Hannelore C. Sauerwein

Deficits in executive functions and motor coordination in children with frontal lobe epilepsy

Frontal lobe dysfunction in adults has been associated with impairments of planning abilities, working memory, impulse control, attention and certain aspects of motor coordination. However, very few studies have attempted to assess these functions in children suffering from frontal lobe epilepsy. The aim of the present study was to determine whether some or all of the components of the frontal lobe syndrome are present in children with this disorder. For this purpose, a neuropsychological test battery was administered to 32 unresected epileptic children, aged 8-16 years: 16 with frontal lobe epilepsy (FLE), eight with temporal lobe epilepsy (TLE) and eight with generalized epilepsy whose principal manifestations were typical absences (GEA). The performances of the three epileptic groups were further compared to normative data derived from 200 French-speaking, healthy children aged 7-16 years, except for standardized tests for which the norms provided in the manual were used. The three epilepsy groups did not differ with respect to conceptual shift and recency memory. However, the FLE children showed deficits in planning and impulse control. Furthermore, they had significantly more coordination problems and exhibited greater rigidity than the other epilepsy groups on the motor tests. These problems were more marked in younger FLE children (8-12 years). The latter were also more impaired on verbal fluency measures. No differences were observed with respect to gender, localization of the epileptic abnormality (unilateral versus bilateral) or medication (monotherapy versus polytherapy). The findings reveal similarities between the neuropsychological profiles of FLE children and adults with frontal lobe lesions.

Attention, memory, and behavioral adjustment in children with frontal lobe epilepsy

To explore whether attention, memory, and behavior would be more affected in children with frontal lobe epilepsy than in children with other types of epilepsy, we compared 16 children with frontal lobe epilepsy (FLE), 8 with temporal lobe epilepsy (TLE), and 8 with generalized absence (GEA) seizures on the Performance Speed (PS) and Freedom of Distraction (FD) indices of the WISC-III, the Continuous Performance Test (CPT), the California Verbal Learning Test (CVLT), and Rey-Osterrieth Complex Figure (ROCF). Parents completed Achenbachs Child Behavior Check List. Children with FLE scored significantly lower than the other two groups on the PS and CPT. On the CVLT they made more intrusion errors and were more prone to interference. Furthermore, they had more difficulties copying and recalling the ROCF. Behavior profiles revealed greater attention problems in this group. This may put children with FLE at greater risk of developing school problems than children with TLE and GEA.

Absence of disconnexion syndrome in callosal agenesis and early callosotomy: Brain reorganization or lack of structural specificity during ontogeny?

Four acallosal subjects, one child, aged 5, and three adults, as well as five epileptic patients who underwent callosotomy between the ages of 6-21 years, were tested on a variety of intra- and intermanual tasks in a study aimed at elucidating the developmental aspects of callosal plasticity. The performance of the clinical sample was compared to that of 48 normal children, aged 5-12 years, an age span generally considered to coincide with the final stages of callosal maturation. As previously reported, interhemispheric integration improved with increasing age in the normal sample. The two patients having undergone callosotomy in childhood performed as well as their normal peers, whereas the three others who had the operation in late adolescence or adulthood showed the typical disconnexion deficits reported in the literature. The acallosal subjects, including the youngest one, outperformed all groups. We speculate that the remarkable plasticity seen in the acallosals and the young callosotomized patients may be related to a critical period in development coinciding with a phase of synaptic overproduction and redundancy that would favor the reinforcement of alternative neural pathways. The compensatory mechanisms appear to become more limited in late adolescence when synaptic distribution presumably assumes adult patterns.

Cognitive and sensori-motor functioning in the absence of the corpus callosum: Neuropsychological studies in callosal agenesis and callosotomized patients

The aim of the present study was to investigate the role of the corpus callosum in cognitive and sensori-motor functioning as measured by a neuropsychological test battery. After a brief review and analysis of the literature, we report our own studies in acallosal subjects (n = 9) and callosotomized patients (n = 25). The main instrument of evaluation was the Michigan Neuropsychological Test Battery. This battery was supplemented by age-appropriate intelligence tests. The performance of the acallosal group was compared to that of two matched control groups: one group consisting of children and adolescents that attended the same school as the acallosals and a second group of subjects recruited from regular schools. The callosotomized patients, tested pre- and post-operatively, served as their own controls. Taken together, the results of the reviewed and personal studies suggest that absence of the corpus callosum does not necessarily impede cognitive functioning. However, samples drawn from clinical populations tend to show a larger variability as to their mental abilities. In keeping with previous findings, our results indicate that the corpus callosum does play a role in bimanual motor coordination although other pathways (probably ipsilateral and/or subcortical) may provide adequate compensation in many cases. The data further suggest that the corpus callosum may be important for interhemispheric transfer of tactuo-motor learning when a spatial component is involved. Finally, our results are consistent with a facilitatory role of the corpus callosum in cognitive and sensori-motor functioning which allows for interhemispheric compensation as part of cerebral reorganization in the case of unilateral brain damage.

Interhemispheric integration of sensory and motor functions in agenesis of the corpus callosum.

Abstract Two siblings with total agenesis of the corpus callosum were compared with matched epileptic and normal controls on bimanual integration of kinesthetic, somesthetic and motor functions. No differences were found between acallosals and control subjects on any of the transfer tasks. However, the acallosal patients were impaired in bimanual operations with regard to speed. The deficit was more pronounced in the younger subject who also showed impairment of differentiated finger movements. Bilateral organization of speech and cross-cuing strategies could have accounted for facilitated performance on some but not all of the transfer tasks. Increased use of ipsilateral and/or subcortical pathways are a more plausible and more parsimonious explanation for the results of this study.

Sensory and Motor Interhemispheric Integration after Section of Different Portions of the Anterior Corpus Callosum in Nonepileptic Patients

OBJECTIVE:We evaluated somatosensory and motor interhemispheric integration in four patients who underwent transection of different portions of the anterior corpus callosum (CC) for removal of an intraventricular cyst. The study goal was to relate their performances to the topographical organization of the CC. METHODS:Experimental tasks included bimanual coordination, tactile cross-localization, and intermanual and interfield comparisons of somesthetic information. Response accuracy and response times were measured. In addition, interhemispheric transmission times were obtained in the somesthetic modality. RESULTS:Section of the middle portion of the genu caused a deficit in motor coordination, which was absent in patients with more posteriorly located lesions, whereas section of more rostral portions of the genu seemed to interfere with motor planning. The most posterior section in our sample, including the anterior portion of the body of the corpus, abolished interhemispheric transfer of simple somesthetic information (perception of touch) but not tactile discrimination (intermanual comparisons of shapes). We speculate that more complex somesthetic information is transferred through the caudal region of the body of the CC, which was spared in all patients. Thus, it seems that section of different portions of the anterior CC (genu and anterior body) produces specific deficits in interhemispheric integration in the motor and somesthetic modalities. These deficits are consistent with the anteroposterior topography of anterior callosal fibers. CONCLUSION:The specific disconnections deficits observed in this study may provide the surgeon with information regarding the consequences of anterior callosotomy and allow for remedial measures to be implemented if required.

Cognitive Functioning in Callosal Agenesis

Agenesis of the corpus callosum results from partial or total failure of callosal axons to cross the midline during the first trimester of fetal development (Rakic and Yakovlev, 1968; Rauch and Jinkins, this volume). Although the resulting structural modifications of the brain are shared by all cases, the clinical expression of this malformation seems to depend on the extent of concomitant pathology of the nervous system (e.g. O’Brien, this volume; Wisniewski and Jeret, this volume). In most cases some neurological dysfunction can be observed. At the cognitive level there seems to exist, as would be expected, a strong relationship between the severity of co-existing CNS defects and level of functioning. Especially affected are those with multiple malformations, such as in Aicardi syndrome (Aicardi and Chevrie, this volume) and other syndromes described in this volume (see Andermann and Andermann; Geoffroy, Wisniewski and Jeret). Recent MRI studies by de Leon et al. (1987) and Atlas et al. (1986) have revealed extensive abnormalities of limbic structures, including absence of the hippocampal formation in some cases, and it has been suggested that these subcortical malformations may be at the basis of the mental deficiencies found in so many syndromes that include callosal agenesis (de Leon et al., 1987).

Verbal Cognitive Functioning and Learning in Girls Treated for Acute Lymphoblastic Leukemia By Chemotherapy With or Without Cranial Irradiation

Neuropsychological problems have frequently been reported following treatment of Acute Lymphoblastic Leukemia (ALL), however, partly because of the heterogeneity of the previously studied samples, the specific nature of these deficits is still a matter of debate. These problems, however, appear to be related more to the combination of cranial radiation therapy (CRT) and intrathecal chemotherapy (ITC) than to ITC alone. In this study, we evaluated a homogenous group of l9 girls between the ages of 7 and 11 years, 30 months after the completion of treatment. Nine received cranial radiation and chemotherapy and 10 were treated with chemotherapy alone. The patients were compared to 10 normal healthy controls. Neuropsychological tests included the Wechsler Intelligence Scale for Children-Third Edition (WISC-III), the California Verbal Learning Test-Childrens Version (CVLT-C), and the Calculation and Passage Comprehension subtests of the Woodcock-Johnson Psycho-Educational Battery-Revised. Results confirmed the presence of a verbal learning deficit in ALL girls treated with the combination of ITC and CRT. The ITC and CRT group scored significantly lower than the healthy controls on the Passage Comprehension subtest and on 5 of the 6 verbal subtests of the WISC-III. Furthermore, compared to nonirradiated patients and healthy normal controls, the ITC and CRT group was impaired on the Freedom from Distractibility index of the WISC-III, indicating an auditory-verbal attention deficit. On the CVLT-C, the ITC and CRT group was particularly impaired on the second half of the learning trials compared to the other two groups, showing a plateau in their performance. The ITC group was not different from the healthy control group, suggesting a less detrimental effect of the ITC alone on verbal abilities. Globally, these results indicate a deficit affecting auditory attention and verbal learning in girls who receive ITC and CRT, which may suggest the necessity for special educational assistance for these children.

Influence of anatomical factors and spatial compatibility on the stimulus-response relationship in the absence of the corpus callosum

Simple and choice reaction times (RTs) to lateralized flashes of light were measured in two acallosal subjects, a callosotomized patient with sparing of the splenium, six IQ-matched controls and six controls with above average IQ. The aim of the study was to investigate the influence of stimulus-response factors on visuomotor integration in the absence of the corpus callosum. The results showed that simple-RTs are faster for all groups when sensory input and motor output are coordinated in the same hemisphere, regardless of the spatial relationship between stimulus (S) and response (R). The difference between ipsilateral and contralateral RTs, taken as a measure of interhemispheric transmission time (ITT), was more than three times longer in the patients lacking the corpus callosum than in the controls. In the choice-RT paradigm, the main determinant of the response speed was S-R spatial compatibility rather than the anatomical relationship between the neural structures receiving the visual input and those controlling the motor output. Spatially compatible S-R pairings were always faster than incompatible pairings. The compatibility effect was present in all subjects but it was significantly larger in the callosum-deprived patients and their IQ-matched controls with respect to the high-IQ controls. The latter finding suggests that cognitive factors may be involved in the production of the spatial compatibility effect.

Developmental outcome after a single episode of status epilepticus

Consequences of status epilepticus (SE) on psychomotor development and the specific impact of the convulsive event on emerging executive functions remain controversial. Infants treated for a single episode of SE, those treated for a single febrile seizure, and healthy infants were tested with respect to motor development, language, personal, and social skills and self-regulation. The children were divided into two age groups to investigate the impact of the convulsive event at different windows of brain maturation. We found that infants who had had SE were inferior to healthy controls on the development scales. Age differentiated SE impact on visuomotor development versus sociolinguistic development. Children who had been treated for SE had significantly more difficulties delaying a response to an attractive stimulus in one of the long-delay conditions. A single episode of SE can interfere with psychomotor and cognitive development in children without previous developmental delay, and it seems that the functions that are emerging at the time of insult are most vulnerable.