Urgencias hematooncológicas: desde la perspectiva del intensivista / Hematology/oncology urgencies: from the perspective of the intensivist

Entre los años 2000 y 2016 en Argentina, se reportaron al Registro Oncopediátrico Hospitalario Argentino (ROHA) 22.450 casos de cáncer en niños menores de 15 años de edad. Las Leucemias constituyen la enfermedad oncológica más frecuente, seguida de los Tumores de Sistema Nervioso Central y los Linfomas. Esta distribución es similar a la descripta en los países desarrollados de Europa y Norteamérica. Su tasa de curación a nivel mundial, llega al 80% debido al uso de quimioterapia intensiva, situación que mejora la supervivencia pero que también aumenta la frecuencia de complicaciones. Estas complicaciones pueden ser debidas tanto al propio cáncer como al tratamiento y en ocasiones ser la primera manifestación de la enfermedad oncológica. Los eventos que amenazan la vida en pacientes inmunocomprometidos son mayores que en la población general, y cuando ocurren tienen una mortalidad elevada. El reconocimiento temprano es clave para el resultado en términos de sobrevida y disminución de la mortalidad. Las acciones deberán centrarse al reconocimiento temprano de eventos críticos en pacientes oncológicos. Los pacientes Hemato-Oncológicos constituyen un gran número de ingresos no planificados a las unidades de cuidados intensivos. Uno de cada 4 pacientes requerirá durante su evolución ingreso a Unidades de Cuidados Intensivos. El propósito de este artículo es describir tres de las urgencias oncológicas que requieren con mayor frecuencia admisión en UCI: la presentación y manejo del shock séptico, Shock Cardiogénico y las complicaciones neurológicas en los pacientes con leucemias agudas (AU)

Between 2000 and 2016, 22,450 cases of cancer in children younger than 15 years of age were reported to the Argentine Hospital Registry of Childhood Cancer (ROHA). Leukemia was the most common cancer reported, followed by central nervous system tumors and lymphoma. This distribution is similar to that described in the developed countries of Europe and North America. The worldwide cure rate is up to 80% due to the use of intensive chemotherapy, which improves survival but also increases the complication rate. These complications may be due both to the cancer itself and to the treatment and are sometimes the first manifestation of the disease. Life-threatening events are more common in immunocompromised patients than in the general population, and when they occur, the mortality rate is high. Early recognition is essential for the outcome in terms of survival and decreased mortality. Interventions should focus on early recognition of critical events in cancer patients. Patients with hematology-oncology diseases account for a large number of unplanned admissions to intensive care units (ICU), while one in four of these patients will require admission to the ICU in the course of their disease. The aim of this study was to describe three oncology emergencies that most frequently require ICU admission: septic shock and its management, cardiogenic shock, and neurological complications in patients with acute leukemia (AU)

Current evidence of neurological features, diagnosis, and neuropathogenesis associated with COVID-19

Abstract Recent reports indicate that besides respiratory and systemic symptoms among coronavirus disease (COVID-19) patients, the disease has a wide spectrum of neurological manifestations (encephalitis, meningitis, myelitis, acute disseminated encephalomyelitis, metabolic and acute hemorrhagic necrotizing encephalopathy, cerebrovascular diseases, Guillain-Barré syndrome, polyneuritis cranialis, dysautonomia, and myopathies). The severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) can spread from the respiratory system to the central nervous system, using transneuronal and hematogenous mechanisms. Although not every COVID-19 patient will test positive for the virus in the cerebrospinal fluid exam, the appearance of neurological symptoms associated with SARS-CoV-2 infection reveals the importance of understanding the neurologic manifestations and capacity for neural invasion associated with the pathogen. These aspects are relevant for correct diagnosis and treatment, and for the potential development of vaccines. This review highlights the latest evidence of SARS-CoV-2 infection with a focus on neurological involvement and potential neuropathogenesis mechanisms.

Síndrome linfoproliferativo postrasplante con compromiso cerebral: reporte de un caso / Post-transplantation lymphoproliferative disorder with brain involvement: a case report

Abstract. Post-transplantation lymphoproliferative disorder is a low incidence complication of transplant recipient patients. However, mortality is high if the diagnosis and management are not appropriate. For this reason the radiologist should be aware when dealing with images of these patients, particularly in the first year following the transplantation. In this article the case is presented of a woman who was recipient of a kidney, and developed post-transplantation lymphoproliferative disorder, affecting the central nervous system.

Resumen. El síndrome linfoproliferativo postrasplante es una complicación que se presenta con una baja incidencia en los pacientes que han sido trasplantados. Sin embargo, si el diagnóstico y manejo no son oportunos su mortalidad es alta. Por esta razón el radiólogo debe estar atento al diagnóstico al interpretar estudios de este tipo de pacientes, especialmente en el año siguiente al trasplante. Presentamos el caso de una paciente con antecedente de trasplante renal y síndrome linfoproliferativo postrasplante con afección del sistema nervioso central.

Neurologic Manifestations of Enterovirus 71 Infection in Korea

Enterovirus 71 frequently involves the central nervous system and may present with a variety of neurologic manifestations. Here, we aimed to describe the clinical features, magnetic resonance imaging (MRI) findings, and cerebrospinal fluid (CSF) profiles of patients presenting with neurologic complications of enterovirus 71 infection. We retrospectively reviewed the records of 31 pediatric patients hospitalized with acute neurologic manifestations accompanied by confirmed enterovirus 71 infection at Ulsan University Hospital between 2010 and 2014. The patients' mean age was 2.9 ± 5.5 years (range, 18 days to 12 years), and 80.6% of patients were less than 4 years old. Based on their clinical features, the patients were classified into 4 clinical groups: brainstem encephalitis (n = 21), meningitis (n = 7), encephalitis (n = 2), and acute flaccid paralysis (n = 1). The common neurologic symptoms included myoclonus (58.1%), lethargy (54.8%), irritability (54.8%), vomiting (48.4%), ataxia (38.7%), and tremor (35.5%). Twenty-five patients underwent an MRI scan; of these, 14 (56.0%) revealed the characteristic increased T2 signal intensity in the posterior region of the brainstem and bilateral cerebellar dentate nuclei. Twenty-six of 30 patients (86.7%) showed CSF pleocytosis. Thirty patients (96.8%) recovered completely without any neurologic deficits; one patient (3.2%) died due to pulmonary hemorrhage and shock. In the present study, brainstem encephalitis was the most common neurologic manifestation of enterovirus 71 infection. The characteristic clinical symptoms such as myoclonus, ataxia, and tremor in conjunction with CSF pleocytosis and brainstem lesions on MR images are pathognomonic for diagnosis of neurologic involvement by enterovirus 71 infection.

Are Preexisting Retinal and Central Nervous System-Related Comorbidities Risk Factors for Complications Following Robotic-Assisted Laparoscopic Prostatectomy?

ABSTRACTPurpose:To assess whether retinal and central nervous system (CNS) comorbidities are risk factors for complications following robotic assisted laparoscopic prostatectomy (RALP).Materials and Methods:A retrospective review of our RALP database identified 1868 patients who underwent RALP by a single surgeon between December 10, 2003-March 14, 2014. We hypothesized that patients with preexisting retinal or CNS comorbidities were at a greater risk of suffering retinal and CNS complications following RALP. Perioperative complications and risk of recurrence were graded using the Clavien and D'Amico systems, respectively.Results:40 (2.1%) patients had retinal or CNS-related comorbidities, of which 15 had a history of retinal surgery and 24 had a history of cerebrovascular accident, aneurysm and/or neurosurgery. One additional patient had a history of both retinal and CNS events.Patients with retinal or CNS comorbidities were significantly older, had elevated PSA levels and CCI (Charlson Comorbidity Index) scores than the control group. Blood loss, length of stay, surgical duration, BMI, diagnostic Gleason score and T-stage were not statistically different between groups.No retinal or CNS complications occurred in either group. The distribution of patients between D'Amico risk categories was not statistically different between the groups. There was also no difference in the incidence of total complications between the groups.Conclusions:RALP-associated retinal and CNS complications are rare. While our RALP database is large, the cohort of patients with retinal or CNS-related comorbidities was relatively small. Our dataset suggests retinal and CNS pathology presents no greater risk of suffering from perioperative complications following RALP.

Neural complications and physical disabilities in leprosy in a capital of northeastern Brazil with high endemicity / Complicações neurais e incapacidades em hanseníase em capital do nordeste brasileiro com alta endemicidade

INTRODUCTION: Leprosy is an infectious disease whose etiologic agent is Mycobacterium leprae, manifested by dermatological and neurological signs and symptoms. OBJECTIVE: To investigate neural changes and the degree of physical disability in the eyes, hands and feet before and after treatment, as well as sociodemographic and clinical profile of patients affected by leprosy. METHOD: A longitudinal epidemiological study comprising 155 patients with leprosy, from a spontaneous demand, diagnosed between March 2010 and February 2011, and treated with multidrug therapy (MDT) between March 2010 and July 2012 in a program for leprosy eradication in São Luis (MA), Brazil. RESULTS: Before treatment, 46.5% of patients were considered as borderline, 51.6% had some alteration in the eyes and 52.3% in the feet, and the radial nerve (18.7%) was the most affected. There was a statistically significant difference between the changes in the radial nerve at the beginning of and after treatment. CONCLUSIONS: The analysis points to late diagnosis, as some patients have had abnormal neural and physical disabilities before treatment. .

INTRODUÇÃO: A hanseníase é uma doença infectocontagiosa cujo agente etiológico é o Mycobacterium leprae, que se manifesta por sinais e sintomas dermatoneurológicos. OBJETIVO: investigar as complicações neurais e o grau de incapacidades físicas nos olhos, mãos e pés antes e após o tratamento, bem como o perfil sociodemográfico e clínico dos pacientes acometidos pela hanseníase. MÉTODO: Estudo epidemiológico do tipo longitudinal constituído por 155 pacientes com hanseníase, a partir da demanda espontânea, diagnosticados no período de março de 2010 a fevereiro de 2011 e tratados com poliquimioterapia (PQT) entre março de 2010 a julho de 2012, em um programa de eliminação da hanseníase, no município de São Luís (MA). RESULTADOS: Antes do tratamento, 46,5% dos pacientes apresentaram forma dimorfa, 51,6% possuíam alguma alteração nos olhos e 52,3% nos pés, sendo o nervo radial (18,7%) o mais acometido. Houve diferença estatisticamente significante entre as complicações do nervo radial no inicio e após o tratamento. CONCLUSÕES: Evidenciou-se a presença do diagnóstico tardio, posto que alguns pacientes já apresentavam complicações neurais e incapacidades físicas antes do tratamento. .

Estudo prospectivo dos fatores de risco para complicações neurológicas na meningite bacteriana infantil / A prospective study of risk factors for neurological complications in childhood bacterial meningitis

OBJETIVO: Análise prospectiva de fatores de prognóstico para complicações neurológicas da meningite bacteriana infantil. MÉTODOS: Este estudo prospectivo recrutou 77 crianças de um mês a 16 anos de idade tratadas de meningite bacteriana durante o período de 1/1/2009 a 31/12/2010. Foram escolhidos 16 preditores relevantes para analisar sua associação com a incidência de complicações neurológicas. Valores P abaixo de 0,05 foram considerados estatisticamente significativos. RESULTADOS: Das 77 crianças tratadas para meningite bacteriana, desenvolveram-se complicações neurológicas em 33 pacientes (43%), e duas crianças morreram (2,6%). A etiologia dos casos de meningite bacteriana foi comprovada em 57/77 (74%) dos casos: foram encontrados 32 isolados de meningococos; 8 de pneumococos; 6 de bacilos gram-negativos; 5 de H. influenzae; 5 de estafilococos e 1 de S. viridans. Os fatores que se mostraram associados a aumento do risco de desenvolvimento de complicações neurológicas foram idade < 12 meses, alteração do estado mental, crises convulsivas antes da admissão, terapia inicial com dois antibióticos, uso de dexametasona, presença de déficit neurológico focal na admissão e aumento das proteínas do líquido cerebrospinal (LCS) (p < 0,05). Pleiocitose inicial > 5.000 células/mm³, pleiocitose > 5.000 células/mm³ depois de 48 horas, baixa relação da glicose no LCS/sangue < 0,20, gênero feminino, tratamento prévio com antibióticos, infecção adquirida na comunidade, duração da doença > 48 horas, presença de comorbidade e foco primário de infecção não se associaram a aumento do risco para o desenvolvimento de complicações neurológicas. CONCLUSÃO: Idade inferior a 12 meses e gravidade da apresentação clínica na admissão foram identificadas como os preditores mais fortes de complicações neurológicas e podem ter valor para selecionar pacientes para tratamento mais intensivo.

OBJECTIVE: To prospectively analyze the prognostic factors for neurological complications of childhood bacterial meningitis. METHODS: This prospective study enrolled 77 children from 1 month until 16 years of age, treated for bacterial meningitis during the period of January 1, 2009 through December 31, 2010. 16 relevant predictors were chosen to analyze their association with the incidence of neurological complications. p-values < 0.05 were considered statistically significant. RESULTS: Of the 77 children treated for bacterial meningitis, 33 patients developed neurological complications (43%), and two children died (2.6%). The etiology of bacterial meningitis cases was proven in 57/77 (74%) cases: 32 meningococci, eight pneumococci, six Gram-negative bacilli, five H. influenzae, five staphylococci, and one S. viridans isolates were found. Factors found to be associated with increased risk of development of neurological complications were age < 12 months, altered mental status, seizures prior to admission, initial therapy with two antibiotics, dexamethasone use, presence of focal neurological deficit on admission and increased proteins in cerebrospinal fluid (CSF) (p < 0.05). Initial pleocytosis > 5,000 cells/mm³, pleocytosis > 5,000 cells/mm³ after 48 hours, CSF/blood glucose ratio < 0.20, female gender, previous treatment with antibiotics, community-acquired infection, duration of illness > 48 hours, presence of comorbidity, and primary focus of infection were not associated with increased risk for the development of neurological complications. CONCLUSION: Age < 12 months and severity of clinical presentation at admission were identified as the strongest predictors of neurological complications and may be of value in selecting patients for more intensive care and treatment.

Complicaciones neurológicas en pacientes adultos sometidos a trasplante hepático ortotópico: experiencia de un centro universitario / Neurological complications following a liver transplantation: experience from a Chilean center

Background: Orthotopic liver transplantation (OLT) is the treatment of choice for multiple acute and chronic end-stage liver diseases as well as for selected cases of liver malignancy and ¡iver-site metabolic disorders. Neurological impairment is a major source of morbidity and moñality following OLT. Aim: To describe the incidence and the type of neurological complications occurring in the post-operative period of OLT in patients transplanted in our hospital. Material and methods: Between March 1994 and August 2007, 76 adult patients underwent OLT. Data on incidence, time of onset, and outcome of central nervous system (CNS) complications have been obtained from our program data base and patient charts. Results: Twenty three patients (30.3 percent) had CNS complications following OLT. The leading complications were immunosuppressive drug-related neurological impairment in nine patients (39.1 percent), peripheral nerve damage in five patients (21.7 percent), central pontine myelinolysis in four patients (17.4 percent), cerebrovascular disease in three (13 percent) and CNS infection in three (13 percent). Most CNS events (90 percent) occurred in the first 2 weeks after OLT. Five patients with neurological complications died (22 percent). Conclusions: CNS complications occurred in almost one fifth of the population studied, and they had a poor outcome, as previously reported).

Emergencies in HIV medicine--part I.

Human immunodeficiency virus (HIV) infection is now a chronic manageable disease due to which is it imperative for reviewing various medical emergencies which an individual case may encounter. Emergencies may occur at any stage of the disease. HIV infection is associated with several opportunistic infections/malignancies that may be life threatening and need quick intervention by health care workers. These emergencies could be related to opportunistic infections that are seen at presentation or that occur as the immune system gets weaker, or may be HIV induced diseases like enteropathy and wasting, diarrhea leading to dehydration and its sequel, neurological complication like PML etc. and from complications resulting from use of anti-HIV medication like lactic acidosis, pancreatitis, bone marrow suppression and may include the immune reconstitution syndromes.

Neurological complications of hematopoietic stem cell transplantation (HSCT): a retrospective study in a HSCT center in Brazil / Complicações neurológicas do transplante de células tronco hematopoiéticas (TCTH): estudo retrospectivo em um centro de TCTH no Brasil

We present the neurological complications evaluated in a series of 1000 patients who underwent hematopoietic stem cell transplantation (HSCT). Central nervous system (CNS) neurological complications, particularly brain hemorrhages, were the most common, followed by seizures and CNS infections. An unusual neurological complication was Wernicke's encephalopathy. Less frequent neurological complications were metabolic encephalopathy, neuroleptic malignant syndrome, reversible posterior leukoencephalopathy syndrome, brain infarct and movement disorders. The most common neurological complication of the peripheral nervous system was herpes zoster radiculopathy, while peripheral neuropathies, inflammatory myopathy and myotonia were very rarely found.

Apresentamos as complicações neurológicas avaliadas em uma série de 1000 pacientes submetidos ao transplante de células tronco hematopoiéticas (TCTH). As complicações neurológicas do sistema nervoso central foram as mais encontradas, particularmente as hemorragias encefálicas, seguidas por crises convulsivas e por infecções. Uma complicação peculiar foi a encefalopatia de Wernicke. Menos freqüentemente foram encontrados casos de encefalopatia metabólica, síndrome maligna neuroléptica, leucoencefalopatia posterior reversível, infarto cerebral e os distúrbios do movimento. Entre as complicações neurológicas do sistema nervoso periférico a mais encontrada foi a radiculopatia pelo herpes zoster, enquanto que raramente se observaram casos de polineuropatias periféricas, miopatia inflamatória e de miotonia.

Enfrentamiento imaginológico de las lesiones cerebrales en pacientes VIH / Imaging approach to brain lesions in HIV patients

Human immunodeficiency virus (HIV) is a neurotropic virus that crosses the blood-brain barrier at early stages of the disease. Thus, the central nervous system (CNS) constitutes the major target of HIV, and according to estimations approximately two-thirds of patients will develop CNS involvement during the course of their disease. CNS diseases spectrum is wide and can be categorized into primary effects of HIV, opportunistic infections, neoplasms and vascular disease. A prompt diagnosis and treatment of such disorders is critical. Imaging studies play a crucial role in the diagnostic process. Therefore, characteristics of neuro-images in patients with HIV and their neurological manifestations have been reviewed.

El virus de inmunodeficiencia humana (VIH) es un virus neurotrópico que cruza la barrera hemato-encefálica en etapas precoces de la enfermedad. Es así como el sistema nervioso central (SNC) es blanco mayor del virus VIH, estimándose que aproximadamente dos tercios de los pacientes desarrollarán compromiso del SNC durante el curso de su enfermedad. El espectro de enfermedades del SNC es amplio y puede ser categorizado en: compromiso directo del virus VIH, infecciones oportunistas, neoplasias y enfermedad vascular. Un pronto diagnóstico y tratamiento de tales desordenes es crítico. Los estudios de imágenes juegan un rol crucial en el diagnóstico. Hemos revisado las características de las neuroimágenes en los pacientes VIH y sus manifestaciones neurológicas.

Traumatismo raquimedular / Spinal cord injury

El Traumatismo Raquimedular (TRM) incluye todas las lesiones traumáticas que afectan las diferentes estructuras osteoligamentosas, cartilaginosas, musculares, vasculares, meníngeas, radiculares y medulares de la columna vertebral a cualquiera de sus niveles. Habitualmente afecta a personas jóvenes en etapa productiva. Representa un problema económico para los sistemas de salud dado el alto costo que implica su tratamiento en la etapa aguda y posterior rehabilitación. La medula espinal es el conducto a través del cual viaja la información entre el cerebro y el cuerpo; está diseñada como eje transmisor, regulador y modulador de múltiples funciones neurológicas. La lesión medular aguda es un proceso dinámico, evolutivo y multifásico a partir del momento en que se produce el traumatismo; par ello el objetivo sobre el manejo del daño espinal esta orientado a reducir el déficit neurológico resultante y prevenir alguna pérdida adicional de la función neurológica. El nivel mas frecuente de lesión es la región cervical media baja, el siguiente lugar es la unión toracolumbar; coincidiendo estos niveles con las áreas de mayor movilidad de la columna espinal. En un paciente con sospecha de daño espinal, la columna debe ser inmovilizada para prevenir el daño neurológico y la anatomía de la lesión debe ser analizada como primer paso al memento de proveer un tratamiento efectivo.

Therapeutic Experience of Bing-Neel Syndrome Associated with Waldenstrom's Macroglobulinemia

Waldenstrom's macroglobulinemia is an uncommon low-grade B-cell lymphoproliferative disorder in which monoclonal immunoglobulin M is produced. Neurological symptoms due to hyperviscosity are frequent manifestations of Waldenstrom's macroglobulinemia. However, central nervous system infiltration by plasmacytoid lymphocytes (Bing-Neel syndrome) has only rarely been reported. We report a case of a 51-yr-old woman suffering from Waldenstrom's macroglobulinemia who complained of persistant headache. Brain magnetic resonance imaging revealed an extra-axial soft tissue mass along the left cavernous sinus, left tentorium, right tentorium, and falx cerebri. A stereotactic biopsy of dural tissue from the falx was performed and showed plasmacytoid lymphocyte infiltration. The patient became symptom- free with irradiation of the whole brain followed by chemotherapy with fludarabine.

Síndromes neuropsiquiátricas pós-estreptocócicas / Post-streptococcal neuropsychiatric syndromes

Nesta revisão narrativa, o nosso objetivo foi descrever as síndromes neuropsiquiátricas pós estreptocócicas e discuti-las à luz das evidências científicas atuais sobre os possíveis mecanismos patogenéticos envolvidos. Nos últimos anos, uma série de distúrbios do movimento, como tiques, distonia, parkinsonismo, e transtornos psiquiátricos, como o transtorno obsessivo-compulsivo (TOC) e o transtorno da hiperatividade com déficit de atenção (THDA), vem sendo considerada parte do espectro das manifestações pós-estreptocócicas. O termo PANDAS (acrônimo do inglês: pediatric autoimmune neuropsychiatric disorder associated with streptococcus) foi inclusive cunhado para descrever um subgrupo de pacientes com TOC e tiques que inibe flutuação clínica dos sintomas associada a infecção estreptocócica. Entretanto a análise crítica das evidências clinicolaboratoriais não apóia esse espectro ampliado das manifestações pós-estreptocócicas. Apenas na coréia de Sydenham há evidências consistentes de patogênese mediada por processo auto-imune pós-estreptocócico.

In this narrative review, our objective was to describe the post-streptococcal neuropsychiatric syndromes and to discuss the possible pathogenetic mechanisms involved in their clinical expression. Recently, several movement disorders, such as tics, dystonia, and parkinsonism, and psychiatric disorders like obsessive-compulsive disorders (OCD) and attention deficit hyperativity disorder (ADHD), are being considered part of a putative spectrum of post-streptococcal infection disorder. The term PANDAS (pediatric autoimmune neuropsychiatric disorder associated with streptococcus) has been introduced to describe a subset of patients with these diagnoses in which onset of symptoms or symptom exacerbations are related to streptococcal infection. However the critical analysis of clinical and laboratory data does not support the hypothesis of an extended spectrum of post-streptococcal neuropsychiatric disorders. Only for Sydenham chorea there is consistent evidence for a post-streptococcal autoimmune mediated pathogenesis.

Enfermedad de Sturge-Weber-Dimitri: del angioma leptomeníngeo a la hemiatrofia cerebral: aporte diagnóstico de la RM cerebral con gadolinio en 7 casos / Disease Sturge-Weber-Dimitri: leptomeningeal angioma of the cerebral hemiatrophy: diagnostic contribution of MRI with gadolinium in 7 cases

Propósito: Demostrar el interés diagnóstico de la RM cerebral con gadolinio en 7 casos con enfermedad de Sturge Weber Dimitri. Material y Métodos: Se estudiaron 7 pacientes, 5 de sexo masculino y 2 de sexo femenino, cuyas edades oscilaron entre 3 meses y 24 años. En 6 casos se observó angioma hemifacial "rojo vinoso" y la RM cerebral fue indicada por la aparición de una o más crisis convulsivas. Sólo en uno de los casos de ésta serie no existía angioma hemifacial ni corporal y la RM cerebral se realizó en un contexto clínico de cefaleas refractarias con nauseas y vómitos. Resultados: En los 7 casos las secuencias SET1 con gadolinio demostraron refuerzo caracteristico de un angioma leptomeningeo de la convesidad temporoparietal ubicado del lado derecho en 6 casos e izquierdo en uno; asociando incremento en el refuerzo de uno o ambos plexos coroideos como signo de angiomatosis. También en los 7 casos hubo asimetría en el tamaño de los hemisferios cerebrales por menor desarrollo en los lactantes, y por hemiatrofia progresiva en niños y adultos jóvenes. Los dos lactantes presentaron además señal anormal en la sustancia blanca del hemisferio cerebral afectado como signo de mielinogénesis acelerada. Las típicas calcificaciones corticales aparecen tardíamente y se observaron en un caso. Conclusión: En un contexto de crisis convulsivas de etiología no aclarada, la RM cerebral con gadolinio puede demostrar alteraciones morfológicas características de la enfermedad de Sturge Weber Dimitri.

La Barrera Hemato-Encefálica en la patología del Sistema Nervioso Central: su importancia en la Respuesta Inflamatoria / The Blood-Brain Barrier and the Pathology of the Central Nervous System: Its importance for the Inflammatory Response

Introducción. La Barrera Hemato-Encefálica (BHE) constituye una estructura histológica bien definida que participa tanto en la homeostasis normal del Sistema Nervioso Central (SNC) como en varios procesos patológicos que lo afectan. Métodos. Se evaluaron más de 50 revisiones y trabajos originales publicados en los últimos años en los que se discuten aspectos del desarrollo, morfología y función normales de la BHE, y alteraciones de su función y su participación en el desarrollo de patologías del SNC. La revisión aborda los mecanismos que pueden determinar la disfunción de la BHE y sus efectos sobre la patología del SNC. Resultados. La función de la BHE como barrera física y metabólica es la de separar al SNC de la periferia. Sin embargo, la BHE, lejos de comportarse como una estructura pasiva, juega un papel activo en la mantención de la función normal del SNC. Dependiendo de la presencia de daño o ciertas señales sistémicas, la integridad de la BHE puede ser comprometida, aumentando su permeabilidad. Conclusiones. Las características morfo-funcionales de la BHE son determinantes en la manifestación de ciertas patologías del SNC, o limitan las aproximaciones terapéuticas para abordarlas. La posibilidad de influir sobre la permeabilidad de la BHE de manera controlada y en forma espacial y temporalmente restringida potencialmente puede modificar tanto la prevención como el tratamiento de muchas enfermedades del SNC.

O sistema glutamatérgico e desordens neuropsiquiátricas: aspectos básicos / The glutamatergic system and neuropsychiatric disorders: basic aspects

O sistema glutamatérgico, um dos mais extensos do sistema nervoso central, é formado por neurônios, vias e receptores específicos. O agonista natural corresponde a um aminoácido excitador de constituição relativamente simples, o glutamato. Este atua em uma ampla gama de subtipos de receptores, ionotrópicos e metabotrópicos, que oferecem a possibilidade de uma variada atividade sináptica, desde a neurotransmissão rápida até às respostas lentas que induzem modificações sinápticas de longa duração. Essa ampla distribuição dos receptores influencia neurônios que transmitem com o mesmo ou com outros neurotransmissores. Os receptores ionotrópicos, os mais bem estudados, apresentam funções diferenciadas, sendo que o do tipo AMPA é responsável pela maior parte das transmissões sinápticas excitadoras rápidas, a do tipo KA contribui nas respostas pós-sinápticas nas sinapses excitadoras, podendo ainda modular a liberação pré-sináptica do transmissor em determinadas sinapses, enquanto o receptor NMDA é fundamental na indução de formas específicas de plasticidade sináptica. Os variados receptores metabotrópicos também contribuem de modo importante em diversas dessas atividades. Dessa maneira, o sistema glutamatérgico encontra-se relacionado a diversas funções normais, como a plasticidade neural, subjacente a processos cognitivos, assim como a situações patológicas, responsáveis por diversas desordens neuropsiquiátricas agudas e crônicas. O conhecimento detalhado do sistema glutamatérgico, portanto, é essencial para a compreensão de variados processos normais e patológicos, assim como das possibilidades de intervenção farmacológica com objetivos terapêuticas

Fístula subaracnoidea-pleural traumßtica por herida de bala no penetrante en tórax / Traumatic subarachnoid-pleural fistula secondary to non-penetrating thoracic gunshot wound

INTRODUCTION: Traumatic subarachnoid-pleural fistula (TSPF) is very unusual and is due to the anomalous communication between the pleural and subarachnoid space. We report a TSPF by Bullet wound that was not penetrating to the thoracic cavity. CLINICAL CASE: Masculine of 34 years-old that receives wounded by bullet in the posterior face of thorax. A pleural effusion was identified and medullar wound with fracture of the fifth thoracic vertebra. The effusion is persist and also added headache appear. TSPF was diagnosed for myelography. The patient die before carrying out the surgical treatment. A massive tromboemboly of lug was the cause. The autopsy confirmed the diagnosis. DISCUSSION: The TSPF should be suspected by the association of medullar lesion with a persistent pleural effusion. The diagnosis should be confirmed by radiology. The treatment can be medical or surgical.

Hypertrophic pachymeningitis: varied manifestations of a single disease entity.

Hypertrophic pachymeningitis is a unique clinical entity characterised by fibrosis and thickening of the duramater with resulting neurological dysfunction. Three cases of this entity are described. Presenting features were headaches and cranial neuropathies in two patients and predominantly cerebellar dysfunction in the third. One of the patients also had evidence of spinal involvement. Lower cranial nerves were chiefly involved in two patients whereas optic nerve was the predominantly affected nerve in one. Except for the presence of rheumatoid arthritis in one of the patients, we could not document clinical or biochemical evidence of any predisposing infective, inflammatory or infiltrative condition in the other two. All three patients had characteristic changes on imaging suggestive of thickened and enhancing duramater. Although variable steroid responsiveness was seen in all the three patients, tendency towards steroid dependence was evident. The clinical presentations, causes, radiological features, management options and differential diagnosis of this unique clinical syndrome have been discussed.