Pediatric neurology
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Pediatric neurology · Feb 1994
Cerebrovascular lesions in infants and children dying after extracorporeal membrane oxygenation.
The neuropathologic autopsy findings of a group of infants and children at Children's Hospital of Pittsburgh who died after treatment with extracorporeal membrane oxygenation (ECMO) were reviewed and tabulated. The study surveyed an 11-year period (February, 1980 to May, 1991); of 268 children receiving ECMO therapy for severe cardiopulmonary failure, 94 patients died, 70 of whom were autopsied and permission for brain examination was granted in only 44. ⋯ The frequency of ischemic and hemorrhagic brain lesions was similar among neonates and older infants and children. This documentation of cerebrovascular lesions in children dying after ECMO may provide a better understanding of potential brain damage in the larger population of infants and children who survive this invasive procedure.
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To determine whether disturbance of myelination is a pathophysiologic feature in patients with treated maple syrup urine disease (MSUD), neurophysiologic studies were performed in 10 MSUD patients ages 4-16 years. Afferent and efferent pathways were studied by visual evoked potentials, somatosensory evoked potentials, motor evoked potentials, stance-stabilizing reflexes, and peripheral nerve conduction velocity. Magnetic resonance imaging was used to detect possible cerebral white matter abnormalities. ⋯ The neurophysiologic findings were not consistently correlated to the neurologic outcome of the patients. Magnetic resonance imaging did not reveal major abnormalities and demonstrated bilateral periventricular high intensity periventricular signals on T2-weighted images in 4 of 10 patients. It is concluded that dysmyelination is not a major pathophysiologic feature in patients with MSUD.
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Pediatric neurology · Jan 1993
Guillain-Barré syndrome in childhood: natural course and efficacy of plasmapheresis.
Eight children with Guillain-Barré syndrome were treated with plasmapheresis. Retrospective comparisons were made with 11 historic control patients. Eight children required mechanical ventilation, 4 of whom were in the plasmapheresis group. ⋯ Discharge Guillain-Barré syndrome scores were significantly lower for those receiving plasmapheresis (P < .05). Patients in the plasmapheresis group had a decrease in the number of days of mechanical ventilation, time until motor recovery, and overall cost. Our results are consistent with published literature and indicate that plasmapheresis for childhood Guillain-Barré syndrome is a safe and effective treatment to shorten the time to recovery.
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The effects of vigabatrin were studied over a 6-month period in 43 patients with intractable epilepsy. Children with complex partial seizures, with or without secondary generalization, responded best with more than one-half achieving a greater than 50% reduction; generalized tonic-clonic seizures also improved but there was no significant change in absence or myoclonic seizures. Four patients are seizure-free on monotherapy with vigabatrin. The drug was well tolerated with few side effects.
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Pediatric neurology · Nov 1991
Case ReportsAlice in Wonderland syndrome as a presenting symptom of EBV infection.
Four Chinese patients (2 boys, 2 girls), ages 4-9 years, who had episodes of perception error of body schema and objects are reported. The metamorphopsia, which had been named Alice in Wonderland syndrome, is the major presentation. ⋯ The duration of the perception disorder ranged from 1 week to 3 months. We believe that any young child presenting with Alice in Wonderland syndrome should undergo examination for Epstein-Barr virus infection; complete recovery from the disorder can be expected.