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Biomedical subjects

E Thiery

Publications and source records attributed to E Thiery.

At least 19 recordsLinked to original sources

Neurocognitive consequences of surgically corrected congenital heart defects: A review.

With advances in surgical procedures, neuropsychological assessment after congenital heart defects and pre, peri- and/or postoperative predictors of adverse outcome has become an important focus in research. We aim to summarize neuropsychological sequelae associated with different types of congenital heart defects, critically review the methodology used in more than 20 empirical studies that were retrieved from biomedical electronic search engines, and identify possible directions for future research. Despite the lack of adequate control groups and long-term studies, there seem to be some cognitive deficits. The largest group of children with isolated congenital heart defects present with normal intellectual capacities. However, they tend to show language deficits and motor dysfunction. Although performances on memory tasks are good, unambiguous conclusions concerning their attentional and executive functioning are still lacking. Serious behavioral problems are not an issue. In addition to a detailed description of the (neuro) psychological consequences of pediatric cardiac surgery, an overview of the predictors of the cognitive defects is provided.

Brain Damage, Chronic↗

Chromosome 21 KIR channels in brain development.

Two KIR (K+ Inwardly Rectifying) channel genes have been identified on chromosome 21, in a region associated with important phenotypic features of trisomy 21, including mental retardation: KIR3.2 (GIRK2) and KIR4.2. We analysed the expression of these channel genes in developing human and mouse brains to determine the possible role of the corresponding channels in brain development and function. KIR3.2, which has been extensively studied in the mouse, was found to be expressed in the human cerebellum during development. The KIR4.2 channel is expressed later in development in both mice and humans. We compared the expression of these channels in terms of RNA and protein levels and discussed the potential synergy and consequences of the overexpression of these channels in Down's syndrome brain development.

Animals↗

Child psychopathology and lower cognitive ability: a general population twin study of the causes of association.

Previous work has demonstrated associations between lower cognitive ability and childhood and adult non-psychotic psychopathology. As both cognitive ability (CA) and child psychopathology (CP) are influenced by genetic factors, one explanation for the association is that they are the pleiotropic manifestations of the same underlying genetic factors. The present paper examines three possible causes of the association: additive genetic factors, common environmental factors and individual-specific environmental factors. Three hundred and seventy-six twin pairs from the East Flanders Prospective Twin Survey were examined with the Child Behaviour Checklist and the Wechsler Intelligence Scale for Children-Revised. The cross-twin within-variable, within-twin cross-variable and cross-twin cross-variable correlations were calculated. Using structural equation modelling, bivariate models were fitted. The best fitting model was chosen, based on likelihood and parsimony. The observed phenotypic correlation between CP and CA was -0.19 (95% CI: -0.09, -0.27), with genetic factors accounting for about 84% of the observed correlation. Bivariate model fitting quantified the genetic correlation between CP and CA at -0.27 (95% CI: -0.12, -0.42) and the individual-specific environmental correlation at -0.17 (95% CI: -0.03, -0.31). In children, three different genetic factors may exist: one that solely affects the liability to CP, one that has only an effect on CA and one that influences both CP and CA. While individual-specific environmental factors can influence the liability to both traits, our results suggest that most of the environmental factors that increase the risk of CP do not influence CA and vice versa.

Adolescent↗

Heritability estimates of intelligence in twins: effect of chorion type.

This study investigates the basic assumption of homogeneity of monozygotic (MZ) twins: are there differences according to the timing of the zygotic splitting, early in dichorionic (DC) and later in monochorionic (MC) pairs? We assessed the IQ of 451 same-sexed twin pairs of known zygosity and chorion type with the Wechsler Intelligence Scale for Children-Revised (WISC-R). The variances of within-pair differences were compared for monochorionic (MC), dichorionic monozygotic (DC-MZ) and dizygotic same-sexed (DZ) twins and structural equation modeling was applied. High heritability estimates were found for almost all subscales and IQ-scores. A significant effect of chorion type was found: the MC twins resembled each other more than the DC-MZ twins on the subscales Arithmetic and Vocabulary. The effect accounts for respectively 14% and 10% of the total variance.

Adult↗

Developmentally regulated expression of the murine ortholog of the potassium channel KIR4.2 (KCNJ15).

The gene KIR4.2 (K(+) inwardly rectifying channel 4.2) has been recently identified in the Down syndrome Chromosome Region 1. We have cloned the mouse ortholog of KIR4.2 and characterized its expression pattern. In situ hybridization showed a restricted and developmentally regulated pattern of expression. The expression is starting at E12.5 and expands at E14.5 in different tissues and organs, which may be affected in Down syndrome: heart, thymus, thyroid gland, and perichondrium. At E17.5, additional epithelia (kidney, bladder, stomach, lung) expressed also strongly the gene.

Animals↗

Intercepting moving objects during self-motion: effects of environmental changes.

The purpose of this study was to examine the role of background texture on an interception task during self-motion. Twenty-six participants modulated tricycle speed along one arm of a V-shaped track so as to intercept a ball approaching horizontally on the other arm of the V. Either a plain or a textured background (consisting of contrasting vertical stripes) was used. Velocity modulations occurred so as to keep the angle beta between the direction of heading and the line head-ball constant (constant bearing angle, or CBA strategy), indicating that this observer-environment relation might regulate the approach phase. In the textured condition, participants initially drove faster than predicted by the CBA model and compensated by slowing down in the second half. This is in line with the texture-induced overestimation of the ball velocity and implies that absolute velocity information is also used.

Analysis of Variance↗

Interictal and ictal video-EEG monitoring.

PURPOSE: The purpose of this paper is to demonstrate the diagnostic efficacy and therapeutic relevance of video-EEG monitoring in an large patient population with long-term follow-up. PATIENTS AND METHODS: Between October 1990 and May 1997, 400 patients were monitored at the Epilepsy Monitoring Unit (EMU) of the University Hospital in Gent. In all patients, the following parameters were retrospectively examined: reason for referral, tentative diagnosis, prescribed antiepileptic drugs (AEDs), seizure frequency, number of admission days, number of recorded seizures, ictal and interictal EEG, clinical and electroencephalographic diagnosis following the monitoring session. During follow-up visits at the Epilepsy Clinic, we prospectively collected data on different types of treatment and post-monitoring seizure control. RESULTS: 255/400 (64%) patients were referred for refractory epilepsy. 145/400 (36%) patients were evaluated for attacks of uncertain origin. Mean follow-up, available in 225 patients, was 28 months (range: 6-80 months). Mean duration of a single monitoring session was 4 days (range: 2-7 days). Prolonged interictal EEG was recorded in all patients and ictal EEG in 258 (65%) patients. Following the monitoring session, the diagnosis of epilepsy was confirmed in 217 patients. Pseudoseizures were diagnosed in 31 patients (8%). AEDs were started in 19 patients, stopped in 6 and left unchanged in 110. The type and/or number of AEDs was changed in 111 patients. Sixty patients underwent epilepsy surgery. In 48 surgery patients, follow-up data were available, 29 of whom became seizure-free, and 16 of whom experienced a greater than 90% seizure reduction. Vagus nerve stimulation was performed in 11 patients, 2 became seizure-free, and 7 improved markedly. Of the non-invasively treated patients in whom follow-up was available (n = 135), 70 became seizure-free or experienced a greater than 50% reduction in seizure frequency; 51 patients experienced no change in seizure frequency. Outcome was unrelated to the availability of ictal video-EEG recording. In patients with complex partial seizures, seizure control was significantly improved when a well-defined ictal onset zone could be defined during video-EEG monitoring. CONCLUSION: Prolonged interictal EEG monitoring is mandatory in the successful management of patients with refractory epilepsy. Ictal video-EEG monitoring is very helpful but not indispensable, except in patients enrolled for presurgical evaluation or suspected of having pseudoseizures.

Adolescent↗

Epilepsy surgery in Belgium, the experience in Gent.

Between January 1992 and July 1998, 320 patients were presurgically evaluated for medically refractory epilepsy at the University Hospital of Gent. All patients underwent a comprehensive presurgical evaluation, including extensive neurological history and examination, video-EEG monitoring of interictal EEG and habitual seizures, and optimum magnetic resonance (MR). In a large subgroup of these patients, a comprehensive neuropsychological examination and interictal 18FDG-PET were performed. Subsequently, a bilateral carotid angiography and intracarotid amytal procedure (Wada-test) were planned in 49 patients to establish hemispheric language dominance and bilateral memory function. After proper selection, 23 patients underwent invasive video-EEG monitoring with intracranial implantation of parenchymal and/or subdural electrodes to further document the area of seizure onset. From the initial group of 320 potential surgical candidates, 75 patients (42 males, 33 females) with mean age of 29 years (range: 2 months-55 years) and mean duration of uncontrolled seizures of 15 years (range: 2 weeks-38 years) eventually underwent a surgical procedure. Sixty of 75 patients were on high dose antiepileptic polytherapy. Optimum MR detected structural abnormalities, confined to a limited brain area, in 71 patients. These abnormalities were of space-occupying nature in 31 cases; an atrophic lesion was suspected in 39 patients; a combination of space-occupying and atrophic lesion was seen in 1 case. Structural abnormalities were most frequently located in the temporal lobe (n = 53) and the frontal lobe (n = 10). Video-EEG monitoring documented complex partial seizures in 67 patients with occasional secondary generalisation in 32. Most patients had complex partial seizures of temporal lobe as defined by clinical and EEG criteria. Two patients had only simple partial seizures. Ultimately, an area of seizure onset could be determined in all patients. Temporal lobectomy with hippocampectomy was the most commonly performed procedure (n = 42). In 13 patients, complete lesionectomies were performed for epileptogenic structural lesions in and outside the temporal lobe. In 2 patients, only partial lesionectomies were possible; in 5 patients, only biopsies in combination with partial lesionectomies could be performed. Anterior 2/3 callosotomy was performed in 4 patients and hemispherectomy was performed in 2 patients. Postsurgical seizure control, after average follow-up of 50 months (range: 12-98 months), was excellent in 49 patients who became seizure-free. In these patients, antiepileptic therapy was tapered 2 years after surgery. Patients in whom only biopsies or partial lesionectomies were performed have poor seizure control. Epilepsy surgery is a rewarding therapeutic alternative for patients with medically refractory epilepsy. Comprehensive presurgical evaluation and epilepsy surgery provide excellent neurological, neurophysiological, neuropsychological and imaging research opportunities.

Adolescent↗

Handedness in twins according to zygosity and chorion type: a preliminary report.

In the course of the East Flanders Prospective Twin Survey (EFPTS), handedness was assessed as part of a genealogical study (Meulemans et al., 1995) in 1616 twins (808 twin pairs) aged 6 to 28. Our findings are that, in this large population-based study with known placentation and zygosity, the often observed higher frequency of left-handedness in twins is confirmed, that it appears to be independent of zygosity and chorion type, and that the belief that discordant handedness in monozygotic twins represents mirror-imaging is mythical.

Adolescent↗

Neuropsychological aspects of Marfan syndrome.

We evaluated the neuropsychological status of 13 adults patients with Marfan syndrome. All subjects were administered the same neuropsychological test battery that included nine measures covering a broad range of cognitive abilities such as attention and concentration, learning and memory, and verbal and non-verbal abilities. Compared to a control group of 13 healthy subjects matched for sex, age and verbal intelligence, Marfan patients only performed significantly worse on tests measuring sustained visual attention and visuoconstruction. Although these tests use visual material and depend on visual perception and processing, the visual acuity problems associated with the syndrome could not explain these differences, nor could the use of beta-blocking medication or the presence of joint hypermobility. The findings suggest that problems with sustained visual attention and visuoconstruction may be present in Marfan syndrome over and above visual acuity problems and other phenomena associated with the disease. Further research on the neuropsychological aspects of Marfan syndrome is needed, using larger patient groups and more adequate control groups such as non-affected siblings and matched controls with similar visual impairment.

Adolescent↗

Epilepsy surgery in Belgium, the Flemish experience.

Between January 1992 and June 1995, 160 patients were presurgically evaluated for medically refractory epilepsy by the Epilepsy Monitoring and Surgery Team at the University Hospital of Gent. All these patients underwent a comprehensive presurgical evaluation, including extensive neurological history and examination, video-EEG monitoring of interictal EEG and habitual seizures, CT and optimum MR. In a large subgroup of these patients a comprehensive neuro-psychological examination and interictal 18FDG-PET were performed. After the non-invasive phase of the presurgical evaluation, a bilateral carotid angiography and intracarotid amytal procedure was planned in 27 patients to establish hemispheric language dominance and bilateral memory function. After proper selection, 14 patients underwent invasive video-EEG monitoring with intracranial implantation of parenchymal and/or subdural electrodes to further document the area of seizure onset. From the initial group of 160 potential surgical candidates, 40 patients (20 M, 20 F) with mean age of 31 years (range: 2 months-55 years) and mean duration of uncontrolled seizures of 16 years (range: 2 months-47 years) eventually underwent a surgical procedure. 30/40 patients were on high dose antiepileptic polytherapy. Optimum MR detected structural abnormalities, confined to a limited brain area, in 39 patients. These abnormalities were of space-occupying nature in 21 cases; an atrophic lesion was suspected in 17 patients. Structural abnormalities were most frequently located in the temporal lobe (n = 26) and the frontal lobe (n = 7). Video-EEG monitoring documented complex partial seizures in 32 patients with occasional secondary generalisation in 14. In most of these patients, seizures could be subclassified as being of temporal lobe origin based on clinical and EEG criteria. Two patients had only simple partial seizures. One patient with Sturge-Weber syndrome and a strictly unilateral angioma had hemiconvulsions. A mentally retarded patient with Lennox-Gastaut syndrome had different types of seizures. After non-invasive and invasive exploration, the area of seizure onset could be determined in all patients. Standard or modified temporal lobectomy +/- hippocampectomy were the most commonly performed procedures (n = 26). In 5 patients complete lesionectomies were performed for epileptogenic structural lesions in and outside the temporal lobe. In 2 patients only partial lesionectomies were possible; in 5 patients only biopsies could be performed. Anterior 2/3 callosotomy and hemispherectomy were each performed in one patient. Postsurgical seizure control, after average follow-up of 20 months (range: 6-40 months), was excellent in 27 patients who became seizure-free. In these patients antiepileptic therapy was tapered 2 years after surgery. An additional 4 patients continue to experience non-disabling simple partial seizures only. Patients in whom only biopsies or partial lesionectomies were performed have poor seizure control. Three patients died as a result of the intrinsic malignancy of their space-occupying lesion. Two patients who are seizure free experienced a moderate postoperative hemiparesis with subtotal recovery. Overall quality of life was substantially improved both in patients who became entirely seizure free or who experienced a very significant reduction in seizure frequency. Presurgical evaluation and epilepsy surgery are a labour intensive but rewarding therapeutic alternative for patients with medically refractory epilepsy. Besides providing therapeutic efficacy, comprehensive presurgical evaluation and epilepsy surgery allow for fruitful clinical neurological research.

Adolescent↗

Clinical and neurophysiological correlations in patients with refractory partial epilepsy and intracranial structural lesions.

Twenty patients (13 males, 7 females), who presented with refractory partial epilepsy and a CT and/or MR detected intracranial intra-axial structural lesion were admitted to the University of Gent Epilepsy Monitoring Unit. Mean duration of the epilepsy was 17 years (2-47 years). All patients were enrolled in a comprehensive presurgical protocol including neurological examination, video-scalp-EEG monitoring with prolonged interictal and ictal recording, neuropsychological assessment and positron emission tomography (PET). Intracranial EEG monitoring was performed in 5 patients in whom discrepancies between different tests were found during the non-invasive evaluation. Clinical neurological examination was normal in 16 patients; 4 patients had a mild contralateral hemiparesis. Lesions were mainly located in the temporal lobe (55%). Most patients presented with complex partial seizures (90%). Clinical seizure characteristics correlated well with the lesion location in 55% of patients. Interictal EEG showed focal epileptic activity and focal slowing in respectively 85% and 30% of patients. Interictal EEG lateralization was congruent with the side of the lesion in 17 patients (85%). Interictal EEG localization was congruent with the lobe of the lesion in 13 patients (65%). Ictal EEG lateralized correctly in 14 patients (70%) and localized correctly in 10 patients (50%). Neuropsychological assessment lateralized and localized congruently in respectively 8/17 (47%) and 7/17 (41%) of patients. Interictal PET showed focal interictal hypometabolism, congruent with the lesion, in 13/16 (81%) of patients. Intracranial EEG was congruent with the lesion location in 3 patients but non-congruent in 2 patients. All patients underwent surgical procedures: average follow-up was 14 months (6-24 months). Complete surgical removal of the lesion with free margins resulted in a more than 90% reduction of seizures without postoperative neurological deficit in 12/13 patients.

Adolescent↗

Personality traits after prolonged vegetative state. Reflections on "Unexpected Improvement after Prolonged Post-traumatic Vegetative State" by W. Arts, H. van Dongen, J. Meulstee.

The consequences of the trauma on the personality traits of the case described by Arts et al. are analysed. Among the biological factors which favoured the restoration of consciousness, youth, normal pretraumatic personality as well as the location and extent of the brain lesions have been of special importance. The psychopathological changes mainly consisted in an impairment of higher mental functions with intellectual regression, loss of abstraction ability and a severe deficit of recall and memory. The pragmatical and ethical consequences of this case are discussed.

Adult↗

Intracerebral osteoma: a clinicopathologic and neuropsychologic case study.

This case report deals with a 16-year-old girl, admitted for headache and Jacksonian seizures. The clinical examination showed no gross disturbances, but the neuropsychologic examination revealed difficulties in speech-linked abstract thinking. Extensive radiologic investigations revealed the presence of a frontal intracallosal bony tumor, protruding into the lateral ventricles. Histologic examination of the surgical specimen showed a large benign osteoma, without any connection with dura or skull. In view of the paucity of symptoms, a dysontogenetic etiology of the tumor seems obvious.

Adolescent↗

Comparative study of the clinical effect of vincamine versus papaverine given parenterally in the acute phase of stroke.

The efficacy of the "oxygenator", vincamine was assessed in comparison to a classic vasodilator, papaverine, after parenteral administration during the first days of an acute thrombo-embolic stroke. All 263 patients admitted to this open trial had a thrombo-embolism of the A. carotis or one of its cerebral branches within the previous 24 h. Patients demonstrating minimal neurological deficit or requiring admission to an intensive care unit, or patients with a suspected hemorrhagic syndrome were excluded. Each patient received i.v. infusions (15 mg X 6) of 6 vials of vincamine per 24 h, or 6 vials of papaverine (40 mg X 6) per 24 h, occasionally in combination with acetylsalicylic acid (ASA), dipyridamol and hydrocortisone. An initial 3-h control was followed by regular surveillance for 5 days, paying special regard to consciousness and motricity. The administration of vincamine alone had a significantly more favourable effect than papaverine alone on consciousness (p less than 0.001). Furthermore, vincamine associated with other drugs was significantly superior (p less than 0.01) to papaverine given in association. A similar tendency was noted for motor recuperation after vincamine compared to papaverine (p less than 0.001). Vincamine and papaverine were well tolerated during the 5-days observation period, only one side effect after vincamine and four after papaverine being noted.

Adult↗

Ultrastructural study of a muscle biopsy from a patient with subacute myelo-optic neuropathy.

A man, aged 46, who had been taking Clioquinol in high doses for a long period, developed a characteristic neurological syndrome of subacute myelo-optic neuropathy rather abruptly. Electron microscopical examination of the muscle biopsy, obtained five months after the onset of the disease, revealed severe degenerative changes of the presynaptic nerve endings and some unique paracrystalline inclusions in the sole plate region. The latter may represent the morphological expression of the toxic agent which is held responsible for the subacute myelo-optic neuropathy.

Clioquinol↗