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Congrès : Spectrum of rolandic epilepsy. Proceeding of the Kiel Symposium
This volume of Epileptic Disorders is dedicated to Hermann Doose
Ulrich Stephani
the participants proposed dedicating the publication of the symposium in this volume to him.
Introduction
Ulrich Stephani, Orvar Eeg-Olofsson
its definite, self limited course (the ILAE classification commission termed it synonymously benign childhood epilepsy with centrotemporal spikes, BECTS) rolandic epilepsy seems not to deserve special interest and many epileptologists, do not appreciate its clinical and scientific challenges. Probably, it is one of the most overlooked epilepsies due to the multiplicity of its clinical presentation (partial and generalised, simple and complex seizures, seizures with uncertain vigilance). Strictly speaking, any neurological finding (e.g. intellectual problems, pareses, MRI-findings in patients with rolandic epilepsy, etc.) prevents its inclusion among the idiopathic epilepsies. Its aetiopathogenesis is multifactorial since genetic influences are present but no Mendelian pattern can be defined. What are the reasons for its self limited course, for the slight male preponderance, and the relation to sleep? What are the borderlines of its semiology? Are astatic seizures (e.g. due to negative myoclonus) and absences reasons to reject the diagnosis? Why do certain genetic syndromes such as Rett's syndrome and fragile X-syndrome express rolandic discharges, the most important diagnostic laboratory proof of rolandic epilepsy? What are the pathological and pathophysiological causes of the migrating and side changing expressions of hypersynchronous activities in rolandic epilepsy? These questions arise immediately during the reading of the proposal of ILAE classification on rolandic epilepsy. Many more questions arise when looking at the details. Some of them are presented and discussed in this volume.
Typical semiology of benign childhood epilepsy with centrotemporal spikes (BCECTS)
Ulrich Stephani
having associated somatosensory symptoms which have a tendency to evolve into generalised tonic clonic seizures (GTCS) [1]. Both seizure types are often related to sleep. Onset occurs between the ages of 3 and 13 years (peak 9-10 years) and recovery occurs before the age of 15-16 years. Genetic predisposition is frequent, and there is a male predominance. The EEG has blunt high-voltage centrotemporal spikes, often followed by slow waves that are activated by sleep and tend to shift or spread from side to side.
Atypical semiology of rolandic epilepsy in some related syndromes
Jean Aicardi
with unusual EEG features, especially a marked activation of paroxysms during sleep that may amount to continuous spike-wave complexes of slow sleep. These electroclinical features are often accompanied by cognitive and/or behavioral disturbances and may belong to several syndromes (atypical benign partial epilepsy, syndrome of continuous spike-waves during sleep, Landau-Kleffner syndrome and status of rolandic epilepsy) whose relationship with typial rolandic epilepsy and among themselves remains to be clarified.
Atypical benign partial epilepsy/pseudo-Lennox syndrome
Andreas Hahn
analyzed the clinical and EEG data of 43 children with ABPE/PLS seen in our department during the last 25 years. Criteria for diagnosis of ABPE/PLS were the occurrence of generalized minor seizures as previously described for ABPE/PLS and the detection of focal sharp waves indistinguishable from those of rolandic epilepsy (RE) with generalization during slow sleep.
Mental development prior to onset of epilepsy was retarded in 26% of patients. In 74%, age at onset of seizures ranged from 2-5 years of age. Atonic-astatic seizures, atypical absences, and more rarely, myoclonic seizures were the predominating seizure types in 67% of patients. Status of minor seizures occurred in 40%. One or several episodes of partial and secondarily generalized seizures were observed. Rolandic seizures occurred in 28%. The EEG was characterized by a normal or slightly slowed background activity and predominantly multi-focal sharp waves (88%) which were activated to a bioelectrical status in 56% during sleep.
No tonic seizures and no fast > 10 Hz spike discharges were observed in any of the 1.291 EEG of the patients. Despite an often temporarily therapy-resistant course which led to use of steroids in 51% of patients, seizures had ceased in 84% and epileptic discharges had disappeared in 72% of patients at last follow-up. All patients older than age 15 were seizure-free. However, the rate of mentally handicapped patients had more than doubled from 26 to 56%.
EEG which could be performed in 41 out of 56 siblings demonstrated shw in 40% of siblings in whom EEG were done at the age of maximum penetrance of sharp waves (3-10 years).
ABPE/PLS overlaps broadly with RE, but also with electrical status epilepticus during sleep and Landau-Kleffner syndrome. Therefore, ABPE/PLS can be ranked alongside RE and other idiopathic partial epilepsies. The high incidence of sharp waves in siblings suggests that ABPE/PLS and RE have a common underlying genetic etiology.
Autosomal dominant rolandic epilepsy with speech dyspraxia
Ingrid E. Scheffer
disorders. Subtle speech and language disorders have recently been well characterised in BRE. ADRESD is associated with long term, more severe speech and language difficulties. The time course of rolandic epilepsy in ADRESD is typical of that of BRE. ADRESD is inherited in an autosomal dominant manner with anticipation. It is postulated that the anticipation may be due to an, as yet unidentified, triplet repeat expansion in a gene for rolandic epilepsy. BRE follows complex inheritance but it is possible that ADRESD may hold some valuable clues to the pathogenesis of BRE.
Rolandic discharges in benign childhood epilepsy with centrotemporal spikes, and in other forms of partial epilepsies
Tateki Morikawa
courses of three cases and the results of magnetoencephalography (MEG) in one patient, with respect to the following points; (1) the electro-clinical characteristics of sylvian seizures and rolandic discharges (RD), and (2) long-term outcome of idiopathic and symptomatic partial epilepsies with RD other than BECT.
The epileptic focus of sylvian seizures is located either in the inferior rolandic cortex or underneath the sylvian fissure, which is strongly supported by the results of clinical seizure manifestations, ictal and interictal EEG findings, and interictal MEG findings. The presence of rolandic discharges is not a hallmark of benign outcome. Instead, the presence of sylvian seizures heralds a benign outcome of partial epilepsy regardless of the presence or absence of an organic lesion. The pathophysiological mechanism of BECT remains unknown. Further neurophysiological as well as genetic investigations are needed.
Epileptiform discharges in benign focal epilepsy of childhood
Andrew Pan, Hans O. Lüders
of its signature electroencephalographic discharge, including morphology, topography, field of distribution, activation and reactivity will be presented and discussed.
Migraine and the benign partial epilepsies of childhood: evidence for an association
Frederick Andermann
not been universally recognized. Clearly further investigation of this association is required. The purpose of this report is to examine the varied diagnostic criteria for common migraine utilized by epileptologists and other neurologists, to review clinical migraine epilepsy relationships, EEG abnormalities in migraine and epilepsy, and the association of seizures with vascular headache in patients with various forms of epilepsy. Hopefully, this will stimulate further research into this intriguing association of two conditions which though they have different pathophysiology frequently coexist.
Benign epilepsies of childhood - distinct syndromes and overlap
Olivier Dulac
with continuous spike waves in slow sleep and myoclonic astatic epilepsy. Genetic predisposition seems to determine whether the epilepsy is partial or generalised. However, some maturational process seems to contribute in the cases with transient worsening including cognitive troubles.
The concept of hereditary impairment of brain maturation
Hermann Doose, Bernd A. Neubauer, Birgit Petersen
and a variety of other syndromes. The broad overlap of the clinical and bioelectrical symptomatology might reflect a pathogenetic background common to these epilepsies. In order to understand the great phenotypic variability, the clinical symptomatology in 56 sibships with focal sharp waves of genetic origin was analyzed. A genetic determination was assumed if, in addition to the index case, at least one sibling or offspring revealed typical focal sharp waves. The 56 index-cases and their 61 sib/offspring/parents showed a broad spectrum of epileptic and non-epileptic conditions ranging from mild selective performance deficits to severe complex mental retardation, from neonatal seizures, febrile convulsions, and simple rolandic epilepsy to severe epilepsies with minor seizures or ESES. The different conditions are not disease entities but sets of variably weighted symptoms of a complex pathogenetic background, in which a genetic disposition to focal anomalies of brain function is of decisive importance. As can be demonstrated by the data, this genetic liability coincides with other widespread genetic traits, expressed in certain EEG patterns, as well as with lesional pathogenetic factors.
MRI in rolandic epilepsy
Orvar Eeg-Olofsson, Staffan Lundberg, Raili Raininko
the latter may be a result of a maturational delay involving a defective myelination. Both abnormalities may cause cognitive dysfunction. In order to get a better understanding of rolandic epilepsy both MRI and neuropsychological studies are wanted in groups of children with typical rolandic seizures with and without rolandic sharp waves, as well as in groups of children with typical rolandic sharp waves and atypical seizures.
Isotope tracer-techniques in rolandic epilepsy and its variants
P. Maquet, M.N. Metz-Lutz, A. de Saint-Martin, C. Marescaux, Edouard Hirsch
with continuous spikes and waves during slow wave sleep (CSWS) which are considered to be included within the spectrum of rolandic epilepsy. The results of studies using isotope tracer-techniques in rolandic epilepsy and its variants are summarized.
Rolandic epilepsy: neuropsychology of the active epilepsy phase
Thierry Deonna
children, particularly in relationship to the paroxysmal electroencephalographic activity.
The difficulty to demonstrate direct effects of the epileptic EEG discharges on some cognitive functions and on learning, as occurs in some cases, is discussed.
Neuropsychological long-term outcome of rolandic EEG traits
Goeran Carlsson, N. Igelbrink-Schulze, B.A. Neubauer, Ulrich Stephani
HASH(0xb9985f8)
The genetics of rolandic epilepsy
Bernd A. Neubauer
has been reported to follow an autosomal dominant mode of inheritance with incomplete penetrance and age dependency. CTS therefore may represent a neurobiological marker for the increased risk of developing BECTS. Several linkage studies exploring candidate loci have rendered negative results. The first positive evidence for linkage in families with centrotemporal spikes was found on chromosome 15q14.
Treatment in typical and atypical rolandic epilepsy
Dietz Rating
study STM was shown to control seizures in BECTS and STM is regarded as first line drug for this epilepsy in contries having access to this drug. Otherwise, CBZ is used most often and very effectively in BECTS. However, CBZ has the disadvantage to worsen clinical and EEG features up to the precipitation of CSWS in few patients. Epileptic seizures are often not the main problem in atypical rolandic epilepsies like CSWS or LKS and the amelioration of cognitive dysfunction by epileptic discharge is the prominent aim of an AED therapy. Steroids seem the have the best efficacy in these cases.
