Maria Isabel Bragatti Winckler

Pediatric neurotoxocariasis with concomitant cerebral, cerebellar, and peripheral nervous system involvement: case report and review of the literature

Objective: To alert pediatricians to the neurologic consequences of toxocariasis and to describe the first pediatric case of neurotoxocariasis with concomitant cerebral, cerebellar and peripheral nervous system involvement. Description: We report a case of neurotoxocariasis in a previously healthy 5-year-old boy with unusual symptoms and multi-site involvement of both the central and peripheral nervous system. Differential diagnoses are discussed and the relevant literature is reviewed. Since the early 1950s, fewer than fifty cases have been described, mostly in adult patients. Comments: Although human toxocariasis is one of the most common zoonotic helminth infections, neurotoxocariasis is a rare condition, especially in pediatric patients. Although toxocariasis usually presents as a self-limiting disease with no central nervous system involvement, when it does occur, it can be devastating. Neurotoxocariasis should be added to the differential diagnosis of pediatric patients with unusual neurologic symptoms accompanied by high levels of eosinophils in the cerebrospinal fluid. Early diagnosis and treatment can prevent long-term neurologic sequelae.

Proteus syndrome associated with hemimegalencephaly and Ohtahara syndrome: Report of two cases

The authors report two cases of Brazilian children with most of the common syndromic features of Proteus syndrome, such as asymmetric overgrowth of tissues, skin abnormalities, hypotonia and mental retardation. In both patients, a refractory epilepsy, compatible with Ohtahara syndrome, as well as hemimegalencephaly, with asymmetric distribution of facial fat, were also diagnosed.

Prognostic factors for recurrence of a first seizure during childhood

This study was designed with the objective of evaluating the chance of recurrence in our area and to answer questions regarding prognostic factors capable of helping in the management of the first seizure in childhood. One hundred and thirty six children from 1 month to 12 years of age seen at the Pediatric Emergency Division of Hospital de Clínicas de Porto Alegre because of a first seizure with or without triggering factors were included in the study. The follow-up included 121 children. We concluded that family history of seizures, presence of triggering factors at first event, seizure type, seizure duration and paroxysmal electroencephalographic abnormalities were predictive factors for seizure recurrence. The recurrence in this sample was 36.36% during the study. Cumulative recurrence risks were 14.88%, 23.14%, 28.93%, 33.06% and 35.54% to 3, 6, 9, 12 and 15 months, respectively.

Treatment of refractory neonatal seizures with topiramate.

OBJECTIVE The objective of this study is to describe the usefulness of topiramate in refractory neonatal seizures. RESULTS We reported the clinical off-label use of topiramate in three cases of refractory neonatal seizures of unclear origin with no response to conventional antiepileptic drugs. In all cases, the seizures were completely controlled with adding topiramate. All patients became seizure free during hospitalization and were followed by approximately 1 year after hospital discharge, with monotherapy with topiramate. COMMENTS The clinical off-label use of topiramate in neonatal seizures is still incipient. When searching publications in this matter, only one report was identified. Because of its efficacy for both seizures and neuroprotection, topiramate could be a useful choice in refractory neonatal seizures.

Early diagnosis of ABO haemolytic disease of the newborn.

To assess the usefulness of cord blood tests in diagnosing ABO-haemolytic disease of the newborn (ABO-HDN), 132 term, adequate for gestational age (AGA) neonates were evaluated. The tests studied and their significant results were: quantitative elution test (≥1/16), direct Coombs test (positive), bilirubin concentration (≥4 mg/dl). In none of the 56 O+ newborn infants delivered by O+ women were the results of any test positive. Of the 76 A+ and B+ newborn infants delivered by O+ women, 17 (22%) developed ABO-HDN. When the combined result of any two tests was positive, the sensitivity, the specificity and the positive predictive accuracy for the diagnosis of ABO-HDN was higher than for any one of the isolated tests. The probablity that ABO-HDN was present when the results of at least two cord blood tests were positive was 70%, and the probablity that ABO-HDN was not present when less than two cord blood tests gave positive results was 93%. It is suggested that the combination of quantitative elution test, bilirubin concentration and direct Coombs test in the cord blood is useful for an early diagnosis of ABO-HDN.

Optic nerve enlargement and leukodystrophy : an unusual finding of the infantile form of Krabbe disease

Pediatric Neurology Unit, Hospital de Clinicas de Porto Alegre (HCPA), Porto Alegre RS, Brazil; Genetic Department, HCPA; Medical Genetic Service, HCPA; Professor of the Genetic Department, HCPA; Neuroradiology Department of Hospital Moinhos de Vento, Porto Alegre RS, Brazil; Pediatric Neurologist, Head of the Pediatric Neurology Unit, HCPA. Adjunct Professor, Universidade Federal do Rio Grande do Sul, Porto Alegre RS, Brazil; Pediatric Neurologist, Adjunct Professor, Universidade Federal do Rio Grande do Sul, Porto Alegre RS, Brazil. Leukodystrophies are a heterogeneous group of inherited neurological disorders characterized by progressive demyelination that causes loss of motor, sensory and intellectual functions leading to a fatal outcome. These disorders result from dysfunctions in myelin metabolism as a consequence of genetic enzymatic defects specific to each leukodystrophy subtype. Krabbe disease (KD), also called globoid cell leukodystrophy (GCL), is inherited in an autosomal recessive pattern and has an estimated incidence of 1 in each 100,000/200,000 live births. It affects the peripheral and central nervous system (CNS). We report on two cases of the infantile form of KD and describe MRI findings that suggest optic nerve enlargement (ONE).

Post‐H1N1 vaccine acute disseminated encephalomyelitis

Acute disseminated encephalomyelitis (ADEM) is a rare autoimmune demyelinating disorder of the central nervous system (CNS) that affects mainly children. Typically it happens after an infection and rarely after vaccination, with compromise of the white matter of the brain and/or spinal cord, characterized by a rapid development of encephalopathy in combination with multifocal and extremely varied neurological deficits. Brain and spinal cord magnetic resonance imaging (MRI) show disseminated demyelization in the CNS. Cerebrospinal fluid (CSF) examination can demonstrate unspecific alterations. We report the case of a previously healthy 8-year-old boy, who had fever, headache and somnolence 12 days after the first dose of vaccine against influenza H1N1. There was no previous history of infection or other vaccines in the previous 30 days, as well as no evidence of ongoing influenza H1N1 infection. The CSF was a crystalline fluid with 45 leukocytes/μL with 100% monocytes, glucose 62 mg/dL, and proteins 27 mg/dL. No infectious pathogens were identified in the blood or in the CSF. The patient had clinical seizures controlled with i.v. diazepam, became aphasic, and developed left hemiparesis. Electroencephalogram indicated slowing background activity with multifocal spikes and one electrographic seizure, after which i.v. phenytoin was initiated. He was transferred to the intensive care unit (ICU) due to coma and necessity of mechanical ventilation and did not have metabolic abnormalities. He was discharged from ICU with residual neurologic deficits, such as aphasia, paresis of extrinsic ocular muscles and ataxia. Brain MRI was compatible with ADEM (Fig. 1). Spine MRI showed no lesion. I.v. methylprednisolone pulse therapy (30 mg/kg per day) was given for 5 days, followed by 4 weeks of oral prednisone (2 mg/kg per day). The patient was discharged from hospital after 30 days. During follow up, a complete remission of symptoms occurred. According to the World Health Organization, in 2009 approximately 40 countries started national campaigns of vaccination against influenza A. Nearly 80 million doses of H1N1 vaccine were distributed and 65 million people were vaccinated. In this sense, rigorous surveillance of side-effects is necessary to establish the vaccine safety. ADEM usually occurs after unspecific viral upper respiratory airway infection. Only 5% are post-vaccine cases, more frequently after vaccination against measles, rubella and mumps. Only two case reports of ADEM after vaccine against H1N1 have been identified on publication database search. Obviously, vaccines are considered safe and provide one of the most cost-effective treatments. Vaccination campaigns have prevented thousands of deaths and disabling consequences, allowing the eradication of some diseases. In contrast, the worrying side-effects, although rare, must be promptly described in order to prevent undesirable events. Even recognizing that this is a description of a single case as well as that the causal association between this vaccine and the neurological alterations could not be totally proved, to our knowledge this paper represents the third description of a case of ADEM identified after H1N1 influenza vaccine. In the present case, early identification and treatment of ADEM were sufficient to ensure complete neurological recovery.

Vigor neurológico de recém-nascidos a termo segundo tipo de parto e realização de manobras obstétricas

PURPOSE to evaluate the effect of delivery type and usual obstetric procedures on the neurologic condition of a sample of consecutive term and healthy neonates, in the first 48 hours of life, using the Neurologic Adaptative Capacity Score (NACS) system. METHODS cohort prospective study with 313 neonates, from a neonatology unit: Unidade de Neonatologia e Alojamento Conjunto. The variables analyzed were obstetric variables; clinical outcome: low neurologic vigor phase, evaluated by NACS, at 4, 24 and 48 hours of life. The data have been assessed twice: once with the whole sample and the other comparing the Vigorous Group, whose neonates kept a score of 35 or more during the three evaluations, and the Low Vigor Group, with less than 35 scores during the three consecutive evaluations. Bivariate and multivariate analyses have been done. Possible associations between low neurologic vigor phase and the type of delivery, as well between the low neurologic vigor phase and obstetric variables have been searched. RESULTS in the bivariate analysis, the delivery type and the obstetric variables were not associated with the low neurologic vigor phase. Nevertheless, the association between the amniotic fluid and the low neurologic vigor phase reached values very close to significance and, then, it was included in the multivariate analysis. In the multivariate analysis, the only variable associated with low neurologic vigor was the presence of meconium stained amniotic fluid, which has shown to be 8.1 times more risky for the neurologic scoring, when Vigorous Group and Low Vigor Group were compared. In the analysis of the whole sample, the same risk was 1.7. CONCLUSIONS neither the delivery type, nor the usual obstetric procedures were associated with low neurologic vigor phase. This is useful information, clinically or legally speaking, mainly for obstetricians. According to this sample data, when the term neonate is healthy, the delivery type and the usual obstetric procedures have no impact in the neurologic condition.

Early Amplitude-Integrated Electroencephalogram as a Predictor of Brain Injury in Newborns With Very Low Birth Weight: A Cohort Study

Purpose: To evaluate the relationship between abnormal early amplitude integrated electroencephalography (EEG) and severe lesions in imaging tests performed during the neonatal period in very low birth weight infants. Methods: An amplitude-integrated EEG was performed in 70 patients with a mean birth weight of 1226 g during the first 48 hours of life. Severe lesions on magnetic resonance imaging (MRI) or ultrasonography (US) during the neonatal period were considered as adverse conditions. Variables were compared using the χ2 test or analysis of variance. Sensitivity, specificity, and positive likelihood ratio were calculated. Results: Adverse outcomes were observed in 6 patients. There was a significant relationship (P < .001) between abnormal amplitude-integrated EEG background and severe lesions on MRI and US. Sensitivity and specificity were 100% and 89%, respectively. Conclusion: Early amplitude-integrated EEG with moderate/severe abnormalities in the background is associated with severe structural lesions detected in imaging studies and should be considered as an auxiliary screening tool for the detection of neonatal brain lesions in very low birth weight infants.

The Role of Amplitude Integrated Electroencephalogram in Very Low-Birth-Weight Preterm Infants: A Literature Review

Neurological sequelae are common in very low-birth-weight preterm infants. The prevention of brain injury and development of neuroprotective strategies have been the main objectives of modern neonatal neurology. Amplitude integrated electroencephalogram (aEEG) is a continuous brain monitoring method that can aid in early diagnosis and detect patients who are at risk. While its role in the assessment of full-term newborn infants is already well established, there are still doubts about its use in preterm infants. The objective of this review was to describe the main recommendations for the use of aEEG in very low-birth-weight preterm infants and its accuracy in assessing the prognosis and detecting epileptic seizures in this population.