James John Riviello

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BACKGROUND Brain derived neurotrophic factor (BDNF) elevation in newborn sera predicts intellectual/social developmental abnormalities. Other autoantibodies (AAs) to endothelial cells (ECs) and myelin basic protein (MBP) are also elevated in some children. We tested relationships between BDNF, BDNF AAs, and other AAs in children with these disorders. (More)
OBJECTIVES The objectives of this study were 1) to determine the frequency of clinically significant abnormal neuroimaging in children coming to the emergency department (ED) with new-onset afebrile seizures (ASZ), and 2) to identify children at high or low risk for clinically significant abnormal neuroimaging. DESIGN/METHODS Five hundred consecutive(More)
Four children with the classic form of maple syrup urine disease (MSUD) died of cerebral edema during an intercurrent infection that caused severe dehydration and acidosis. The diagnosis of MSUD had been established during the neonatal period in all four patients, on day 1 of life in three of them. All were in satisfactory control before the intercurrent(More)
Subacute necrotizing encephalomyelopathy (SNE) is a genetic disorder of pyruvate metabolism. Until recently the diagnosis of SNE could only be made at autopsy. However, an antemortem diagnosis can now be suggested by the correlation of clinical and laboratory data with computed tomography (CT) and/or magnetic resonance imaging (MRI). Five children with(More)
Macrocrania (i.e., macrocephaly) is a common manifestation of von Recklinghausen neurofibromatosis (NF-1), occurring in approximately 30% of patients with this disorder. Megalencephaly is the usual basis for NF-1-associated macrocrania, although obstructive hydrocephalus secondary to a brain tumor or cranial vault dysplasia may occur. We describe 2 patients(More)
OBJECT Neurophysiological monitoring of motor evoked potentials (MEPs) during complex spine procedures may reduce the risk of injury by providing feedback to the operating surgeon. While this tool is a well-established surgical adjunct in adults, clinical data in children are sparse. The purpose of this study was to determine the reliability and safety of(More)
Primidone can be used for seizures refractory to standard antiepileptic drugs. We administered primidone, 25 mg/kg/day in 3 divided doses, to 10 patients and obtained serum levels of primidone, phenobarbital, and phenylethylmalonic acid at 1, 2, 4, 6, and 8 hours on day 1, alternate days until discharge, and after 6 weeks. Other antiepileptic drugs were(More)
Spinal cord injuries in children usually result from significant trauma. Two children with a delayed cervical central cord syndrome following trivial trauma are reported, one with no predisposing factor and one with a Chiari I malformation. Although dissimilar, these cases emphasize the importance of an evaluation for an anatomic defect or congenital(More)
The spectrum of clinical disease in juvenile Huntington's disease differs from that seen in adults. Younger patients often present with seizures, dystonia and rigidity. The mechanism and type of seizures, timing of onset and electrographic features have not been well characterized in either adults or children. We describe the electroencephalographic(More)
Plasmapheresis is used for treating the complications of liver failure. We performed plasmapheresis on 6 children with hepatic encephalopathy resulting from acute hepatic failure and prospectively assessed its effects on neurologic and electrophysiologic (electroencephalography and evoked potentials) function. Clinical improvement was observed in 3 of 6(More)