Encefalitis inmunomediada en un niño con antecedente de infección por SARS-CoV-2 n / Immune-mediated encephalitis in a child with a history of SARS-CoV-2 infectio

Las encefalitis son cuadros clínicos frecuentes en la edad pediátrica. Pueden dividirse en aquellas causadas por la infección del sistema nervioso central y en las de etiología inmunomediada (algunas de las cuales pueden ser para- o posinfecciosas). En marzo de 2020 la Organización Mundial de la Salud declaró la pandemia por el coronavirus de tipo 2 del síndrome respiratorio agudo grave (SARS-CoV-2, por su sigla en inglés). Los reportes pediátricos de enfermedad por dicho agente describen una amplia gama de manifestaciones clínicas: compromiso respiratorio, gastrointestinal, síntomas neurológicos, entre otros; y el síndrome inflamatorio multisistémico asociado a COVID-19 (SIM-C). Describimos el caso de un niño de 2 años con diagnóstico de encefalitis por anticuerpos antirreceptor N-metil-d-aspartato (anti-NMDAR), en quien se comprobó, mediante serología, una infección reciente por SARS-CoV-2. La presencia de marcadores serológicos positivos para SARS-CoV-2 en un paciente que presentó encefalitis por anticuerpos anti-NMDAR podría interpretarse como una asociación temporal, estableciéndose la posibilidad de que el virus haya actuado como gatillo de una enfermedad autoinmunitaria.

Encephalitis are frequent clinical pictures in pediatric age. They can be divided into those caused by infection of the central nervous system and those of immune-mediated etiology (some of which may be para- or post-infectious). In March 2020, the WHO declared a SARS-CoV-2 pandemic. Pediatric reports of disease caused by this agent describe a wide range of clinical manifestations: respiratory and gastrointestinal compromise, neurological symptoms, among others; and a multisystemic inflammatory syndrome in children associated with COVID-19 (MIS-C).We describe the case of a 2-year-old boy with a diagnosis of anti-NMDAR antibody encephalitis, in whom a recent SARSCoV-2 infection was serologically proven. The presence of positive serological markers for SARS-CoV-2 in a patient who presented encephalitis due to anti-NMDAR antibodies could be interpreted as a temporal association; establishing the possibility that the virus has acted as a trigger for an autoimmune disease

¿Encefalopatía o encefalitis por varicela-zóster? Una paciente con una punción lumbar engañosa / ENCEPHALOPATHY OR VARICELLA-ZOSTER ENCEPHALITIS? A PATIENT WITH A TRICKY LUMBAR PUNCTURE

La encefalopatía es un cuadro clínico característico de múltiples procesos neurológicos y sistémicos que no hay que confundir con la encefalitis, que es una inflamación cerebral, normalmente causadas por infecciones virales. Se presenta el caso de una mujer de 58 años con enfermedad renal crónica en diálisis peritoneal, que ingresa por sepsis de origen peritoneal con clínica de encefalopatía y crisis epilépticas parciales. La paciente presenta lesiones de herpes zóster en zona lumbar y se practica punción lumbar, con resultado del líquido cefalorraquídeo positivo para virus varicela-zóster, por lo que completa tratamiento con aciclovir. En la resonancia magnética no presenta ninguna alteración, y una segunda punción lumbar tras mejoría de las lesiones cutáneas es negativa. El curso de la paciente es fluctuante durante el ingreso, con mejoría significativa tras antibióticos, hemodiálisis y tratamiento antiepiléptico, y no respondiendo al aciclovir. La etiología sospechada es la debida al contexto infeccioso y metabólico de la paciente. Probablemente el resultado del líquido fue contaminado por la proximidad de las lesiones herpéticas, ya que además no es frecuente encontrar encefalitis infecciosas agudas sin alteraciones en las pruebas de imagen. La mejoría final fue debida tanto a la medicación antiepiléptica como al inicio de hemodiálisis

Encefalopathy is a clinical syndrome ocurring in multiple neurologic and systemic diseases which must not be mistaken with encephalitis, that is a cerebral inflammatory process, often caused by viral infections. We present the case of a 58-year-old woman with chronic renal failure receiving peritoneal dyalisis, who was admitted into hospital for sepsis secondary to infectious peritonitis, with encefalopathy and epileptic partial seizures. The patient presented lumbar herpetic cutaneous lesions and a lumbar punction is practiced, with a positive result in the cerebrospinal fluid for varicella-zoster virus. Treatment with aciclovir was completed. Her cerebral magnetic resonance was absolutely normal, and a second lumbar puncture when herpetic lesions got better was negative. The course is fluctuating during the stay, and a significant clinical improvement occurs after antibiotics, hemodyalisis and antiepileptic treatment. The patient did not respond to aciclovir. The suspected ethiology is the infectious and metabolic context. Positivity for the virus is thought to be a contamination from the nearby herpetic lesions. Also, it is rare for an infectious acute encephalitis to present with normal radiologic imaging. The final clinical improvement was probably due to hemodyalisis initiation and the antiepileptic treatment.

Encefalitis asociada a COVID-19 en una niña de 13 años: reporte de caso / Encephalitis associated with COVID-19 in a 13-year-old girl: A case report

Existen limitados reportes de síntomas neurológicos en la población pediátrica con COVID-19. Reportamos el caso de una niña de 13 años con tres días de enfermedad caracterizada por cefalea, vómitos no explosivos, fiebre y trastorno del sensorio de inicio brusco asociado a dificultad para la bipedestación y hemiparesia en extremidades, sin evidencia de signos meníngeos. La tomografía cerebral reveló edema cerebral difuso y el estudio de líquido cefalorraquídeo era concordante con una infección viral. El diagnóstico de COVID-19 fue mediante pruebas serológicas. La paciente tuvo una evolución clínica desfavorable a pesar del tratamiento con hidroxicloroquina, azitromicina y corticoides, falleciendo al tercer día de hospitalización. La encefalitis en un paciente con COVID-19 es una asociación poco descrita en la población pediátrica. Esta infección debe ser considerado como diagnóstico diferencial en los pacientes que llegan a la emergencia con trastorno de sensorio o sintomatología neurológica en el contexto de la actual pandemia.

There are limited reports of neurological symptoms in the pediatric population with COVID-19. We report a 13-year-old girl with three days of illness characterized by headache, non-explosive vomiting, fever, and sudden-onset sensory disorder associated with difficulty in standing and hemiparesis in limbs without evidence of meningeal signs. Brain tomography revealed diffuse brain edema, and the cerebrospinal fluid study was consistent with a viral infection. COVID-19 was diagnosed based on serology. The patient had an untoward clinical course despite treatment with hydroxychloroquine, azithromycin, and corticosteroids, dying on the third day of hospitalization. Encephalitis in a patient with COVID-19 is not frequently reported in the pediatric population. It should be considered in the differential diagnosis in patients who arrives at the emergency with a sensory disorder or neurological symptomatology in the context of the COVID-19 pandemic.

Neumonía y miositis aguda benigna de la infancia por Mycoplasma pneumoniae asociado a encefalitis: reporte de caso / Pneumonia and benign acute myositis of childhood due to Mycoplasma pneu-moniae associated with encephalitis: a case report

Una de las principales causas de neumonía en niños entre 5 y 15 años es el Mycoplasma pneumoniae, una bacteria que causa manifestaciones clínicas atípicas como la miositis y encefalitis. Reportamos un caso de una niña de cinco años que presentó limitación funcional en extremidades inferiores precedida por una infección respiratoria superior. Posteriormente, se complicó con neumonía y encefalitis. Se administraron antibióticos y antivirales debido al deterioro clínico del paciente. La serología de inmunoglobulinas para Mycoplasma pneumoniae fue positiva; mientras que los demás estudios virales fueron negativos. El curso clínico fue favorable con disminución progresiva de la dificultad respiratoria, trastorno del sensorio y mejoría en la limitación funcional en las extremidades inferiores a los 15 días de tratamiento.

One of the leading causes of pneumonia in children between 5 to 15 years is Mycoplasma pneumoniae, a bacterium that causes atypical clinical manifestations such as myositis and encephalitis. We report a 5-year-old girl who presented functional limitations of the lower extremities preceded by an upper respiratory infection. Later on, she developed pneumonia and encephalitis. Antibiotics and antivirals were administered due to the clinical deterioration of the patient. IgM serology for Mycoplasma pneumoniae was positive, while the other viral studies were negative. The clinical course was favorable with a progressive decrease in respiratory distress, sensorial disorder, and improvement in the functional limitations of the lower limbs after 15 days of treatment.

Encefalitis autoinmunes: Receptor anti-NMDA y nuevos inmunofenotipos / Autoimmune encephalitis. Anti-NMDA receptor and new immunophenotypes

Las encefalitis autoinmunes (EA) se definen como síndromes neurológicos de instalación subaguda de compromiso de conciencia, alteración de la memoria de trabajo y trastornos psiquiátricos frecuentemente asociados a movimientos anormales y crisis epilépticas y que se producen por la acción de anticuerpos anti neuronales específicos que se fijan a receptores de neurotransmisores o proteínas de membrana. El anticuerpo anti NMDAR es el que origina la mayoría de los casos de EA en niños y adultos jóvenes, seguido por el anticuerpo anti LGI1 de presentación en el adulto. Las EA han aumentado en la última década, en la que se ha descrito un gran número de nuevos anticuerpos que producen en su mayoría síndromes neurológicos que involucran al sistema nervioso central, con predominio de signología psiquiátrica, excepto en niños en los que predominan movimientos anormales, crisis epilépticas y compromiso de conciencia. Se asocian frecuentemente a tumores en el adulto pero en los niños esta asociación es más rara. Todas las EA responden a terapia inmunomoduladora aunque en diferente medida según el tipo de anticuerpo involucrado. Generalmente la evolución a la mejoría es lenta y puede completarse en meses o incluso en un año o más. En esta revisión se destaca los principales cuadros de EA relacionados con anticuerpos específicos mencionando también los inmunofenotipos descubiertos recientemente.

Autoimmune encephalitis (AE) is defined as neurological syndromes of subacute installation of compromise of consciousness, alteration of working memory and psychiatric disorders associated with abnormal movements and epileptic seizures and that are produced by the action of anti-neuronal antibodies. They bind to neurotransmitter receptors or membrane proteins. Antibody to NMDAR is the origin of the majority of cases of AD in children and young adults, followed by anti-LGI1 antibody for presentation in adults. The AE has increased in the last decade, with a large number of new agents described that produce mostly neurological syndromes that involve the central nervous system, with predominance of psychiatric signaling, except in children and the predominant abnormal movements, epileptic seizures and compromise of conscience. They are frequently associated with tumors in adults but in children this association is more infrecuent. All AEs respond to immunomodulatory therapy although in different measures depending on the type of antibody involved. In general, the evolution to improvement is slow and can be completed in months or even in one year or more. In this review, the main EA clinical pictures related to specific antibodies are highlighted, also mentioning recently discovered immunophenotypes.

Encefalitis por virus influenza B en un paciente adulto: reporte de caso / Encephalitis caused by type B influenza virus in an adult: report of one case

Neurological manifestations associated with influenza virus infection include encephalitis, encephalopathy, acute necrotizing encephalitis, transverse myelitis, acute disseminated encephalomyelitis, mild encephalitis with reversible splenial syndrome (MERS), and Guillaín Barré syndrome. We report a 16-year-old female who was admitted at our emergency department with seizures, confusion, nystagmus and motor clumsiness five days after an upper a respiratory tract infection. Influenza type B virus infection was confirmed by chain polymerase reaction analysis. The initial electroencephalogram demonstrated a pattern of global slowness without epileptic discharges. One week later, it showed a progression to slow-wave focal bilateral discharges at both temporal and occipital lobes. The patient had a favorable evolution and was discharged 19 days after admission with phenytoin to prevent seizures.

Encefalitis autoinmunes: Un nuevo diagnóstico para una antigua enfermedad / Autoimmune encephalitis. New diagnosis for an old condition

Las encefalitis autoinmunes son un nuevo grupo de enfermedades de gran trascendencia clínica y terapéutica debido a la buena respuesta en gran parte de los casos a la terapia inmunomoduladora indicada, con un gran porcentaje de curación, sin secuelas neurológicas importantes (cognitivo, motor, crisis o movimientos involuntarios). En el año 2007 se demostró la presencia de auto anticuerpos neuronales en la patogenia de este grupo de enfermedades, con síntomas psicóticos y de movimientos involuntarios como indicadores de la enfermedad. La presente revisión enfatiza el salto crucial y el cambio de paradigmas suscitados tras el descubrimiento de estas encefalitis asociadas a anticuerpos.

Autoimmune encephalitis is a new group of diseases of great clinical and therapeutic importance due to the good response in most cases to the immunomodulatory therapy indicated, with a large percentage of healing without significant neurological effects (cognitive, motor, seizures or involuntary movements). Since 2007, the presence of neuronal autoantibodies in the pathogenesis of this group of diseases has been demonstrated, with psychotic symptoms and involuntary movements as clinical markers of the disease. The present review emphasizes the crucial leap and change of paradigms arising after the discovery of these encephalitis associated with antibodies.

Encefalitis autoinmunes: criterios diagnósticos y pautas terapéuticas / Autoimmune encephalitis. a review

Autoimmune encephalitis are one of the emergent causes of subacute changes in the level of consciousness, behavior, cognitive impairment and seizures, mainly in young people. They are a consequence of inflammation or dysfunction of parts of the brain caused by antibodies against specific brain antigens, usually located in the limbic system, resulting in clinical presentation as a limbic encephalitis. The objectives of this article are to show the clinical presentation, complementary studies and treatment of this entity, considering that the patient's prognostic depends on a high level of clinical suspicion, and on an early initiation of immunosuppressive therapy. We did a nonsystematic review of the literature on autoimmune encephalitis between 2005 and 2017. We conclude that the prevalence of autoimmune encephalitis is increasing, even surpassing infectious causes of encephalitis in developed countries. Clinical presentation includes sub-acute cognitive and behavioral impairment, with or without alterations in consciousness and seizures. Fever and inflammation of the cerebrospinal fluid are less common than in the infectious causes but psychiatric symptoms are more frequent. There are specific clinical presentations according to the particular type of antigen/antibody present, which also determines the association with cancer, constituting a paraneoplastic syndrome only in some cases. Immunosuppressive therapy has been standardized in steps, and should be initiated early to improve prognosis.

Autoimmune encephalitis: a review of diagnosis and treatment / Encefalites autoimunes: uma revisão sobre diagnóstico e tratamento

ABSTRACT Autoimmune encephalitis (AIE) is one of the most common causes of noninfectious encephalitis. It can be triggered by tumors, infections, or it may be cryptogenic. The neurological manifestations can be either acute or subacute and usually develop within six weeks. There are a variety of clinical manifestations including behavioral and psychiatric symptoms, autonomic disturbances, movement disorders, and seizures. We reviewed common forms of AIE and discuss their diagnostic approach and treatment.

RESUMO As encefalites autoimunes (EAI) são a principal causa de encefalite não-infecciosa. As manifestações neurológicas são variadas, incluindo alterações comportamentais ou psiquiátricas, disautonomia, transtornos do movimento e epilepsia. Habitualmente a instalação dos sintomas ocorre em até 6 semanas, de forma aguda ou subaguda. As EAI podem ser desencadeadas por tumores, quadros infecciosos virais ou ainda apresentar etiologia criptogênica. Este artigo revisa as principais EAI, estratégias de diagnóstico e tratamento.

Conocimientos actuales en encefalopatía de Hashimoto: revisión de la literatura / Current knowledge on Hashimoto's encephalopathy: a literature review

Resumen La encefalopatía de Hashimoto es una enfermedad rara. Se reporta una prevalencia de 2,1 por cada 100 000 habitantes. Entre las manifestaciones clínicas se describen confusión, disminución del estado de consciencia, déficit cognitivo, convulsiones, mioclonus, ataxia y/o déficits neurológicos focales. Debido a la amplia variedad de signos y síntomas, la sospecha clínica diagnóstica es fundamental. El diagnóstico se basa en tres pilares: la presencia de manifestaciones clínicas neurológicas, con la exclusión de otras causas de encefalopatía; presencia de anticuerpos antitiroideos aumentados; una mejoría clínica notable luego de la administración de inmunomoduladores. El tratamiento de la encefalopatía de Hashimoto tiene dos objetivos: controlar el proceso autoinmune y controlar las complicaciones de la enfermedad. Aunque en la mayoría de los casos la recuperación es completa con el tratamiento, el riesgo de recaídas puede oscilar entre 12,5 a 40% en seguimientos a dos años.

Abstract Hashimoto's encephalopathy is a rare disease, with a reported prevalence of 2.1 per 100 000. Clinical manifestations include confusion, decreased state of consciousness, cognitive deficit, seizures, myoclonus, ataxia, and focal neurological deficits. Due to the wide variety of signs and symptoms, clinical diagnostic suspicion is essential. Diagnosis is based on three pillars: the presence of neurological clinical manifestations after ruling out other causes of encephalopathy. 2) Presence of increased antithyroid antibodies. 3) Significant clinical improvement after the administration of immunomodulation. The treatment of Hashimoto's encephalopathy pursues two objectives: to control the autoimmune process and to control the complications of the disease. Although in most cases recovery is complete with treatment, the risk of relapse can range from 12.5 to 40% in follow-ups to 2 years.

Potential role of dengue virus, chikungunya virus and Zika virus in neurological diseases

This study showed that laboratory markers of recent infection by dengue, Zika or chikungunya arboviruses were detected in the biological samples of approximately one-third of patients with encephalitis, myelitis, encephalomyelitis or Guillain-Barré syndrome, in a surveillance programme in Piauí state, Brazil, between 2015-2016. Fever and myalgia had been associated with these cases. Since in non-tropical countries most infections or parainfectious diseases associated with the nervous system are attributed to herpesviruses, enteroviruses, and Campylobacter jejuni, the present findings indicate that in tropical countries, arboviruses may now play a more important role and reinforce the need for their surveillance and systematic investigation in the tropics.

Clinical and cerebrospinal fluid findings contribute to the early differentiation between infectious and noninfectious encephalitis

Early recognition and prompt specific treatment are crucial factors influencing the outcome of patients with acute encephalitis. The aim of this study was to determine the main causes of acute encephalitis in our population and to find predictors that may lead to specific diagnosis. Adult patients admitted to our hospital with suspected diagnosis of encephalitis in the period 2006-2013 were included. One hundred and five medical records were analyzed. Eighty-two patients with infectious encephalitis were identified (78% of total cases), 53 (65%) men and 29 (35%) women, mean age 47.8 years. The most common microorganisms identified were: HSV-1 (11%), VZV (10%), HSV-2 (5%) and EBV (5%). Twenty-three patients (22% of the series) had non-infectious encephalitis. Headache (p < 0.0001) and fever (p = 0.008) were more frequent in encephalitis of infectious origin. Protein levels and white blood cell counts in the cerebrospinal fluid were significantly higher in patients affected by infectious encephalitis than in those affected by noninfectious encephalitis (OR 95% CI 12.3 [2.9-51.7] and OR 95% CI 7.4 [2-27], respectively). Identifying specific causal agents of acute encephalitis remains a major challenge. Cerebrospinal fluid markers, as well as specific clinical findings, may however contribute to initial differentiation between infectious and noninfectious causes.

El reconocimiento temprano y la instauración del tratamiento adecuado son dos elementos de gran relevancia en el pronóstico de las encefalitis agudas. El objetivo del presente trabajo es determinar las principales causas de encefalitis aguda en nuestro medio, así como buscar predictores que permitan orientar a un diagnóstico determinado. Se revisaron de manera retrospectiva las historias clínicas de todos los pacientes adultos que consultaron en nuestro centro entre 2006 y 2013 con el diagnóstico presuntivo de encefalitis. Ciento cinco pacientes fueron finalmente incluidos en nuestro estudio. Se identificaron 82 pacientes con encefalitis de origen infeccioso (78%), 53 (65%) fueron hombres y 29 (35%) mujeres, con una edad promedio de 47.8 años. Los agentes infecciosos más frecuentes fueron virus: HSV-1 12 (11%), VZV 11 (10%), HSV-2 5 (5%) y EBV 5 (5%). Se diagnosticó encefalitis no infecciosa en 23 (22%) pacientes. La cefalea (p < 0.0001) y la fiebre (p = 0.008) fueron más frecuentes en las encefalitis de origen infeccioso. Además, los niveles de proteínas y células en el LCR fueron significativamente mayores en los casos de etiología infecciosa que en los de etiología no infecciosa (OR 12.3 95%CI [2.9-51.7] y OR 7.4 95%CI [2-27], respectivamente). La identificación de la etiología específica de las encefalitis agudas continúa siendo un gran desafío y en la mayoría de los casos no se identifica el agente causal. Determinados marcadores en el LCR pueden contribuir a la identificación inicial de las encefalitis de etiología infecciosa versus no infecciosa.

Diagnóstico etiológico en meningitis y encefalitis por técnicas de biología molecular / Etiologic diagnosis in meningitis and encephalitis molecular biology techniques

Introducción: El estudio etiológico de las infecciones del sistema nervioso central se ha realizado tradicionalmente con cultivos bacterianos y con reacción en cadena de la polimerasa (PCR) para virus herpes simple (VHS). Los cultivos bacterianos pueden disminuir su rendimiento en pacientes que hayan usado antibióticos previos a la toma de muestra, y el solicitar PCR solo para virus VHS reduce el diagnóstico etiológico a un solo agente. El objetivo de este trabajo fue determinar las causas infecciosas en meningitis y encefalitis en niños, utilizando conjuntamente la microbiología convencional y la biología molecular, con el fin de mejorar el diagnóstico etiológico de estas enfermedades. Pacientes y método: Se estudiaron 19 pacientes con sospecha de meningitis y encefalitis, de manera prospectiva, hospitalizados en el hospital Luis Calvo Mackenna en Santiago de Chile, entre el 1 de marzo de 2011 y el 30 de marzo de 2012. Luego de obtener el consentimiento informado, a las muestras de LCR se les realizó examen citoquímico, cultivo, PCR múltiple bacteriana (N. meningitidis, S. pneumoniae, H. influenzae) y PCR en tiempo real para HSV-1 y 2, VVZ, VEB, CMV, VHH-6 y enterovirus. Se recabaron datos clínicos y epidemiológicos desde la ficha clínica del paciente. Resultados: De los 19 pacientes analizados 2 (10%) fueron diagnosticados por métodos microbiológicos convencionales y 7 (37%) al adicionar biología molecular (p = 0,02). Tres pacientes presentaron meningitis por S. pneumoniae, uno por Enterobacter cloacae, 2 pacientes meningoencefalitis por VHS-1 y uno meningitis por VVZ. Conclusiones: La adición de la PCR a los métodos microbiológicos convencionales de diagnóstico en las infecciones del sistema nervioso central aumenta significativamente la probabilidad de detectar el agente causal. La incorporación rutinaria del diagnóstico molecular permitiría un manejo más oportuno y racional.

Introduction: The aetiological study of infections of the central nervous system has traditionally been performed using bacterial cultures and, more recently, using polymerase chain reaction (PCR) for herpes simplex virus (HSV). Bacterial cultures may not have good performance, especially in the context of patients who have received antibiotics prior to sampling, and a request for HSV only by PCR reduces the information to only one aetiological agent. The aim of this study is to determine the infectious causes of meningitis and encephalitis, using traditional microbiology and molecular biology to improve the aetiological diagnosis of these diseases. Patients and method: A prospective study was conducted on 19 patients with suspected meningitis, admitted to the Luis Calvo Mackenna Hospital in Santiago, Chile, from March 1, 2011 to March 30, 2012. After obtaining informed consent, the CSF samples underwent cytochemical study, conventional culture, multiplex PCR for the major producing bacterial meningitis (N. meningitidis, S. pneumoniae, H. influenzae), real-time single PCR for HSV-1 and 2, VZV, EBV, CMV, HHV-6 and enterovirus. Clinical and epidemiological data were also collected from the clinical records. Results: Of the 19 patients analysed, 2 were diagnosed by conventional methods and 7 by adding molecular biology (increase to 37%). Three patients had meningitis due to S. pneumoniae, one due to Enterobacter cloacae, 2 patients meningoencephalitis HSV-1, and one VZV meningitis. Conclusions: The addition of PCR to conventional diagnostic methods in CNS infections increases the probability of finding the causal agent. This allows a more adequate, timely and rational management of the disease.

Rasmussen's encephalitis: a case report / Encefalitis de Rasmussen: reporte de caso

Rasmussen's encephalitis (RE) is a rare but severe immune-mediated brain disorder leading to unilateral hemispheric atrophy, associated progressive neurological dysfunction with intellectual decline, and intractable seizures. It is a well-established cause of pharmacologically intractable epilepsy. The report is on a 17-month-old infant, treated at the Mario Catarino Rivas Hospital Honduras. Family history: grandfather epileptic secondary trauma from 20 years. Personal history: two previous emergency visits (at 16 months and 16 months 8 days) for convulsions for which she was admitted three days and was treated with valproic acid 30 mg/kg per day. The infant is admitted in the emergency, with a history of about three hours after onset of tonic convulsions, focused on left-side with drooling, oculogiros and relaxation of sphincters and fever of 38.5 ° C. Entered as convulsive syndrome in the study, however, as the days passed the number of seizures increased to 60 per day and was gradually presenting alterations in neurodevelopment. MRI reported leukoencephalopathy of undetermined origin and biopsy reported findings consistent with Rasmussen's syndrome. She was treated with immunoglobulin every two weeks for six doses after two months of hospitalization with achieved improvement. Currently, episodes of seizures have decreased significantly and almost not convulsing, she presented alterations in neurodevelopment.

La encefalitis de Rasmussen (ER)es un trastorno poco frecuente pero grave, mediado inmunológicamente, que afecta al cerebro y conduce a la atrofia hemisférica unilateral, disfunción neurológica progresiva asociada con deterioro intelectual y convulsiones intratables. Lactante de 17meses de edad, atendida en el Hospital Mario Catarino Rivas, Honduras. Con antecedentes familiares: abuelo epiléptico secundario a trauma a partir de los 20 años. Antecedentes personales: dos ingresos previos por cuadros convulsivos por los cuales estuvo ingresada tres días, en tratamiento con ácido valproico 30 mg/kg por día. Es recibida en la emergencia, con historia de aproximadamente tres horas de evolución de convulsiones tónicas, focalizadas en hemicuerpo izquierdo con sialorrea, oculogiros y relajación de esfínteres, febril 38,5 °C. Ingresada como síndrome convulsivo en estudio, sin embargo, al pasar los días incrementó el número de convulsiones hasta 60 diarios y progresivamente fue presentando alteraciones en su neurodesarrollo. La Resonancia Magnética reportó leucoencefalopatia de origen no determinado y la biopsia reportó hallazgos compatibles con Síndrome de Rasmussen. Fue tratada con inmunoglobulina cada quince días por seis dosis y después de dos meses de hospitalización, se logró egresar. Actualmente, los episodios de convulsiones han disminuido considerablemente y casi no convulsiona, presenta alteraciones en su neurodesarrollo.

Acute measles encephalitis in an immigrant Syrian child: case report and review of the literature

The introduction of measles vaccination programs and broad coverage worldwide has meant this infection a rare encounter for pediatricians. In Oman, with almost 100% measles vaccination coverage for children, this infection disappeared from the list of fever and rash differential diagnoses. Encephalitis is a well-known complication of measles infection and sometimes can be the only manifestation especially in adults. We report a seven-year-old Syrian immigrant who was admitted to the Royal Hospital, Muscat, with acute encephalitis secondary to wild measles infection. Although she had a classical presentation of measle infection, the diagnosis was missed in the private and regional hospital she attended before getting referred to Royal Hospital. She was later identified to be exposed to an outbreak of the infection in an unvaccinated population. Magnetic resonance imaging showed high signal intensity of both basal ganglia suggestive of measles encephalitis. The diagnosis was confirmed by detection of measles virus from her urine and blood, and a throat swab. The isolated measles virus was D8 serotype, which was prevalent in Syria around the same time. The child was treated with steroids and vitamin A. She achieved full recovery despite her severe presentation. A high degree of suspicion for measles infection should be maintained in unvaccinated children with a compatible presentation of the infection or its complications. There might be a role for steroid use in cases of acute measles encephalitis

Binswanger: mais que um epônimo / Binswanger: more than an eponym

Otto Ludwig Binswanger (1852-1929) foi um médico suíço, psiquiatra e neurologista, oriundo de uma linhagem de psiquiatras. Além de ser talentoso, recebeu educação privilegiada de mestres excepcionais, lembrados até hoje. Como psiquiatra, foi muito dinâmico e inovador na reorganização e modernização da Clínica Psiquiátrica e Neurológica que dirigiu, em Jena, durante 37 anos. Escreveu livros importantes e numerosos artigos científicos, entre os quais A delimitação da paralisia geral progressiva, no qual descreveu a "Encefalite subcortical progressiva crônica". Alzheimer denominou a condição, em 1902, de "Encefalite subcortical progressiva crônica de Binswanger", mais tarde conhecida por "doença de Binswanger". Apesar de críticas, chegando mesmo a se argumentar que o epônimo poderia ser impróprio, concluiu-se por sua manutenção, conservada até os dias de hoje.

Otto Ludwig Binswanger (1852-1929) was a Swiss physician, psychiatrist and neurologist, who stemmed from an extraction of psychiatrists. Besides being talented, he received a privileged education by exceptional masters, remembered up to the present days. As a psychiatrist he was very dynamic and innovative in the reorganization and modernization of the Psychiatric and Neurologic Clinic he directed, in Jena, for 37 years. He wrote several important books and numerous scientific papers, among them The delimitation of the progressive general paralysis, where he described the "Chronic progressive subcortical encephalitis". Alzheimer was the one that designated the condition, in 1902, as "Chronic progressive subcortical encephalitis of Binswanger", later acknowledged as "Binswanger's disease". Despite the critics, even reaching to arguments that the eponym was a misnomer, it was concluded for its maintenance, retained up to the present days.

Encefalitis de Bickerstaff: informe de caso y revisión de la literatura / Bickerstaff brain encephalitis: Case report and literature review

En este artículo se presenta el caso clínico de un paciente de sexo masculino de 34 años que fue remitido al Instituto Neurológico de Colombia e internado en la unidad de cuidados intensivos neurológicos, por la sospecha inicial de un síndrome de Guillain-Barré. La presencia de alteraciones cognitivas durante su evolución, llevó a reconsiderar el diagnóstico presuntivo inicial por el de encefalitis de Bickerstaff. Se describen el tratamiento instaurado y la evolución del paciente, se hacen la revisión de la literatura y la discusión.

We describe the case of a 34-year-old male patient, who was referred to the Instituto Neurológico de Colombia with probable Guillain-Barré syndrome, requiring intensive care management. The presence of cognitive alterations during his evolution, lead the team to reconsider the initial diagnosis for the Bickerstaff´s brainstem encephalitis diagnosis. We aim to describe the patient´s treatment and evolution, as well as a brief review and discussion.

Encefalitis del tallo cerebral y mielitis por Listeria monocytogenes / Brainstem encephalitis and myelitis due to Listeria monocytogenes : a case report and literature review

La romboencefalitis por Listeria monocytogenes es una presentación poco común de la listeriosis del sistema nervioso central; sin embargo, es la presentación más común en personas inmunocompetentes. Aun más rara es la combinación de romboencefalitis con mielitis causada por L. monocytogenes ; no obstante, en este artículo se reporta un caso de encefalitis del tallo y mielitis grave en un paciente sin compromiso del sistema inmunitario. Se presenta un paciente de 21 años de edad, sin deficiencias del sistema inmunitario, que consumió productos lácteos no pasteurizados y, posteriormente, presentó un cuadro de cefalea, vómito, deterioro de su estado general y, finalmente, alteración del estado de conciencia y muerte. Consultó al Instituto Neurológico de Colombia y se hizo diagnóstico de encefalitis del tallo y mielitis por L. monocytogenes . Se discuten las diferencias entre el caso presentado y los reportados en la literatura científica. Ante un paciente con signos de compromiso del tallo cerebral, de posible origen infeccioso, es prudente iniciar tratamiento antibiótico para L. monocytogenes y, en caso de poca respuesta, escalar rápidamente en dicho tratamiento. También lo es extender el estudio radiológico hacia la columna vertebral, con el fin de descartar compromiso de la médula espinal.

Brainstem encephalitis caused by Listeria monocytogenes is an uncommon form of central nervous system listeriosis; however, it is the most common presentation in immunocompetent individuals. Here, we describe an even more rare combination of rhombencephalitis with severe myelitis caused by L. monocytogenes in an immunocompetent patient. We report the case of a 21-year-old immunocompetent patient who consumed unpasteurized dairy products and experienced headache and vomiting that progressed to an impaired general condition, altered consciousness and ultimately death. The patient had presented to the Neurological Institute of Colombia (INDEC in Spanish) for consultation and was diagnosed with brainstem encephalitis and myelitis caused by Listeria monocytogenes . The differences between this particular case and those reported in the literature will be discussed. It is advisable to initiate antibiotic treatment for Listeria monocytogenes if a patient shows signs of brainstem compromise of possible infectious origin and quickly intensify treatment if there is no or minimal response. It is also necessary to extend radiological assessment to include the spinal column to rule out spinal cord involvement.

Síndrome anti-GQ1b: Descripción de cuatro pacientes y revisión de la literatura / Anti-GQ1b syndrome: Report of four cases

Anti-GQ1b syndrome includes Miller Fisher Syndrome (MFS), Guillain Barré Syndrome (GBS), Bickerstaff`s brain stem encephalitis (BBE) and Acute Ophtamoplegia (AO). We report four patients aged 16 to 76 years, with anti-GQ1b syndrome. All presented with MFS, one of them evolved to GBS pharyngeal-cervical-brachial variant and other to GBS with BBE. All had a previous history of diarrhea or upper respiratory tract infection. All had positive anti-GQ1b serum antibodies. Both brain magnetic resonance imaging and cerebrospinal fluid analysis were normal. Electrophysiology studies were compatible with a demyelinating disease. Two patients needed airway protection with an orotracheal tube and developed dysautonomia. All four patients were treated with immunomodulation. On the sixth month follow-up, patients had only minimal alterations in the neurological examination.

Enfermedad de Von Economo: Caso clínico / Lethargic Encephalitis: Report of one case

Lethargic encephalitis (LE) is a Central Nervous System disorder following an upper respiratory tract infection, characterized by sleep disturbances, clinical symptoms corresponding to basal ganglia involvement and in some cases, neuropsychiatric sequelae. We report a 18-year-old mole with a history of sinusitis treated with azithromycin, two weeks before, presenting with fever, headache, confusion and myoclonus. Urine analysis was positive for cannabis. Cerebro spinal fluid analysis showed mononuclear pleiocytosis (109xmm³) and an increase in protein concentration ofl.6 g/dl. Forty eight hours after admission, the patient required mechanical ventilation and subsequently a status epilepticus appeared. Ten days later, fever, rigidity and resting tremor appeared. A magnetic resonance imaging showed hyperintensities in FLALR sequence in the right insular cortex. The patient continued with extreme rigidity, catatonia and mutism. Considering the possibility ofa LE, methyl prednisolone 1 g/day was administered for five consecutive days followed by prednisone 40 mgl day, observing a dramatic improvement of rigidity and tremors.