Search

Of the classic syndromes of idiopathic (benign) focal epilepsies in childhood, more than half of children with Rolandic epilepsy and Panayiotopoulos syndrome do not need antiepileptic drug (AED) treatment. Monotherapy with carbamazepine, levetiracetam, sodium valproate, or clobazam is usually recommended for those with seizures that are unusually frequent (more than three), lengthy, distressing, or otherwise significantly interfering with the child's life. Other factors in favor of treatment include a short interval between the first three seizures, a younger age of onset (less than 4 years), or a presence of recurrent tonic–clonic seizures and diurnal seizures. In contrast to Rolandic epilepsy and Panayiotopoulos syndrome that may not need treatment, idiopathic childhood occipital epilepsy of Gastaut should be treated because seizures are frequent and secondarily generalized convulsions are probably unavoidable without medication. It is likely that all AEDs licensed as monotherapy for focal seizures and mainly carbamazepine and levetiracetam are suitable.The electroencephalogram does not predict prognosis or AED treatment needs, though there are views supporting that interictal spikes have an adverse effect on cognition.Strategies of withdrawing medication in idiopathic focal epilepsies in childhood differ among experts, although all agree that there is no need to continue medication 1 to 3 years after the last seizure, and certainly not after the age of 14 years, when most benign childhood focal seizures remit, or age 16, when they are practically nonexistent.In the acute stage of status epilepticus, control of the seizures is of paramount importance. Convulsive status epilepticus is a life threatening condition and should be terminated as soon as possible. Autonomic status epilepticus needs thorough evaluation for proper diagnosis and management. Benzodiazepines, intravenously, in rectal or buccal preparations are commonly used to terminate long lasting seizures and status epilepticus. Early parental treatment is more effective than late emergency treatment. Buccal midazolam is probably the first choice medication for out-of-hospital termination of status epilepticus.Parental education and psychological support are the cornerstone of the optimal management because despite excellent prognosis, idiopathic focal epilepsies in childhood usually have a dramatic impact to parents.

This critical review refers to the new report on terminology and concepts for the organization of epilepsies by the Commission of the International League Against Epilepsy (ILAE). It is unfortunate that most of the proposals in the Commission's report are modified interpretations and nomenclature of previous ILAE classifications; new terms are not better than the old ones, and recent advances have not been incorporated. Hence, the new ILAE report met with considerable dissatisfaction from several expert epileptologists. The Commission abandoned (1) the disease-syndrome distinction, although "disease" is generally differentiated from "syndrome" in most medical texts as well as in the ILAE classification itself; (2) the distinction of "generalized" and "focal" for epileptic syndromes, despite maintaining this distinction for epileptic seizures and despite the fact that most epileptic syndromes manifest exclusively either with generalized or focal epileptic seizures; (4) the terms "idiopathic,""symptomatic," and "cryptogenic," although these terms have been well defined in the previous ILAE classifications; reiterating their true meaning would be sufficient. Genetic epilepsy could be a new category and (5) the designation of "benign" epilepsies, despite the recommendations of experts at the Monreale workshop. In addition, the Commission proposed that "age at onset" be used as a primary dimension for organizing the epilepsies. However, (a) this runs counter to classification efforts of other diseases in medicine and neurology; (b) syndromes that are likely to be linked together on the basis of electroclinical (and often genetic) evidence are now separated and intermixed with a number of heterogeneous epilepsies; and (c) a considerable number of epileptic syndromes have a wide range of age at onset from childhood to adulthood. Furthermore, epilepsy syndromes were given by name only, without definition; thus we remain dependent on previous ILAE definitions, which are often broad and imprecise. The ILAE should commission consensus of opinion from experts in specific fields in order to define each syndrome. Areas of certainties and uncertainties and of agreements and disagreements should be identified and explained. This approach may be the only way toward achieving a scientifically sound and clinically meaningful organizational system for the epileptic seizures and the epilepsies-a process that would incorporate the tremendous advances in our field and would be accepted by the wider community of clinicians and scientists.

The International League Against Epilepsy (ILAE) standardized classification and terminology for "epileptic seizures" of 1981 and "epilepsies and epileptic syndromes" of 1989 provide a fundamental framework for organizing and differentiating the epilepsies. However, a revision of these classifications is mandated by recent major technologic and scientific advances. Since 1997, the relevant ILAE Commissions have made significant efforts to achieve better and internationally uniform classifications as reflected in their reports of 2001, 2006, and 2010. Their initial aim to construct a "new scientific classification from application of methods used in biology that determines separate species and natural classes" proved elusive and, therefore, the last Commission in their report of 2010 confined their revisions to "new terminology and concepts" instead of "proposing a new classification (in the sense of organization) of epilepsies." It is unfortunate that most of the proposals in this report are modified interpretations and nomenclature of previous ILAE classifications; new terms are not better than the old ones, and recent advances have not been incorporated. Hence, the new ILAE report met with considerable protest from several expert epileptologists. This critical review refers mainly to the epileptic seizures, the classification of which may be an easier and less controversial task in the ILAE revisions. A revised classification should incorporate advanced knowledge of seizure pathophysiology, and clinical, interictal, and ictal manifestations. Such an attempt was made and detailed in the 2006 report of the ILAE Classification Core Group. However, these changes were largely discarded in the new ILAE report of 2010, without justification. This is inexplicable considering that the scientific advances that were available to the two Commissions were the same or had improved between 2006 and 2010. Of major concern is that "No specific classification is recommended for focal seizures which should be described according to their manifestations." Such a proposition defies the essence and the principle of any classification that requires an organization and a common language for communication. Free text descriptions are fine in a manual of differential diagnosis but not as a classification system. Another striking weakness is that even the accepted types of epileptic seizure are listed by name only, without defining them. The result is avoidable confusion. Furthermore, the report fails to consider reflex epileptic seizures. Status epilepticus is the most conspicuous omission despite immense advances of our understanding of it and its relevance on the classification. It appears that the new ILAE report does not fulfill its intent to improve the previous classifications and it may be premature to submit anything similar to this for approval by the ILAE General Assembly. The ILAE Commission could benefit by asking experts in basic and clinical science to provide a concise statement in their field of expertise as, for example, what are focal, myoclonic, or absence seizures, and their subtypes, their manifestations, and their possible pathophysiology. Areas of certainties and uncertainties, agreements and disagreements should be identified and stated clearly, with documentation of the reasons for it. Probably this is the only way forward for a truly scientific, sound, and clinically meaningful organizational system for the epileptic seizures and the epilepsies.

Purpose. To define the spectrum of the epileptic syndromes and epilepsies (other than the idiopathic epilepsies of childhood with occipital paroxysms) that can be associated with fixation-off sensitivity (FOS), delineate the electrographic types of FOS abnormalities and identify the patterns that can be associated with clinical seizures, and examine whether there may be a pure form of fixation-off sensitive epilepsy. Methods. We reviewed the clinical and video EEG data of all our patients with FOS over the last 12 years. Children with idiopathic focal epilepsies and occipital EEG paroxysms were excluded. Results. From January 1995 to December 2006, 19 of about 8,500 patients had had one or more video-EEGs with FOS, yielding an approximate incidence of 0.2%. From the 14 patients with full clinical and EEG data available, 12 had various epilepsies that included IGE phenotypes (7), symptomatic or probably symptomatic focal (3), cryptogenic generalised (1), and adult onset idiopathic photosensitive occipital (1), and two had no seizures. Seven patients (50%) were photosensitive. FOS EEG abnormalities were occipital in six patients, generalised in eight, and generalised with posterior emphasis in two patients. Seven of these patterns were associated with habitual seizures in seven patients, but actual FOS-induced seizures (absences) were documented with video EEG in only one patient; three others had some historical evidence suggesting that, under some circumstances, their FOS EEG abnormalities might generate clinical seizures. Conclusions. Despite the association of FOS with generalised and focal, symptomatic and cryptogenic and mild or pharmaco-resistant epilepsies, closer analysis of our data, and supportive evidence from functional imaging and physiological observations on alpha rhythm generation, disclose a prominent role of the occipital areas, even when FOS EEG abnormalities and seizures are ostensibly generalised. Although FOS appears to be of relatively low epileptogenicity, an electroclinical profile of pure FOS epilepsy may exist [Published with video sequences].

Behavioural disturbances and psychotic reactions are commoner in patients with epilepsy than in the general population and may be precipitated by the majority of antiepileptic drugs, including the newer ones. These reactions may be more frequent in patients with complex partial seizures, reflecting underlying temporal lobe pathology. A review of the literature on vigabatrin found an incidence of severe abnormal behaviour in controlled trials in adults of 3.4%. In children open studies gave an incidence of around 6%. This may be related to dosage and speed of introduction. Such reactions may be related to changes in seizure control, either unaccustomed good control (force normalisation) or breakdown in control, implying non-specific causative mechanisms. Alternatively, any relationship to control may be fortuitous and specific, unknown pharmacological mechanisms may be involved. Appropriate risk reduction measures include slow introduction, limiting the dose to that required for seizure control, slow withdrawal and increased vigilance in those on polytherapy or with psychiatric histories. Such advice is pertinent to all antiepileptic medications. Additionally, vigabatrin is probably contraindicated in idiopathic generalised epilepsies. Behavioural reactions are uncommon with vigabatrin, and have not been shown to be greater with it than with other antiepileptic agents. Therefore, it maybe inappropriate to withhold the drug from those who may benefit from it. Language: en

A big advance in epileptology has been the recognition of syndromes with distinct aetiology, clinical and EEG features, treatment and prognosis. A prime and common example of this is rolandic epilepsy that is well known by the general paediatricians for over 50 years, thus allowing a precise diagnosis that predicts an excellent prognosis. However, rolandic is not the only benign childhood epileptic syndrome. Converging evidence from multiple and independent clinical, EEG and magnetoencephalographic studies has documented Panayiotopoulos syndrome (PS) as a model of childhood autonomic epilepsy, which is also common and benign. Despite high prevalence, lengthy and dramatic features, PS as well as autonomic status epilepticus had eluded recognition because emetic and other ictal autonomic manifestations were dismissed as non-epileptic events of other diseases. Furthermore, PS because of frequent EEG occipital spikes has been erroneously considered as occipital epilepsy and thus confused with the idiopathic childhood occipital epilepsy of Gastaut (ICOE-G), which is another age-related but rarer and of unpredictable prognosis syndrome. Encephalitis is a common misdiagnosis for PS and migraine with visual aura for ICOE-G. Pathophysiologically, the symptomatogenic zone appears to correspond to the epileptogenic zone in rolandic epilepsy (sensory-motor symptomatology of the rolandic cortex) and the ICOE-G (occipital lobe symptomatology), while the autonomic clinical manifestations of PS are likely to be generated by variable and widely spread epileptogenic foci acting upon a temporarily hyperexcitable central autonomic network. Rolandic epilepsy, PS, ICOE-G and other possible clinical phenotypes of benign childhood focal seizures are likely to be linked together by a genetically determined, functional derangement of the systemic brain maturation that is age related (benign childhood seizure susceptibility syndrome). This is usually mild but exceptionally it may diverge to serious epileptic disorders such as epileptic encephalopathy with continuous spike and wave during sleep. Links with other benign and age-related seizures in early life such as febrile seizures, benign focal neonatal and infantile seizures is possible. Overlap with idiopathic generalized epilepsies is limited and of uncertain genetic significance. Taking all these into account, benign childhood focal seizures and related epileptic syndromes would need proper multi-disciplinary re-assessment in an evidence-based manner.

To evaluate the clinical associations of adult-onset photosensitivity, we studied the clinical and EEG data of patients who were referred due to a possible first seizure and who had a photoparoxysmal response on their EEG. Patients with clinical evidence of photosensitivity before the age of 20 were excluded. Of a total of 30 patients, four had acute symptomatic seizures, two had vasovagal syncope, and 24 were diagnosed with epilepsy. Nine of the 24 patients had idiopathic (genetic) generalized epilepsies and predominantly generalized photoparoxysmal response, but also rare photically-induced seizures, while 15 had exclusively, or almost exclusively, reflex photically-induced occipital seizures with frequent secondary generalization and posterior photoparoxysmal response. Other important differences included a significantly older age at seizure onset and paucity of spontaneous interictal epileptic discharges in patients with photically-induced occipital seizures; only a quarter of these had occasional occipital spikes, in contrast to the idiopathic (genetic) generalized epilepsy patients with typically generalized epileptic discharges. On the other hand, both groups shared a positive family history of epilepsy, common seizure threshold modulators (such as tiredness and sleep deprivation), normal neurological examination and MRI, a generally benign course, and good response to valproic acid. We demonstrated that photosensitivity can first occur in adult life and manifest, either as idiopathic (possibly genetic) photosensitive occipital epilepsy with secondary generalization or as an EEG, and less often, a clinical/EEG feature of idiopathic (genetic) generalized epilepsies. Identification of idiopathic photosensitive occipital epilepsy fills a diagnostic gap in adult first-seizure epileptology and is clinically important because of its good response to antiepileptic drug treatment and fair prognosis.

This chapter assesses probable epileptic syndromes within the idiopathic generalized epilepsies (IGE) that have not yet been recognized by the International League Against Epilepsy (ILAE). Jeavons syndrome, a purely reflex IGE that predominantly manifests with eyelid myoclonia and electroencephalogram (EEG) abnormalities on eye closure, is the most distinct and undisputed of the syndromes. Another is autosomal-dominant cortical tremor, myoclonus, and epilepsy, a purely monogenic disorder that has been documented in numerous reports, mainly from Japan and Italy. Perioral myclonia with absences is certainly a seizure type that may constitute an IGE syndrome when it is associated with a number of other clinical and EEG manifestations. Similarly, many patients suffer for years from phantom absences, a type of mild absence, before a first generalized tonic-clonic seizure that usually occurs in adulthood. Both perioral myoclonia with absences and phantom absences are clinically significant because they are probably lifelong and are associated with a very high incidence (around 50%) of absence status epilepticus that may escape diagnosis and appropriate treatment. The position of early childhood IGE, which manifests mainly with typical absence seizures that are distinctly different from childhood absence epilepsy and other recognized IGE syndromes, is less clear. The prevalence of these syndromes is significant. Their identification allows better clinical management and is important for genetic research and counselling. In addition, their recognition permits application of exclusion criteria for a more purified definition and a better understanding of the true boundaries of the other IGE syndromes already accepted by the ILAE.

Autonomic seizures and autonomic status epilepticus in children have a high prevalence, manifest with dramatic clinical symptoms, and have important clinical and management implications. They probably affect approximately 13% of children aged 3-6 years with one or more nonfebrile seizures, or 6% in the age group 1-15. The primary cause is an idiopathic age-dependent epileptogenic susceptibility (Panayiotopoulos syndrome), but 10-20% are due to cerebral pathology. Autonomic seizures and autonomic status epilepticus have been best studied in Panayiotopoulos syndrome, which has been confirmed worldwide in more than 800 cases and recently recognized in the new classification scheme of the International League Against Epilepsy. Seizures start with autonomic symptoms, mainly emesis, while the child is usually fully conscious. Other more conventional seizure manifestations often ensue, but autonomic manifestations commonly predominate to the end of the seizure. Ictal syncope (transient loss of consciousness and postural tone) is an intriguing common symptom. Half of the seizures last longer than 30 minutes, constituting autonomic status epilepticus. Prognosis is invariably excellent except for the symptomatic cases. The interictal EEG shows great variability from normal to severely epileptogenic, often with multifocal spikes. Pathophysiology of Panayiotopoulos syndrome is unknown, but it is likely that they are due to diffuse maturation-related epileptogenicity activating susceptible-for-children emetic centers and the hypothalamus. Thus, Panayiotopoulos syndrome is not occipital epilepsy, with which it is often erroneously equated. Autonomic seizures and autonomic status epilepticus are frequently misdiagnosed and often treated as encephalitis, atypical migraine, cardiogenic syncope, or other unrelated medical conditions such as gastroenteritis. This review examines the existing evidence, provides a means of improving diagnostic yield, and proposes practice parameters and guidelines for the diagnosis and management of autonomic seizures and autonomic status epilepticus in children.

Childhood absence epilepsy (CAE) is an idiopathic generalised epilepsy (IGE) characterised by onset of typical absence seizures in otherwise normal children of school age. A genetic component to aetiology is well established but the mechanism of inheritance and the genes involved are unknown. Available evidence suggests that mutations in genes encoding GABA receptors or brain expressed voltage-dependent calcium channels (VDCCs) may underlie CAE. The aim of this work was to test this hypothesis by linkage analysis using microsatellite loci spanning theses genes in 33 nuclear families each with two or more individuals with CAE. Seventeen VDCC subunit genes, ten GABA(A)R subunit genes, two GABA(B) receptor genes and the ECA1 locus on 8q24 were investigated using 35 microsatellite loci. Assuming locus homogeneity, all loci gave statistically significant negative LOD scores, excluding these genes as major loci in the majority of these families. Positive HLOD scores assuming locus heterogeneity were observed for CACNG3 on chromosome 16p12-p13.1 and the GABRA5, GABRB3, GABRG3 cluster on chromosome 15q11-q13. Association studies are required to determine whether these loci are the site of susceptibility alleles in a subset of patients with CAE.

Introduction - Lamotrigine is licenced in many countries for use in patients with partial seizures. Evidence suggests that it may also be effective in generalised epilepsies. Material & methods - We analysed retrospectively our patients with idiopathic generalised epilepsy with refractory absences. Results - Fifteen patients with idiopathic generalised epilepsies were identified who had been treated with lamotrigine for 3 months or more. All patients were also treated with sodium valproate. Fourteen patients had active absences. Nine (64%) had a total or virtual cessation of absences and in a further patient they became milder and less frequent. One patient reported an increase in seizures. The effective dose of lamotrigine was 1.6-3.0 mg/kg/day in children and 25-50 mg/day in adults. Patients who responded did so after the first or second dose. Lamotrigine was well tolerated. Conclusion - Low-dose lamotrigine added to sodium valproate appears to be effective in typical absence seizures. A therapeutic interaction of the two drugs seems likely.

Typical absences (previously known as petit mal) are generalised seizures that are distinctively different from any other type of epileptic fit.1 They are pharmacologically unique2-5 and demand special attention in their treatment.6 The prevalence of typical absences among children with epilepsies is about 10%, probably with a female preponderance.6 Typical absences are easy to diagnose and treat. Therefore, it is alarming that 40% of children with typical absences were inappropriately treated with contraindicated drugs, such as carbamazepine and vigabatrin, according to a recent report from London, UK.7 The purpose of this paper is to offer some guidance to paediatricians regarding diagnosis and management of typical absence seizures. This is also important because of the introduction of new antiepileptic drugs. These are mainly tested in partial (focal) epilepsies and there are inappropriate generalisations regarding their use in the treatment of other epilepsies. Typical absence seizures are defined according to clinical and electroencephalogram (EEG) ictal and interictal expression.1 6 Clinically, the hallmark of the absence is abrupt and brief impairment of consciousness, with interruption of the ongoing activity, and usually unresponsiveness. The seizure lasts for a few to 20 seconds and ends suddenly with resumption of the pre-absence activity, as if had not been interrupted. Although some absence seizures can manifest with impairment of consciousness only, this is often combined with the following

The authors studied the functional anatomy of the déjà vu (DV) experience in nonlesional temporal lobe epilepsy (TLE), using interictal fluorine-18 fluorodeoxyglucose PET in 14 patients with and 17 patients without DV. Several clinical conditions, such as age at PET study, side of ictal onset zone, and dominance for language, were no different between the two groups. The patients with DV showed significant relative reductions in glucose metabolism in the mesial temporal structures and the parietal cortex. The findings demonstrate that ictal DV is of no lateralizing value. They further suggest that temporal lobe dysfunction is necessary but not sufficient for the generation of DV. Extensive association cortical areas may be involved as part of the network that integrates this distinct experience.

Early-onset benign childhood occipital seizures (EBOS) described by Panayiotopoulos constitute the commoner after the rolandic phenotype of a childhood seizure susceptibility syndrome. EBOS are the clinical representative of occipital spikes. Their cardinal features are infrequent (often single) partial seizures manifested with deviation of the eyes and vomiting, frequently evolving to hemi- or generalized convulsions. Ictal behavioral changes, irritability, pallor, and rarely cyanosis, and eyes wide open are frequent. Retching, coughing, aphemia, oropharyngolaryngeal movements, and incontinence may occur. Consciousness is usually impaired or lost, either from the onset or the course of the fits, but in a few children, it may be preserved. Duration varies from a few minutes to hours (partial status epilepticus). Seizures are usually nocturnal, but semiology is similar in nocturnal or diurnal fits. Onset is between 1 and 12 years with a peak at 5 years. One third of children have a single seizure, the median total number of fits is two to three, and the prognosis is invariably excellent, with remission usually occurring within 1 year from onset. A few children may later develop rolandic or other benign partial seizures. The likelihood to have seizures after age 12 years is exceptional and rarer than that of febrile convulsions. EEG shows occipital paroxysms demonstrating fixation-off sensitivity, but random occipital spikes, occipital spikes in sleep EEG alone, or normal EEG may occur. Centrotemporal and other spike foci may appear in the same or more frequently in subsequent EEGs. The EEG does not reflect clinical course and severity.

This is a qualitative and chronological analysis of ictal and postictal symptoms, frequency of seizures, family history, response to treatment, and prognosis in nine patients with idiopathic occipital epilepsy and visual seizures. Ictal elementary visual hallucinations are stereotyped for each patient, usually lasting for seconds. They consist of mainly multiple, bright coloured, small circular spots, circles, or balls. Mostly, they appear in a temporal hemifield often moving contralaterally or in the centre where they may be flashing. They may multiply and increase in size in the course of the seizure and may progress to other non-visual occipital seizure symptoms and more rarely to extra-occipital manifestations and convulsions. Blindness occurs usually from the beginning and postictal headache, often indistinguishable from migraine, is common. It is concluded that elementary visual hallucinations in occipital seizures are entirely different from visual aura of migraine when individual elements of colour, shape, size, location, movement, speed of development, duration, and progress are synthesised together. Postictal headache does not show preference for those with a family history of migraine. Most of the patients are misdiagnosed as having migraine with aura, basilar migraine, acephalgic migraine, or migralepsy simply because physicians are not properly informed of differential diagnostic criteria. As a result, treatment may be delayed for years. Response to carbamazepine is excellent and seizures may remit.

In a previous report of 900 patients with epileptic seizures, 24 children had ictal vomiting. Twelve had a previously unrecognised syndrome of early onset benign childhood occipital seizures (EBOS) and three had symptomatic epilepsy. The other nine children with extraoccipital EEG foci or normal EEG are described in this paper based on a prospective follow up for a median of 9 years after their first seizure. All had normal neurology, mental state, and development. All seizures of all but one patient occurred in sleep. Seizures manifested with ictal vomiting (nine), deviation of the eyes (four), speech arrest (three), hemiconvulsions (five), oropharyngolaryngeal symptoms, and hypersalivation (one) with or without impairment of consciousness. Median age at onset was 5 years, seizures were infrequent and remitted at a median age of 6. Four children had a single fit, four had two to three, and only one child had many seizures before the initiation of treatment. This study certifies that idiopathic childhood partial seizures with ictal vomiting may occur with EEG spike foci in other than the occipital regions or EEG may be normal. Despite some clinico-EEG differences from the EBOS, childhood seizures with ictal vomiting, and extraoccipital EEG foci are of equally excellent prognosis. Their existence on the border between rolandic and occipital seizures is consistent with a unified concept of a benign childhood partial seizure susceptibility syndrome.

portrays eminent contributions(Caraballo et al., 2007; Ferrie et al., 2007; Koutromanidis,2007; Martinovic, 2007) on a common childhood seizuresusceptibility syndrome that primarily involves the auto-nomic nervous system (ANS). I have been honored withits eponymous nomenclature and the editors’ invitation towrite a personal note with the above title.It all started in 1975 with two otherwise normal chil-dren, A and B, who had EEG occipital paroxysms (Fig. 1)but different clinical manifestations (Panayiotopoulos,1980, 1981). I saw them in my part-time private prac-tice and followed their progress for over two decades(Table 1).My scientific curiosity of why occipital paroxysms oc-curredonlywhentheeyeswereclosedwassatisfiedwithaseries of simple tests that documented the activating roleof the elimination of central vision and fixation, whichI called “fixation-off sensitivity”(Panayiotopoulos, 1980,1981, 2002).The clinician’s responsibility of making an accu-rate diagnosis was more difficult to satisfy. Child Ahad brief visual occipital seizures or blindness oftenfollowed by headache (Panayiotopoulos, 1980; case 24in Panayiotopoulos, 1999a), which should be differen-tiated from migraine. Child B was a different diagnos-tic problem (case 1 in (Panayiotopoulos, 2002). In early

Summary: Purpose: To study the electroclinical features of typical absence status (TAS) in adults with syndromes of idiopathic generalized epilepsies (IGEs). Methods: Twenty-one patients with one or more spells of TAS were identified among 136 consecutive adult patients with IGEs. All patients with TAS had comprehensive electroclinical investigations and EEG or video-EEG recorded absences. Results: TAS occurred in 24.4% of 86 patients who had IGEs with typical absences alone or in combination with other seizures presisting in adult life. The prevalence of TAS appeared to be syndrome related, ranging from as high as 57.1% in perioral myoclonia with absences and 46.2% in “phantom” absences with GTCS to as low as 6.7% in juvenile myoclonic epilepsy. A varying degree of impairment of cognition was the cardinal clinical symptom shared in all TAS, but corresponding syndromes of IGE were often betrayed by other symptoms such as eyelid or perioral myoclonia. In phantom absences with GTCS, TAS was more numerous (p S 0.05) and more frequently the first overt seizure type (p = 0.006) than in any other IGE. Only in the syndrome of eyelid myoclonia with absences, TAS was always situation related, mainly as a result of antiepileptic drug discontinuation. Conclusions: The clinical EEG semiology and prevalence of TAS appear to be syndrome related with the highest prevalence in the syndromes of perioral myoclonia with absences and phantom absences with GTCS (p = 0.0024). Key Words: Typical absence status-Nonconvulsive status epilepticusIdiopathic generalized epilepsies-Epileptic syndromesAdults.

OBJECTIVES—The phenomenon of interictal regional slow activity (IRSA) in temporal lobe epilepsy and its relation with cerebral glucose metabolism, clinical data, MRI, and histopathological findings was studied. METHODS—Interictal 18F-fluorodeoxyglucose positron emission tomography (FDG PET) was performed under continuous scalp EEG monitoring in 28 patients with temporal lobe epilepsy not associated with intracranial foreign tissue lesions, all of whom subsequently underwent resective surgery. Regions of interest (ROIs) were drawn according to a standard template. IRSA was considered lateralised when showing a 4:1 or greater ratio of predominance on one side. RESULTS—Sixteen patients (57%) had lateralised IRSA which was always ipsilateral to the resection and of maximal amplitude over the temporal areas. Its presence was significantly related to the presence of hypometabolism in the lateral temporal neocortex (p=0.0009). Logistic regression of the asymmetry indices for all measured cerebral regions confirmed a strong association between IRSA and decreased metabolism of the posterior lateral temporal neocortex only (p=0.009). No significant relation could be shown between slow activity and age at onset, duration of the epilepsy, seizure frequency, and MRI evidence for hippocampal atrophy. Furthermore, IRSA was not specifically related to mesial temporal sclerosis or any other pathology. CONCLUSIONS—Interictal regional slowing in patients with temporal lobe epilepsy not associated with a mass lesion is topographically related to the epileptogenic area and therefore has a reliable lateralising, and possibly localising, value. Its presence is irrelevant to the severity or chronicity of the epilepsy as well as to lateral deactivation secondary to neuronal loss in the mesial temporal structures. Although slow EEG activity is generally considered as a non-specific sign of functional disturbance, interictal regional slowing in temporal lobe epilepsy should be conceptualised as a distinct electrographic phenomenon which is directly related to the epileptogenic abnormality. The strong correlation between interictal regional slowing and lateral temporal hypometabolism suggests in turn that the second may delineate a field of reduced neuronal inhibition which can receive interictal and ictal propagation.

A 16-year-old boy of normal intelligence had brief absence and myoclonic jerks since age 7 years. Video-EEG documented the unexpected combination of fixation-off sensitivity (scotosensitivity) with photosensitivity.

This 24-year-old woman of normal physical and mental state had from the age of 12 years brief seizures of visual hallucinations of multicolored circular flashing bright lights followed by severe headache and vomiting. These were initially misdiagnosed as migraine. One year later the visual hallucinations were followed by generalized convulsions. Electroencephalogram (EEG) consistently showed brief posterior paroxysmal fast spiking activity occurring immediately after closing of the eyes and not persisting with the eyes closed. These eye-closure related abnormalities were totally inhibited in complete darkness and elimination of central vision. Intermittent photic stimulation induced time-locked occipital spikes. This patient is of interest because she has an unusual form of idiopathic regional occipital epilepsy that may be misdiagnosed as migraine with aura.

A 33-year-old woman of borderline normal intelligence had catamenial absence status epilepticus and absences since early teens, diagnosed as temporal lobe epilepsy. Clinical and video-EEG evaluation documented that she suffers from a rare epileptic condition manifested with fixation-off sensitivity, absences, absence status, and infrequent generalized tonic-clonic seizures.

A 4-year-old boy had an autonomic seizure of Panayiotopoulos syndrome during video-EEG recording. Interictal EEG showed multifocal spikes in the centrotemporal and left posterior regions. Ictal electrographic onset included fast rhythms in the left posterior regions progressing to a mixture of high-amplitude spikes and fast and slow rhythms, bilaterally. The clinical symptoms (sighing, arousal with eyes opening, eye-deviation, and emesis with possible alteration of consciousness) started two minutes after the electrographic onset. These symptoms were mild and their characterisation as epileptic seizure behaviour would have been difficult without the ictal EEG documentation. The clinical manifestations of the child's previous epileptic seizures were mainly from the Rolandic area. [Published with video sequences].

This report comments on methodological issues related to the use of F-wave in clinical neurophysiology. An F-wave study aims to describe with relative accuracy the properties of the compound F-wave population which is the population of F-waves consecutively recorded from a muscle. This can only be achieved if an adequate number of F-waves is sampled. In order to avoid inaccuracies, correction of F-wave latency measurements for height or limb length and age is also required. Differences in the recording procedure could account for the variability in F-wave measurements. The usefulness of F-wave parameters other than latency is discussed. F-chronodispersion and F-tacheodispersion are more sensitive than conventional neurophysiological methods in detecting mild nerve lesions. F-persistence provides valuable information only if the findings are interpreted in correlation with the particular clinical setting. The suitability of the F-wave technique for routine studies is examined and a current view on the clinical applications is briefly recounted. There is an urgent need for the standardization of F-wave methodology.

A prospective study of 32 children with epileptic encephalopathies 12 years or younger revealed a high incidence of focal cortical metabolic defects on 18-fluorodeoxyglucose positron emission tomography (PET) not suspected from clinical, EEG, or magnetic resonance imaging findings. PET scans were normal in all five children with typical de novo Lennox-Gastaut syndrome but showed cortical metabolic abnormalities in three out of four with atypical de novo Lennox-Gastaut syndrome, five out of six with Lennox-Gastaut syndrome following infantile spasms, six out of eight with severe myoclonic epilepsy in infancy, one out of two with epilepsy with myoclonic-astatic seizures, and four out of six with an unclassified epileptic encephalopathy. This suggests that some children with epileptic encephalopathies previously thought to have primary generalised seizures or seizures due to multifocal pathology may have unifocal cortical origin for their seizures. Such an origin may be amenable to surgery.

A prospective electrophysiological study of phrenic nerve was performed in 59 subjects undergoing open-heart surgery. The nerve was stimulated percutaneously at the neck and the diaphragmatic response was recorded with surface electrodes placed over the 8th intercostal space. The latency, amplitude, duration and area of the evoked response were measured before and after the operation. Post-operatively no response was elicited in 2 patients bilaterally, in 5 from the left and in 2 from the right. Comparison of the post-operative with the pre-operative group values in the remaining subjects showed that the amplitude and area of the left phrenic were lower in the post-operative study, indicating that some of the nerve fibres were not conducting. There were no statistically significant differences between pre and post-operative values of latency or duration on the left or any of the parameters on the right. Our findings suggest that the amplitude and area of the diaphragmatic response are more sensitive than latency in detecting phrenic nerve paresis associated with open-heart surgery.

Occipital lobe epilepsies (OLEs) manifest with occipital seizures from an epileptic focus within the occipital lobes. Ictal clinical symptoms are mainly visual and oculomotor. Elementary visual hallucinations are common and characteristic. Postictal headache occurs in more than half of patients (epilepsy-migraine sequence). Electroencephalography (EEG) is of significant diagnostic value, but certain limitations should be recognized. Occipital spikes and/or occipital paroxysms either spontaneous or photically induced are the main interictal EEG abnormalities in idiopathic OLE. However, occipital epileptiform abnormalities may also occur without clinical relationship to seizures particularly in children. In cryptogenic/symptomatic OLE, unilateral posterior EEG slowing is more common than occipital spikes. In neurosurgical series of symptomatic OLE, interictal EEG abnormalities are rarely strictly occipital. The most common localization is in the posterior temporal regions and less than one-fifth show occipital spikes. In photosensitive OLE, intermittent photic stimulation elicits (1) spikes/polyspikes confined in the occipital regions or (2) generalized spikes/polyspikes with posterior emphasis. In ictal EEG, a well-localized unifocal rhythmic ictal discharge during occipital seizures is infrequent. A bioccipital field spread to the temporal regions is common. Frequency, severity, and response to treatment vary considerably from good to intractable and progressive mainly depending on underlying causes.

Many generalized tonic-clonic seizures are accompanied by profound autonomic changes. However, autonomic seizures and autonomic status epilepticus can also be seen with specific electroclinical syndromes (Panayiotopoulos syndrome), etiologies, and localizations. Such autonomic symptoms may impact cardiorespiratory function. While it is likely that several factors contribute to SUDEP, further study of both ictal respiratory and cardiac changes and underlying neuroanatomical mechanisms involved in autonomic seizure semiology are likely to provide important data to improve our understanding of the pathophysiology of this devastating condition. This paper will review the association between autonomic symptoms and epileptic seizures and will highlight the work of three young investigators. Drs. Lisa Bateman and Brian Moseley will review their work on cardiorespiratory effects of recorded seizures and how this assists in our understanding of SUDEP. Dr. John Millichap will review autonomic seizures and autonomic dysfunctions related to childhood epilepsy and will discuss the importance of expanded research efforts in this field.

Objective: To describe the clinical features of syncope-like epileptic seizures (SLES) and their frequency in Panayiotopoulos syndrome (PS). Methods: This was a 6-year prospective study of all children aged 1−15 years referred for an EEG. PS was defined by the occurrence of at least one autonomic seizure (AS) in a neurodevelopmentally normal child and at least one EEG with focal spikes. SLES were defined as self-terminating events of sudden loss of postural tone and unresponsiveness, occurring either concurrently with other ictal autonomic symptoms and signs that characterize PS (AS + SLES) or on their own (pure SLES). Results: PS was diagnosed in 33 of 394 consecutive children with at least one afebrile seizure (8.4%). SLES occurred at least once in 17 of 33 children (51.5%); 12 presented SLES in all their AS, and 5 had also AS without SLES. Overall, 53 of 74 AS manifested with SLES (71.6%); 25 were AS + SLES and 28 were pure SLES. The latter occurred in 7 children suddenly and without premonition or obvious triggers while standing, sitting, lying down, or asleep, did not resolve in the horizontal position, and were not associated with stiffening or any involuntary movements, even when longer than a few minutes. Concurrent autonomic symptoms during AS + SLES included emesis, incontinence, mydriasis, miosis, and cardiorespiratory abnormalities. Conclusions: SLES is a common ictal manifestation of PS and should be considered in the differential diagnosis of suspected syncope, particularly when clinical signs are atypical for neurocardiogenic syncope and the EEG shows focal spikes.

Fifteen patients who experienced epileptic seizures while playing video games are described together with a review of 20 cases in the English literature. Nine of the 15 cases and all but two of the reported cases experienced their first seizure while playing video games. Two thirds of patients had idiopathic generalised epilepsy and mainly reported generalised tonic clonic seizures, but some had typical absence seizures and myoclonic jerks while playing video games. In this series, 30% with idiopathic generalised epilepsy had juvenile myoclonic epilepsy. Overall, 70% of patients with idiopathic generalised epilepsy were photosensitive to intermittent photic stimulation and the mechanism of seizure provocation was probably similar to that of television induced seizures, although sensitivity to specific patterns was sometimes important. Two children had self induced video game seizures. Non-photic factors such as excitement, fatigue, sleep deprivation, cognitive processing, and diurnal variation in susceptibility seemed to be important seizure precipitants, particularly in non-photo-sensitive patients. Twenty nine per cent of patients had partial (mainly occipital) video game associated seizures. Occipital spikes were common in the EEG of these patients. Photosensitivity to intermittent photic stimulation may have been important in two patients but in the others, who all played arcade video games, other mechanisms need to be considered. Video game associated seizures are a feature of several epileptic syndromes and differ in precipitants and appropriate management.

Summary: We made a long term prospective study of 66 patients with juvenile myoclonic epilepsy (JME). Prevalence was 10.2% among 672 patients with epilepsies. Sex distribution was equal. Sixty-three were not diagnosed on referral; JME was not initially recognized in the epilepsy clinic in 22. Clinical typical absence seizures were reported in 33.3%, myoclonic jerks in 97% and generalized tonic-clonic seizures (GTC) in 78.8% of the patients. Mean age (±SD) at onset was 10.5 ± 3.4 years (range 5–16 years) for absence seizures, 15 ± 3.5 years (range 8–26 years) for myoclonic jerks, and 16 ± 3.5 years (9–28) for GTC. Absence predated myoclonic jerks by 3.9 ± 2.3 years (range-1–9 years) and GTC by 4.4 ± 2.7 years (range 1–8 years) in 14 (21.2%) patients who manifested all three types of seizure. Absence were never antedated by myoclonic jerks or GTC. Myoclonic jerks occurred on awakening in 87.5% of the patients. GTC occurred mainly on awakening, but other patients had nocturnal or diurnal GTC with no circadian distribution. Neurologic examination was normal for all patients except for tremor of the hands similar to essential tremor, noted in 35% of patients. Computed tomography (CT) brain scans were normal: 93% of patients had precipitating factors: sleep deprivation (89.5%), fatigue (73.7%), photosensitivity (36.8%; television and video games 8.8%), menstruation (24.1% of women), mental concentration (22.8%), and stress (12.3%). Incidence of JME among siblings (13 of 41 examined families) implies an autosomal recessive mode of inheritance for this Arab population. EEGs were frequently normal in treated patients. At least one abnormal EEG was recorded in 56 (84.9%) patients. Abnormalities consisted mainly of generalized discharges of spike/double spike and/or polyspike and slow wave. Frequent multiple spikes and discharge fragmentations varied from 0.5to 20-s duration (mean 6.8 s). Twenty (30.3%) had focal abnormalities, and 18 (27.3%) had photoconvulsive discharges. Eighty-eight percent of patients remained seizure-free for 3 years of follow-up. Effective treatment was achieved with valproate (VPA); control of myoclonic jerks was improved with clonazepam (CZP). CZP monotherapy did not consistently prevent GTC. Adding small doses of CZP with simultaneous reduction of VPA was the most effective and better tolerated form of medication, particularly in patients demonstrating an adverse reaction or requiring a large VPA dosage. VPA dosage was successfully reduced in 15 patients who were seizure-free for >2 years and had infrequent seizures before treatment, but 9 of 11 patients relapsed after VPA discontinuation. ReSUMe Les auteurs ont entrepris une etude prospective a long terme de 66 patients presentant une epilepsie myoclonique juvenile (EMJ). La prevalence etait de 10.2% parmi 672 patients presentant une epilepsie. La distribution entre les sexes etait egale. Soixante trois patients n'etaient pas diagnostiques au moment de leur premier contact; l'epilepsie myoclonique juvenile n'avait pas ete reconnue initialement dans la consultation epileptologique chez 22 patients. Les absences cliniques etaient reporetes chez 33.3%, des secousses myocloniques chez 97% et des crises tonico-cloniques chez 78.8% des patients. L'âge moyen du debut etait de 10.5 ± 3.4 ans (±SD, limite 5 a 16) pour les absences, 15 ± 3.5 (8 a 26) pour les secousses myocloniques et 16 ± 3.5 (9 a 28) pour les crises generalisees tonico-cloniques. Les absences precedaient les myoclonies de 3.4 ± 2.3 annees (de 1 a 9) et les crises generalisees tonico-cloniques de 4.4 ± 2.7 annees (1 a 8) chez les 14 patients (21.2%) qui presentaient les trois types de crises. Les absences n'ont jamais ete precedees par des secousses myocloniques ou par des crises generalisees tonicocloniques. Les secousses myocloniques etaient constatees au reveil chez 87.5% des patients. Les crises generalisees tonicocloniques survenaient principalement au reveil, mais d'autres patients avaient des crises nocturnes ou diurnes sans distribution circadienne. L'examen neurologique etait normal chez tous les patients, en dehors d'un tremblement des mains semblable au tremblement essentiel, constatt chez 35% des patients. Les scanners cerebraux ont toujours ete normaux. Les facteurs declenchant des crises ont ete constates chez 93% des patients: privation de sommeil chez 89.5%, fatigue chez 73.7%, photosensibilite chez 36.8% (television et jeux video 8.8%), menstruation chez 24.1% des femmes, concentration mentale chez 22.8%, stress chez 12.3%. L'incidence de l'EMJ dans les fratries (13 des 41 familles examinees) implique un mode de transmission autosomique recessif dans cette population d'origine arabe. Les EEG etaient souvent normaux chez les patients traites. Au moins un EEG anormal a ete enregistre chez 56 patients (84.9%). Les anomalies ttaient representtes surtout par des decharges generalisees de pointes, doubles pointes ou poly pointes-ondes lentes. La duree des pointes multiples et des fragmentations des decharges variaient de 0.5 a 20 secondes (en moyenne 6,8 secondes). Les anomalies focales ont ete constatees chez 20 patients (30.3%) et des decharges photoconvulsives chez 18 patients (27.3%). Quatre vingthuit pour cent des patients sont restes sans crise pendant 3 ans ou plus de suivi. Le traitement efficace a utilise le valproate (VPA). Le controle des secousses myocloniques a ete ameliore avec le clonazepam (CZP). Une monotherapie par CZP n'a pas permis de prevenir de facon constante les crises generalisees tonico-clonique. Une substitution partielle utilisant le valproate, avec de petites doses de CZP, a ete le traitement le plus efficace et le mieux tolere, particulierement chez les patients presentant des effets secondaires ou necessitant de fortes doses de VPA. La dose de VPA a ete reduite avec succes chez 14 patients qui n'avaient plus de crises depuis plus de 2 ans et qui n'avaient que de rares crises avant le traitement. Cependant, 9 patients sur 11 ont recidive aprts l'arret du VPA. RESUMEN Los autores han llevado a cabo un estudio prospectivo a largo plazo de 66 enfermos con Epilepsia Mioclonica Juvenil (JME). La prevalencia fue de 10.2% en 672 casos con epilepsia. La distribucion entre sexos fue equivalente. Sesenta y tres casos no habian sido diagnosticados por quienes enviaban los enfermos y la JME no fue reconocida inicialmente en la clinica de epilepsia en 22 sujetos. Los tipicos ataques de ausencia clinica aparecieron en 33.3%, los movimientos mioclonicos en 97% y los ataques generalizados tonico-clonicos (GTCs) en 78.8% de los enfermos. La edad media (tSD)a1 comienzo fue de 10.5 ± 3.4 (rango 5–16) ahos para las ausencias, 15 ± 3.5 (8–26) para los movimientos mioclonicos y 16 ± 3.5 (9–28) para los GTCs. Las ausencias precedieron a los movimientos mioclonicos en 3.4 ± 2.3 anos (rango 1–9) y en 4.4 ± 2.7 a los GTCs (rango 1–8) y en 14 anos (21.2%) en enfermos que presentaban los tres tipos de ataques. Las ausencias nunca fueron precedidas de ataques mioclonicos o GTCs. Las mioclonias se presentaron durante la vigilia en 87.5% de los enfermos. Los GTCs predominaron durante la vigilia pero otros enfermos tenian GTCs nocturnos o diurnos sin distribucion circadiana. La exploracidn neurologica fue normal en todos los pacientes con la excepcion de temblor en las manos semejante al temblor esencial que aparecio en el 35% de los enfermos. El CT de cerebro fue normal. En el 93% de los enfermos se encontraron factores precipitantes: la deprivacion del suedo en 89.5%, la fatiga en 73.7%, la fotosensibilidad en 36.8% (television y juegos de video en 8.8%), la menstruation en el 24.1% de las mujeres, el retraso mental en el 22.8% y el stress en el 12.3%. La incidencia de los JME entre hermanos (13 en 41 de las familias examinadas) sugiere un modo autosomico recesivo de herencia para esta poblacion Qrabe. Los EEGs fueron frecuentemente normales en los enfermos tratados. A1 menos un EEG anormal fue registrado en 56 enfermos (84.9%). Las anomalias consistieron, generalmente, en descargas generalizadas de puntddoble punta ylo polipuntas y ondas lentas. La frecuencia de las mliltiples puntas y la fragmentacion de las descargas variaron de 0.5–20 s (promedio 6.8 s) de duracion. Las anomalias focales se observaron en 20 (30.3%) y las descargas fotoconvulsivas en 18(27.3%) pacientes. El 88% de los enfermos permanecieron libres de ataques durante 3 o mas anos de seguimiento. La eficacia del tratamiento se consiguio con valproato (VPA) que controlo los movimientos mioclonicos y mejoro con clonazepam (CZP). La monoterapia con CZP no evito de mod0 consistente las GTCs. La sustitucion parcial de VPA por dosis bajas de CZP fue la medicacion mas eficaz y mejor tolerada particularmente en enfermos que demostraban reacciones adversas o requerian dosis altas de VPA. La reduccibn de las dosis de VPA fue posible en 14 enfermos que estaban libres de ataques durante mas de 2 afios y habian tenido ataques infrecuentes antes del tratamiento. Sin embargo 9 de 11 pacientes volvieron a tener episodios a1 interrumpir el VPA.

A detailed study of EEGs of patients with an unequivocal diagnosis of juvenile myoclonic epilepsy (JME) showed a high prevalence of focal EEG abnormalities. Focal slow waves, spikes, and sharp waves and focal onset of the generalized discharge were present in 36.7% of EEGs in our patients with JME. In more than half of the patients, at least one EEG showed focal abnormalities. These features should not be misconstrued as indicative of partial epilepsy.

\s=b\ Juvenile myoclonic epilepsy is a common idiopathic generalized epileptic syndrome that occurs in 5% to 10% of patients with epilepsy. Despite this, it is still frequently unrecognized and misdiagnosed, even as epilepsy of focal onset. Juvenile myoclonic epilepsy usually responds well to treatment with appropriate anticonvulsants, and misdiagnosis often results in unnecessary morbidity. This article reviews the syndrome, including the clinical and electroencephalographic features, the misinterpretation of which contributes to misdiagnosis. (Arch Neurol. 1993;50:594-598)

Any functional or structural cerebral derangement is a potential cause of epileptic seizures. Axis 4 of the ILAE Diagnostic Scheme (Table 1.1)1 is to specify the aetiology of epilepsies when known.1,2