Pediatric neurology
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Pediatric neurology · Mar 2010
Case ReportsIntracerebral blood and MRS in neonatal nonketotic hyperglycinemia.
Nonketotic hyperglycinemia is an inborn error of glycine metabolism leading to the accumulation of glycine in the brain. The neonatal form presents in the first days after birth with encephalopathy, seizures. and characteristic "hiccups." Rapid progression can lead to intractable seizures, coma, and respiratory failure. The outcome is invariably poor, and many die before age 1 year. ⋯ However, there have been no reports of intracerebral blood confounding the cerebral glycine detected on long echo time proton magnetic resonance spectroscopy. We report on an infant who presented with neonatal encephalopathy and imaging findings consistent with hypoxic-ischemic encephalopathy, as well as intracerebral hemorrhage and an abnormal glycine peak on spectroscopy. The source of the abnormal glycine on long echo time proton magnetic resonance spectroscopy was intracerebral blood, highlighting an important confounder in the interpretation of long echo time proton magnetic resonance spectroscopy in suspected nonketotic hyperglycinemia.
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Pediatric neurology · Mar 2010
Review Case ReportsDiaphragmatic paralysis associated with neonatal brachial plexus palsy.
Phrenic nerve palsy can occur in the context of neonatal brachial plexus palsy, yet neither outcomes nor definitive treatment guidelines have been established. Diaphragmatic paralysis alone in the newborn results in significant respiratory sequelae and failure to thrive. Reviewing the available literature revealed little information about the incidence of phrenic nerve palsy associated with neonatal brachial plexus palsy, or whether outcomes are associated with the severity of the brachial plexus palsy. ⋯ The severity of brachial plexus palsy failed to correlate with severity of respiratory consequences. None of the patients underwent nerve repair or reconstruction. We suggest that diaphragmatic paralysis should not be overlooked during a brachial plexus examination, and diaphragmatic paralysis in the very young may require aggressive intervention before the treatment of brachial plexus palsy.
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Pediatric neurology · Feb 2010
Comparative StudyEfficacy and safety of adjunctive levetiracetam therapy in pediatric intractable epilepsy.
To investigate the efficacy and safety of levetiracetam adjunctive therapy in childhood intractable epilepsy, data were reviewed for 130 children who had >or=4 seizures per month, whose seizures were intractable to an initial >or=2 antiepileptic drugs, and who could be monitored for at least 6 months after levetiracetam add-on. Reduction in seizure frequency and related variables were investigated. Sixty-two of the 130 patients (48%) showed a seizure reduction of >or=50%, and 28 patients (22%) became seizure-free. ⋯ Levetiracetam was discontinued in 24 children at last visit (retention rate, 82%). The most common complaint was irritability (5%), and none of the adverse events were life threatening. In conclusion, levetiracetam adjunctive therapy is effective and safe for childhood intractable epilepsy.
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Pediatric neurology · Feb 2010
Comparative StudyDiffusion features of white matter in tuberous sclerosis with tractography.
Normal-appearing white matter has been shown via diffusion tensor imaging to be affected in tuberous sclerosis complex. Under the hypothesis that some systems might be differentially affected, including the visual pathways and systems of social cognition, diffusion properties of various regions of white matter were compared. For 10 patients and 6 age-matched control subjects, 3 T magnetic resonance imaging was assessed using diffusion tensor imaging obtained in 35 directions. ⋯ Mean and radial diffusivity of the splenium of corpus callosum were higher in the tuberous sclerosis complex group. The differences in diffusion properties of white matter between tuberous sclerosis complex patients and control subjects suggest disorganized and structurally compromised axons with poor myelination. The visual and social cognition systems appear to be differentially involved, which might in part explain the behavioral and cognitive characteristics of the tuberous sclerosis complex population.
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We investigated the incidence and 30-day case-fatality of childhood stroke in Estonia, and clinical signs and risk factors of childhood stroke. A retrospective (1995-2003) and prospective study (2004-2006) of childhood stroke (arterial ischemic, hemorrhagic, and sinovenous thrombosis) and transient ischemic attack was conducted. Stroke-incidence calculation was based on the prospective study. ⋯ Risk factors were identified in 35/48 (73%) children with cerebrovascular attacks. Six children with arterial ischemic stroke (6/24, 25%) manifested more than one risk factor. The incidence rate of childhood stroke in Estonia is similar to that in earlier data.