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Editorial
This issue of Epileptic Disorders marks the beginning of the second year of the journal.
J. Aicardi
our readers: we shall continue our effort to further improve the contents and interest of Epileptic Disorders. This first year of publication has shown that the use of videotapes to illustrate the clinical phenomena of seizures and their differential diagnosis has been well received. Clearly, the image is going to play an increasing role in the next century and we shall make every effort to improve the quality of the visual material. At the end of this first year, I wish to thank those who generously gave their time and effort to support us, especially the members of the Editorial and Advisory Boards and to the ad hoc reviewers who helped them. The quality of a journal is critically dependent on that of its reviewers and we greatly value their generous contribution. Sadly, this year has been marked by the untimely death of a prominent member of the Board, Claudio Munari, a tragic loss for Epileptic Disorders. New, distinguished members are joining the Boards this year. I am confident they will contribute to increasing the interest and quality of the journal.
Synthèse
Neuropsychology of childhood epilepsy: pre- and postsurgical assessment
M. Lassonde, H.C. Sauerwein, I. Jambaque, M.L. Smith, C. Helmstaedter
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.
Articles originaux
Nocturnal paroxysmal dystonia due to a subfrontal cortical dysplasia
C.T. Lombroso
of Sleep Disorder includes NPD within the parasomnias [1]. Others have opined for its epileptic nature, although mainly on circumstantial evidence. The location of the epileptogenic area has been so far unknown. A child with NPD was studied extensively, and in spite of normal scalp EEGs, all of his attacks were shown to originate from his right orbitofrontal cortex. Surgical ablation of an unsuspected cortical dysplastic lesion led to full control.
”Dual pathology” and the significance of surgical outcome in “Dostoewsky epilepsy“
C.L. Vera, S.J. Patel, W. Naso
the AVM was embolized. Thereafter, seizures began with motor arrest and lip smacking, but never again with “light and ecstasy”. A right temporal lobectomy and electrocorticogram (E.Co.G) were performed under local anesthesia four years after occipital lobectomy. Abundant spiking activity was recorded from the right hippocampus which showed gliosis and neuronal loss in the pathology studies. Electrical stimulation of the lateral and basal temporal cortices failed to elicit the vision of “lights” or the experience of “ecstasy”. The patient has had two seizures in the last two years as a result of a lapse in taking his anti-convulsant medication. He now lives alone, seizure-free. The possible abnormally induced functional network organization and structures involved in the production of the “ecstasy” phenomenon are discussed.
Is it juvenile myoclonic epilepsy?
P. Gelisse, P. Genton, C. Raybaud, P. Thomas, F. Bartolomei, C. Dravet
that had begun 2 years before, and for a recent generalized tonic-clonic seizure preceded by a cluster of MJ. Physical examination revealed a small stature, bilateral pyramidal signs, severe mental retardation, and retinis pigmentosa. Etiological factors for this encephalopathy were not found (muscle and skin biopsies, karyotype and extensive blood chemistry). Waking interictal EEG showed a normal background activity and generalized poly-spike-and wave (PSW) discharges. Photic stimulation disclosed a marked photoparoxysmal response, sometimes associated with myoclonic jerks. Three spontaneous jerks accompanied by a burst of generalized PSW were recorded on awakening from a nap. The MRI disclosed wide ventricles, a thin corpus callosum, brainstem atrophy and a so-called “redundant gyration”; these changes were evocative of acquired perinatal damage. Juvenile myoclonic epilepsy (JME) was diagnosed and valproate was started resulting in complete control of seizures. During a 5-year follow-up, the patient has remained seizure-free and the EEG consistently normal. In our opinion, JME can be diagnosed in very uncommon settings, including patients with significant brain damage, as long as all the other criteria for the diagnosis are present.
Hypothalamic hamartoma, precocious puberty and gelastic seizures: a special model of “epileptic” developmental disorder
T. Deonna, A.L. Ziegler
suggesting that the acquired cognitive and behavioral symptoms seen in the majority of cases of this special epileptic syndrome result from a direct effect of the seizures. The early neurobehavioral profile of the case presented in this paper and that of a previous study is particular and combines features of a pervasive developmental and an attention deficit disorder which are probably closely related to the particular location of the epilepsy and its spread from the hypothalamus.
Article spécial
Treatment of children with “ordinary“ epilepsy
P. Camfield, C. Camfield
children are at the time of diagnosis, but they do not have catastrophic epilepsy. Epilepsy is best defined as two unprovoked seizures. Not all of these children require treatment and treatment is motivated by fear of brain damage, injury, death, kindling of additional seizures, and social consequences. None of these fears provides an absolute indication for treatment. The decision to start medication should be considered on an individual basis. The choice of a first AED is arbitrary with most AEDs having equal efficacy. Follow-up schedules have not been well studied. However, there is fairly convincing evidence that routine blood and urine screening for toxicity is of no benefit, if the child is asymptomatic. Serum drug levels are of little clear benefit. Once the child has been seizure-free for 6 months to 12 months, it is reasonable to consider stopping medication. Only rarely does seizure control fail to return if there are recurrences without medication.
Compte-rendu de cas
Carbamazepine-induced combined phonic and motor tic in a boy with Down’s syndrome
M. Holtmann, E. Korn-Merker, H.E. Boenigk
development of tics seems to be a rare, idiosyncratic side effect of CBZ in children.
Ictal SPECT hyperperfusion reflects the activation of the symptomatogenic cortex in spontaneous and electrically induced non-habitual focal epileptic seizures: correlation with subdural EEG recordings
S. Arnold, S. Noachtar, R. Linke, K.J. Werhahn, T.A. Yousry, P.A. Winkler, K. Tatsch
focal seizure. A SPECT investigation during an habitual seizure originating in the left temporal lobe showed a left temporal hyperperfusion. Electrical stimulation of the parietal cortex adjacent to the location of a previously resected angioma using subdural electrodes resulted in a non-habitual seizure beginning with a unilateral somatosensory aura. Ictal SPECT of this seizure demonstrated contralateral central hyperperfusion. We conclude from our findings that ictal SPECT hyperperfusion reflects the activation of symptomatogenic cortex, which can be induced by both epileptic discharge and electrical stimulation.
Conférence mondiale sur la neurochirurgie pédiatrique
Pediatric epilepsy surgery: state of the art, recent developments and future perspectives
I. Tuxhorn, A. Moch, H. Holthausen
[1]. Seizure outcome after surgery may, however, be quite variable and is influenced by numerous factors of which the etiology or morphologic substrate causing the epilepsy, the localization and extent of the epileptogenic zone are probably the most important factors. In general, the goals of surgical treatment may be defined as improving the patient's quality of life (QOL). There are currently few comprehensive data studying the effect of pediatric epilepsy surgery on behaviour, longterm cognitive development, academic performance and psychosocial adaptive functioning. As the outcome results of epilepsy surgery in childhood are so variable, and as improved QOL may not depend entirely on a totally seizure-free outcome, it is going to be important to define new aims and to develop new categories for measuring outcome, as has been suggested by David Taylor. The overall positive impact of epilepsy surgery on global, health-related quality of life has been reported in a pilot study, but prospective studies with epilepsy-specific quality of life measures, assessing the effects of surgery outcome in children of different ages are still lacking [2].
Electrocorticography
D.L. Keene, S. Whiting, E.C.G. Ventureyra
It has been used 1) to localize the epileptogenic tissue; 2) map out cortical functions; and 3) predict the success of the surgery. Despite its common use, few studies have been done to prove its effectiveness in these areas.
The technique used in children for recording ECOG is very similar to that used in adults except for the limitations imposed by the child’s age. Anaesthesia must often be used.
Based upon a computerized medical literature search, a review of this procedure was done. Pre-resection localization, and post-resection prediction of outcome was done for temporal and extra-temporal resection, both lesional and nonlesional. Most of the available studies were in adult patients. All were retrospective in nature.
Evidence for the role of pre-resection ECOG in determining the degree of resection felt necessary to obtain good clinical outcome was limited. Similarly the post-resection ECOG predication of surgical outcome was restricted.
Semiological seizure classification: relevance to pediatric epilepsy
J.F. Bautista, H. Lüders
the wide diversity and richness of seizure expression. In the field of epilepsy surgery, the use of a seizure classification system that emphasizes ictal semiology in an invaluable first step in the precise localization of ictal onset. This proves to be particularly true in pediatric epilepsy, because seizure semiology is more subtle and difficult to characterize. The following article describes a semiological seizure classification and its relevance to both pediatric epilepsy surgery and status epilepticus.
Analyse d'ouvrages
Benign childhood partial seizures and related epileptic syndromes
J. Aicardi
and clinical neurophysiologists who care for children with seizures. The author rightly underlines that these syndromes are both a fascinating and a rewarding topic in paediatric epileptology.
Panayiotopoulos starts with a reminder of the concept of epilepsy syndrome and an excellent review on the significance of the EEG in the diagnosis and management of the epilepsies. He then describres in considerable detail the main benign epilepsy syndromes beginning with benign rolandic seizures, then dealing with the various forms of benign occipital seizures and their differentiation from migraine. The book ends with the description of some less common benign seizures syndromes.
For each syndrome, the author gives a complete account of the historical aspects of each syndrome with extensive quotations from the most important original articles. The clinical description of the clinical EEG features of the syndromes is superb and the relevant literature is exhaustively reviewed (the reference list includes over 830 items). Case reports drawn from the extensive experience of the author are appended thus giving a personal flavor to the descriptions.
Two chapters are dedicated to benign rolandic epilepsy and the problems posed by the presence in tracings of centrorolandic spikes with and without seizures and lucidily consider the significance of this finding and its relationship with conditions other than epilepsy, with age of occurrence and with associated EEG abnormalities. There is also an informative section on ictal EEG in this syndrome.
The chapters on benign occipital epilepsy, a field in which Panayiotopoulos has long been a pioneer, are particularly developed and their clinical presentation and course carefully reviewed. The author gives precises criteria for differentiation of the early type from the late-onset form, from photic-induced benign occipital epilepsy and from the symptomatic and probably symptomatic forms. This nosological discussion is richly documented with an abundance of personal examples of the different types. There is a very good chapter on the occipital EEG paroxysms both in children with occipital epilepsies and in those without seizures and a review of the reflex epilepsies triggered by visual stimuli.
Because of the frequency of headaches and/or vomiting in occipital epilepsies, Panayiatopoulos reviews in detail the differential diagnosis between epilepsy and migraine, a theme to which his contribution has been important. I found the exposition of this usually rather murky topic illuminating and the criteria for distinction between these two conditions are undoubtedly of great practical value.
The rarer syndromes of partial benign epilepsies of different localization (e.g. frontal, parietal and temporal) and their still uncertain nosology are adequately discussed.
The last chapters on severe syndromes possibly related to the benign partial epilepsies, namely the Landau-Kleffner syndrome and the syndrome of continous spike-waves of sleep are less exhaustive than those of the benign types and the discussion of the nature of their relationship to the more usual types is somewhat perfunctory. These syndromes clearly do not belong to the benign partial epilepsies proper but the problem posed by their relation to typical rolandic spikes and the question as the whether they can be predicted early in the course of the epilepsy is not dealt with.
Likewise, I believe that the recently described types of dominantly inherited, partial epilepsy such as frontal nocturnal and temporal familial epilepsies deserved at least a brief mention, even though the available information is still limited, the benign uncertain nature and the age of occurrence not limited to childhood.
Panayiotopoulos’book will remain a landmark in the study of the benign partial epilepsy by its completeness, lucidity and original documentation. It is of major interest for all those who see children with these common, yet often misdiagnosed and mismanaged syndromes, whether epileptologists, neurologists, paediatricians or clinical neurophysiologists who will have many occasions to use it and will find it useful on their bookshelf.
Panayiotopoulos CP. Benign Childhood Partial Seizures and Related Epileptic Syndromes. London, Current Problems in Epilepsy: 15, John Libbey & Company LTD; 1999.
Books proposed for review can be addressed to the Supplement Editor:Dr Arzimanoglou A. Child Neurology Department, Hôpital Robert-Debré, 48, boulevard Sérurier, 75019 Paris, France.
Lettre de l'éditeur
Paroxysmal non-kinesigenic dyskinesia
L. Vercueil
strongly suggesting dopaminergic striatal dysfunction during clinical episodes. However, not much is described about dopaminergic agents used in this young boy, although the therapeutic issue is discussed. Contrary to the author’s suggestion, it would be of great interest to test dopamine antagonists, i.e. neuroleptics, in this context. In the patient reported, striatal oversensitivity to dopamine might be supported by post-synaptic D2 upregulation as shown on PET images. According to the current model of levodopa-induced dyskinesias, one might propose that paroxysmally higher amounts of endogenous striatal dopamine (compared to a low basic level, according to F-DOPA PET findings) could overstimulate upregulated, post-synaptic D2 receptors. This could transiently lead to subthalamic nucleus inactivation, through the “indirect” striatopallidal pathway. In this case, the tonic drive from subthalamic to GPi will be transiently suppressed, and phasic inactivation of GPi will occur, leading to choreoballic movements. Choreoballism is known to be correlated with silent periods during electrophysiological recording in the internal pallidum (GPi) [2]. Coulter et al. reported a good response to haloperidol in a case with paroxysmal dyskinesia, which might support this view [3].
1. Lombroso CT, Fischman A. Paroxysmal non-kinesigenic dyskinesia: pathophysiological investigations. Epileptic Disorders 1999; 1: 187-93.
2. Vitek GL, Chockkan V, Zhang JY, et al. Neuronal activity in the basal ganglia in patients with generalized dystonia and hemiballismus. Ann Neurol 1999; 46: 22-35.
3. Coulter DL, Donofrio P. Haloperidol for non-kinesigenic paroxysmal dyskinesia. Arch Neurol 1980; 37: 325-6.
