Smell and Taste Dysfunction in Pediatric Patients With SARS-CoV-2 Infection.

INTRODUCTION: Anosmia and hypogeusia are frequent symptoms in severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection in adults, but their incidence in children is unknown. OBJECTIVE: Describe the incidence and associated characteristics of olfactory and gustatory dysfunction in children with SARS-CoV-2 infection. MATERIAL AND METHODS: Descriptive study carried out by telephone survey of patients aged between five and 18 years with SARS-CoV-2 infection confirmed between March and December, 2020. RESULTS: Two hundred eighty Spanish patients (female: 42.2%) with a mean age of 10.4 years (±3.54, range: 5 to 17) were analyzed, 22.5% with other diseases (mostly respiratory: 11.8%). The most frequent symptoms were fever (55.36%) and neurological symptoms (45.7%). Forty-four (15.7%) were hospitalized due to the infection, in intensive care unit (ICU): 7.1%. Forty-five patients (16.1%) had anosmia and/or hypogeusia: 32 both, eight with hypogeusia only, and five with exclusively anosmia. The mean symptom duration in days for anosmia was 36.4, and for hypogeusia it was 27.6. Either symptom was the initial manifestation in 15 patients. None had anosmia/hypogeusia with no other symptoms. Anosmia/hypogeusia was related to the presence of respiratory infection, gastroenteritis, chills, odynophagia, myalgia, asthenia, and anorexia, but not severity (hospitalization/ICU admission). Cohabitation with another infected individual was associated with a higher incidence of anosmia/hypogeusia (P = 0.041) and duration of anosmia (P = 0.006). The presence of anosmia/hypogeusia in cohabitants was associated with longer duration of anosmia (P < 0.001). CONCLUSIONS: The incidence of anosmia/hypogeusia in children with SARS-CoV-2 was lower than that reported in adults, although with a longer duration. Although no association was found between anosmia/hypogeusia and greater disease severity, recognition of these symptoms could help identify paucisymptomatic patients.

Efficacy of Brivaracetam in children with epilepsy.

BACKGROUND AND OBJECTIVES: To determine the efficacy, tolerance, and safety of BRV in children with epilepsy. METHODS: A retrospective study of patients with epilepsy who received treatment with BRV before age 16 years and underwent a minimum follow-up of 3 months. METHOD AND RESULTS: Sixty-six patients were included in the study. Patients received BRV at a mean age of 8.8 years (range 1-16 years). The majority (93.4 %) had refractory epilepsy, 27 with epileptic encephalopathy. The median maximum dose used was 4.3 mg/kg/day. In 30.3 % of the cases, seizure frequency was reduced by over 50 %, and 9 % remained seizure-free. Greater efficacy was observed in those patients who received higher doses and when a direct switch from levetiracetam (LEV) to BRV was performed. The ineffectiveness of LEV was not related to a failure to respond to BRV treatment. Side effects were identified in 24.2 % of the cases, the most frequent being irritability and drowsiness. CONCLUSIONS: BRV appears to be an effective, safe, and well-tolerated AED in children with refractory epilepsy.

Recommendations for the radiological diagnosis and follow-up of neuropathological abnormalities associated with tuberous sclerosis complex.

Tuberous sclerosis complex (TSC) is a genetic condition with multisystem involvement, characterized by the development of tumors and other abnormalities in organs such as the brain, retina, skin, heart, kidneys, and lungs. Most patients have neuropathological abnormalities such as cortical tubers, white matter radial migration lines, subependymal nodules, and subependymal giant cell astrocytomas (SEGAs). These lesions are associated with different neurological manifestations that are frequently associated with TSC. These manifestations consist of epilepsy, intellectual disability, and neurobehavioral and psychiatric problems, including autism spectrum disorder. Hydrocephalus may also develop in patients with SEGAs due to ventricular obstruction, when this usually slow-growing tumor reaches sufficient size. Surgery has been the classical approach to treat SEGAs, although this treatment is associated with substantial morbidity and does not completely prevent tumor recurrence. Recently, the mammalian target of rapamycin (mTOR) inhibitor, everolimus, has been approved by the Food and Drug Administration and the European Medicines Agency for the treatment of patients with SEGAs associated with TSC. However, the treatment of SEGAs with these agents requires the development of guidelines that establish a differential diagnosis between SENs and SEGAs, in which neuroradiological examinations play an essential role. With the aim of improving the neuroradiological diagnosis and follow-up of the neuropathological abnormalities associated with TSC, a group of experts in this field has reviewed different aspects related to these issues and put together, a series of statements and recommendations intended to provide guidance to specialists involved in the management of TSC.

Seizure and developmental outcomes after hemispherectomy in children and adolescents with intractable epilepsy.

PURPOSE: The aim of this study is to describe a series of pediatric hemispherectomies, reviewing pathologic substrate, epilepsy characteristics and seizure outcome as well as developmental profiles, before and after surgery, in different domains. METHODS: Seventeen patients with full pre-surgical work-up, minimum follow-up of 12 months, and at least one post-surgical neuropsychological evaluation were selected. Three had Rasmussen encephalitis (RE), five hemispheric malformations of cortical development (MCD), and nine hemispheric vascular lesions. RESULTS: At latest follow-up, all patients with RE and 66.7 % of those with vascular lesions are in Engel's class I; in the latter group, pre-surgical independent contralateral EEG discharges statistically correlated with a worse seizure outcome. Patients with MCD showed the worst seizure outcome. Pre-surgical language transfer to the right hemisphere was confirmed in a boy with left RE, operated on at 6 years of age. Patients with MCD and vascular lesions already showed severe global developmental delay before surgery, which persists afterwards. A linear correlation was found between earlier age at surgery and better outcome in personal-social, gross motor, and adaptive domains, in the vascular lesions group. The case with highest cognitive improvement had continuous spike and wave during sleep on pre-surgical EEG. CONCLUSIONS: Pathologic substrate was the main factor related with seizure outcome. In children with MCD and vascular lesions, although developmental progression is apparent, significant post-surgical improvements are restricted by the severity of pre-surgical neuropsychological disturbances and a slow maturation. Early surgery assessment is recommended to enhance the possibilities for a better quality of life in terms of seizure control, as well as better autonomy and socialization.

Síndrome de deleción de genes contiguos en Xp21 / No disponible

No disponible