Pediatric neurology
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Pediatric neurology · May 2017
Multicenter StudyScoliosis in Rett Syndrome: Progression, Comorbidities, and Predictors.
Scoliosis is prominent in Rett syndrome (RTT). Following the prior report from the US Natural History Study, the onset and progression of severe scoliosis (≥40° Cobb angle) and surgery were examined regarding functional capabilities and specific genotypes, addressing the hypothesis that abnormal muscle tone, poor oral feeding, puberty, and delays or absence of sitting balance and ambulation may be responsible for greater risk in RTT. ⋯ Scoliosis requires vigilance regarding the risk factors noted, particularly specific mutations and the role of puberty and motor abilities. Bracing is recommended for moderate curves and surgery for severe curves in accordance with published guidelines for scoliosis management.
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Pediatric neurology · Jan 2016
Multicenter Study Observational StudyClinical Electroencephalographic Biomarker for Impending Epilepsy in Asymptomatic Tuberous Sclerosis Complex Infants.
We assessed the clinical utility of routine electroencephalography (EEG) in the prediction of epilepsy onset in asymptomatic infants with tuberous sclerosis complex. ⋯ Serial routine EEGs in infants with tuberous sclerosis complex is a feasible strategy to identify individuals at high risk for epilepsy. The most frequent clinical presentation was epileptic spasms followed by focal seizures, and then a combination of both seizure types.
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Pediatric neurology · Jul 2014
Multicenter StudyTherapeutic hypothermia in neonates and selective hippocampal injury on diffusion-weighted magnetic resonance imaging.
Hippocampal injury is most often observed in conjunction with basal ganglia injury after hypoxia-ischemia in term newborns. Objective was to determine perinatal characteristics leading to selective hippocampal injury vs basal ganglia injury on diffusion-weighted imaging in term encephalopathic infants following intrapartum hypoxia-ischemia treated with selective head cooling and to correlate specific injury to subsequent neurodevelopmental outcome. ⋯ Infants with hippocampal injury on diffusion-weighted imaging recovered from an intrapartum asphyxial insult more rapidly as reflected by an earlier correction of acid-base status, were less likely to need cardiopulmonary resuscitation, and were less severely encephalopathic. These findings highlight the exquisite vulnerability of the hippocampus to acute hypoxia unaffected by selective head cooling, whereas the normal appearance of the basal ganglia in these infants suggests a neuroprotective effect of cooling.
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Pediatric neurology · Apr 2014
Multicenter StudyThe role of continuous electroencephalography in childhood encephalitis.
Seizures are a known complication of encephalitis. We sought to determine the incidence of seizures and the relative utility of routine and continuous electroencephalography in children with suspected encephalitis. ⋯ Children with suspected encephalitis are at high risk for seizures. Continuous electroencephalography is better able than routine electroencephalography to determine whether seizures are present. Further, continuous electroencephalography can guide treatment by classifying a clinical event as seizure or seizure-mimic. Our findings support the expanded use of continuous electroencephalography in children with suspected encephalitis.
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Pediatric neurology · Jul 2013
Multicenter StudyCerebrospinal fluid brain injury biomarkers in children: a multicenter study.
Cerebrospinal fluid (CSF) biomarkers reflecting neuronal and astroglial injury, such as total tau (T-tau), glial fibrillary acidic protein (GFAP), and neurofilament light (NFL), have been extensively investigated in neurologic diseases in adults, but no large study has investigated these biomarkers in children. ⋯ CSF T-tau, GFAP, and NFL are differently altered across different neurologic diseases in children. Importantly, the biomarker pattern distinguishes between progressive and static neurologic disorders.