Hillary A. Shurtleff

Family functioning and children's academic performance and behavior problems in the year following traumatic brain injury

This study examined the roles of preinjury family and child functioning and injury severity in predicting 1-year outcomes and changes in academic performance and behavioral problems following childhood traumatic brain injury (TBI). Families of 94 children (ages 6 to 15) with TBI (mild = 50, moderate = 25, severe = 19) were consecutively enrolled from emergency departments of two regional medical centers. Standardized measures of family and child functioning and interviewer ratings were completed within 3 weeks of injury (measuring preinjury status), at 3 months, and 1 year. Mean ratings of preinjury child functioning were within normal range. Whereas injury severity was associated with substantial declines in academic functioning, there was no association of injury severity with change in behavior problems. Interview ratings showed declines at all severity levels, however. Poor academic and cognitive outcomes at 1 year were associated with injury severity and, to a lesser degree, poor preinjury family and child functioning. In contrast, most of the variation in behavioral outcomes was explained by preinjury child or family factors. Preinjury functioning must be assessed and support services provided for optimal academic and behavioral outcomes following childhood TBI.

Family functioning and injury severity as predictors of child functioning one year following traumatic brain injury

This study examined changes in childrens functioning in the year following traumatic brain injury (TBI) and the preinjury family and injury factors most predictive of childrens overall adaptive functioning and social competence at 1 year. Ninety-four children with TBI (mild = 50, moderate = 25, severe = 19) and their families were consecutively enrolled from two regional medical centers. The age range was from 6 years to 15 years. Interviewer ratings and standard measures of family and child functioning were completed within 3 weeks of injury (measuring preinjury status), at 3 months and 1 year. Mean preinjury parent and teacher ratings of child functioning were within normal range. Older children (> or = 12 years) had worse preinjury functioning than younger children. Declines in child functioning were significantly associated with injury severity. Mild and moderately injured children had few declines in overall functioning. Severely injured children had the most dramatic early declines and improved only slightly between 3 months and 1 year; however, older children from poorly functioning families deteriorated in the same period. Injury severity and preinjury family functioning explained from 25% to 39% of the variation in child functioning at 1 year and up to 57% when the childs preinjury status was included. Children at risk for poorer adaptation following TBI can be identified and for optimal recovery should receive appropriate support services for optimal recovery.

Severity of pediatric traumatic brain injury and neurobehavioral recovery at one year--a cohort study.

As part of an ongoing longitudinal cohort study of children with mild, moderate, and severe traumatic brain injury and their matched controls, the neurobehavioral status of 94 case-control pairs was assessed one year after initial postinjury testing. There was a statistically significant dose-response association of severity with performance in all six domains of neurobehavioral functioning (intelligence, adaptive problem solving, memory, academic performance, motor performance, and psychomotor problem solving) with Spearman correlation coefficients of up to -.35, p < .001. The strongest correlations between severity and outcome were in the domains of intelligence, academic performance, and motor performance. Recovery over the year was also dependent on the severity of brain injury. Because mildly injured cases had negligible initial deficits, recovery was not at issue. However, for moderately and severely injured children, the degree of initial impairment was related to the magnitude of both recovery and residual deficit. Results showed that the use of population normative values to evaluate impairment was misleading. Although the mean scores of all severity groups fell within the normal range of standardized tests, the means for the moderately and severely injured were substantially below those of their matched controls on many tests.

Use of dexmedetomidine in awake craniotomy in adolescents: report of two cases

Awake craniotomy is a key tool in resection of lesions near critical functional regions, particularly the speech area. Craniotomy with an awake portion for mapping may be performed in carefully selected adolescents and preteenaged children. A number of different regimens may be used for sedation and anesthesia in these cases. We describe two adolescent patients in whom awake craniotomy was performed using an intravenous anesthesia technique with dexmedetomidine and without need for airway instrumentation.

Functional magnetic resonance imaging for presurgical evaluation of very young pediatric patients with epilepsy

OBJECT The authors describe their experience with functional MR (fMR) imaging in children as young as 5 years of age, or even younger in developmental age equivalent. Functional MR imaging can be useful for identifying eloquent cortex prior to surgical intervention. Most fMR imaging clinical work has been done in adults, and although children as young as 8 years of age have been included in larger clinical series, cases in younger children are rarely reported. METHODS The authors reviewed presurgical fMR images in eight patients who were 8 years of age or younger, six of whom were 5 or 6 years of age. Each patient had undergone neuropsychological testing. Three patients functioned at a below-average level, with adaptive functioning age scores of 3 to 4 years. Self-paced finger tapping (with passive movement in one patient) and silent language tasks were used as activation tasks. The language task was modified for younger children, for whom the same (not novel) stimuli were used for extensive practice ahead of time and in the MR imaging unit. Patient preparation involved techniques such as having experienced staff present to work with patients and providing external management during imaging. Six of eight patients had extensive training and practice prior to the procedure. In the two youngest patients, this training included use of a mock MR unit. RESULTS All cases yielded successful imaging. Finger tapping in all seven of the patients who could perform it demonstrated focal motor activation in the frontal-parietal region, with expected activation elsewhere, including in the cerebellum. Three of four patients had the expected verb generation task activations, with left-hemisphere dominance, including a 6-year-old child who functioned at the 3-year, 9-month level. The only child (an 8-year-old) who was not prepared prior to the imaging session for the verb generation task failed this task due to movement artifact. CONCLUSIONS Despite the challenges of successfully using fMR imaging in very young and clinically involved patients, these studies can be performed successfully in children with a chronological age of 5 or 6 years and a developmental age as young as 3 or 4 years.

Cognitive and Neuropsychological Correlates of Academic Achievement: A Levels of Analysis Assessment Model

Regression techniques were used to evaluate whether combining a subtest of a cognitive battery and a subtest of a neuropsychological battery contributes to educational asessment. The Wechsler Intelligence Scale for Children-Revised (WISC-R), the Halstead Reitan Battery for Older Children (9-14), the Wide Range Achievement Test, and the Analytical Reading Inventory were administered to 49 children aged 10 to 12 referred to a medical center for assessment of school learning problems. Correlations between subtests in the cognitive battery (WISC-R) and the neuropsychological battery (Halstead-Reitan) indicated both common and unique variance between these batteries. When combinations of a cognitive and a neuropsychological subtest were compared to a single cognitive or neuropsychological subtest alone, significantly more variance in word decoding, reading comprehension, and arithmetic was explained by combinations than by single subtests. These findings show that cognitive and neuropsychological subtests are not redundant and that inclusion of both improves educational assessment. Both cognitive and neuropsychological testing are needed for inferences about levels of function within working brain systems related to school achievement.

Developmental changes in interrelationships of visible language codes, oral language codes, and reading or spelling

Abstract Structural equation modeling (EQS, Bentler 1985) was used to analyze covariance structures incorporating a visible language factor underlying memory for a word and memory for a letter in a word, an oral language factor underlying access to phonemic and semantic codes, and a reading or spelling factor underlying letterby-letter and whole word presentation on tasks dependent on both visible and oral language codes. Competing theoretical models, one with the visible-oral language covariance set to zero and the other with this covariance freely estimated, were evaluated for two reading tasks (lexical decision or naming) and one spelling task (written reproduction) at the beginning (2nd month) and end (8th month) of first grade ( N = 42). At the beginning of first grade, allowing a covariance between the visible and oral language factors improved the fit, but at the end of first grade it did not. When the covariance between the visible language and oral language factors was set to zero at the end of first grade, the direct effect of the visible language factor was significant for only the naming task. Results provide evidence for (a) developmental differentiation of the visible language and oral language systems as children gained in reading skill, and (b) the importance at the end of first grade of the visible language factor in prelexical but not postlexical processes.

Impact of epilepsy surgery on development of preschool children: identification of a cohort likely to benefit from early intervention

OBJECT Outcomes of focal resection in young children with early-onset epilepsy are varied in the literature due to study differences. In this paper, the authors sought to define the effect of focal resection in a small homogeneous sample of children who were otherwise cognitively intact, but who required early surgical treatment. Preservation of and age-appropriate development of intelligence following focal resection was hypothesized. METHODS Cognitive outcome after focal resection was retrospectively reviewed for 15 cognitively intact children who were operated on at the ages of 2-6 years for lesion-related, early-onset epilepsy. Intelligence was tested prior to and after surgery. Effect sizes and confidence intervals for means and standard deviations were used to infer changes and differences in intelligence between 1) groups (pre vs post), 2) left versus right hemisphere resections, and 3) short versus long duration of seizures prior to resection. RESULTS No group changes from baseline occurred in Full Scale, verbal, or nonverbal IQ. No change from baseline intelligence occurred in children who underwent left or right hemisphere surgery, including no group effect on verbal scores following surgery in the dominant hemisphere. Patients with seizure durations of less than 6 months prior to resection showed improvement from their presurgical baseline in contrast to those with seizure duration of greater than 6 months prior to surgery, particularly in Wechsler Full Scale IQ and nonverbal intelligence. CONCLUSIONS This study suggests that surgical treatment of focal seizures in cognitively intact preschool children is likely to result in seizure remediation, antiepileptic drug discontinuation, and no significant decrement in intelligence. The latter finding is particularly significant in light of the longstanding concern associated with performing resections in the language-dominant hemisphere. Importantly, shorter seizure duration prior to resection can result in improved cognitive outcome, suggesting that surgery for this population should occur sooner to help improve intelligence outcomes.

Advances in the Care of Children with Spina Bifida

Susan D. Apkon, MD*, Richard Grady, MD, Solveig Hart, PT, MSPT, PCS, Amy Lee, MD, Thomas McNalley, MD, MA, Lee Niswander, PhD, Juliette Petersen, MS, Sheridan Remley, PT, DPT, Deborah Rotenstein, MD, Hillary Shurtleff, PhD, Molly Warner, PhD, ABPP-CN, William O. Walker Jr, MD Rehabilitation Medicine, University of Washington, Seattle, WA, USA; Rehabilitation Medicine, Seattle Children’s Hospital, 4800 Sand Point Way Northeast, M/S OB-8414, Seattle, WA 98105, USA; Section of Pediatric Urology, Seattle Children’s Hospital, University of Washington School of Medicine, 4800 Sand Point Way Northeast, Seattle, WA 98105, USA; Rehabilitation Services, Seattle Children’s Hospital, 4800 Sand Point Way Northeast, Seattle, WA 98105, USA; Pediatric Neurosurgery, Seattle Children’s Hospital, University of Washington, 4800 Sand Point Way Northeast, M/S W7729, PO Box 5371, Seattle, WA 98105, USA; Rehabilitation Medicine, Seattle Children’s Hospital, University of Washington, 4800 Sand Point Way Northeast, M/S OB-8404, Seattle, WA 98105, USA; Department of Pediatrics, Children’s Hospital Colorado, Howard Hughes Medical Institute, University of Colorado School of Medicine, Mail Stop 8133, Building RC1 South, Room L18-12106, 12801 East 17th Avenue, Aurora, CO 80045, USA; Molecular Biology Program, University of Colorado Denver Anschutz Medical Campus, Mail Stop 8133, Building RC1 South, L18-12400D, 12801 East 17th Avenue, Aurora, CO 80045, USA; Pediatric Endocrinology, Endocrine Division, Pediatric Alliance, 1789 South Braddock Avenue, Suite 294, Pittsburgh, PA 15218, USA; Department of Neurology, University of Washington School of Medicine, Seattle, WA, USA; Department of Child Psychiatry, Seattle Children’s Hospital, 4800 Sand Point Way Northeast, Seattle, WA 98105, USA; Neuropsychology Consult Service, Department of Psychiatry, Seattle Children’s Hospital, 4800 Sand Point Way Northeast, Seattle, WA 98105, USA; Division of Developmental Medicine, Seattle Children’s Hospital, University of Washington School of Medicine, 4800 Sand Point Way Northeast, M/S OC.9.940, Seattle, WA 98105, USA

High-fat diets and seizure control in myoclonic-astatic epilepsy: A single center's experience

PURPOSE To determine the efficacy of the Modified Atkins Diet (MAD) and Ketogenic Diet (KD) in seizure control within a population of myoclonic-astatic epilepsy (MAE) patients. METHODS This was a retrospective, single center study evaluating the seizure control by high fat diets. Seizure diaries kept by the parents performed seizure counts. All patients met the clinical criteria for MAE. RESULTS Nine patients met the clinical criteria. We found that both the MAD and KD were efficacious in complete seizure control and allowed other medications to be stopped in seven patients. Two patients had greater than 90% seizure control without medications, one on the KD and the other on the MAD. Seizure freedom has ranged from 13 to 36 months, and during this time four patients have been fully weaned off of diet management. One patient was found to have a mutation in SLC2A1. CONCLUSION Our results suggest that strictly defined MAE patients respond to the MAD with prolonged seizure control. Some patients may require the KD for seizure freedom, suggesting a common pathway of increased requirement for fats. Once controlled, those fully responsive to the Diet(s) could be weaned off traditional seizure medications and in many, subsequently off the MAD or KD.