[The rapid development of child neuropsychology]. / L'essor de la neuropsychologie de l'enfant.

The past few years have seen important progress and new developments in the field of child neuropsychology. Children can exhibit acquired or learning disorders, but in all cases such deficits take place in a developmental trajectory that requires taking into account cerebral maturation and plasticity processes. Child neuropsychology finds its specificity in the perspective of developmental neuropsychology and has close connections with health and education. This article focuses on two pathologies - childhood epilepsies and developmental dyslexia - that highlight the recent progress in this specialty and its perspectives.

[Quality of life in children with epilepsy: a review]. / La qualité de vie chez l'enfant avec épilepsie: revue de la littérature.

Health-related quality of life (HRQOL) is a multidimensional construct, without general agreement on the number of domains and the content of each domain. In children with epilepsy, the HRQOL evaluation includes both nonspecific aspects, such as behavioral, psychological and cognitive difficulties and the impact of a chronic pediatric illness on the child and its family and specific aspects, such as the perception of the severity of the seizures and of the undesirable effects of the antiepileptic treatments, as well as the social impact of a negative attitude toward epilepsy. The present article presents a review of the methods proposed for the assessment of HRQOL in children with epilepsy. Most methods rely on parental reports; however, there is an increasing effort to develop tools taking the child's point of view into account. HRQOL tools have often been used in clinical trials and, especially, to evaluate the surgical treatments of epilepsy. For the clinician, HRQOL tools may be a preliminary approach to the patient's problems to be interpreted in relation to the patient's medical, psychological, cognitive, social and familial context. In France, few large-scale studies on HRQOL in children with epilepsy have been conducted. We present the preliminary results of a French study based on parental reports.

[Neuropsychological assessment in epilepsy surgery of children]. / Chirurgie de l'épilepsie: études neuropsychologiques chez l'enfant.

In children with epilepsy, surgical treatment has been recognized as a valuable therapeutic option that can improve the cognitive prognosis and facilitate schooling. Most of the earlier studies focused on general intellectual outcome and behavior. However, substantial progress and new developments in the neuropsychological evaluation of epileptic children treated with surgery have been made over the past few years. Several recent studies explored language, memory, executive functions and behavior in patients undergoing surgery, taking into account cerebral maturation and plasticity processes. Neuropsychological studies have demonstrated greater functional recovery after surgery in children than in adults. Notably, there is evidence of particular reorganization processes for language dominance. Other studies demonstrate that early surgery of temporal lobe epilepsy in children is associated with better memory than in adults. Longitudinal studies are still needed to describe developmental trajectories and optimize neuropsychological rehabilitation.

Devastating epileptic encephalopathy in school-aged children (DESC): a pseudo encephalitis.

PURPOSE: To describe the characteristics of a previously overlooked devastating epileptic encephalopathy that presents as intractable bilateral perisylvian epilepsy starting with prolonged status epilepticus (SE) in normally developing school-aged children. METHODS: Retrospective study over 7 years of all normally developing children admitted in our institution for a prolonged SE following non-specific febrile illness with at least one seizure recorded on EEG. RESULTS: Fourteen children were included at a median age of 7.5 years (4-11) (median follow-up of 4 years (1-7)). Intractable SE lasted 4-60 days (median 30). CSF cell count was normal in five cases and moderately increased in the others. During SE, seizures were recorded in 11 patients and involved temporal lobes in 7; the other 4 patients exhibited perisylvian clinical features with secondary generalization. Intractable epilepsy followed SE in all cases without any latent period. Persisting seizures were recorded in 10 patients and involved temporo-perisylvian regions in 8, frontal regions in 2; 3 others had perisylvian ictal semiology. Spiking was bilateral in 10 cases. MRI showed bilateral hippocampal hypersignal and/or atrophy in 10 cases (extended to the neocortex in 3). All children had major cognitive sequelae. When feasible (six patients), detailed neuropsychology suggested fronto-temporal impairment. CONCLUSIONS: Among so called grey matter encephalitis patients, we identified a recognizable pattern we propose to call Devastating Epileptic encephalopathy in School-age Children (DESC) that begins with prolonged SE triggered by fever of unknown cause, and persists as intractable perisylvian epilepsy with severe cognitive deterioration.

Verbal and visual memory impairment in children with epilepsy.

Verbal and visual memory performances were evaluated in 60 epileptic children and 60 normal control subjects with Signoret's Memory Battery scale. Eighteen patients had idiopathic generalized epilepsy and 42 had partial epilepsy, mostly of the temporal (n = 28) and frontal (n = 10) lobes. Memory scores were statistically lower in epileptics than in controls and significant differences were found within each group: (1) children with idiopathic generalized epilepsy had a slight depression of visual memory; (2) memory disorder was more severe in partial epilepsy; and (3) children with left and right temporal lobe epilepsy had marked memory deficits related to hemispheric specialization.

Long-term intellectual outcome in children with posterior fossa tumors according to radiation doses and volumes.

PURPOSE: To analyze the relationship between craniospinal irradiation (CSI) and intellectual outcome in children with posterior fossa (PF) tumors. METHODS AND MATERIALS: A neuropsychological evaluation was performed retrospectively in 31 children, aged 5-15 years, who had received radiotherapy for PF tumors, and who had been off therapy for at least 1 year. Factors evaluated for impact on intellectual outcome were: socioeconomic status, disease presentation, histology, complications, chemotherapy, age at radiotherapy, interval between radiotherapy and testing, and radiation doses and volumes. Patients were divided into 3 subgroups according to the CSI doses (0 Gy [i.e., PF irradiation only], 25 Gy, and 35 Gy), with 11, 11, and 9 patients, respectively. RESULTS: Long-term cognitive impairment occurred in most of the patients, even after PF irradiation only. Moreover, there was a significant correlation between the full-scale IQ score (FSIQ) and the CSI dose, with mean FSIQ scores at 84.5 (SD = 14.0), 76.9 (SD = 16.6), and 63.7 (SD = 15.4) for 0 Gy, 25 Gy, and 35 Gy of CS1, respectively. A marked drop in verbal comprehension scores was noted in children who had received the higher dose. CONCLUSION: This preliminary study further supports the rationale for de-escalation of CSI doses and volumes in standard-risk PF tumors.

Randomized trial comparing vigabatrin and hydrocortisone in infantile spasms due to tuberous sclerosis.

Vigabatrin has been shown to be efficient in infants with infantile spasms and tuberous sclerosis, in open studies. In order to compare vigabatrin to oral steroids, a prospective randomized multicenter study was implemented using both drugs as monotherapy in newly diagnosed patients with infantile spasms and tuberous sclerosis. Eleven infants received vigabatrin (150 mg/kg per day) and 11 hydrocortisone (15 mg/kg per day) for 1 month. Spasm free patients continued vigabatrin or progressively stopped hydrocortisone in 1 month, non-responders were crossed to the other drug for a new 2 month-period. All vigabatrin patients (11/11) were spasm-free versus 5/11 hydrocortisone infants (P < 0.01). Seven patients were crossed to vigabatrin (six for inefficacy, one for adverse events) and became also totally controlled. Mean time to disappearance of infantile spasms was 3.5 days on vigabatrin versus 13 days on hydrocortisone (P < 0.01). Five patients exhibited side effects on vigabatrin but nine on hydrocortisone (P = 0.006). Vigabatrin should therefore be considered as the first choice treatment for infantile spasms due to tuberous sclerosis.

Mental and behavioural outcome of infantile epilepsy treated by vigabatrin in tuberous sclerosis patients.

Vigabatrin (VGB) has demonstrated high efficacy in infantile spasms (IS) due to tuberous sclerosis. Our first objective was to evaluate the cognitive long term effect outcome of children whose refractory spasms definitely disappeared when VGB was given as an add on drug. Our second objective was to determine the response of generalized epilepsy (infantile spasms) compared to partial epilepsy on cognitive impairment. A non selected series of 13 children underwent psychometric and behavioural evaluation before VGB initiation at a mean of 3 years on VGB treatment. Eight of them could perform detailed neuropsychological tests at follow-up. Seven had infantile spasms (Group I), they all were spasm free before 2 years of age and five remained with rare partial seizures (mean age, 5.5 years). Six others had partial epilepsy without spasms (Group II) and five remained with rare seizures (mean age, 7.5 years). Patients of Group I experienced dramatic changes. Developmental quotient (DQ) significantly rose in six out of seven by ten to more than 45 points (P = 0.03) and autistic behaviour disappeared in five out of the six who presented with. The four tested children had normal verbal level after 5 years and could integrate at school but they remained with marked visuospatial disabilities. By contrast, patients of Group II remained with an unchanged DQ of about 60 so that both groups had similar DQ levels on follow-up. The cessation of spasms with VGB is therefore associated with significant improvement of cognition and behaviour in children with tuberous sclerosis. Controlling secondary generalization induced by infantile spasms seems to be a key factor for mental development.

A serial study of regional cerebral blood flow before and after hemispherectomy in a child.

Hemimegalencephaly (HME) is a severe unilateral brain malformation the prognosis of which may be improved by hemispherectomy. HME also provides a unique opportunity to compare normal and pathological hemispheric function in the same patient. We performed a serial functional cerebral imaging study in a child suffering from a neuropathologically confirmed left HME. He was hemispherectomized at 11 months because of intractable epilepsy; this led to cessation of seizures and dramatic psychomotor improvement. Regional cerebral blood flow (rCBF) was studied at 1, 7, 10, 12 and 25 months with single photon computed tomography (SPECT) using 133-Xenon and with simultaneous EEG recording. At one month of age SPECT was performed ictally. During left EEG discharges, rCBF was 40% higher on the left hemisphere than on the right, even in occipital and frontal regions, usually immature at this age. A crossed cerebellar hyperperfusion was also found. At 7 and 10 months, SPECT was performed interictally; rCBF was 45% lower in the left hemisphere than in the right. During follow-up, global and regional CBF values showed normal levels and normal maturation in the right hemisphere except for a mild and transient decrease observed one month after hemispherectomy. SPECT provides an additional procedure for studying hemispheric function in vivo. Serial SPECT imaging may be useful for the preoperative and postoperative evaluation in unilateral cerebral malformation.

Early diagnosis of severe myoclonic epilepsy in infancy.

Of 329 epileptic patients referred in a six year period with the first seizure occurring in the first year of life, 20 met the following criteria: generalized seizures excluding infantile spasms, myoclonic, tonic or absence seizures, at least one afebrile seizure, normal development prior to the first seizure, normal CT scan, and no etiology. Seventeen of these 20 patients developed the full pattern of severe myoclonic epilepsy in infancy (SMEI). This syndrome was recognizable from the second or third seizure in the first year of life, although epileptiform EEG abnormalities were lacking until the age of 11 to over 30 months. Therefore, based on the clinical pattern, the diagnosis of SMEI can be made with quite good reliability by the end of the first year of life.

Neuropsychology of childhood epilepsy: pre- and postsurgical assessment.

Childhood epilepsy is one of the most prevalent forms of chronic and disabling childhood disorders. Because it disrupts brain maturation, it has long been thought to produce non-specific consequences such as mental deficiency and behavioral problems. However, advances in medical knowledge have shown that childhood epilepsy should not be considered as a single disorder, and it is now becoming apparent that various clinical entities have different cognitive expressions that yet need to be specified. The purpose of this paper is to provide an up-to-date analysis of this multi-faceted pathology. The first section is devoted to the characterization of the neuropsychological profile that accompanies focal epilepsies, as defined by the site of the epileptic process. We report the first group study of children with frontal lobe epilepsy. The results indicate that frontal lobe epilepsy produces symptoms (deficits of planning, attention and motor dexterity) that are akin to those found in frontal-lesioned adults. Similarly, like in adults, temporal lobe epilepsy produces memory impairment in children as well as behavioral and academic disturbances. Occipito-parietal lobe epilepsy is rare in children and its effects still need to be specified. The second section deals with the neuropsychological techniques used in presurgical evaluation. Finally, the various neurosurgical procedures that are increasingly being used as part of the arsenal of epilepsy treatment are described along with the neuropsychological findings that are associated with these interventions. It can be concluded that the beneficial effects of epilepsy surgery (callosotomy, hemispherectomy, temporal and extra-temporal resections) by far outweigh the few cognitive deficits that are occasionally reported following these interventions.

[Optic pathway gliomas in neurofibromatosis type I. Longitudinal study of 30 cases in two multidisciplinary practices]. / Les gliomes des voies optiques dans la neurofibromatose de type 1. Etude longitudinale de 30 cas suivis dans deux consultations multidisciplinaires.

UNLABELLED: The aim of this study was to analyse the outcome of optic pathway gliomas in 30 children with neurofibromatosis type 1, the indications of treatment, and the follow-up and screening protocol. PATIENTS AND METHODS: All patients with a minimal two years follow-up (median six years, range two to 19 years), in two multidisciplinary consultations of Saint-Vincent-de-Paul (Paris) and Purpan (Toulouse) hospitals, were included in the study. In our series, we practiced systematic screening MRI in children under six years' of age or with neuropsychological deficiency that may imply an unreliable ophthalmological examination. RESULTS: Thirty-seven percent (11 patients) had progressive ophthalmological signs and were treated, and 63% (19 patients) were not progressive. Our study confirmed that most of optic pathway gliomas were stable during evolution, but rare cases may have bad prognosis. CONCLUSION: Our study supported the importance of close ophthalmological follow-up during childhood for which screening methods are discussed. There is a consensus to limit treatment for patients with progressive ophthalmological symptoms.

[Problems related to neurosurgical treatment of epilepsy in children]. / Problèmes posés par la neurochirurgie de l'épilepsie de l'enfant.

Neurosurgery of epilepsy in children has undergone rapid development during the past decade. This was justified by the high incidence of intractable epilepsy and its consequences on cognitive development. Any decision in the area of surgery must take in account specific etiology and the rapid maturation of the infant's brain. As in adulthood, both the epileptogenic and the functional zones must be identified, but this is a challenge when the brain is not mature and the functions not yet developed. In addition, the ability to recover must be determined. At the present time no precise methodology can be advised for presurgical work-up and surgical indications.

Cognitive disorders in pediatric epilepsy.

Childhood epilepsy may cause cognitive disorders and the intellectual quotient is indeed not normally distributed in epileptic children, a fair proportion of whom show an IQ in the deficient range. Some epileptic syndromes happen during vulnerability periods of brain maturation and interfere with the development of specific cognitive functions. This is the case for the Landau-Kleffner syndrome, which generally appears during speech development and affects language. Similarly, West syndrome - or infantile spasms - is an epileptogenic encephalopathy appearing during the first years of life and induces a major delay in social and oculo-motor development. Specific impairments can also be identified in partial childhood epilepsies in relation with seizure focus localization. For instance, left temporal and frontal epilepsies are frequently associated with verbal impairments. Moreover, episodic memory disorders have been described in children suffering from temporal lobe epilepsy whereas executive deficits (planning, self-control, problem solving) have been reported in frontal lobe epilepsy. In most cases, including its mildest forms, childhood epilepsy induces attention deficits, which may affect academic achievement. These observations militate in favor of individual neuropsychological assessments as well as early interventions in order to provide the child with an optimal individualized treatment program.

The neuropsychology of the Klüver-Bucy syndrome in children.

The Klüver-Bucy syndrome (KBS) is characterized by a number of peculiar behavioral symptoms. The syndrome was first observed in 1939 by Heinrich Klüver and Paul Bucy in the rhesus monkey following removal of the greater portion of the monkey's temporal lobes and rhinencephalon. The animal showed (a) visual agnosia (inability to recognize objects without general loss of visual discrimination), (b) excessive oral tendency (oral exploration of objects), (c) hypermetamorphosis (excessive visual attentiveness), (d) placidity with loss of normal fear and anger responses, (e) altered sexual behavior manifesting mainly as marked and indiscriminate hypersexuality, and (f) changes in eating behavior. In humans, KBS can be complete or incomplete. It occurs as a consequence of neurological disorders that essentially cause destruction or dysfunction of bilateral mesial temporal lobe structures (i.e., Pick disease, Alzheimer disease, cerebral trauma, cerebrovascular accidents, temporal lobe epilepsy, herpetic encephalopathy, heat stroke). As for epilepsy, complete and incomplete KBS are well documented in temporal lobe epilepsy, temporal lobectomy, and partial status epilepticus. KBS can occur at any age. Children seem to show similar symptoms to adults, although some differences in the manifestations of symptoms may be related to the fact that children have not yet learned certain behaviors.