Seizure as Presenting Symptom of Multisystem Inflammatory Syndrome in Children.

We describe the case of a 13-year-old girl who presented with a new-onset seizure and fever and subsequently developed severe cardiac dysfunction, coronary artery dilation, and shock due to the surprising diagnosis of multisystem inflammatory syndrome in children (MIS-C). Although the clinical entity we now call MIS-C was first mentioned in the medical literature in April 2020, the full picture of this disease process is still evolving. Neurologic involvement has been described in cases with MIS-C; however, seizures are not a typical presenting symptom. Additionally, because children infected with SARS-CoV-2 are often asymptomatic, a documented preceding COVID-19 infection might not be available to raise suspicion of MIS-C early on. Febrile seizures, meningitis, and encephalitis are childhood illnesses that pediatricians are generally familiar with, but associating these clinical pictures with MIS-C is uncommon. Given the possibility of rapid clinical cardiogenic decline, as seen in our patient, a prompt diagnosis and appropriate monitoring and treatment are of utmost importance. This case report aims to raise awareness that new-onset seizures with fevers can be early or the first presenting symptoms in children with MIS-C, and further workup and close monitoring may be required.

[Autoimmune encephalitis in pediatric population]. / Encefalitis autoinmune en población pediátrica.

Autoimmune encephalitis can be defined as central nervous system inflammation, secondary to multiple causes, where we can possibly identify the formation of auto-antibody against neurotransmitter receptors or neuronal surface proteins. Approximately 50% of patients are seropositive; the auto-antibody against N-methyl-D-aspartate receptor (NMDAR) are the most common. In the pediatric population, the clinical presentation is characterized by movement disorders and seizures, psychiatric manifestations are more commonly found in young adults. An early intervention is associated with a better prognosis in these patients. In contrast to the seropositive group, seronegative autoimmune encephalitis is linked with less movement alterations and is related with a worse cognitive outcome. Much remains to be discovered about possible etiologies, molecular processes, detection, and interaction of yet undescribed antibodies,as well as increasing our knowledge about clinical manifestations in early disease and new diagnostic techniques that could improve the diagnosis of autoimmune encephalitis. The main goal of this document is to review the updates of the molecular field about the antibody against GluK2 and its clinical presentation in pediatric population; COVID-19 as a possible cause of autoimmune encephalitis; recognize the importance of psychiatric manifestation in early disease, especially catatonia as a marker of severity; additionally consider new imaging diagnostic method such as positron emission tomography (PET), which has shown to be more sensible than MRI (goal standard).

La encefalitis autoinmune se puede definir como el proceso inflamatorio del sistema nervioso central, secundario a múltiples causas donde se consigue identificar o no, la creación de auto-anticuerpos contra receptores de neurotransmisores o proteínas de la superficie neuronal. Aproximadamente un 50% de pacientes son seropositivos, el anticuerpo contra el receptor N-metil-D-aspartato (NMDAR) es encontrado con mayor frecuencia. La presentación clínica característica en la población pediátrica es la alteración del movimiento, seguido por episodios convulsivos; en edades más avanzadas, priman las manifestaciones psiquiátricas. Muestra buen pronóstico si se impone un pronto tratamiento. En la encefalitis autoinmune seronegativa, se observa una menor alteración de movimiento, la cual, contrario al grupo seropositivo, se asocia con peor pronóstico cognitivo. Falta mucho por conocer sobre las posibles etiologías, procesos moleculares, la interacción y detección de anticuerpos aún no descritos,al igual que es necesario incrementar nuestro conocimiento sobre las manifestaciones clínicas en etapa temprana de la enfermedad e investigar propuestas que podrían mejorar el diagnóstico de la encefalitis autoinmune. El objetivo de este documento es revisar las actualizaciones en el ámbito molecular sobre el nuevo anticuerpo descrito (GluK2) y su presentación clínica en la población pediátrica; COVID-19 como posible causa del desarrollo de encefalitis autoinmune; reconocer la importancia de las manifestaciones psiquiátricas en etapa temprana, en especial la catatonia como marcador de gravedad, de igual manera, considerar nuevas propuestas para el diagnóstico de encefalitis autoinmune como: tomografía por emisión de positrones (PET), que ha mostrado mayor sensibilidad al detectar anomalías cerebrales que la RMN (estudio de elección).

[Pediatric therapeutic options in autoimmune neurologic diseases]. / Opciones terapéuticas para enfermedades neurológicas autoinmunes en pediatría.

The pediatric neuroimmunology field has made significant progress in the last decade. Now, is possible to recognize primary demyelinating diseases, paraneoplastic syndromes, inflammatory (vasculitis), and granulomatous disorders that affect the central nervous system; at the same time, it is important to exclude neurologic manifestations caused by infections, toxic agents, and metabolic problems. An early diagnosis is imperative to institute treatment as soon as possible, improving outcomes. Treatment may include both, specific drugs if the etiology has been established, as well as drugs to treat potential complications, for example anticonvulsants, anti-inflammatory drugs, transfusions, or albumin replenishment within others. The main objective of this review is to provide guidance about the therapeutic options in pediatric autoimmune neurological diseases. We review the evidence and recommendations for the use of steroids in autoimmune demyelinating diseases, acute disseminated encephalomyelitis, optic neuritis, neuromyelitis optica, multiple sclerosis, among others. We will focus on current therapies, including high doses of intravenous methylprednisolone, followed by its progressive reduction, as well as intravenous immunoglobulin or plasmapheresis as second line therapies. Early institution of these treatments can save the patient's life and decrease their risk of permanent disability.

El campo de la pediatría neuro-inmunológica ha progresado significativamente en la última década. Ahora es posible reconocer con prontitud enfermedades desmielinizantes primarias, síndromes para-neoplásicos, enfermedades inflamatorias, autoinmunes y granulomatosas, que afectan el sistema nervioso central. Excluir con gran rapidez posibles causas infecciosas, agentes tóxicos, problemas metabólicos que se presenten con manifestaciones neurológicas es imperativo, ya que al hacer un diagnóstico preciso y temprano del paciente se puede instituir un tratamiento lo más pronto posible e incrementar las probabilidades de éxito. El tratamiento puede ser dirigido a la etiología específica, si se conoce. Adicionalmente, es importante tratar las complicaciones relacionadas a la propia enfermedad o efectos secundarios de los tratamientos que se impongan. El tratamiento puede incluir tanto fármacos específicos si se ha establecido la etiología, así como medicamentos para tratar posibles complicaciones, por ejemplo, anticonvulsivos, antiinflamatorios, transfusiones, o reposición de albúmina dentro de otros. El objetivo principal de esta revisión es brindar una guía sobre las opciones terapéuticas en enfermedades neurológicas autoinmunes en fase aguda. Revisamos la evidencia y recomendaciones acerca del uso de esteroides en enfermedades autoinmunes desmielinizantes, encefalomielitis aguda diseminada, neuritis óptica, neuromielitis óptica, esclerosis múltiple, entre otras, donde altas dosis de metilprednisolona, seguida por su disminución progresiva son esenciales, así como el uso de inmunoglobulina humana intravenosa y plasmaféresis, como tratamiento de segunda línea. La institución temprana de estos tratamientos puede salvar la vida del paciente y disminuir su discapacidad permanente.

Professional and Demographic Profile of Spanish-Speaking Child Neurologists in the United States.

>Objective: To ascertain the prevalence of culturally native Spanish-speaking child neurologists in the United States. Methods: Prevalence statistics regarding demographic and work profile were applied to data obtained from a cross-sectional electronic survey of Child Neurology Society (CNS) members. Results: Demographics of the 135 respondents were comparable to a similar CNS survey except for ethnicity as shown in Table 1. Fifty- three percent were male and 24% were over age 60. Approximately a quarter were represented each from East, South, Midwest, and Western US. 42% self-identified as Spanish, Hispanic, or Latino. 62% spoke English as their primary language and 39% spoke Spanish as their primary language. Two-thirds graduated from a US medical school, 51% practice general neurology, and epilepsy was the most common subspecialty (18%). Two-thirds of respondents practice at a major teaching hospital, and 93% hold university academic appointments. 79% are AAN members. 76% did not have medical student debt at the time of the survey. 29% report signs consistent with burnout. 87% would choose Child Neurology again and 96% would recommend Child Neurology to a medical student. Conclusion: 40% of survey respondents self-identified as Hispanic, Latino or Spanish and spoke Spanish as the primary language and the majority practice in Academic Medicine. Nearly a third of those in the current survey identify burnout symptoms. Consideration of distinctive language and cultural characteristics across the US may lead to provision of a more patient-centered and equitable care.

"Your Child Has Cerebral Palsy": Parental Understanding and Misconceptions.

IMPORTANCE: Caregivers of children with cerebral palsy can best help their child if they understand the disorder and the correct terminology. OBJECTIVE: To assess caregiver understanding of cerebral palsy. DESIGN: This was a cross-sectional study from a large tertiary medical center in Boston, to assess understanding of the term cerebral palsy by primary caregivers of children and adolescents with cerebral palsy. All cases were obtained from hospital electronic medical records. Telephone surveys were conducted. Caregiver understanding of cerebral palsy was assessed by open-ended responses (50%) and success in answering true/false questions about cerebral palsy (50%). PARTICIPANTS: Primary caregivers of children 18 years and younger with cerebral palsy. RESULTS: Thirty-three percent of caregivers denied ever being told that their child had cerebral palsy. Most caregivers identified cerebral palsy as a brain problem (79%), lifelong condition (73%), often caused by a perinatal (60%) or gestational (40%) insult. Fifty-two percent knew that cerebral palsy was nonprogressive. Sixty-two percent of caregivers believed they had a good, very good, or excellent understanding of cerebral palsy, whereas the investigators found 69% of caregivers had a good, very good, or excellent understanding of cerebral palsy (P = .006). Most caregivers rated very good or excellent the setting where cerebral palsy was discussed (58%), the explanations provided (55%), and the amount of time spent (45%), yet using a Pearson correlation coefficient, most important was the time spent (r = 0.53). CONCLUSIONS: Following discussion with their child's physician, most primary caregivers of children with cerebral palsy have a good, very good, or excellent understanding of cerebral palsy. Most critical to a good understanding of cerebral palsy was the time spent in explaining the diagnosis.

Ages and Stages Questionnaire as a screening tool for developmental delay in Indian children.

OBJECTIVE: To evaluate the ability of Ages and Stages Questionnaire, a parent completed developmental screening questionnaire to detect developmental delay in Indian children. DESIGN: Cross-sectional study. SETTING: Child Development Clinic of a tertiary care center located in Northern India. PARTICIPANTS AND METHODS: 200 children, 50 each in the age groups of 4±1, 10±1, 18±1 and 24±1 months were recruited (20 high risks and 30 low risks in each age group). The Ages and Stages Questionnaire (ASQ) was translated into Hindi and administered to the parents, followed by standardized development assessment using Developmental Assessment Scale for Indian Infants (DASII). RESULTS: 102 (51%) children failed on ASQ and 90 (45%) children failed on DASII. The overall sensitivity of ASQ for detecting developmental delay was 83.3% and specificity was 75.4%. The sensitivity was best for the 24-months questionnaire (94.7%) and specificity was best for the 4-month questionnaire (86.4%). The sensitivity of ASQ was much higher in the high risk group (92.3%) as compared to the low risk group (60%). CONCLUSION: ASQ has strong test characteristics for detecting developmental delay in Indian children, especially in high risk cases. It may be easily converted into other Indian languages and used widely for developmental screening.