Anesthetic considerations in patients with Dyke-Davidoff-Masson syndrome: a case report and review of literature

Dyke-Davidoff-Masson syndrome [DDMS] is a rare disorder of cerebral hemiatrophy. The clinical presentation may consist of facial asymmetry, contralateral atrophy [including the trunk and extremities], hemiparesis, speech difficulties, mental retardation and epilepsy. It involves multiple systems, especially problems of the airway, occult myopathy and seizure disorder. Anesthesia for such patients is a challenge to the anesthesiologist. We report the anesthetic care of 9 year old female child of DDMS for fractional lengthening of tendons of the forearm. Airway management, induction technique, pathophysiology of the disease, drug selection and other concerns of anesthesia have been discussed reviewing the sparse literature

Clinical electroencephalogram (EEG) evaluation is improved by the amplitude asymmetry index / Avaliação da assimetria de amplitudes amplia o uso clínico do eletroencefalograma (EEG)

ABSTRACT Cerebral hemispheres, although similar, are neither completely symmetrical in structure nor equivalent in function. EEG asymmetry studies have been directed more to frequency than to amplitude analysis. Objective Better definition of normal amplitude asymmetry values on the classical EEG frequency bands. Results EEG amplitude asymmetry index (AAI) is physiologically low in normal adults, differences usually lesser than 7%. Conclusion Persistent or intermittent amplitude asymmetry regional differences higher than 7% may be suggestive of pathology after adequate correlation with clinical data and EEG classical visual analysis.

RESUMO Os hemisférios cerebrais não são simétricos em morfologia ou funções. Tradicionalmente, os estudos de assimetria do EEG focaram mais na análise de frequências do que na de amplitudes. Objetivo Contribuir para melhor definição dos valores normais de assimetria de amplitudes nas faixas de frequência clássicas do EEG. Resultados O índice de assimetria de amplitudes é fisiologicamente baixo em adultos, usualmente menor do que 7%. Conclusão Se adequadamente correlacionadas aos dados clínicos e à análise visual do EEG, assimetrias persistentes ou intermitentes de amplitude acima de 7% podem ser sugestivas de patologia.

Caracterización sociodemográfica y clínica de la población atendida en el Instituto Teletón de Santiago / Sociodemographic and clinical characteristic of the population attended in the Instituto Teletón de Santiago

Los Institutos Teletón atienden al 85% de la población infantil chilena con discapacidad neuro-músculo-esquelética, concentrando el mayor porcentaje de esta población. Sin embargo, no existen registros que permitan caracterizar esta población. Objetivo: Caracterizar la población de pacientes atendidos en el Instituto Teletón de Santiago durante el año 2012. Pacientes y método: Se analizaron las características sociodemográficas de los registros electrónicos del Instituto Teletón de Santiago de los pacientes activos que se atendieron durante el año 2012. Resultados: Durante el año 2012 se atendió un total de 8.959 pacientes en el Instituto Teletón de Santiago. En relación con el nivel socioeconómico, un 33,3% de estos correspondieron a extrema pobreza, y un 28,7% a nivel medio bajo. Con respecto a los diagnósticos clínicos principales se encontró que la parálisis cerebral y otras encefalopatías que también llevan a discapacidad motora concentran el 55,4% de los casos. Conclusiones: Como producto de esta caracterización, sería adecuado fomentar la necesidad de optimizar el registro nacional de la población infantil con discapacidad y sus características particulares, para así poder tomar decisiones de políticas públicas, como destinación de fondos o programas de apoyo.

The Institutos Teletón care for 85% of the Chilean child population with neuromusculoskeletal disability, the large percentage concentrating in this population. However, there are no registers that enable a profile to be determined on this population. Objective: To determine the profile of patients attending the Instituto Teletón de Santiago during the year 2012. Patients and method: The sociodemographic characteristics were analyzed from the computerised records of the Instituto Teletón de Santiago on active patients who were seen during the year 2012. Results: A total of 8,959 patients were seen during the study year in the Instituto Teletón de Santiago. As regards socioeconomic level, 33.3% were in extreme poverty, 28.7% to low-middle level. The main clinical diagnoses were cerebral palsy and other encephalopathies that also lead to motor disability, and accounted for 55.4% of the cases. Conclusions: As a result of determining this profile, it would be appropriate to encourage the need for a national register of the child population with disability, as well as their particular characteristics in order to make decisions on public policy, as a destination for funds or support programs.

One-year follow-up of neurological status of patients after cardiac arrest seen at the emergency room of a teaching hospital / Um ano de seguimento da condição neurológica de pacientes pós-parada cardiorrespiratória atendidos no pronto-socorro de um hospital universitário

ABSTRACT Objective: To describe neurological status and associated factors of survivors after cardiac arrest, upon discharge, and at 6 and 12 month follow-up. Methods: A cohort, prospective, descriptive study conducted in an emergency room. Patients who suffered cardiac arrest and survived were included. A one-year consecutive sample, comprising 285 patients and survivors (n=16) followed up for one year after discharge. Neurological status was assessed by the Cerebral Performance Category before the cardiac arrest, upon discharge, and at 6 and 12 months after discharge. The following factors were investigated: comorbidities, presence of consciousness upon admission, previous cardiac arrest, witnessed cardiac arrest, location, cause and initial rhythm of cardiac arrest, number of cardiac arrests, interval between collapse and start of cardiopulmonary resuscitation, and between collapse and end of cardiopulmonary resuscitation, and duration of cardiopulmonary resuscitation. Results: Of the patients treated, 4.5% (n=13) survived after 6 and 12 months follow-up. Upon discharge, 50% of patients remained with previous Cerebral Performance Category of the cardiac arrest and 50% had worsening of Cerebral Performance Category. After 6 months, 53.8% remained in the same Cerebral Performance Category and 46.2% improved as compared to discharge. After 12 months, all patients remained in the same Cerebral Performance Category of the previous 6 months. There was no statistically significant association between neurological outcome during follow-up and the variables assessed. Conclusion: There was neurological worsening at discharge but improvement or stabilization in the course of a year. There was no association between Cerebral Performance Category and the variables assessed. .

RESUMO Objetivo: Identificar a condição neurológica e os fatores associados de sobreviventes pós-parada cardiorrespiratória na alta hospitalar, após 6 e 12 meses de seguimento. Métodos: Estudo de coorte, prospectivo e descritivo, realizado em um pronto-socorro. Foram incluídos pacientes em parada cardiorrespiratória que sobreviveram à alta. A amostra foi consecutiva por um ano, sendo composta por 285 pacientes, e os sobreviventes (n=16) foram acompanhados por um ano após alta. O estado neurológico foi avaliado pela Categoria de Performance Cerebral antes da parada, na alta, 6 e 12 meses após alta. Foram investigados os seguintes fatores: comorbidades, presença de consciência na admissão, parada cardiorrespiratória prévia, parada cardiorrespiratória testemunhada, local, causa e ritmo inicial da parada, número de paradas, intervalo entre colapso e início da ressuscitação cardiopulmonar, e entre colapso e término da ressuscitação, e duração da ressuscitação. Resultados: Dos pacientes atendidos, 4,5% (n=13) sobreviveram após 6 e 12 meses de seguimento. Na alta, 50% dos pacientes permaneceram com Categoria de Performance Cerebral prévia à parada, e 50% tiveram piora da mesma. Após 6 meses, 53,8% permaneceram com mesma Categoria de Performance Cerebral, e 46,2% tiveram melhora em relação à alta. Após 12 meses, a totalidade dos pacientes permaneceu com mesma Categoria de Performance Cerebral em relação aos 6 meses anteriores. Não houve associação estatisticamente significativa entre evolução neurológica durante o seguimento e variáveis de interesse. Conclusão: Observou-se piora neurológica na alta, mas houve melhora ou estabilização no decorrer de 1 ano. Não foi encontrada associação entre Categoria de Performance Cerebral e variáveis de interesse. .

Aristides Leão: a birth centennial homage with comments on his spreading depression / Aristides Leão: homenagem ao centenário de nascimento com comentários sobre sua depressão alastrante

The year of 2014 is the birth centenary of Aristides Azevedo Pacheco Leão (1914-1993), and also marks seventy years of the publication of his discovery of the novel electrophysiological phenomenon, named by him “spreading depression” (SD), soon designated “Leão’s wave” or “Leão’s spreading depression”. This was a remarkable scientific milestone, and the author must be celebrated for this achievement, as the studies he triggered proceeded worldwide, with new concepts, as spreading depolarization, until the present days. Robust experimental and clinical evidence emerged to suggest that these and related electrophysiological phenomena are involved in the mechanisms of migraine aura, acute cerebrovascular diseases, traumatic brain injury, transient global amnesia, epileptic seizures, and their pathophysiological characteristics come to offer new therapeutic perspectives. He was a remarkable and complex personality, and the authors remit the readers to a paper where his personal life is contemplated.

O ano de 2014 é o centenário de nascimento de Aristides Azevedo Pacheco Leão (1914-1993), e também assinala setenta anos da publicação de sua descoberta, que ele denominou “depressão alastrante” (DA), logo designada “onda de Leão” ou “depressão alastrante de Leão”. Foi um notável marco científico e o autor deve ser celebrado por esse feito, considerando que estudos que desencadeou continuaram no mundo todo, com novos conceitos, como a despolarização alastrante, até os dias atuais. Evidência experimental e clínica robusta emergiram sugerindo que esses fenômenos eletofisiológicos e outros relacionados encontram-se envolvidos nos mecanismos da aura da enxaqueca, doenças cerebrovasculares agudas, lesão cerebral traumática, amnésia global transitória, crises epiléticas, sendo que suas características fisiopatológicas vêm oferecer novas perspectivas terapêuticas. Foi uma personalidade complexa e notável, e os autores remetem os leitores para um artigo no qual sua vida pessoal é contemplada.

La encefalopatía de la prematuridad, una entidad nosológica en expansión / Encephalopathy of prematurity, a developing nosological entity

Con frecuencia se emplean los términos leucomalacia periventricular, daño a la sustancia blanca y encefalopatía de la prematuridad como sinónimos; sin embargo, no son exactamente lo mismo. Se realiza esta revisión con el objetivo de fundamentar la consistencia del término encefalopatía de la prematuridad, a partir de una actualización sobre su fisiopatología, diagnóstico y pronóstico. La leucomalacia periventricular tiene 2 componentes principales, uno macroscópico quístico y otro microscópico, que puede ser focal o difuso. El daño a la sustancia blanca es el hallazgo neuropatológico más común de parálisis cerebral en los recién nacidos pretérminos, que identifica cualquier anormalidad en la sustancia blanca. La encefalopatía de la prematuridad, en cambio, es un término mucho más abarcador, porque en su origen parecen tener importancia los trastornos destructivos primarios y los relacionados con el desarrollo cerebral. Su fisiopatología se ha tratado de explicar mediante 2 modelos teóricos: el infeccioso-inflamatorio y el hemodinámico. Para su diagnóstico resultan necesarias la evaluación neuroimaginológica y la del neurodesarrollo, en la que a menudo se diagnostica la presencia de parálisis cerebral o trastornos del desarrollo intelectual, aisladamente o en asociación. Es muy probable que la encefalopatía de la prematuridad tienda a afianzarse cada día más como una entidad nosológica independiente, dadas sus implicaciones médicas y sociales.

Some terms such as periventricular leukomalacia (PVL), white matter damage (WMD) and encephalopathy of prematurity are used as synonyms; however, they are not exactly the same. This review was aimed at substantiating the consistency of the term encephalopathy of prematurity, on the basis of its physiopathology, diagnosis and prognosis. Periventricular leukomalacia has two main components, one macroscopic cystic and the other microscopic one which can be focal or diffuse. The white matter damage is the most common neuropathological feature of cerebral palsy in preterm infants, which identifies any anomalies in the white matter. In turn, encephalopathy of prematurity is a much more comprehensive term because it seems that both primary destructive disorders and those related to brain development are influential in its origin. Its physiopathology has been explained by two theoretical models: the infective-inflammatory one and the hemodynamic one. Neuroimaginological and neurodevelopmental evaluations are necessary for the diagnosis, where the presence of cerebral palsy and of intellectual development disorders, either isolated or combined, is detected. It is much likely that encephalopathy of prematurity tends to increasingly consolidate as an independent nosological entity, taking into account its medical and social implications.

Disentangling the effects of insomnia and night work on cardiovascular diseases: a study in nursing professionals

Cardiovascular diseases (CVDs) are known to be associated with poor sleep quality in general populations, but they have not been consistently associated with specific work schedules. Studies of CVD generally do not simultaneously consider sleep and work schedules, but that approach could help to disentangle their effects. We investigated the association between insomnia and a self-reported physician diagnosis of CVD in day and night workers, considering all sleep episodes during nocturnal and diurnal sleep. A cross-sectional study was conducted in 1307 female nursing professionals from 3 public hospitals, using baseline data from the “Health and Work in Nursing - a Cohort Study.” Participants were divided into two groups: i) day workers with no previous experience in night shifts (n=281) and whose data on insomnia were related to nocturnal sleep and ii) those who worked exclusively at night (n=340) and had data on both nocturnal and diurnal sleep episodes, as they often sleep at daytime. Multiple logistic regression analysis was performed. Among day workers, insomnia complaints increased the odds of CVD 2.79-fold (95% CI=1.01-6.71) compared with workers who had no complaints. Among night workers, reports of insomnia during both nocturnal and diurnal sleep increased the odds of reported CVD 3.07-fold (95% CI=1.30-7.24). Workers with insomnia had similar probabilities of reporting CVD regardless of their work schedule, suggesting a relationship to insomnia and not to night work per se. The results also highlighted the importance of including evaluation of all sleep episodes (diurnal plus nocturnal sleep) for night workers.

Quantitative EEG evaluation in patients with acute encephalopathy / Analise quantitativa do EEG em pacientes com encefalopatias agudas

Objective To investigate the use of quantitative EEG (qEEG) in patients with acute encephalopathies (AEs) and EEG background abnormalities. Method Patients were divided into favorable outcome (group A, 43 patients) and an unfavorable outcome (group B, 5 patients). EEGLAB software was used for the qEEG analysis. A graphic of the spectral power from all channels was generated for each participant. Statistical comparisons between the groups were performed. Results In group A, spectral analysis revealed spectral peaks (theta and alpha frequency bands) in 84% (38/45) of the patients. In group B, a spectral peak in the delta frequency range was detected in one patient. The remainder of the patients in both groups did not present spectral peaks. Statistical analysis showed lower frequencies recorded from the posterior electrodes in group B patients. Conclusion qEEG may be useful in the evaluations of patients with AEs by assisting with the prognostic determination. .

Objetivos Investigar o uso do EEG quantitativo (qEEG) em pacientes com encefalopatias agudas (EAs ) e anormalidades da atividade de base no EEG. Método s pacientes foram divididos em prognóstico favorável (grupo A, 43 pacientes) e desfavorável (grupo B, 5 pacientes). O programa EEGLAB foi utilizado para a análise do qEEG. Um gráfico da potência espectral de todos os canais foi gerado para cada participante. Os dois grupos foram comparados estatisticamente. Resultados No grupo A, a análise espectral revelou picos (frequências teta e alfa) em 84% (38/45) dos pacientes. No grupo B, um pico espectral na frequência delta foi detectado em um paciente. Os pacientes remanescentes dos dois grupos não apresentaram picos espectrais. A análise estatística mostrou menores frequências registradas nos eletrodos posteriores dos pacientes do grupo B. Conclusão O qEEG pode ser útil na avaliação de pacientes com EAs auxiliando na determinação do prognóstico. .

Encefalopatias: etiologia, fisiopatologia e manuseio clínico de algumas das principais formas de apresentação da doença / Encephalopathies: etiology, pathophysiology, and clinical management of some major forms of disease presentation

JUSTIFICATIVA E OBJETIVOS: As encefalopatias compõem um grupo heterogêneo de etiologias, onde a pronta e correta atuação médica direcionada à causa da doença, pode modificar o prognóstico do paciente. O objetivo deste estudo foi rever os aspectos fisiopatológicos das diferentes encefalopatias bem como seus principais fatores desencadeantes e manuseio clínico.CONTEÚDO: Foram selecionadas as mais frequentes encefalopatias observadas na prática clínica e discutir sua fisiopatologia, bem como sua abordagem terapêutica, destacando: encefalopatia hipertensiva, hipóxico-isquêmica, metabólica, Wernicke-Korsakoff, traumática e tóxica.CONCLUSÃO: Trata-se de uma complexa condição clínica que exige rápida identificação e preciso manuseio clínico com o intuito de reduzir sua elevada taxa de morbimortalidade. O atraso no reconhecimento dessa condição clínica poderá ser extremamente prejudicial ao paciente que estará sofrendo lesão cerebral muitas vezes irreversível.

BACKGROUND AND OBJECTIVES: Encephalopathies comprise a heterogeneous group of clinical conditions, in which the prompt and adequate medical intervention can modify patient prognosis. This paper aims to discuss the pathophysiological aspects of different encephalopathies, their etiology, and clinical management.CONTENTS: We selected the main encephalopathies observed in clinical practice, such as hypertensive, hypoxic-ischemic, metabolic, Wernicke-Korsakoff, traumatic, and toxic encephalopathies, and to discuss their therapeutic approaches.CONCLUSION: This is a complex clinical condition that requires rapid identification and accurate clinical management with the aim of reducing its high morbidity and mortality rates. Delay in recognizing this condition can be extremely harmful to the patient who is suffering from often irreversible brain injury.

A New Disability-related Health Care Needs Assessment Tool for Persons With Brain Disorders / 예방의학회지

OBJECTIVES: This study aimed to develop a health needs assessment (HNA) tool for persons with brain disorders and to assess the unmet needs of persons with brain disorders using the developed tool. METHODS: The authors used consensus methods to develop a HNA tool. Using a randomized stratified systematic sampling method adjusted for sex, age, and districts, 57 registered persons (27 severe and 30 mild cases) with brain disorders dwelling in Seoul, South Korea were chosen and medical specialists investigated all of the subjects with the developed tools. RESULTS: The HNA tool for brain disorders we developed included four categories: 1) medical interventions and operations, 2) assistive devices, 3) rehabilitation therapy, and 4) regular follow-up. This study also found that 71.9% of the subjects did not receive appropriate medical care, which implies that the severity of their disability is likely to be exacerbated and permanent, and the loss irrecoverable. CONCLUSIONS: Our results showed that the HNA tool for persons with brain disorders based on unmet needs defined by physicians can be a useful method for evaluating the appropriateness and necessity of medical services offered to the disabled, and it can serve as the norm for providing health care services for disabled persons. Further studies should be undertaken to increase validity and reliability of the tool. Fundamental research investigating the factors generating or affecting the unmet needs is necessary; its results could serve as basis for developing policies to eliminate or alleviate these factors.

Protocolo de avaliação da amusia: exemplo português / Evaluation protocol for amusia: portuguese sample

A amusia é uma disfunção que compromete o processamento musical. Parte desse processamento é feito a nível do cortéx auditivo primário. O estudo dessa afecção permite-nos avaliar também as vias auditivas centrais. OBJETIVO: Explorar os testes de avaliação diagnóstica da amusia. MÉTODO: Os autores propõem um protocolo de avaliação para doentes com suspeita de amusia (após lesão cerebral ou por queixas de má percepção musical), paralelamente com a avaliação do processamento auditivo central, já implementada no serviço. A base dos testes de avaliação assenta na Montreal Battery of Evaluation of Amusia. Desta ampla bateria de testes, foram selecionados alguns dos exemplos musicais para avaliação de diferentes vertentes da área musical, nomeadamente memória e percepção musical, capacidade de reconhecimento e discriminação musical. Em termos de memória, foi criado um teste de avaliação da memória tardia, adaptado à cultura portuguesa. Estudo prospectivo. RESULTADOS e CONCLUSÕES: Embora ainda em fase experimental e com possibilidades de ajustes na avaliação realizada, considera-se que esta avaliação, aliada ao estudo do processamento auditivo central, permitirá compreender algumas lesões e disfunções centrais, congênitas ou adquiridas, que limitam a percepção auditiva.

Amusia is a disorder that affects the processing of music. Part of this processing happens in the primary auditory cortex. The study of this condition allows us to evaluate the central auditory pathways. OBJECTIVE: To explore the diagnostic evaluation tests of amusia. METHOD: The authors propose an evaluation protocol for patients with suspected amusia (after brain injury or complaints of poor musical perception), in parallel with the assessment of central auditory processing, already implemented in the department. The Montreal Evaluation of Battery of amusia was the basis for the selection of the tests. From this comprehensive battery of tests we selected some of the musical examples to evaluate different musical aspects, including memory and perception of music, ability concerning musical recognition and discrimination. In terms of memory there is a test for assessing delayed memory, adapted to the Portuguese culture. Prospective study. RESULTS AND CONCLUSIONS: Although still experimental, with the possibility of adjustments in the assessment, we believe that this assessment, combined with the study of central auditory processing, will allow us to understand some central lesions, congenital or acquired hearing perception limitations.

Non-epileptiform EEG abnormalities: an overview / Anormalidades eletrencefalográficas não epileptiformes: uma visão geral

More than 80 years after its introduction by Hans Berger, the electroencephalogram (EEG) remains as an important supplementary examination in the investigation of neurological disorders and gives valuable and accurate information about cerebral function. Abnormal EEG findings may include ictal patterns, interictal epileptiform activity and non-epileptiform abnormalities. The aim of this study is to make an overview on the main non-epileptiform EEG abnormalities, emphasizing the pathologic findings and the importance of their recognition, excluding periodic patterns and EEG physiologic changes. Scientific articles were selected from MEDLINE and PubMed database. The presence of non-epileptiform EEG abnormalities provide evidence of brain dysfunction that are not specific to a particular etiology and may be related to a number of disorders affecting the brain. Although these abnormalities are not specific, they can direct attention to the diagnostic possibilities and guide the best treatment choice.

Mais de 80 anos após sua introdução por Hans Berger, o eletrencefalograma (EEG) permanece como importante exame complementar na investigação de transtornos neurológicas e fornece informações valiosas e precisas a respeito da função cerebral. Achados eletrencefalográficos anormais podem incluir padrões ictais, atividade epileptiforme interictal e anormalidades eletrencefalográficas não epileptiformes. O objetivo deste estudo é fazer uma revisão das principais anormalidades eletrencefalográficas não epileptiformes, enfatizando os achados patológicos e a importância de seu reconhecimento, excluindo padrões periódicos e alterações eletrencefalográficas fisiológicas. Foram selecionados artigos científicos por meio de pesquisa nas bases de dados MEDLINE e PubMed. A presença de anormalidades eletrencefalográficas não epileptiformes fornece evidências de disfunção cerebral, as quais não são específicas para uma etiologia particular e podem estar relacionadas a uma série de desordens que afetam o encéfalo. Embora essas anormalidades não sejam específicas, elas podem direcionar a atenção para as possibilidades diagnósticas e guiar a escolha do melhor tratamento.

Afasia de conducción como consecuencia de un astrocitoma anaplásico parieto–temporo–occipital izquierdo: estudio de caso / Conduction aphasia as a result of left parietal-temporal-occipital anaplastic astrocytoma: a case study

La afasia de conducción es un trastorno caracterizado por un lenguaje espontáneo relativamente fluido, buena comprensión, pero dificultades en la repetición asociadas con parafasias fonológicas. Se ha atribuido a lesiones del fascículo arqueado por desconexión entre el lóbulo temporal posterior y el frontal, sin embargo se ha debatido esta postura, planteando que la integridad y funcionamiento del fascículo arqueado no es indispensable en la repetición verbal. Se presenta un caso de un sujeto varón de 23 años que, como consecuencia de un astrocitoma anaplásico recidivante que abarca áreas parietales y temporo-occipitales, presenta una afasia de conducción. Se plantea una reconceptualización de esta afasia, analizándola en términos clínicos, neuropsicológicos y en las redes neuronales existentes entre áreas cerebrales posteriores ipsilaterales y contralaterales.

Conduction aphasia is a language disorder characterized by an impaired ability to repeat verbal material associated with phonological paraphasias but a relatively fluent spontaneous speech and preserved comprehension. It has been attributed to lesions of the arcuate fasciculus by disconnection between posterior temporal lobe and frontal lobe, however, this idea has been debated, because the integrity and function of the arcuate fasciculus does not seem to be essential in verbal repetition. We report a case of a 23 year old male, with conduction aphasia as a result of a recurrent anaplastic astrocytoma in parietal and temporo-occipital areas. We propose a reconceptualization of the aphasia, analyzing it in terms of clinical neuropsychological and neural networks between ipsilateral and contralateral posterior brain areas.

Sepsis-associated encephalopathy: not just delirium

Sepsis is a major cause of mortality and morbidity in intensive care units. Organ dysfunction is triggered by inflammatory insults and tissue hypoperfusion. The brain plays a pivotal role in sepsis, acting as both a mediator of the immune response and a target for the pathologic process. The measurement of brain dysfunction is difficult because there are no specific biomarkers of neuronal injury, and bedside evaluation of cognitive performance is difficult in an intensive care unit. Although sepsis-associated encephalopathy was described decades ago, it has only recently been subjected to scientific scrutiny and is not yet completely understood. The pathophysiology of sepsis-associated encephalopathy involves direct cellular damage to the brain, mitochondrial and endothelial dysfunction and disturbances in neurotransmission. This review describes the most recent findings in the pathophysiology, diagnosis, and management of sepsis-associated encephalopathy and focuses on its many presentations.

Encefalopatía inducida por ácido valproico: serie de siete casos / Valproic acid-induced encephalopathy: series of seven cases

Introducción: La encefalopatía inducida por ácido valproico (AV) es una complicación infrecuente caracterizada por disminución del nivel de conciencia, déficits neurológicos focales, enlentecimiento cognitivo, vómitos, somnolencia y letargia, con o sin hiperamonemia. El electroencefalograma (EEG) muestra enlentecimiento difuso. Los hallazgos EEG, las manifestaciones clínicas y la hiperamonemia tienden a normalizarse con la suspensión del AV. Pacientes y Métodos: Se presenta una serie de 7 pacientes que desarrollaron encefalopatía por AV, en el Servicio de Neurología del Hospital del Salvador, entre 2003 y 2010. Se detallan dos casos clínicos ilustrativos. Resultados: La serie está compuesta por 5 mujeres y 2 hombres. Cinco pacientes desarrollaron hiperamonemia (amonemia sobre 50 ug/dl). El promedio de edad fue de 55 años (37 a 82 años). Las dosis de AV fueron de 375 a 2.000 mg (promedio = 903). La latencia entre el inicio o ajuste significativo del AVfue de 3 días hasta 16 años y un mes. Todos los pacientes presentaban daño orgánico cerebral. La politerapia con fenobarbital, fenitoína y carbamazepina fue significativa. El patrón de EEG más frecuente fue el enlentecimiento difuso. Una paciente de 82 años desarrolló actividad pseudoperiódica sugerente de un status epilepticus no convulsivo. En todos los pacientes hubo normalización clínica, de laboratorio y del EEG con la suspensión del AV. Conclusiones: La encefalopatía inducida por ácido valproico es una reacción adversa reversible pero potencialmente fatal que requiere un alto índice de sospecha. El daño orgánico cerebral y la politerapia parecen ser importantes factores de riesgo para su producción.

Introduction: Valproic acid (VA) induced encephalopathy is an unusual complication characterized by decreasing level of consciousness, focal neurological deficits, cognitive slowing, vomiting, drowsiness, and lethargy, with or without hyperammonemia. Electroencephalography (EEG) is characterized by continuous generalized slowing. The EEG findings, as well as clinical manifestations and hyperammonemia, tend to normalize after VA withdrawal. Patients and Methods: We present a series of seven patients who developed VA-induced encephalopathy at the Neurology Department of Hospital Salvador between 2003 and 2010. We report two illustrative cases in extenso. Results: Our series is composed by five women and two men. Five patients developed hyperammonemia (ammonemia above 50 ug/dl). 55years was the average of patients (range: 37 to 82 years). VA dose was between 375 and 2.000 mg (average 903 mg). Latency between start or important change in VA dose was 3 days to 16 years and a month. All patients had brain damage. Polytherapy with phenobarbital, phenytoin and carbamazepine was significant. The most frequent EEG pattern was diffuse slowing. A 82-year-old female developed a seudo-periodic activity suggesting a non-convulsive status epilepticus. The clinical manifestations, EEG findings and laboratory normalized after VA withdrawal. Conclusions: Acid valproic-induced encephalopathy is a reversible but potentially fatal adverse reaction that requires a high index of suspicion. Brain damage and polytherapy seem to be important risk factors.

Encefalopatia associada a sepse: disfunção comportamental, metabólica e mitocondrial em modelo de sepse abdominal / Sepsis associated encephalopathy: behavioral, metabolic and mitichondrial dysfunction in a model of abdominal sepsis

A sepse e síndrome de disfunção orgânica múltipla representam um problema clínico de alta relevância, principalmente devido a sua grande incidência em pacientes críticos e aos seus altos índices de mortalidade. Apesar de medidas terapêuticas terem sido capazes de diminuir a mortalidade, observamos que muitos sobreviventes a sepse não são capazes de retomar suas atividades usuais. Estudos experimentais em animais são uma ferramenta importante para o estudo da fisiopatologia e da terapêutica da sepse. A encefalopatia associada à sepse (EAS) é muitas vezes a primeira disfunção orgânica a se manifestar. Clinicamente pode se apresentar como sonolência, agitação, delírium e coma. A presença da EAS está associado a maior mortalidade e pior prognóstico. Muitos pacientes apresentam dano cognitivo a médio e longo prazo que pode ser irreversível. Neste trabalho, padronizamos o modelo de injeção de fezes intraperitoneal em camundongos Swiss para facilitar o estudo da EAS. No modelo utilizado podemos caracterizar o choque com queda da pressão arterial 6 e 24h após a indução da sepse, disfunção metabólica caracterizada pelo aumento de lactato, disfunção hepática e renal com aumento de uréia, creatinina, TGO e TGP em 24h. a resposta inflamatória foi caracterizada pelo aumento de citocinas plasmática e no líquido peritoneal. IL-6 e MIP-1alfa aumentaram em 6h e24h tanto no plasma quanto no líquido peritoneal. IL-1beta, citocina de liberação precoce, teve aumento no plasma nas primeiras 6h. Leucócitos totais, mononucleares e neutrófilos mais evidente em 24h. Dano cognitivo foi verificado em animais sobreviventes após 10 dias do insulto da sepse. Observamos aumento no consumo de oxigênio pelo córtex cerebral em 6h e seguido de queda em 24h. Houve aumento na...

Diagnosis of regional cerebral blood flow abnormalities using SPECT: agreement between individualized statistical parametric maps and visual inspection by nuclear medicine physicians with different levels of expertise in nuclear neurology

INTRODUCTION: Visual analysis is widely used to interpret regional cerebral blood flow (rCBF) SPECT images in clinical practice despite its limitations. Automated methods are employed to investigate between-group rCBF differences in research studies but have rarely been explored in individual analyses. OBJECTIVES: To compare visual inspection by nuclear physicians with the automated statistical parametric mapping program using a SPECT dataset of patients with neurological disorders and normal control images. METHODS: Using statistical parametric mapping, 14 SPECT images from patients with various neurological disorders were compared individually with a databank of 32 normal images using a statistical threshold of p<0.05 (corrected for multiple comparisons at the level of individual voxels or clusters). Statistical parametric mapping results were compared with visual analyses by a nuclear physician highly experienced in neurology (A) as well as a nuclear physician with a general background of experience (B) who independently classified images as normal or altered, and determined the location of changes and the severity. RESULTS: Of the 32 images of the normal databank, 4 generated maps showing rCBF abnormalities (p<0.05, corrected). Among the 14 images from patients with neurological disorders, 13 showed rCBF alterations. Statistical parametric mapping and physician A completely agreed on 84.37 percent and 64.28 percent of cases from the normal databank and neurological disorders, respectively. The agreement between statistical parametric mapping and ratings of physician B were lower (71.18 percent and 35.71 percent, respectively). CONCLUSION: Statistical parametric mapping replicated the findings described by the more experienced nuclear physician. This finding suggests that automated methods for individually analyzing rCBF SPECT images may be a valuable resource to complement visual inspection in clinical practice.

Planificación quirúrgica avanzada en neurocirugía: metodología de procesamiento de imágenes integradas / Advanced surgical planning in neurosurgery: methodology of integrated image processing

El riesgo de provocar morbilidad incapacitante y permanente en el paciente neuroquirúrgico ha sido el motor del desarrollo de la imagenología actual. Además de la adquisición de imágenes anatómicas muy detalladas, hoy se puede tener información funcional para cada paciente individual y utilizarla para la planificación del abordaje quirúrgico. La integración de esta información y su utilización racional en un sistema de despliegue tridimensional es el objetivo final de todo el estudio preoperatorio del paciente. La capacidad que tenga el neurocirujano de evaluar esta información antes y durante el acto quirúrgico son determinantes en el resultado. Se analizan la integración de imágenes derivadas de la TAC RM funcional, anatómica y tractografía en la evaluación preoperatoria. A pesar de que la tecnología está ampliamente disponible y al alcance, el conocimiento sobre su empleo correcto para el manejo rutinario no siempre está presente. Actualmente la mayoría de los clínicos deben revisar interminables secuencias de cortes para efectuar una reconstrucción mental del problema del paciente, y con esta información enfrentar el caso. El desafío educacional actual es incorporar el manejo de esta tecnología en la práctica clínica rutinaria, para construir una memoria organizacional que sea capaz de mantener el ritmo de avance que cada vez parece más acelerado.

The risk of incapacitating and permanent morbidity in the neurosurgical patient has driven modern times medical imaging. Besides rich anatomical images, today we can have functional information about the individual patient, to be used in pre-operative planning. The integration of this information for its rational use in a three dimensional planning system is the final goal of all the preoperative study of the patient. The capacity of the neurosurgeon to evaluate and use this information during the actual surgical procedure is determinant in the results. The integration of images from computed tomography, magnetic resonance, anatomic and functional, and lately tractography are analyzed. Although the technology is widely available, knowledge about its use is lacking in the clinical field. Many neurosurgeons still must review hundreds of slices to make a mental composition of the pathology and the anatomy and with this, plan the operation. The challenge is to incorporate this technology in routine clinical practice, to build enough organizational memory to keep pace with the fast rhythm imposed by progress in neuroimaging.

The role of spontaneous general movement assessment in the neurological outcome of cerebral lesions in preterm infants

OBJETIVO: Estudar a relação entre a qualidade, tipos e trajetória dos movimentos generalizados espontâneos em recém-nascidos pré-termo com os achados da ultra-sonografia de crânio neonatal e com a evolução neurológica. MÉTODO: 40 recém-nascidos pré-termo, com idade gestacional < 35 semanas, tiveram seus movimentos generalizados registrados através de videoteipe durante os períodos pré-termo, a termo (37ª - 42ª semana de idade pós-concepcional) e pós-termo (49ª - 56ª semana de idade pós-concepcional), e foram seguidos prospectivamente até um ano de idade pós-concepcional. RESULTADOS: Nossos resultados mostraram que a qualidade dos movimentos generalizados, particularmente no período pós-termo (p = 0,009), foram relacionados com a presença de lesões cerebrais detectadas à ultra-sonografia e com a evolução neurológica. Enquanto a presença de lesões ultra-sonográficas graves foi associada à evolução neurológica desfavorável (p = 0,01), o achado de padrões de movimentos generalizados espontâneos normais foi associado à normalidade neurológica no seguimento, com valores preditivos negativos de 100 por cento, para o período pré-termo, e 80 por cento para ambos os períodos a termo e pós-termo. CONCLUSÕES: Quando usados conjuntamente, esses métodos podem aumentar a especificidade e a sensibilidade na detecção de um grupo de recém-nascidos pré-termo de alto risco para seqüelas neurológicas, no seguimento a longo prazo.