säo amplamente discutidas

Opioides endogenos e epilepsia: geradores ou controladores de crises?: uma revisao critica da literatura

Desde a descoberta dos opioides endogenos, muita pesquisa tem sido feita no sentido de investigar sua possivel participacao nos fenomenos epilepticos humanos. Uma revisao sobre o assunto e realizada e, a partir de dados da literatura e dos dados experimentais dos autores nesta area, as hipoteses atuais sobre o papel dos peptidios endogenos na epilepsia, bem como possiveis implicacoes clinicas, sao amplamente discutidas.