Eileen B. Fennell

Correlations between assessed manual laterality and predicted speech laterality in a normal population

Abstract The correlation of handedness and lateral speech representation was investigated in an adult normal population of left- and right-handers. Handedness was assessed by multivariate analysis of manual test scores. Assessment of speech laterality was based on scores derived from the Dichotic Listening Test. Results showed that: (1) self-reports of “left-handedness” were unreliable estimates of manual performance; (2) self-classified left-handers, in contrast to right-handers, varied along levels of both manual and speech laterality; (3) the association between speech and manual laterality was greater when handedness was based on multiple performance scores; (4) familial sinistrality was twice as frequent in those test-classified left- and right-handers who showed speech representation on the same side as the dominant hand.

Molar Tooth Sign in Joubert Syndrome: Clinical, Radiologic, and Pathologic Significance:

Joubert syndrome is a rare autosomal-recessive condition characterized by early hyperpnea and apnea, developmental delay, and truncal ataxia. We previously described key ocular motor signs in Joubert syndrome and the molar tooth sign resulting from dysplasia of the isthmic segment of the brain stem, superior cerebellar peduncles, and vermis. In this study, we obtained clinical and developmental data in 61 cases, and radiologic data in 46 of these, to determine the prevalence of the molar tooth sign in a large sample, and to ensure that magnetic resonance images obtained for study were representative of the Joubert syndrome population at large. We studied the morphology of the isthmic segment of the pontomesencephalic junction, the segment of the brain stem derived from the primitive isthmus. Portions of the cerebellum analyzed included the superior cerebellar peduncles, the anterior and posterior lobes of the vermis, and the flocculonodular lobe. In one case, autopsy of the brain was performed. The average age at diagnosis was 33 months. All patients were hypo-tonic and developmentally delayed. The molar tooth sign was present in 85% of cases with 13% of these showing additional malformations. All patients without the molar tooth sign had other mimicking conditions such as neocerebellar dysgenesis, isolated vermian atrophy, cerebellar aplasia, and cystic dilation of the cisterna magna. Autopsy showed aplasia of the cerebellar vermis with dysplasia of the dentate nucleus, elongated locus coeruleus, and marked dysplasia of the caudal medulla. A better understanding of the clinical, radiologic, and pathologic features of Joubert syndrome should help uncover the genetic basis for the syndrome. (J Child Neurol 1999;14:368-376).

Planum temporale asymmetry and language dominance

Language is lateralized to the left hemisphere in most individuals, and leftward asymmetry of the planum temporale has been reported in postmortem and imaging studies. In this study we measured the planum temporale on magnetic resonance imaging (MR) scans of patients (11 right-handers, 1 non-right-hander) who had selective hemispheric anesthesia or Wada testing performed for language lateralization. All subjects who had language lateralized to the left hemisphere (11 right-handers) had a leftward asymmetry of the planum temporale. One subject who had language lateralized to the right hemisphere (non-right-hander) had a strong rightward asymmetry of the planum temporale. These data suggest that planum temporale asymmetries determined by MR are associated with language dominance and may predict language laterality.

Order of Report, Ear Asymmetry and Handedness in Dichotic Listening

When different numbers were presented simultaneously to each ear, Ss tended to report all the numbers from one channel before reporting any from the other (Broadbent effect). The results contradicted Kimuras findings on the low incidence of the ear order effect, but showed that when verbal input is increased, the frequency of the effect and over-all accuracy decrease. Kimuras ear asymmetry hypothesis, however, was shown to be true for varying amounts of verbal input. The fact that the ear asymmetry occurred for recall on the delayed “storage” half-spans suggested a rapproachement between both Broadbent and Kimura. The striking relationship between handedness and ear asymmetry was discussed in relation to speech-brainedness.

The Test of Everyday Attention for Children (TEA-Ch): patterns of performance in children with ADHD and clinical controls.

The present study explores the utility of the Test of Everyday Attention for Children (TEA-Ch) as a measure of the attentional impairments displayed by children with Attention Deficit Hyperactivity Disorder (ADHD). Sixty-three children with ADHD and 23 non-ADHD Clinical Control children were compared on subtests of the TEA-Ch reflecting three attentional domains: sustained, selective, and attentional control. Results show that children with ADHD performed significantly worse than clinical controls on subtests of sustained attention and attentional control. The groups did not differ, however, on subtests of selective attention. These findings suggest that the TEA-Ch is sensitive to attentional deficits unique to ADHD and holds promise as a useful tool in the assessment of ADHD. Performance patterns and future directions are discussed.

Cognitive and Neuropsychological Functioning in Children With Cerebral Palsy

This article reviews the extant literature on intellectual functioning in different subtypes of cerebral palsy. Following a definition of the characteristics of each of three major cerebral palsy groups, typical neurologic and magnetic resonance imaging findings are reported. More recent studies that examine the intellectual and neuropsychological functioning of children within these classification groups are also reviewed. This review concludes that there remains a significant lack of precise information about the impact of cerebral palsy on the intellectual, motor, and neuropsychological functioning of children and that neuropsychological assessment can provide the necessary tools for such studies. (J Child Neurol 2001;16:58-63).

Topical Review Article: Brainstem Glioma: I. Pathology, Clinical Features, and Therapy

Gliomas that arise in the brain stem have been associated with a poor prognosis. Diagnostic neuroimaging readily identifies the tumor as it extends between normal brainstem structures. Histologic sampling of tumor with stereotactic methods is notoriously unreliable in establishing a definitive prognosis. Clinical trials that incorporate high-dose chemotherapy, autologous bone marrow rescue, and irradiation hold promise of better tumor control by overcoming the inaccessibility of the central nervous system to standard doses of chemotherapy. We review the pathology, clinical features, neuroimaging features, and current therapeutic concepts relative to brainstem glioma. The pediatric neurologist has a pivotal role in identifying and monitoring children with this malignancy. (J Child Neurol 1993;8:112-128).

Daytime behavior and sleep disturbance in childhood epilepsy

The purpose of this research is to further explore the relationship between sleep disturbance and daytime behavior in children with epilepsy. Parent-rating questionnaires and child symptom self-report measures were employed to evaluate daytime behavior in 30 children with epilepsy and sleep-disordered symptoms. Overnight polysomnography was used to assess for nocturnal sleep problems such as obstructive sleep apnea, nocturnal seizures, periodic leg movements, and sleep fragmentation. We hypothesized that children with epilepsy would exhibit both clinically significant behavioral and sleep problems. Results indicate that 80% of children with epilepsy exhibited sleep disruption because of either clinically significant obstructive apnea syndrome, disturbance of sleep architecture, or sleep fragmentation. These findings further suggest that daytime behavior problems encountered in children with epilepsy may be attributed to specific disruptions in sleep regulation.

Sleep disturbance in children with epilepsy

This study employed a validated pediatric sleep questionnaire to evaluate for sleep-disordered symptoms in children with epilepsy (n=14). Results were compared with those for age-matched children (n=14) with known obstructive sleep apnea. Subjects were enrolled from an outpatient epilepsy clinic and a pediatric sleep disorders clinic. We hypothesized that children with epilepsy would (1) report symptoms of sleep disturbance similar to those of children with documented obstructive sleep apnea, and (2) display behavioral disturbances on parent and child behavioral symptom measures. Results indicate that more than 50% of children with epilepsy had behavioral problems (n=14). No differences in snoring, excessive daytime sleepiness, and restless sleep were seen between the two subject groups. Results suggest that neurobehavioral problems in children with epilepsy may in part be attributed to an underlying sleep disturbance.

Posttraumatic stress disorder, dissociation, and sexual abuse history in epileptic and nonepileptic seizure patients

Rates of posttraumatic stress disorder (PTSD), dissociative symptoms, and childhood sexual abuse (CSA) are believed to be higher among patients with nonepileptic seizures (NES) than those with epileptic seizures (ES). However, women have higher rates of CSA and sexual assault than men. Comparisons of seizure type controlling for gender may produce different results. Data from an opportunity sample of 34 patients with ES and 17 with NES evaluated during or following phase 1 video-EEG monitoring demonstrated that patients with NES had statistically higher rates of PTSD and CSA and higher Dissociative Experiences Scale (DES) scores than patients with ES. No differences were found between women with NES and ES for histories of PTSD or CSA. Mean between-group DES score differences remained. Results indicated that PTSD, dissociation, and CSA may be common among women presenting to a tertiary epilepsy referral center for both ES and NES. Neither a positive history of CSA nor a history of PTSD was evidence against epilepsy.