In November 1996, a panel of pediatric neurologists met to update the consensus statement issued in 1989 by a panel of neurologists and metabolic experts on L-carnitine supplementation in childhood epilepsy. The panelists agreed that intravenous L-carnitine supplementation is clearly indicated for valproate (VPA)-induced hepatotoxicity, overdose, and other acute metabolic crises associated with carnitine deficiency. Oral supplementation is clearly indicated for the primary plasmalemmal carnitine transporter defect. The panelists concurred that oral L-carnitine supplementation is strongly suggested for the following groups as well: patients with certain secondary carnitine-deficiency syndromes, symptomatic VPA-associated hyperammonemia, multiple risk factors for VPA hepatotoxicity, or renal-associated syndromes; infants and young children taking VPA; patients with epilepsy using the ketogenic diet who have hypocarnitinemia; patients receiving dialysis; and premature infants who are receiving total parenteral nutrition. The panel recommended an oral L-carnitine dosage of 100 mg/kg/day, up to a maximum of 2 g/day. Intravenous supplementation for medical emergency situations usually exceeds this recommended dosage.
Journal Article Verbal and Nonverbal Skill Discrepancies in Children with Hydrocephalus: A Five-Year Longitudinal Follow-Up Get access Bonnie L. Brookshire, Bonnie L. Brookshire 2 2All correspondence should be sent to Bonnie Brookshire, Department of Pediatrics, MSB 7.142. The University of Texas Medical School at Houston, 6431 Fannin, Houston, Texas 77030. Search for other works by this author on: Oxford Academic PubMed Google Scholar Jack M. Fletcher, Jack M. Fletcher Search for other works by this author on: Oxford Academic PubMed Google Scholar Timothy P. Bohan, Timothy P. Bohan Search for other works by this author on: Oxford Academic PubMed Google Scholar Susan H. Landry, Susan H. Landry Search for other works by this author on: Oxford Academic PubMed Google Scholar Kevin C. Davidson, Kevin C. Davidson University of Houston Search for other works by this author on: Oxford Academic PubMed Google Scholar David J. Francis David J. Francis University of Houston Search for other works by this author on: Oxford Academic PubMed Google Scholar Journal of Pediatric Psychology, Volume 20, Issue 6, December 1995, Pages 785–800, https://doi.org/10.1093/jpepsy/20.6.785 Published: 01 December 1995 Article history Received: 16 September 1994 Accepted: 05 June 1995 Published: 01 December 1995
Examined the relationship of hydrocephalus and behavioral adjustment in three groups of 5- to 7-year-old children (N = 84) with a history of early hydrocephalus (spina bifida, prematurity, aqueductal stenosis) and three non-hydrocephalic comparison groups (spina bifida, prematurity, normals). Results revealed no significant group differences on measures of behavioral adjustment and a variety of family and sociodemographic variables. Children with hydrocephalus were more likely to meet criteria for behavior problems, obtained lower scores on measures of adaptive behavior, and perceived themselves as less physically competent. Categorical modeling analyses showed that hydrocephalus and its treatment, gender, family variables, and motor skills were related to the presence of behavior problems.
Abstract Measures of executive functions (Tower of London, Wisconsin Card Sorting Test), focused attention (cancellation tasks), and selective attention (Stroop test) were administered to 116 school‐age children with shunted hydrocephalus, arrested (un‐shunted) hydrocephalus, and no hydrocephalus. The results revealed that children with shunted hydrocephalus solved fewer problems on the Tower of London task and achieved fewer categories on the Wisconsin Card Sorting Test than children with arrested and no hydrocephalus. However, most indices of executive functions derived from these two measures did not significantly differentiate the three groups. Children with shunted hydrocephalus also performed more poorly on the focused and selective attention tasks. However, their poorer performance was related to brain defects influencing motor speed and the transfer of information across the corpus callosum and not to specific effects of hydrocephalus. Children with hydrocephalus do demonstrate problems with attention and problem‐solving skills. These differences are not as large as those observed on measures of spatial cognition and are not readily explained by hypotheses involving frontal lobe control of executive functions.
Cranial magnetic resonance (MR) imaging was performed in 124 male patients (aged 7-19 years), from 14 institutions, in whom a diagnosis of moderate to severe hemophilia was made. Blood tests in all subjects were negative for human immunodeficiency virus. Findings in MR studies were abnormal in 25 (20.2%) subjects. Six lesions in five subjects were classified as congenital. The most commonly identified congenital lesion was a posterior fossa collection of cerebrospinal fluid (five cases). Twenty-two subjects had acquired lesions that were probably related to the hemophilia or its treatment. The most commonly acquired lesions were single- or multifocal areas of high signal intensity within the white matter on T2-weighted images noted in 14 (11.3%) subjects. Two subjects had large focal areas of brain atrophy, and six had some degree of diffuse cerebral cortical atrophy. Three subjects (2.4%) had hemorrhagic lesions. To the authors' knowledge, the unexpected finding of small, focal, nonhemorrhagic white matter lesions has not previously been reported.
Abstract This study evaluated a large sample (N = 90) of 5- to 7-year-old children with hydrocephalus caused by aqueductal stenosis or prematurity-intraventricular hemorrhage or associated with spina bifida. Comparison groups of normal controls, children with spina bifida and no shunt, and premature children with no hydrocephalus were also evaluated. Comparison of skill discrepancies at two occasions separated by 1 year revealed that hydrocephalic children, as a group, showed poorer nonverbal than verbal skills on measures from the McCarthy Scales of Children's Abilities, the WISC-R and composites of neuropsychological skills. No discrepancies in verbal-nonverbal memory were found nor were any discrepancies attributable to etiology or motor demands of the tasks. Consistent with current hypotheses concerning the role of the cerebral white matter in cognitive development, these results sho the hydrocephalic children in this age range genrally have poorer development of nonverbal cognitive skills relative to their language development.
