Auditory central pathways in children and adolescents with multiple sclerosis.

Background: Multiple sclerosis (MS) is an inflammatory demyelinating disease. Auditory evoked potential studies have demonstrated conduction and neural processing deficits in adults with MS, but little is known about the electrophysiological responses in children and adolescents.

Objective: to evaluate the central auditory pathway with brainstem auditory evoked potentials (BAEP) and long-latency auditory evoked potentials (LLAEP) in children and adolescents with MS.

Asymptomatic MRI lesions in pediatric-onset AQP4-IgG positive NMOSD.

Background And Purpose: Around 5% of all Neuromyelitis Optica Spectrum Disorders (NMOSD) cases start before 18 years of age. Clinical and radiological manifestations of AQP4-IgG positive NMOSD were revised in 2015, and the importance of neuroimaging in the diagnosis is well recognized. Neuroimaging findings in pediatric-onset NMOSD were scarcely described, and longitudinal evaluation of NMOSD lesions was only accessed in a few adult-onset cohorts.

Post-varicella arterial ischemic stroke in children and neurocognitive performance: a 4-year follow-up study.

Objective: To analyze data from children who were previously healthy and presented with post-varicella arterial ischemic stroke upon arrival when admitted to the emergency room, with focus on the clinical/laboratory aspects, and neurocognitive performance after four-year follow-up.

Methods: Seven children presenting with arterial ischemic stroke after varicella were evaluated at pediatric emergency services in the city of São Paulo (SP), Brazil. Ischemic stroke was determined by magnetic resonance imaging/magnetic resonance angiography in a topography compatible with the areas supplied by the middle cerebral or internal carotid arteries.

Auditory evoked potentials in children and adolescents with multiple sclerosis and neuromyelitis optica spectrum disorders.

Background: In children, an acute demyelinating disease may evolve as a multiphasic disease with multiple relapses, such as multiple sclerosis (MS) and neuromyelitis optica spectrum disorder (NMOSD). The Brainstem Auditory Evoked Potentials (BAEP) and Long-Latency Auditory Evoked Potentials (LLAEP) contribute to the identification of either retrocochlear changes or other central auditory nervous system (CANS) changes.

Objectives: To characterize BAEP and LLAEP in children and adolescents with MS and NMOSD and verify the diagnostic values of these potentials in each of the demyelinating diseases.

Reduced quality of life in a pediatric-onset Neuromyelitis optica spectrum disorders cohort.

Background: Neuromyelitis optica spectrum disorders (NMOSD) is a severe condition associated with high disability and low quality of life (QoL) in adults. Since this evaluation had been rarely perfomed in children, this study aimed to describe QoL in pediatric-onset NMOSD with positive aquaporin4 antibody (AQP4-IgG) patients.

Methods: This was a cross-section evaluation of patients and parents' proxy QoL from individuals enrolled in a longitudinal cohort of AQP4-IgG positive NMOSD with onset ≤ 18 years of age.

Arteriopathy in pediatric stroke: an underestimated clinical entity.

Background: Pediatric arterial ischemic stroke (AIS), which was thought to be a rare disorder, is being increasingly recognized as an important cause of neurological morbidity, thanks to new advances in neuroimaging.

Objective: The aim of this study was to review the main etiologies of stroke due to arteriopathy in children.

Methods: Using a series of cases from our institution, we addressed its epidemiological aspects, physiopathology, imaging findings from CT, MR angiography, MR conventional sequences and MR DWI, and nuclear medicine findings.

Treatment and outcome of aquaporin-4 antibody-positive NMOSD: A multinational pediatric study.

Objective: To describe the clinical phenotypes, treatment response, and outcome of children with antibodies against aquaporin-4 (AQP4-Ab) neuromyelitis optica spectrum disorder (NMOSD).

Methods: Retrospective, multicenter, and multinational study of patients with AQP4-Ab NMOSD aged <18 years at disease onset from a center in Brazil and 13 European centers. Data on demographics, clinical findings, and laboratory results were analyzed; calculation of annualized relapse rates (ARRs) pre- and on-treatment with disease-modifying therapies (DMTs) and of ORs for predictors of poor outcome was performed.

Mowat-Wilson syndrome: neurological and molecular study in seven patients.

Objective: To present a seven-cases serie of Mowat-Wilson syndrome (MWS).

Method: All patients with positive mutation for the ZEB2 were evaluated by a geneticist and a neurologist, with clinical and laboratorial characterization.

Results: A peculiar facies and mental retardation were present in all patients.

Acute hemorrhagic encephalomyelitis in childhood: Case report and literature review.

Acute disseminated encephalomyelitis (ADEM) is an inflammatory immune-mediated disorder which is more common in pediatric patients. The clinical setting is characterized by a rapid onset of encephalopathy and multifocal neurological features. Acute hemorrhagic encephalomyelitis (AHEM) is considered a rare form of ADEM.

Möbius sequence in a girl and arthrogryposis in her half-brother: distinct phenotypes caused by prenatal injuries.

Möbius sequence is a congenital facial and abducens nerve palsy, frequently associated to abnormalities of extremities. Arthrogryposis multiplex congenital is defined as a congenital fixation of multiple joints seldom of neurogenic origin. Both sequences must have a genetic origin, but usually are sporadic cases related to environmental factors such as drugs exposition and maternal trauma.

Guillain-Barré syndrome in children: clinic, laboratorial and epidemiologic study of 61 patients.

The aim of the study was to analyze the epidemiologic, clinical, laboratory and development profile of Guillain-Barré syndrome series studied at the Child Institute, between 1989 and 2000. From the 61 patients that fulfilled the selection criteria, aged between 7 months and 13 years old, no sexual or seasonal variation was observed. Clinical events prior to neurological symptoms (with an average time gap of 20.

Ketogenic diet for the treatment of refractory epilepsy: a 10 year experience in children.

Ketogenic diet (KD) is a high fat and low carbohydrate diet, which controls refractory epilepsy. We analyzed the KD effects on 54 children of the Children's Institute of the University of São Paulo. Efficacy, tolerability, and adverse effects were studied.

Spinal cord schistosomiasis in children: analysis of seven cases.

We describe seven cases of children (ages 2 to 14 years) with myeloradiculopathy caused by infection with S. mansoni. None of them presented hepatosplenic involvement and one presented an intestinal picture.