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Disabling amnestic syndrome following stereotactic laser ablation of a hypothalamic hamartoma in a patient with a prior temporal lobectomy

Zubkov, Sarah; Del Bene, Victor A; MacAllister, William S; Shepherd, Timothy M; Devinsky, Orrin
A 19-year-old man with cortical dysplasia and intractable focal seizures underwent a right temporal lobectomy. A hypothalamic hamartoma was subsequently recognized, and he then underwent MRI-guided stereotactic laser ablation. Unfortunately, he sustained damage to the bilateral medial mammillary bodies and suffered significant memory loss. We review laser ablation therapy for hypothalamic hamartomas and the anatomy of the memory network. We postulate that his persistent memory disorder resulted from a combination of the right temporal lobectomy and injury to the bilateral medial mammillary bodies.
PMCID:4536301
PMID: 26288758
ISSN: 2213-3232
CID: 1732292

Safety of Staged Epilepsy Surgery in Children

Roth, Jonathan; Carlson, Chad; Devinsky, Orrin; Harter, David H; Macallister, William S; Weiner, Howard L
BACKGROUND:: Surgical resection of epileptic foci relies on accurate localization of the epileptogenic zone, often achieved by subdural and depth electrodes. Our epilepsy center has treated selected children with poorly localized medically refractory epilepsy with a staged surgical protocol, with at least one phase of invasive monitoring for localization and resection of epileptic foci. OBJECTIVE:: To evaluate the safety of staged surgical treatments for refractory epilepsy among children. METHODS:: Data were retrospectively collected, including surgical details and complications of all patients who underwent invasive monitoring. RESULTS:: 161 children underwent 200 admissions including staged procedures (>1 surgery during one hospital admission), and 496 total surgeries. Average age at surgery was 7y (8m-16.5y). 250 surgeries included resections (and invasive monitoring), and 189 involved electrode placement only. Cumulative total number of surgeries per patient was 2-10 (average 3). Average duration of monitoring was 10 days (1-30). There were no deaths. Follow-up ranged from 1m to 10y. Major complications included unexpected new permanent mild neurological deficits (2%/admission), CNS or bone flap infections (1.5%/admission), intracranial hemorrhage, CSF leak, and a retained strip (each 0.5%/admission). Minor complications included bone absorption (5%/admission), positive surveillance sub/epidural cultures in asymptomatic patients (5.5%/admission), non-infectious fever (5%/admission), and wound complications (3%/admission). 30 complications necessitated additional surgical treatment. CONCLUSION:: Staged epilepsy surgery, with invasive electrode monitoring, is safe in children with poorly localized medically refractory epilepsy. The rate of major complications is low, and appears comparable to that associated with other elective neurosurgical procedures.
PMID: 24149978
ISSN: 0148-396x
CID: 688012

Attention and Executive Functions in Children With Epilepsy: What, Why, and What to Do

Macallister, William S; Vasserman, Marsha; Rosenthal, Joshua; Sherman, Elisabeth
Abstract Attention and executive function deficits are a common sequelae of many neurological conditions of childhood. Those with epilepsy frequently show such deficits, as executive dysfunction is common in all epilepsy syndromes of childhood. The purpose of this article is to review what is known about attention and executive functions, including the neurological underpinnings of these skills. Then, general cognitive function and dysfunction in childhood epilepsy is discussed with a special focus on attention and executive function impairment. Finally, treatment considerations for children and adolescents with these deficits are reviewed and future directions are discussed.
PMID: 24559518
ISSN: 2162-2973
CID: 844962

Motor skills development in children with inattentive versus combined subtypes of ADHD

Vasserman, Marsha; Bender, H Allison; Macallister, William S
The relations between attention-deficit hyperactivity disorder (ADHD) and motor skills are well documented, with research indicating both early and lifelong motor deficits in children with this disorder. Despite neuroanatomical and neurodevelopmental differences, which may predict differential rates of motor impairment between ADHD subtypes, evaluation of motor skill deficits in children with different presentations are limited in scope and equivocal in findings. The present investigation evaluated early motor development history and objectively measured motor skills in children with ADHD-Inattentive subtype (ADHD-I) and ADHD-Combined subtype (ADHD-C). One hundred and one children with ADHD-I (n = 53) and ADHD-C (n = 48) were included. Variables included Full-Scale IQ (FSIQ), history of motor delays, and utilization of early intervention services, as well as objectively measured motor impairment as assessed via tasks of fine-motor coordination. No between-group differences were found for FSIQ, but differences in age emerged, with the ADHD-I group being older. No differences in early motor delays were observed, though a considerably higher percentage of children with ADHD-C demonstrated early difficulties. Surprisingly, although children and adolescents with ADHD-C reported more frequent utilization of early intervention services, those with ADHD-I exhibited greater levels of current motor impairment on objective tasks. Given the over-representation of older children in the ADHD-I group, data were reanalyzed after excluding participants older than 10 years of age. Although the between-group differences were no longer significant, more than twice the number of parents of children with ADHD-C reported early motor delays, as compared with the ADHD-I group. Overall, children with ADHD-I were more likely to exhibit current objectively measured motor impairment, possibly due to later identification, less intervention, and/or different neurodevelopmental substrates underlying this disorder subtype.
PMID: 24716873
ISSN: 2162-2973
CID: 900322

Pediatric Multiple Sclerosis: What we know and where are we headed?

Macallister, William S; Christodoulou, Christopher; Milazzo, Maria; Preston, Thomas E; Serafin, Dana; Krupp, Lauren B; Harder, Lana
Multiple Sclerosis (MS), an autoimmune mediated disease of the central nervous system, has historically been considered a disease of young adulthood. However, there has been increasing recognition that the disease can occur in adolescence and even early childhood and recent years have witnessed a surge of studies documenting the clinical features of the disease as it pertains to this young population. The purpose of this article is to review the literature on MS in childhood and adolescence, including the clinical presentation of the disease in this group, neuropathology and pathogenesis, magnetic resonance imaging findings, as well as neuropsychological and psychosocial considerations.
PMID: 22375830
ISSN: 0929-7049
CID: 203792

Multiple sclerosis

Chapter by: MacAllister, William S; Harder, Lana
in: Pediatric neuropsychology: Medical advances and lifespan outcomes by Baron, Ida Sue; Rey-Casserly, Celiane [Eds]
New York, NY, US: Oxford University Press; US, 2013
pp. 197-218
ISBN: 0-19-982932-2
CID: 334372

Clinical utility of reliable digit span in assessing effort in children and adolescents with epilepsy

Welsh, Antoinette J; Bender, H Allison; Whitman, Lindsay A; Vasserman, Marsha; Macallister, William S
The assessment of effort is an important aspect of a comprehensive neuropsychological evaluation, as this can significantly impact data interpretation. While recent work has validated the appropriateness of adult-derived cutoffs for standalone effort measures in younger populations, little research has focused on embedded effort measures in children. The present study includes 54 clinically referred children and adolescents (32 males/22 females; aged 6-17) with a confirmed diagnosis of epilepsy. Reliable Digit Spans (RDSs) were calculated and the Test of Memory Malingering (TOMM) was administered in the context of a comprehensive neuropsychological evaluation. Using a previously published RDS cutoff of
PMID: 22777141
ISSN: 0887-6177
CID: 184452

Detecting epilepsy-related cognitive problems in clinically referred children with epilepsy: is the WISC-IV a useful tool?

Sherman, Elisabeth M S; Brooks, Brian L; Fay-McClymont, Taryn B; MacAllister, William S
PURPOSE: The Wechsler Intelligence Scale for Children - Fourth Edition is the most widely used intelligence quotient (IQ) test in use today. However, despite numerous studies on IQ in childhood epilepsy, data exist almost exclusively from prior editions of the test, and no studies to date provide information on the sensitivity of specific WISC-IV scores (full-scale IQ [FSIQ], index, and subtest scores) to epilepsy-related cognitive impairments. The goal of this study was to determine the relative sensitivity of WISC-IV index and subscale scores in detecting cognitive problems in a group of clinically referred children with epilepsy compared to matched controls, and to define the relationship among WISC-IV scales, demographic factors, and epilepsy-related variables. METHODS: WISC-IV data for children with epilepsy and high seizure burden were obtained from the Alberta Children's Hospital (ACH) and the New York University Comprehensive Epilepsy Center (NYU), two tertiary care medical centers for pediatric epilepsy. All children were clinically referred and received a standard assessment including WISC-IV. Matched controls were obtained from the WISC-IV Canadian and American standardization samples. KEY FINDINGS: WISC-IV scores from 212 children were included: 106 children with epilepsy (46 girls, 60 boys; mean age 11.0 years, standard deviation [SD] 3.1; parental education 14.5 years, SD 2.8), and 106 controls matched for age, gender, ethnicity, and parental education. Of the children with epilepsy, 44 had a clearly lateralized focus on electroencephalography (EEG) involving either the right or left hemisphere (26 left, 18 right). FSIQ for the epilepsy group was significantly lower than for controls, and 36.8% of children had IQs compatible with intellectual disability (FSIQ < 70), versus <1% of controls. In children with epilepsy, Working Memory and Processing Speed Index scores were lower than those for Verbal Comprehension and Perceptual Reasoning (p < 0.01). At the subtest level, scores for children with epilepsy were highest on visual and verbal subtests measuring reasoning skills such as Matrix Reasoning, and lowest on Coding (mean 5.93, SD 3.6). In terms of percentage of children on each subtest with low scores (i.e., scores below 2 SDs from the expected normative mean of 10), the Coding subtest identified the most children (28.3%) with low scores, and the Similarities subtest identified the fewest (16%). Later age at onset and shorter epilepsy duration were both correlated with higher WISC-IV FSIQ and index scores (r correlation coefficient values ranging from 0.36 to 0.44, p < 0.0001), and number of current and previous antiepileptic drug trials were both inversely correlated with FSIQ and index scores (r -0.27 to -0.47, all p-values < 0.01). Neither the FSIQ nor the index scores were significantly related to seizure frequency. A similar pattern was found for subtest scores. No differences in FSIQ, index scores, or subtest scores were found between children with left- and right-hemisphere seizure foci, or between those with positive or negative magnetic resonance imaging (MRI) findings. SIGNIFICANCE: The WISC-IV is sensitive to epilepsy-related cognitive problems in clinically referred children with high seizure burden, particularly problems relating to expressive verbal, working memory, and processing speed difficulties. Compared to healthy children, these children have a very high rate of cognitive difficulties as assessed by the WISC-IV. The usefulness of the WISC-IV in detecting cognitive deficits in children with milder forms of epilepsy remains to be determined.
PMID: 22554239
ISSN: 0013-9580
CID: 959002

Assessment of executive functioning in childhood epilepsy: The Tower of London and BRIEF

Macallister, William S; Bender, H Allison; Whitman, Lindsay; Welsh, Antoinette; Keller, Shari; Granader, Yael; Sherman, Elisabeth M S
Children and adolescents with epilepsy are known to demonstrate executive function dysfunction, including working memory deficits and planning deficits. Accordingly, assessing specific executive function skills is important when evaluating these individuals. The present investigation examined the utility of two measures of executive functions-the Tower of London and the Behavioral Rating Inventory of Executive Functioning (BRIEF)-in a pediatric epilepsy sample. Ninety clinically referred children and adolescents with seizures were included. Both the Tower of London and BRIEF identified executive dysfunction in these individuals, but only the Tower of London variables showed significant relations with epilepsy severity variables such as age of epilepsy onset, seizure frequency, number of antiepileptic medications, etc. Further, the Tower of London and BRIEF variables were uncorrelated. Results indicate that objective measures of executive function deficits are more closely related to epilepsy severity but may not predict observable deficits, as reported by parents. Comprehensive evaluation of such deficits, therefore, should include both objective measures as well as subjective ratings from caregivers.
PMID: 21961902
ISSN: 0929-7049
CID: 172999

Neuropsychological endophenotypes in ADHD with and without epilepsy

MacAllister, William S; Vasserman, Marsha; Vekaria, Pooja; Miles-Mason, Eavan; Hochsztein, Natanya; Bender, Heidi A
Attention-deficit hyperactivity disorder (ADHD) is a frequent comorbidity in children with epilepsy. Despite similarities in behavioral manifestations of inattention and hyperactivity, it is unclear whether the neuropsychological endophenotypes of children with developmental ADHD differ from those with ADHD in the context of epilepsy. The present study compared groups of clinically referred children with both ADHD-Inattentive subtype (ADHD-I) and ADHD-Combined subtype (ADHD-C) to children with ADHD-I and ADHD-C and epilepsy on neuropsychological measures of intellectual functioning, auditory attention, working memory, and sustained attention and response inhibition. Those with ADHD and epilepsy performed more poorly on measures of intellectual function (e.g., Full-Scale IQ, Verbal IQ, Performance IQ) as well as auditory attention and working memory. Differences across the groups were also seen on a continuous performance test. Follow-up correlational analyses showed that variables such as seizure frequency and number of antiepilepsy medications predicted cognitive dysfunction in the epilepsy groups. Overall results suggest that the neuropsychological endophenotypes in developmental ADHD versus ADHD in epilepsy differ with seizure-related variables predicting cognitive dysfunction.
PMID: 23428299
ISSN: 2162-2973
CID: 271282