Tratamiento y evolución de la encefalitis inmunomediada en edad pediátrica, serie de casos / Treatment and outcome of immune-mediated encephalitis in children, a case series

Introducción: La encefalitis por anticuerpos contra el receptor N-metil.D.aspartato (NMDA-R) es un trastorno inflamatorio del sistema nervioso central (SNC) en el cual autoanticuerpos dirigidos hacia la subunidad NR1 del receptor N-metil-D aspartato (NMDA) desarrollan un conjunto de síntomas neuropsiquiátricos, convulsiones y movimientos anormales. El tratamiento recomendado incluye metilprednisolona (MP) y gamaglobulina (IVIg), y/o recambio plasmático terapéutico (RPT); y en caso de no respuesta: rituximab (RTX) y/o ciclofosfamida (CFM). Objetivos: Analizar características clínicas, bioquímicas, electroencefalograma (EEG), resonancia magnética (RM) cerebral, tratamientos recibidos y resultados observados en una serie de pacientes con encefalitis autoinmune (EA) probable o confirmada. Materiales y métodos: Analizamos las historias clínicas de pacientes menores a 17 años que cumplían criterios diagnósticos de Graus (2016) para EA probable, con seguimiento mayor a 6 meses, internados en el Hospital Garrahan entre 2008 y 2023. El diagnóstico se definió por la identificación de anticuerpos anti-NMDAR (N-metil D-aspartato) en líquido cefalorraquídeo (LCR) por ensayo basado en células - cell bassed assay (CBA). Resultados: Reunieron criterios de EA probable 94 pacientes con una edad media de 89.5 meses, 51% mujeres. Se dividieron en dos grupos: seropositivos y seronegativos de acuerdo al resultado del biomarcador. Seropositivos 45/94. El síntoma inicial más frecuente fue: convulsiones. El 28% requirió ingreso a Unidad de Cuidados Intensivos (UCI). 4 pacientes seropositivos y 1 seronegativo tuvieron encefalitis por el virus del herpes simple (Om) previamente. En una paciente seronegativa se diagnosticó teratoma ovárico. Hallazgos de estudios complementarios: LCR patológico en el 29%, RM cerebral en el 52%, EEG en el 74%. El tratamiento de primera línea más empleado fue MP + IVIg. El 46% de los pacientes presentó recuperación completa. Entre los pacientes que recibieron RTX, el 65% tuvo una recuperación completa. Ningún paciente que recibió RTX presentó recaída. Conclusión: Ante la sospecha de EA se debe considerar el inicio temprano de inmunoterapia para favorecer la rápida recuperación funcional. Se recomienda el uso temprano de RTX en los casos con presentación grave o respuesta subóptima al tratamiento de primera línea para beneficiar la respuesta clínica y reducir el riesgo de recaída (AU)

Introduction: Encephalitis due to antibodies against the N-methyl-D-aspartate receptor (NMDA-R) is an inflammatory disorder of the central nervous system (CNS) in which autoantibodies directed against the NR1 subunit of the N-methyl-D-aspartate (NMDA) receptor develop a set of neuropsychiatric symptoms, seizures, and abnormal movements. The recommended treatment includes methylprednisolone (MP) and intravenous immunoglobulin (IVIg), and/or therapeutic plasma exchange (TPE); and in case of non-response: rituximab (RTX) and/or cyclophosphamide (CFM). Objectives: To analyze clinical, biochemical, electroencephalogram (EEG), magnetic resonance imaging (MRI) of the brain, treatments received, and outcomes observed in a series of patients with probable or confirmed autoimmune encephalitis (AE). Materials and methods: We analyzed the medical records of patients under 17 years of age who met Graus' diagnostic criteria (2016) for probable AE, with follow-up of more than 6 months, hospitalized at Hospital Garrahan between 2008 and 2023. Diagnosis was defined by the identification of anti-NMDAR antibodies (N-methyl D-aspartate) in cerebrospinal fluid (CSF) by cell-based assay (CBA). Results: Ninety-four patients met criteria for probable AE with a mean age of 89.5 months, 51% female. They were divided into two groups: seropositive and seronegative according to the biomarker result. Seropositive 45/94. The most frequent initial symptom was seizures. Twenty-eight percent required admission to the Intensive Care Unit (ICU). Four seropositive patients and one seronegative patient had previously had herpes simplex encephalitis (Om). Ovarian teratoma was diagnosed in one seronegative patient. Findings of complementary studies: Pathological CSF in 29%, brain MRI in 52%, EEG in 74%. The most commonly used first-line treatment was MP + IVIg. Forty-six percent of patients experienced complete recovery. Among patients who received RTX, 65% had complete recovery. No patient who received RTX experienced relapse. Conclusion: In the suspicion of AE, early initiation of immunotherapy should be considered to promote rapid functional recovery. Early use of RTX is recommended in cases with severe presentation or suboptimal response to first-line treatment to benefit clinical response and reduce the risk of relapse (AU)

Encefalitis aguda con síndrome cerebeloso secundario a neuroborreliosis / Cerebellar syndrome associated to Lyme neuroborreliosis: case report

La enfermedad de Lyme es la infección transmitida por garrapatas más común en Norteamérica y Europa. Sin embargo, en América Latina se han reportado pocos datos. Borrelia burgdorferi es capaz de invadir el sistema nervioso central causando la neuroborreliosis de Lyme. Se comunica el caso de una mujer joven con encefalitis y síndrome cerebeloso, una presentación poco frecuente, que se manifestó con cefalea, marcha atáxica, nistagmo y ptosis palpebral. Se realizó el diagnóstico con serología positiva por Western blot para Borrelia burgdorferi en suero. Recibió tratamiento con mejoría clínica. El diagnóstico de neuroborreliosis es difícil, ya que no existen hallazgos neurológicos o de imagen específicos. Se recomienda tener un mayor nivel de vigilancia epidemiológica en Latinoamérica, así como los antecedentes recreativos o viajes, que deben incluirse en la historia clínica para mejorar la aproximación diagnóstica.

Lyme disease is the most common tick-borne disease in North America and Europe. However, in Latin America, few data have been reported. Borrelia burgdorferi is capable of invading the central nervous system causing Lyme neuroborreliosis. This is a case report of a female with encephalitis and cerebellar syndrome, a rare manifestation, presented with generalized headache ataxic gait, nystagmus and palpebral ptosis. Serologic diagnosis was made for Borrelia burgdorferi and treatment was initiated with clinical improvement. Neuroborreliosis is a very difficult diagnosis, since there are no neurologic or imaging findings specific. It is recommended to physicians to have an enhanced level of surveillance in Latin America, as well as recreational/travel history of the patient, which should be included in the clinical record to improve the diagnostic approach.

Enfermedad por virus sincitial respiratorio en pediatría: más allá del compromiso respiratorio / Respiratory syncytial virus disease in children: beyond respiratory involvement

Introducción: el virus sincitial respiratorio (VSR) suele asociarse con la enfermedad respiratoria en la población pediátrica, sin embargo se han documentado múltiples manifestaciones extrapulmonares, en especial neurológicas. Objetivo: presentar un panorama de los reportes en la literatura indexada sobre el compromiso neurológico en la infección por VSR. Materiales y Métodos: revisión sistemática exploratoria formal de la literatura en las bases de datos Pubmed y SCOPUS, que incluyó estudios primarios y secundarios hasta el 17 de julio 2022, publicadas en inglés, español y francés. Resultados: se consideraron 30 publicaciones que describían la experiencia en 1.681 pacientes con una edad mediana de 17 meses y mayor compromiso del sexo masculino (64.59%), en el marco de la enfermedad respiratoria grave por VSR. Las convulsiones y la apnea central aparecieron como principales manifestaciones, ya sea por invasión viral e inflamación secundaria del sistema nervioso central (SNC) o por la compensación derivada de los ajustes neuroendocrinos ante el daño respiratorio severo. Las secuelas a largo plazo incluyeron retraso en el desarrollo y discapacidad cognitiva. Conclusión: el síndrome por infección respiratoria grave por VSR puede ocurrir con compromiso neurológico, posiblemente derivado de la acción directa del virus a nivel del SNCo de los efectos del proceso adaptativo. Estos dos mecanismos deben considerarse en presencia de enfermedad respiratoria grave por VSR que cursen con deterioro neurológico, para una correcta aproximación diagnóstica y manejo oportuno, con el fin de disminuir el riesgo de secuelas significativas.

Introduction: respiratory syncytial virus (RSV) is usually associated with respiratory disease in the pediatric population, however, multiple extrapulmonary manifestations especially at neurological level, have been documented. Objective: to present an overview of reports included in the indexed literature on RSV-related neurological involvement. Materials and Methods: formal exploratory, systematic review of the literature in the Pubmed and SCOPUS databases, including primary and secondary studies as of July 17, 2022, published in English, Spanish and French. Results: 30 publications which describe the experience in 1681 patients with a median age of 17 months and greater involvement of the male sex (64.59%), in the setting of severe RSV respiratory disease, were considered. Convulsions and central apnea appeared as the main manifestations, either due to viral invasion and secondary inflammation of the central nervous system (CNS), or due to compensation derived from neuroendocrine adjustments to severe respiratory damage. Long-term sequelae included developmental delay and cognitive disability. Conclusion: Severe RSV respiratory syndrome can result in neurological involvement, possibly derived from direct action of the virus in the CNS or from the effects of the adaptive process. These two mechanisms should be considered in the presence of severe RSV respiratory disease with neurological impairment, for a correct diagnostic approach and timely management, in order to, reduce the risk of significant sequelae.

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

Plasmaféresis terapéutica en paciente pediátrico con encefalitis autoinmune / Therapeutic apheresis in pediatric patient with autoimmune encephalitis

La encefalitis autoinmune es un cuadro con una expresión neuropsiquiátrica especialmente en pediatría, aunque existen diversas opciones de tratamiento, otras alternativas terapéuticas se relacionan con procedimientos que pueden tener un mayor beneficio para el paciente, como es la plasmaféresis. Este procedimiento, representa una de las terapias de primera línea en este padecimiento. Hace poco se cuenta con la disponibilidad de estos equipos, motivo por el cual describimos este primer procedimiento en pediatría a nivel Bolivia describiendo el manejo en un paciente con encefalitis autoinmune.

Autoimmune encephalitis is a condition with a neuropsychiatric expression, especially in pediatrics, although there are various treatment options, other therapeutic alternatives are related to procedures that may have a greater benefit for the patient, such as plasmapheresis. This procedure represents one of the first line therapies in this condition. This equipment has recently become available, which is why we describe this first procedure in pediatrics at the Bolivian level, describing the management of a patient with autoimmune encephalitis.

Encefalopatía asociada a enfermedad tiroidea autoinmune: reporte de caso y revisión de la literatura / Encephalopathy associated with autoimmune thyroid disease: Case report and literature review

RESUMEN INTRODUCCIÓN: La encefalopatía asociada a enfermedad tiroidea autoinmune se caracteriza por un inicio insidioso de síntomas neuropsiquiátricos que incluyen alteración de la función cognoscitiva, cambios del comportamiento, crisis convulsivas y trastornos del movimiento. REPORTE DE CASO: Hombre de 69 años con antecedente de hipotiroidismo primario que consultó por dos semanas de alteración de la memoria, confusión y trastorno del lenguaje, asociados a hiperreflexia y temblor generalizado. Los estudios mostraron nivel de hormona tiroidea y títulos de anticuerpos antitiroideos elevados, líquido cefalorraquídeo con aumento de proteínas y anticuerpos antineuronales negativos, neuroimagen normal y electroencefalograma con alteraciónes inespecíficas. Posterior a descartar otras etiologías, se hizo el diagnóstico de encefalopatía asociada a enfermedad tiroidea autoinmune y se inició manejo con esteroides, con los que presentó una mejoría clínica significativa. DISCUSIÓN: La encefalopatía asociada a enfermedad tiroidea autoinmune es un trastorno complejo que requiere un diagnóstico oportuno y rápido. En todos los pacientes con síntomas neuropsiquiátricos agudos o subagudos, y sin causa clara, es pertinente solicitar un perfil de anticuerpos antitiroideos independiente de la función tiroidea basal.

ABSTRACT INTRODUCTION: Thyroid autoimmune disease-associated encephalopathy is characterized by an insidious onset of neuropsychiatric symptoms which may include impaired cognitive function, behavioral changes, seizures, and movement disorders. CASE REPORT: A 69-year-old man with a history of primary hypothyroidism who consulted for two weeks of memory impairment, confusion, and language disorder, associated with hyperreflexia and generalized tremor. Studies showed elevated thyroid hormone levels and antithyroid antibody titers, cerebrospinal fluid with increased protein and negative antineuronal antibodies, normal neuroimaging, and electroencephalogram with nonspecific abnormalities. After ruling out other etiologies, a diagnosis of encephalopathy associated with autoimmune thyroid disease was made and management with steroids was started, with which he presented significant clinical improvement. DISCUSSION: Encephalopathy associated with autoimmune thyroid disease is a complex disorder that requires timely and rapid diagnosis. In all patients with acute or subacute neuropsychiatric symptoms, and without a clear cause, it is pertinent to request an antithyroid antibody profile independent of baseline thyroid function

Encefalitis por anticuerpos contra el receptor N-metil-D-aspartato secundaria a teratoma ovárico en una paciente pediátrica / Encephalitis due to antibodies against the N-methyl-D-aspartate receptor secondary to ovarian teratoma in a pediatric patient

La encefalitis por anticuerpos contra el receptor N-metilD-aspartato es un proceso inmunomediado en el que autoanticuerpos se dirigen contra la subunidad GluN1 del receptor de glutamato del sistema nervioso central. Se caracteriza por la aparición aguda o subaguda de síntomas psiquiátricos, como confusión, pérdida de la memoria a corto plazo, cambios de conducta, catatonía, seguidos por manifestaciones neurológicas, tales como convulsiones, alteraciones del movimiento, disfunciones autonómicas, coma y depresión respiratoria. Es grave y potencialmente mortal. Su asociación con teratoma de ovario como síndrome paraneoplásico fue descrita en mujeres jóvenes. En la población pediátrica, es mucho menos frecuente y se reporta en comunicaciones de 1 o 2 pacientes y en series de pocos casos. Se presenta una paciente de 13 años con encefalitis paraneoplásica por anticuerpos contra el receptor N-metil-Daspartato, secundaria a un teratoma ovárico maduro.

The encephalitis due to antibodies against the N-methylD-aspartate receptor is a process immune-mediated in which antibodies are directed against the GluN1 subunit of the glutamate receptor in the central nervous system. It is characterized by an acute or subacute onset of psychiatric symptoms such as confusion, short-term memory loss, behavioral changes, catatonia followed by neurological manifestations such as seizures, movement disturbances, autonomic dysfunctions, coma, and respiratory depression. It is serious and life threatening. Its association with ovarian teratoma as a paraneoplastic syndrome was described in youngwomen. In the pediatric population it is much less frequent and is reported in publications of one or two patients and in series of few cases. We present a 13-year-old patient with encephalitis paraneoplastic due to antibodies against the N-methyl-Daspartate receptor, secondary to a mature ovarian teratoma.

Encefalitis como complicación neurológica por dengue / Encephalitis as neurological complication due dengue

Resumen Se presenta el caso de un varón de 32 años, previamente sano, que consultó por fiebre de cinco días, cefalea, dolor retro-ocular, rash, petequias, mialgias, artralgias y dolor abdominal. Presentaba leucopenia, trombocitopenia intensa, transaminitis y tiempo de coagulación prolongado. Se diagnosticó un dengue grave con coagulopatía que requirió manejo en Unidad de Cuidados Intensivos. Evolucionó con alteración del estado de conciencia, agitación psicomotora y agresividad. Se descartaron alteraciones estructurales, isquémicohemorrágicas, infecciones bacterianas y micóticas. Se confirmó finalmente una encefalitis por dengue por una RPC para virus dengue positiva en LCR. Se brindaron medidas de soporte con una evolución favorable. La encefalitis es la complicación neurológica más grave tras la infección por virus del dengue.

Abstract We present the case of a 32-year-old male, previously healthy, with a 5-day history of fever, frontal-occipital headache, retro-ocular pain, rash, petechiae, myalgia, arthralgia, and abdominal pain. Blood tests with leukopenia, severe thrombocytopenia, transaminitis, long clotting times. Severe dengue with associated coagulopathy was diagnosed, indicating transfer to ICU. Presents torpid evolution, altered state of consciousness, psychomotor agitation, and aggressiveness. Structural, ischemic-hemorrhagic alterations, bacterial and fungal infections were ruled out. Finally diagnosing dengue encephalitis, confirmed by DENV PCR in CSF. Support measures are provided with favorable evolution. Encephalitis is the most serious neurological complication after dengue virus infection.

Giant immature teratoma in a 15 year old causing anti-n-methyl-d-aspartate (anti-nmda) receptor encephalitis-like and Guillain-Barré Syndrome as paraneoplastic manifestations: A case report / Philippine Journal of Obstetrics and Gynecology

@#Germ cell tumor is the most prevalent ovarian tumor in young women between 10 and 30 years of age.[1] However, immature teratomas account for only 20% of the malignant ovarian tumors found in the adolescent age group.[2] More uncommon is the occurrence of immature teratoma causing anti‑N‑methyl‑D‑aspartate receptor (Anti‑NMDAR) encephalitis and Guillain‑Barré Syndrome (GBS). This is a case of a 15‑year‑old female, nulligravid, who initially presented sudden behavioral change and symmetrical weakness of both lower and upper extremities with concomitant seizure episodes and with palpable lower abdominal mass. The patient was diagnosed to have GBS and treated with intravenous immunoglobulin causing resolution of neurologic symptoms months after. During the management of GBS, the patient noted increasing abdominal girth. Tumor markers showed elevated alpha‑fetoprotein, CA125, and lactate dehydrogenase. An imaging study revealed predominantly solid ovarian mass hence malignancy was considered. Once the medical condition stabilized, the patient underwent fertility‑sparing surgery with the final histopathologic result of immature teratoma. The aim of this report is to present a case of anti‑NMDAR encephalitis and GBS as paraneoplastic manifestation of immature teratoma.

More than teen angst: A case of ovarian teratoma presenting as anti-N-methyl-d-aspartate receptor encephalitis / Philippine Journal of Obstetrics and Gynecology

@#A 17‑year‑old nulligravid presented with labile mood, insomnia, and hyper‑productive speech for 2 days, which progressed to seizures. Cerebrospinal fluid was positive for anti‑N‑methyl‑d‑aspartate (NMDA) receptor antibodies. Despite immunotherapy and seizure prophylaxis, she had status epilepticus and rapid decrease in sensorium. She was placed on ventilatory support and was admitted in the intensive care unit. On the 44th hospital day, an incidental finding of an ovarian teratoma was seen on abdominal computed tomography scan. She underwent right salpingo‑oophorectomy, which revealed an immature teratoma. The absence of an intensive care admission and prompt surgery and immunotherapy are known predictors of good outcome in anti‑NMDA receptor (NMDAR) encephalitis, with improvement seen in 80% of patients. Because most ovarian teratomas are only diagnosed as an incidental finding, anti‑NMDAR encephalitis with a concurrent ovarian teratoma should be suspected in young females presenting with sudden‑onset neuropsychiatric symptoms, to provide the timely intervention necessary for a good prognosis.

Clinico-Epidemiologic features and outcome of Infectious and Immune-mediated Pediatric Encephalitis

Introduction@#The etiology of encephalitis involves an enormous range and can be classified as infectious or immune-mediated. There are several factors influencing its prognosis and has been associated with significant morbidity and mortality. This study aims to evaluate the clinico-epidemiologic characteristics and outcomes of infectious and immune-mediated encephalitis among pediatric patients.@*Methodology@#Retrospective descriptive cross-sectional study that included patients aged 6 months to 17 years old with encephalitis in a tertiary hospital between January 2010 to December 2020@*Results@#A total of 23 cases were reviewed and 60.87% were infectious while that of immune-mediated was 39.13%. Among those with identified infectious cause, Mycoplasma pneumonia was the most common (28.57%). Infectious encephalitis was more common among younger males (35.71%) while immune-mediated affected female adolescents more (55.56%). The most common neurologic manifestation was altered mental status and/or behavioral changes. Treatment such as antibiotics (78.26%), anticonvulsant therapy (78.26%), and steroids (43.48%) were given. All immune-mediated cases received steroids. More than half of patients had complete recovery (56.52%). @*Conclusion@#Pediatric encephalitis should be considered among patients with neurologic dysfunction with or without systemic involvement. Behavioral changes in an apparently well child should prompt clinicians to consider anti-NMDAR encephalitis, especially if viral studies are negative and with no other known cause. Viruses remain to be the most common etiology, but other possible causes should be highly considered such as anti-NMDAR and Mycoplasma. A normal CSF analysis, imaging and/or encephalography (EEG) may not totally exclude encephalitis. Prognosis is relatively good hence an early diagnosis and initiation of appropriate management is important.

Hashimoto's encephalopathy presenting with isolated cerebellar ataxia in 13 children / 中华儿科杂志

Objective: To analyze the clinical characteristics, treatment and prognosis of Hashimoto's encephalopathy presenting with isolated cerebellar ataxia in children. Methods: A retrospective analysis was performed on the clinical features, laboratory tests, neuroelectrophysiological examination, imaging, treatment and outcomes of 13 patients with Hashimoto's encephalopathy presenting with isolated cerebellar ataxia, who were admitted to the Department of Pediatric Neurology of Guangzhou Women and Children's Medical Center from January 2016 to May 2021. Results: Among the 13 cases, 6 were males and 7 were females. The onset age was 2.6 (2.0,3.3) years, 9 children had precursor infection or vaccination before the first course of disease. All the 13 children had gait abnormalities or unsteady sitting, 10 had intentional tremor, 6 had dysarthria, 3 had body tremor, 2 had nystagmus, 3 had fatigue, 3 had hypotonia, 2 had vomiting and 1 had irritability. Thyroglobulin antibody (TgAb) was 500.0 (298.9,587.2) kU/L and thyroid peroxidase antibody (TPOAb) was 621.9 (449.6,869.4) kU/L in 13 cases. Autoantibodies were positive in 9 cases, and cerebrospinal fluid leukocytosis was seen in 4 cases. Regarding electroencephalography result, 4 cases had background slowing and 1 case had occasional sharp waves. Among the 3 patients who had relapses, 1 had cerebellar atrophy shown on cranial magnetic resonance imaging (MRI) during the recurrence. All the patients received intravenous immunoglobulin (IVIG) and intensive methylprednisolone therapy during the first onset, followed by the disappearance of the symptoms, 1 patient had repeated episodes which was decreased after immunosuppressive treatment with Rituximab.Followed up for 25.0 (22.5,33.3) months after the last episode, 12 achieved complete remission and 1 had a wide base gait. Conclusions: Trunk ataxia is the common symptom of Hashimoto's encephalopathy presenting with isolated cerebellar ataxia in children.Children with cerebellar ataxia should be tested for TgAb and TPOAb to detect Hashimoto's encephalopathy, avoiding missed diagnosis and treatment delays; IVIG and intensive steroid therapy is effective, and immunosuppressive therapy for patients with multiple relapses could reduce the recurrence.

Etiology and epidemiology of hand, foot and mouth disease in China / 中华流行病学杂志

Hand, foot and mouth disease (HFMD) has been widespread in the world, and caused fever, rashes and other clinical symptoms of children, and sometimes inducing respiratory failure, brainstem encephalitis, and other complications, even death. The disease is mainly caused by enterovirus 71 (EV-A71) and coxsackievirus 16 (CV-A16). Since 2013, the proportion of HFMD cases caused by other enteroriruses has gradually increased, causing severe and even fatal cases. This paper summarizes the research progress in the epidemiological and etiological characteristics of HFMD in China since 2008.

Estudio de caso de una persona con encefalitis autoinmune basado en la teoría déficit de autocuidado de Dorothea Orem / Case study of a person with autoimmune encephalitis based on the theory of self-care deficit of Dorothea Orem

Introducción: este caso describe el abordaje y aplicación de intervenciones especializadas de enfermería en una mujer de 49 años con encefalitis autoinmune teniendo como base el modelo de Dorothea Orem. Objetivo: identificar los requisitos universales alterados en la persona para restablecerlos mediante acciones de cuidado. Material y métodos: tras la selección de la persona y autorización del cuidador primario se aplicó un instrumento de valoración que permitió realizar diagnósticos utilizando el formato PESS, planeación y ejecución de intervenciones de enfermería, así como evaluación de cada una, por último, la elaboración de un plan de alta. Descripción del caso clínico: mujer de 49 años presentó súbitamente cefalea, alteración del lenguaje, agitación, inatención y labilidad emocional, punción lumbar arrojó leve proceso inflamatorio, descartando etiología vascular e infecciosa sospechando de autoinmunidad. Relevancia: analizar diferentes situaciones clínicas permitiendo plantear soluciones y estrategias para la mejora en el cuidado enfermero. Resultados y conclusiones: resultados satisfactorios, la evolución fue poco a poco, con grandes cambios que le permitieron ser funcional en las actividades de la vida diaria; ahora, a un año de su enfermedad a pesar del deterioro cognitivo puede deambular y alimentarse por sí misma.

Introduction: This case study describes the approach and application of specialized nursing interventions applied to a 49-year-old woman with autoimmune encephalitis based on the Dorothea Orem model. Objective: Identify the universal requirements altered in the person to restore them through care actions. Methodology: After the selection of the person and authorization of the primary caregiver, an assessment instrument was applied that allowed diagnoses to be made using the PESS format, planning and execution of nursing interventions, as well as the evaluation of each one, ending with the elaboration of a high plan. Description of the clinical case: a 49-year-old woman who suddenly presented headache, language alteration, agitation, inattention and emotional lability, lumbar puncture showed a mild inflammatory process, ruling out vascular and infectious etiology, suspecting autoimmunity. Relevance: The case study analyzes different clinical situations allowing solutions and strategies to be proposed to improve nursing care. Results and conclusions: The results were satisfactory, the evolution of the person was slow but with great changes that allowed her to be functional in the activities of daily life, currently one year after his illness, despite the cognitive deterioration, she can walk and feed herself by herself.

Neurological rehabilitation for a patient with chronic Rasmussen encephalitis: a case report / Reabilitação neurológica em paciente com encefalite de Rasmussen crônica: um estudo de caso

ABSTRACT Rasmussen encephalitis is a rare disease consisting of a chronic, slowly progressive inflammatory reaction of brain tissues. The objective of this study was to describe the case of an 18-year-old female patient, studying on the fourth grade of elementary school, and right-handed, who underwent left hemispherectomy in a neurological rehabilitation program. Her seizures began at 10 years of age and were unresponsive to drug treatment, with functional repercussions. She underwent hemispherectomy in March 2019, with 7 years of ongoing disease, and was admitted for the rehabilitation program with a multidisciplinary team in June 2020. The quality-of-life questionnaire (WHOQOL-BREF) was applied as a comparison measure before and after the rehabilitation program, along with measures of participation on the program activities. Despite the patient's short time in a rehabilitation program, data show the importance of an interdisciplinary intervention through the establishment of specific and contextualized objectives in chronic patients.

RESUMO A encefalite de Rasmussen representa uma doença rara, que consiste em uma reação inflamatória dos tecidos cerebrais, de modo crônico e lentamente progressivo. O objetivo deste estudo foi descrever o caso de uma paciente, submetida à hemisferectomia à esquerda, em março de 2019, em programa de reabilitação neurológica. Paciente do sexo feminino, 18 anos de idade, 4ª série do ensino fundamental, destra, que participou de programa de reabilitação neurológica interdisciplinar após hemisferectomia. Início das crises convulsivas aos 10 anos de idade, refratárias ao tratamento medicamentoso, com repercussão funcional. Foi submetida à hemisferectomia em março de 2019, com sete anos de doença em curso. Foi internada para programa de reabilitação, com equipe multiprofissional, em junho de 2020. Aplicou-se o questionário de qualidade de vida (WHOQOL-BREF), como medida de comparação antes e após o programa de reabilitação, além de medidas de participação nas atividades do programa. Os dados, apesar do pouco tempo da paciente em programa de reabilitação, mostram a importância da intervenção interdisciplinar, por meio do estabelecimento de objetivos realísticos e contextualizados.

Encefalitis por ameba de vida libre: reporte de caso / Free-living amoeba encephalitis: case report

La encefalitis amebiana granulomatosa es una enfermedad considerada atípica debido a la baja incidencia desde que fue descubierta. Sin embargo, conforme han avanzando los métodos diagnósticos, en retrospectiva, se ha descrito la presencia de esta enfermedad desde principios del siglo XX, identificando a Acanthamoeba spp y Balamuthia mandrillaris como agentes más comunes.Es una enfermedad de difícil diagnóstico debido al amplio abanico clínico que puede presentar, siendo el sitio anatómico dentro del sistema nervioso central quien determinará estas características. El conocimiento y sospecha de la misma permitirá un abordaje terapéutico oportuno aunque, en la mayoría de los casos, con un desenlace fatal para el paciente.A continuación se presenta el caso clínico de un paciente masculino de 26 años de edad originario de la ciudad de Durango, México, sin antecedentes de importancia, de ocupación ladrillero, quien sufre golpe con ladrillo en región naso-orbitaria, ingresando al hospital al tercer día del evento con diagnóstico de celulitis periorbitaria izquierda y nasal, cefalea de leve intensidad, rinorrea abundante, hemiparesia fasciocorporal izquierda, parestesia de extremidad superior izquierda, diplopía y vértigo postural. Los estudios imagenológicos revelaron lesiones heterogéneas en el lóbulo occipital, hemisferio cerebeloso izquierdo y tallo cerebral. Se realizó escisión de lesión tumoral cerebelar, con estudio anatomopatológico, reportando encefalitis granulomatosa por presencia de ameba de vida libre. El paciente evolucionó hacia deterioro rostro-caudal declarándose su fallecimiento a los 16 días de su estancia intrahospitalaria.En el Estado de Durango, México, no se cuenta con casos reportados por infección de ameba de vida libre

Granulomatous amoebic encephalitis is a disease considered atypical due to the low incidence since it was discovered. However, as diagnostic methods have progressed, in retrospect, the presence of this disease has been described since the beginning of the 20th century, identifying Acanthamoeba spp. and Balamuthia mandrillaris as the most common agents.A disease that is difficult to diagnose due to the wide clinical range that it can present, being the anatomical site within the central nervous system that will determine these characteristics. The knowledge and suspicion of it will allow a timely therapeutic approach although, in most cases, with a fatal outcome for the patient.The clinical case is presented below of a 26 year old male patient from the city of Durang, México, with no significant history, of brick occupation, who suffers a blow with a brick in the naso-orbital region, follows, entering our hospital on the third day of the event with a diagnosis of left periorbital and nasal cellulitis, mild headache, profuse rhinorrhea, left fasciocorporeal hemiparesis, left upper limb parestesia, diplopia, and postural vertigo.Imaging studies were performed observing heterogeneous lesions in the occipital lobe, left cerebellar hemisphere and brain stem; excision of the cerebellar tumor lesion being carried out, with pathological study, reporting granulomatous encephalitis due to the presence of free-living amoeba. The patient progresses to face-caudal deterioration, declaring his death after 16 days of hospital stay.Within the state of Durango, Mexico, there are no reported cases of free-living amoeba infection

Manifestaciones neurológicas graves de la gripe durante la temporada gripal 2018-2019: serie de casos de 13 pacientes pediátricos / Severe neurological manifestations of influenza during 2018-2019 influenza season: Case series of 13 pediatric patients

La gripe se asocia al aparato respiratorio, especialmente en invierno, y puede causar complicaciones neurológicas. Se evaluó a pacientes pediátricos con manifestaciones neurológicas graves por gripe desde septiembre de 2018 hasta febrero de 2019 para determinar características clínicas, neuroimagenología, tratamiento y resultados. El objetivo fue evaluar la encefalitis asociada a la gripe y destacar diferentes manifestaciones neurológicas y cambios de neuroimagenología. El estudio incluyó a 13 pacientes. Los síntomas neurológicos ocurrieron tras los síntomas típicos de la gripe. Los cambios de neuroimagenología incluyen alteraciones de señal de la sustancia blanca cortical y subcortical, edema localizado o generalizado y lesiones multifocales simétricas bilaterales en el tálamo y la médula del cerebelo. Las opciones terapéuticas incluyen metilprednisolona en inyección intravenosa, inmunoglobulina intravenosa, plasmaféresis y oseltamivir. Es fundamental considerar la encefalitis asociada a la gripe en pacientes con convulsiones, la encefalopatía con hallazgos radiológicos compatibles, e iniciar el tratamiento lo antes posible

Influenza is mostly associated with the respiratory tract system, especially in the winter season. Various neurological complications could occur due to influenza infection. Pediatric patients who had severe neurological manifestations due to influenza infection from September 2018 to February 2019 were evaluated for clinical characteristics, neuroimaging studies, treatment, and outcome. We aimed to assess Influenza-associated encephalitis in children, emphasize different neurological manifestations and neuroimaging changes. Thirteen patients were included in the study. Neurological symptoms occurred after flu-like symptoms. Neuroimaging changes of influenza-associated encephalitis/encephalopathy include cortical and subcortical white matter signal alterations, localized or generalized edema, and bilateral symmetrical multifocal lesions on the thalamus and cerebellar medulla. Pulse methylprednisolone, intravenous immunoglobulin, plasma exchange, and oseltamivir are the therapy choices. It is essential to consider influenza-associated encephalitis in patients with seizures, encephalopathy with supporting radiological findings, especially during the influenza season and starting treatment as fast as possible for better outcomes.

Influence of euthanasia, the intensity of inflammatory lesion and viral load in the laboratory diagnosis of rabies in cattle / Influência da eutanásia, intensidade da lesão inflamatória e carga viral no diagnóstico laboratorial da raiva em bovinos

This research reports the use of different diagnostic tests in cattle, naturally infected by Rabies lyssavirus (RABV), and correlates the positivity of the tests with the clinical moment of euthanasia, the intensity of the inflammatory lesion and viral load. It also highlights the possibility of euthanasia in early stages of the disease as a way to improve animal welfare. For that, samples of 34 bovine brains were collected for analysis, preserved in 10% buffered formaline and refrigerated with subsequent freezing. The samples were subjected to direct immunofluorescence antibody technique (DFAT) tests, viral isolation in cell culture (VICC), histopathology with hematoxylin and eosin staining (HE), immunohistochemistry (IHC), Shorr stainied neural tissue smears (DSS), Reverse transcription polymerase chain reaction (RT-PCR) and polymerase chain reaction by quantitative reverse transcriptase (qRT-PCR). The areas used for analysis were the cerebellum, parietal telencephalon and thalamus. Samples with Negri bodies (NBs) or immunostaining in at least one of the analyzed areas were considered positive. For the study of the intensity of histological lesions, the lesions were classified into grades 0, 1, 2 and 3 and the positivity of the test in the presence or absence of NBs in one of the three areas analyzed. To verify the influence of the disease clinical evolution, 4-four groups of analysis were created according to the animal's clinical status at moment of the euthanasia, being: M1 = animal euthanized while standing, M2 = euthanized when in sternal recumbence, M3 = euthanized when in lateral recumbence, M4 = animal with natural death. Of the 34 brains evaluated, IHC was positive in 100% of cases, DFAT was positive in 97.05% of them, and in this negative sample the presence of RABV was confirmed by VICC. NBs ere seen in 88.23% of the cases, and the DSS test was positive in 82.35% of them. All diagnostic techniques showed positive cases in all groups analyzed. Each case was positive in at least two diagnostic methods. All cases that contained NBs were positive for rabies in the other tests. In this study, it was observed that the variables analyzed (intensity of injury and clinical evolution at the moment of euthanasia) had an influence only on HE and DSS techniques, which are based on NB research to form the diagnosis, but did not interfere with the effectiveness of the diagnosis performed by detecting the viral antigen performed by DFAT and IHC. All isolated RABV samples included in the present study have a genetic lineage characteristic of hematophagous bats Desmodus rotundus. The evaluation of qRT-PCR showed that the amount of virus did not interfere in the positivity of the tests. This work shows that IHC and DFAT are safe diagnostic techniques. They are capable of detecting RABV even in euthanized animals in the early stages of clinical evolution with mild intensities of histological lesions.(AU)

Esta pesquisa relata a utilização de diferentes testes de diagnóstico em bovinos, naturalmente infectados pelo Rabies lyssavirus (RABV), e correlaciona a positividade dos testes com o momento clínico da eutanásia, a intensidade da lesão inflamatória, e a carga viral. Salienta também a possibilidade da eutanásia em estágios precoces da doença como forma de melhorar o bem-estar animal. Para isso amostras de 34 encéfalos bovinos foram coletados para análise, conservadas em formol tamponado 10% e sob refrigeração com posterior congelamento. As amostras foram submetidas aos testes de imunofluorescência direta (IFD), isolamento viral em cultivo de células (IVCC), histopatologia com coloração de hematoxilina e eosina (HE), imuno-histoquímica (IHQ), esfregaço direto com coloração de Shorr (EDS), reação da polimerase em cadeia por transcriptase reversa (RT-PCR) e reação da polimerase em cadeia por transcriptase reversa quantitativo (qRT-PCR). As áreas utilizadas para análise foram o cerebelo, telencéfalo parietal e tálamo. Foram consideradas positivas as amostras que apresentaram Corpúsculo de Negri (CNs) ou imuno-marcação em ao menos uma das áreas analisadas. Para o estudo da intensidade das lesões histológicas, as lesões foram classificadas em graus 0, 1, 2 e 3 e a positividade do teste na presença ou ausência de CN em uma das três áreas analisadas. Para verificar a influência da evolução clínica da doença foram criados 4 grupos de análise conforme o estado clínico do animal no momento da eutanásia, sendo: M1 = animal eutanasiado em estação, M2 = eutanasiado em decúbito esternal, M3 = eutanasiado em decúbito lateral, M4 = animal com morte natural. Dos 34 encéfalos avaliados a IHQ foi positiva em 100% dos casos, a IFD foi positiva em 97,05%, sendo que na amostra negativa a presença de RABV foi confirmada por IVCC. A histologia com HE, através da visualização das CNs, foi positiva em 88,23 % dos casos, e o teste de EDS, foi positivo em 82,35%. Todas as técnicas de diagnóstico apresentaram casos positivos em todos os grupos analisados. Cada caso foi positivo em, pelo menos, dois métodos de diagnóstico. Todos os casos que continham CN foram positivos para raiva nos demais testes. Nesse estudo observou-se que as variáveis analisadas intensidade de lesão e evolução clínica no momento da eutanásia tiveram influência somente nas técnicas de HE e EDS, que se baseiam na pesquisa do CN para formação do diagnóstico, mas não interferiram na eficácia do diagnóstico realizado através da detecção do antígeno viral realizado por IFD e IHQ. Todas as amostras RABV isoladas incluídas no presente estudo apresentam linhagem genética característica de morcegos hematófagos Desmodus rotundus. A avaliação de qRT-PCR demostrou que a quantidade de vírus não interferiu na positividade dos testes. Esse trabalho mostra que a IHQ e a IFD são técnicas seguras de diagnóstico e que mesmo em animais eutanasiados em estágios iniciais de evolução clínica com intensidades leve de lesões histológicas, são capazes de detectar o RABV.(AU)