A case of Panayiotopoulos syndrome showing an atypical course

We describe herein a patient with Panayiotopoulos syndrome (PS) showing an atypical course. The patient initially had seizures typical of this syndrome from 3 to 5 years of age. EEG showed right occipital high-amplitude sharp and slow-wave complexes followed by brief generalized discharges of slow waves. Sequential EEGs obtained from 5 to 11 years of age showed both multifocal discharges and generalized spike and wave complexes. With these changes in EEG findings, the patient experienced various types of seizures. The seizures were frequent and showed oculocephalic deviation followed by absence, atonic seizures, generalized tonic clonic convulsions and clonic seizures of the eyelids, which were observed between 7 and 10 years of age. Antiepileptic drugs were only partially effective for these seizures. Ictal EEG recorded at 8 years of age revealed high-voltage slow waves from the bilateral frontal and occipital regions prior to diffuse high-amplitude spike-wave bursts. At 9 years of age, magnetoencephalography (MEG) revealed the calculated dipoles of the preceding bifrontal spike-wave discharges to be in the frontal areas, while those of the following generalized spike-wave bursts were in the bilateral mid-temporal areas. In PS, reportedly, dipoles of multifocal epileptic discharges are usually located in the occipital and Rolandic areas. The unique clinical evolution in our case may be associated with the unusual frontal localization of dipoles detected by MEG.     

Epileptiform EEG Activity of the Centromedian Thalamic Nuclei in Children with Intractable Generalized Seizures of the Lennox-Gastaut Syndrome

Centromedian thalamic nuclei (CM) epileptiform EEG activities were recorded in children with intractable generalized seizures of Lennox-Gastaut syndrome (LGS) through implanted recording-stimulating electrodes used for seizure control. Ictal CM epileptiform activities were consistently correlated to widespread surface cortical EEG activities and symptoms in all patients and all types of generalized seizures; i.e., fast spike discharges at CM correlated at onset of tonic and tonic-clonic generalized seizures; slow (1-2 Hz) spike-wave complex discharges at CM correlated for atypical absence seizures; slow polyspike-wave complex discharges correlated for myoclonic seizures; and spike bursts and suppression patterns correlated for combined tonic-atonic-myoclonic seizures. Ictal EEG activities occurred simultaneously at right and left CM and surface at onset of all seizure types, with the exception of myoclonic seizures where CM complete discharges and individual spike-wave complexes significantly lead those of the surface. Brief tonic-atonic spasms clinically undistinguishable from "real" epileptic seizures showed no EEG counterparts at CM and surface. Interictal CM spike-wave complete discharges and individual spike-wave complexes showed variable amplitude-temporal patterns. Amplitude emphasis on CM and frontopolar regions was observed in most of complete discharges, however, and phase shifts between CM and frontopolar regions were observed in individual spike-wave complexes.     

Unilateral closed-lip schizencephaly and epilepsy: a comparison with cases of unilateral polymicrogyria

We compared the electroclinical features and evolution of patients with two different types of abnormal cortical organization: unilateral closed-lip schizencephaly (SCHZ) and unilateral polymicrogyria (PMG). Between February 1990 and June 2002, 51 children with either unilateral PMG or closed-lip SCHZ were selected through neuroradiological analysis for investigation at our service. We evaluated the frequency of epilepsy, electroclinical features and evolution. The mean time of follow-up was 7 years (range 1-12 years). All patients underwent neurological examination, computed tomography scan and magnetic resonance imaging, serial electroencephalographic (EEG) recordings and neuropsychological assessment. Thirty-six of the 51 patients had unilateral PMG. All patients had hemiparesis with mild spasticity. Mental retardation was mild in 20 and moderate in 14. In two patients IQ was normal. Partial motor seizures were recorded in 28 patients, with secondary generalization in 20. The median age at onset of seizures was 2 years (range 4 months-7 years). Interictal EEGs showed unilateral spikes in all patients. In 21 patients epilepsy worsened between the ages of 4 and 8 (mean 5.6 years) with frequent atonic seizures, atypical absences, epileptic negative myoclonus and gait difficulties. EEGs showed continuous spike-wave activity or bilateral high-frequency spike discharges during slow-wave sleep. Frequent relapses of atonic and myoclonic seizures were seen in nine patients. At present, 16 patients are seizure-free. Fifteen patients with unilateral SCHZ were included in the study. Focal motor seizures were registered in seven cases, in three of them with secondary generalization. The median age at onset of epilepsy was 2.5 years (range 1-4 years). Interictal EEGs showed unilateral spikes in these seven cases. All patients except one presented mild spastic hemiparesis. Mental retardation was mild in ten children, moderate in two and IQ was normal in three. Although the underlying mechanisms leading to PMG and SCHZ are probably similar, the electroclinical phenomenon of secondary bilateral synchrony with frequent negative myoclonus was not present in our cases with unilateral closed-lip SCHZ.     

Diagnostic issues and treatment of cryptogenic or symptomatic generalized epilepsies

To clarify the diagnostic issues and treatment of patients with cryptogenic or symptomatic generalized epilepsies, not including West syndrome (WS), we investigated electroclinical change during the clinical course, and treatment effects in these patients. The selection criteria were minor generalized seizures as their main seizure type and diffuse epileptic discharges as their main EEG findings. Regarding EEG, we included EEGs that predominantly displayed multifocal spike-waves because of the inclusion of severe epilepsy with multiple independent spike foci (SE-MISF). We divided the subjects into two groups according to their main seizure types: Group A (54 patients) with brief tonic seizures and Group B (24 patients) with myoclonic seizures and/or atypical absences. The main epileptic syndromes were considered to be Lennox-Gastaut syndrome and SE-MISF in Group A, and epilepsy with myoclonic-astatic seizures in Group B. A history of WS was often seen in Group A, but it was exceptional in Group B. During the clinical course, seizure types did not basically change in Group A. EEG patterns were changeable in both groups. Although there was some overlap in electroclinical manifestations among epileptic syndromes, a transition between the two groups was not seen. High-dose valproate and ethosuximide were the most effective in Groups A and B, respectively. Long-term prognosis was significantly more favorable in Group B than in Group A.     

4p- Syndrome: A Chromosomal Disorder Associated with a Particular EEG Pattern

Summary: We report an electroclinical and cytogeneticstudy of 4 patients with Wolf-Hirschhorn syndrome (WHS). In all cases, we observed a stereotyped EEG and clinical picture characterized by generalized or unilateral myoclonic seizures followed later by brief atypical absences. Electrographically, these were accompanied by a sequence of centroparietal or parietotemporal sharp waves; high-voltage wave with a superimposed spike becoming unusual spike-wave complexes, often elicited byeye closure; bursts of diffuse spikes and waves; and frequent jerks. This electroclinical pattern is very similar to the one described in Angelman syndrome (AS) in which a defect in GABA_A receptor function has been suggested. Moreover, the genes encoding the GABA_A receptor subunit have been mapped to the p12–pl3 bands of chromosome 4. Even though the deletion in these cases does not encompass the 4p12–p13 region, we suggest that the electroclinical picture common to WHS and AS might represent a characteristic type of epilepsy linked to a common genetic abnormality.     

A case of epilepsy with myoclonoic absences

A case of epilepsy with myoclonic absences is reported. A boy, 3 years and 8 months old, had the first attack at the age of 1 year and 8 months. He was mentally retarded, but had no evidence of organic brain damages. He had been said to have "absence" at another hospital for 2 years until he was referred to our hospital. The attack was characterized clinically by sudden loss of consciousness accompanied with rhythmical massive myoclonias. The ictal EEGs showed synchronous diffuse 3 c/s spike-wave discharges. There was a strict and constant relation between spike-wave discharges and the myoclonia. The polygraphy recording (EEG and EMG of various muscles) was very helpful for the diagnosis of epilepsy with myoclonic absences. We think that epilepsy with myoclonic absences should be considered in any case of "absence" with concomitant 3 c/s spike-wave discharges which is resistant to appropriate treatment, or is accompanied with mental retardation.     

Myoclonic encephalopathy in the CDKL5 gene mutation.

OBJECTIVE: Epilepsy with mutation of the CDKL5 gene causes early seizures and is a variant of Rett syndrome (MIM (312750), which is reported typically as infantile spasms. The purpose of this study was to analyze the epileptic histories and EEGs of patients with the CDKL5 mutation. METHODS: We reviewed the epilepsy histories and electroclinical analyses of three girls aged 9.5, 7.4, and 9.4 years, each with a mutation of the CDKL5 gene. RESULTS: We revealed the presence of an encephalopathy that started by 1.5 months of age. At first, seizures involved tonic spasms or complex partial seizures, and were complicated by the later appearance of complex partial, tonic, and unexpectedly, myoclonic seizures. This form of epilepsy was drug resistant. Routine and prolonged video EEGs both displayed a homogeneous electroclinical pattern consisting of (a) unique background with diffuse high voltage sharp waves of 6-7 Hz, and absence of the typical rhythmic frontal-central theta activity present in Rett syndrome; (b) unique awake and sleep background, with diffuse, high voltage, continuous sharp waves with multifocal and diffuse spikes; (c) rhythmic, diffuse, 15 Hz activity accompanied clinically by tonic seizures; (d) intercritical pattern with pseudoperiodic, diffuse, sharp waves or pseudoperiodic, diffuse spike and polyspike or wave discharges; and (e) diffuse, spike, polyspike and wave discharges accompanied by massive or focal myoclonias or both. CONCLUSIONS: Patients with the CDKL5 mutation have an early onset, epileptic encephalopathy in infancy that evolves into myoclonic seizures in childhood with a unique EEG pattern. SIGNIFICANCE: Recognizing this type of encephalopathy could be useful in prompting clinicians to proceed further with their diagnostic work in patients not fitting the criteria of classical Rett syndrome.     

The Long-Term Prognosis of Minor Seizures in Childhood Epilepsy

The long–term clinical and electroence-phalographic follow-up studies were carried out for more than three to 13 years on 72 cases with minor seizures in childhood epilepsy at the Department of Pediatrics of Keio University from January 1963 to January 1973. Minor seizures were classified into typical absences, infantile spasms, Lennox syndrome, myoclonic seizures and atonic seizures. The results of each group were summarized as follows, but atonic seizures were excluded because there were too few cases of this group.

• 1 Some patients with typical absences were not preceded by other epileptic seizures, while some others had generalized tonic-clonic seizures, including febrile convulsions (mixed form by Fukuyama), prior to the onset of typical absences. Typical absences were not transformed into other epileptic seizures excluding generalized tonic-clonic seizures at follow-up. Twenty-one (84%) out of 25 cases with typical absences had ceased having seizures over one year at follow-up. Spontaneous, generalized spike and wave discharges including 3 c/s spike and wave complexes were recorded in about one half of all cases at the final interictal EEG recordings, but focal components were never seen. Mental retardation was very rare.
• 2 The attacks of infantile spasms had a tendency to disappear or be converted to variously other epileptic fits, excluding typical absences, with the development of age. EEG-findings were transformed into focal or Muse paroxysmal discharges and basic dysrhythmia from hyp-sarhythmia after many years, but classical 3 c/s spike and wave discharges were never seen. Moderately or severely mental retardation was found in most of these patients.
• 3 It was characteristic of Lennox syndrome that the improvements or successions of tonic seizures and atypical absences were repeated and diffuse slow spike and wave discharges were sustained in most of children during the periods of follow-up. Mental retardation was found in half of all cases.
• 4 Myoclonic seizures had ceased in most of children without transformations to other epileptic fits at follow-up, but some tendency toward relative dirsculty in control of clinical seizures was noted. Two thirds of these patients were of normal intelligence, while one third had retarded intelligence at follow-up.

Based on the above–mentioned results, the literatures concerning prognosis of various types of minor seizures in childhood epilepsy were studied.     

Epileptic seizures difficult to differentiate from alternating hemiplegia in infants: a case report

A child with epileptic seizures had a clinical course similar to that of alternating hemiplegia of infancy (AHI). Tonic hemiplegia began at 2 months of age, and atonic alternating hemiplegia and tetraplegic attacks began at 2 years of age. Clinical findings were paroxysmal ocular movement abnormalities, choreoathetotic involuntary movements, and severe developmental retardation. An interictal EEG at 6 years of age showed multiple independent spike discharges. An ictal EEG showed diffuse irregular spike-wave and slow wave bursts following focal spikes. The present case suggests that a long-term extensive follow-up is necessary to differentiate epileptic seizures from AHI.     

Epilepsy With Myoclonic Atonic Seizures: An Electroclinical Study of 69 Patients

Epilepsy with myoclonic-atonic seizures is characterized by myoclonic-atonic, absence, tonic-clonic, and eventually tonic seizures, appearing in previously normal children at ages 18-60 months. We analyzed the electroclinical features, treatment, and outcome of 69 patients with myoclonic-atonic seizures; these patients were followed between 1990 and 2012 at the Juan P. Garrahan Pediatric Hospital, Buenos Aires, Argentina. No structural or metabolic etiology was identified. Based on the electroclinical features and evolution, two groups could be distinguished. The first group of 39 patients with myoclonic and myoclonic-atonic seizures with or without generalized tonic-clonic seizures and absences associated with generalized spike- and polyspike-and-wave paroxysms had excellent prognoses. The second group of 30 patients had myoclonic jerks and myoclonic-atonic seizures associated with other seizure types including tonic seizures; some had myoclonic status epilepticus and cognitive deterioration. The interictal EEG showed frequent generalized spike- and polyspike-and-wave paroxysms. In 16 patients, the seizures remitted within 3.6 years. The two groups were distinguished in retrospect, when enough time had elapsed to evaluate cognitive deterioration and different seizure types. In conclusion, epilepsy with myoclonic atonic seizures is an epileptic syndrome with a broad clinical spectrum and variable prognosis.     

Hidden focal EEG seizures during prolonged suppressions and high-amplitude bursts in early infantile epileptic encephalopathy.

OBJECTIVE: We report on a 27-month-old female with atypical early infantile epileptic encephalopathy (EIEE), who developed tonic spasms, partial seizures and myoclonic jerks along with episodic bradycardia at 5 days. METHODS: We recorded digital electroencephalography (EEG) using either an 11-channel neonatal montage or 19 channel scalp electrodes, at 200 Hz sampling rate, and a single reference for a minimum of 30 min. RESULTS: At 18 days EEG showed suppression-burst (SB) patterns during wakefulness and sleep. Tonic spasms concomitant with bursts recorded as brief, low-amplitude fast waves. EEG at 8 months showed increased amplitude of bursts to 1 mV and extension of suppression periods to 65 s. By increasing recording sensitivity, we detected focal epileptiform discharges of slow rhythmic sharp and slow waves building to 30 microV during suppression periods. Status epilepticus occurred at 16 months. EEG at 27 months returned to the previous SB pattern with rare partial seizures. CONCLUSIONS: This report is the first to demonstrate clinically silent focal EEG seizures during prolonged suppression periods in atypical EIEE by off-line digital EEG. SIGNIFICANCE: Digital EEG sensitivity can reveal covert electrical activity during suppression periods in epileptic neonates and infants.     

Aggravation of epilepsy by valproate sodium in a child with cryptogenic localization-related epilepsy

We report a paradoxical effect of valproate sodium (VPA) observed in a 3-year-old girl with cryptogenic localization-related epilepsy. On admission she experienced two types of seizures that were confirmed by ictal EEGs : complex partial seizures (CPSs) originating from the left hemisphere and combined seizures that began with repetitive myoclonic seizures immediately followed by a CPS. These myoclonic seizures did not possess asymmetrical features, but the ictal EEGs showed left-side dominant multiple spike-waves. The patent's interictal EEGs on admission showed left posterior temporal- parietal spikes during wakefulness and frequent diffuse spike-waves during sleep. In the process of introduction and increase in the dosage of VPA, an aggravation of epileptic discharges, especially a dramatic increase in diffuse spike-waves during sleep, was observed. In the same period of time, myoclonic seizures not followed by CPS newly appeared, and there was an increase in the frequency of CPSs and combined seizures. Marked improvement of epileptic discharges, namely the disappearance of diffuse discharges, and complete suppression of all types of seizures were achieved by the introduction of carbamazepine (CBZ) along with the withdrawal of VPA. During the clinical course, the patient did not display any signs or symptoms of VPA encephalitis, overdose of VPA or metabolic aberration. The paradoxical effect of CBZ in localization-related epilepsy is well-known, yet in this case, VPA displayed a similar paradoxical effect. Additionally, CBZ was efficacious in the suppression of secondary bilateral synchrony on EEG and also successfully controlled CPSs, combined seizures and myoclonic seizures.     

Distinct behavioral and EEG topographic correlates of loss of consciousness in absences

PURPOSE: To describe the behavioral and EEG topographic correlates of absences with 3-Hz generalized spike-waves and partitioned impairment of consciousness. METHODS: Two adult women had so-called "phantom" absences, characterized by brief and mild impairments of consciousness that were previously inconspicuous to both patient and physician. Neuropsychological examination was performed under video-EEG monitoring during absence status. EEG topographic mapping of spike-wave discharges was obtained in the two cases. RESULTS: Only mild attentional and executive disturbances were observed during absence status despite prolonged discharges. Spike-wave bursts were associated with selective impairment in the initiation of response and self-generated action, whereas short-term storage of external information during discharges was fully preserved. This is consistent with a predominant involvement of frontomesial cortex demonstrated by topographic mapping of spike-wave discharges in the two cases. By contrast, in two other patients with typical absences and a complete lack of retention for information given during the discharges, topographic mapping found a more lateral frontal involvement by spike-wave activity. CONCLUSIONS: Different types of absence seizures may impair distinct components of conscious behavior. A predominant involvement of frontomesial thalamocortical circuitry may underlie an "inconspicuous" disorder of consciousness as seen in phantom absences with selective loss of initiation and goal-oriented behavior, whereas involvement of more lateral frontal areas in typical absences may additionally disrupt working memory processes.     

SYNGAP1-DEE: A visual sensitive epilepsy

ObjectiveTo further delineate the electroclinical features of individuals with SYNGAP1 pathogenic variants.MethodsParticipants with pathogenic SYNGAP1 variants and available video-electroencephalogram (EEG) recordings were recruited within five European epilepsy reference centers. We obtained molecular and clinical data, analyzed EEG recordings and archived video-EEGs of seizures and detailed characteristics of interictal and ictal EEG patterns for every patient.ResultsWe recruited 15 previously unreported patients and analyzed 72 EEGs. Two distinct EEG patterns emerged, both triggered by eye closure. Pattern 1 (14/15 individuals) consisted of rhythmic posterior/diffuse delta waves appearing with eye-closure and persisting until eye opening (strongly suggestive of fixation-off sensitivity). Pattern 2 (9/15 individuals) consisted of diffuse polyspike-and-wave discharges triggered by eye closure (eye-closure sensitivity). Both patterns presented in 8/15. Including archived video-EEG clips of seizures from 9/15 patients, we analyzed 254 seizures. Of 224 seizures experienced while awake, 161 (72%) occurred at or following eye closure. In 119/161, pattern 1 preceded an atypical absence, myoclonic seizure or myoclonic absence; in 42/161, pattern 2 was associated with eyelid myoclonia, absences and myoclonic or atonic seizures.ConclusionsFixation-off and eye closure were the main triggers for seizures in this SYNGAP1 cohort.SignificanceCombining these clinical and electroencephalographic features could help guide genetic diagnosis.     

Oxcarbazepine and atypical evolution of benign idiopathic focal epilepsy of childhood

Patients that have benign epilepsy with centrotemporal spikes (BECTS) may occasionally experience an atypical development in their course when treated with drugs such as carbamazepine. Three patients with electroclinical patterns consistent with BECTS showed seizure exacerbation during oxacarbazepine (OXC) therapy. Two manifested atypical absences, neuropsychological disturbances, and generalized spike-and-wave discharges in their electroencephalograms (EEGs) that became continuous during sleep. The third patient showed, during OXC therapy, more frequent partial motor seizures which ended with ictal vomiting and post-ictal obnubilation. EEGs recorded during sleep showed discontinuous paroxysmal activity in the right centrotemporal area. Symptoms were reversed following discontinuation of the OXC therapy. Although electroclinical findings were consistent with a BECTS diagnosis, all patients had some atypical features. Our observations show that BECTS patients, in particular those presenting with atypical findings, might be at risk for developing paradoxical reactions to OXC therapy. We suggest that OXC should be included in the list of drugs that may cause electroclinical deterioration in these patients.     

Electroclinical patterns and evolution of epilepsy in the 4p- syndrome

BACKGROUND: Wolf-Hirschhorn syndrome (WHS) is a well-known clinical entity caused by partial deletion of the short arm of one chromosome 4 (4p- syndrome). Seizures occur in almost all the cases, but studies on the electroclinical disorder and its evolution are still scarce. We present a longitudinal study of the electroclinical features in 10 children with WHS. METHODS: Ten patients (five boys and five girls) underwent a detailed clinical assessment and a prolonged EEG study. Six of the 10 also had video-polygraphy. RESULTS: Nine of the 10 patients had seizures; they were generalized or unilateral clonic and tonic-clonic, and atypical absences associated with myoclonic jerks. Age at onset of seizures varied from 1 day to 2.5 years. In all the patients, including the only one without seizures, two stereotyped EEG patterns were observed, consisting of (a) bursts of rhythmic (3-5 Hz), high-voltage slow waves located in the posterior regions and increased by sleep, or bursts of rapid spike-wave complexes in the centroparietal and parietooccipital regions; and (b) repetitive rapid posterior spikes. Sleep organization was constantly absent or very poor. The evolution of epilepsy was frequently good, with four seizure-free cases at the end of follow-up, two of them weaned from antiepileptic drugs (AEDs). CONCLUSIONS: Seizure onset in WHS also can occur at neonatal age. At least two electrical stereotyped patterns of the epileptic disorder are associated with a relevant disorganization of the sleep states. Prognosis of epilepsy is generally good both for the seizure control and for its evolution.     

Myoclonic-astatic epilepsy of early childhood--clinical and EEG analysis of myoclonic-astatic seizures, and discussions on the nosology of the syndrome.

Purpose: The aim of this study is to elucidate the clinical and neurophysiological characteristics of the myoclonic, myoclonic-astatic, or astatic seizures in patients with myoclonic-astatic epilepsy (MAE) of early childhood, and to discuss on the nosology of this unique epileptic syndrome.Subjects: The subjects included 30 patients, who fulfilled the following modified International League Against Epilepsy (ILAE) criteria for MAE, and whose main seizures were captured by video-electroencephalographs (EEG) or polygraphs. The modified ILAE criteria includes: (1) normal development before onset of epilepsy and absence of organic cerebral abnormalities; (2) onset of myoclonic, myoclonic-astatic or astatic seizures between 7 months and 6 years of age; (3) presence of generalized spike- or polyspike-wave EEG discharges at 2-3 Hz, without focal spike discharges; and (4) exclusion of severe and benign myoclonic epilepsy (SME, BME) in infants and cryptogenic Lennox-Gastaut syndrome based on the ILAE definitions.Results: The seizures were investigated precisely by video-EEG (n=5), polygraph (n=2), and video-polygraph (n=23), which identified myoclonic seizures in 16 cases (myoclonic group), atonic seizures, with or without preceding minor myoclonus, in 11 cases (atonic group), and myoclonic-atonic seizures in three cases. All patients had a history of drop attacks, apart from ten patients with myoclonic seizures. Myoclonic seizures, involving mainly the axial muscles were classified into those with mild intensity not sufficient to cause the patients to fall (n=10) and those that are stronger and sufficient to cause astatic falling due to flexion of the waist or extension of the trunk (n=6). Patients in the atonic group fell straight downward, landed on their buttocks, and recovered immediately. Analysis of the ictal EEGs showed that all attacks corresponded to the generalized spike or polyspikes-and-wave complexes. In the atonic form, the spike-and-wave morphology was characterized by a positive-negative-deep-positive wave followed by a large negative slow wave. In two patients, the intensity of the atonia appeared to correspond to the depth of the positive component of the spike-and-wave complexes. We did not detect any significant differences in the clinical and EEG features and prognosis, between the atonic and myoclonic groups.Conclusions: Although the determination of exact seizure type is a prerequisite for diagnosing an epileptic syndrome, the strict differentiation of seizure type into either a myoclonic or atonic form, does not appear to have a significant impact on the outcome or in delineating this unique epileptic syndrome. At present, we consider it better to follow the current International Classification of Epileptic Syndromes and Epilepsies until a more appropriate system than the clinico-electrical approach for classifying patients with MAE is available.     

Late-onset, "Gastaut type", childhood occipital epilepsy: an unusual evolution.

We report on two girls and one boy with clinical and electroencephalographic features of late-onset childhood epilepsy with occipital paroxysms of the "Gastaut type", showing an unusual evolution. Neurological examination and brain imaging were normal in all three. At the age of 7.5 years, eight years and ten years respectively, the three children presented with episodes of visual symptoms when awake, and in one of them, the seizures were occasionally followed by oculocephalic deviation. The interictal EEG showed bilateral occipital spike-wave activated by eye closing. In two patients, the occipital seizures had been immediately followed by typical absences, since onset; in the other patient, five months after onset. The ictal EEG showed irregular bilateral occipital spike-wave discharges during the visual symptoms, followed by generalized spike-wave activity during the typical absences. The typical absences were activated by hyperventilation; the EEG did not show continuous spikes and waves during slow sleep. These three patients, with typical electroclinical features of "Gastaut type", childhood occipital epilepsy, demonstrated an evolution which, to our knowledge, has not been previously described. We investigated whether this unusual, age-dependent evolution was due to secondary bilateral synchrony or if these electroclinical features represent two types of idiopathic epileptic syndromes in the same patients.     

Video-EEG Analysis of Drop Seizures in Myoclonic Astatic Epilepsy of Early Childhood (Doose Syndrome)

We studied 36 drop seizures in 5 patients with myoclonic astatic epilepsy of early childhood (MAEE) with simultaneous split-screen video recording and polygraph. Sixteen were falling attacks and 20 were either less severe attacks exhibiting only deep head nodding or seizures equivalent to drop attacks in terms of ictal pattern but recorded in the supine position. All seizures except those that occurred in patients in the supine position showed sudden momentary head dropping or collapse of the whole body downward. Recovery to the preictal position was observed in 0.3-1 s. As a result of carefully repeated observations, the 36 seizures were classified as myoclonic flexor type in 9, myoclonic atonic type in 2, and atonic type, with and without transient preceding symptoms in the remaining 25. The MF seizure was characterized by sudden forward flexion of the head and trunk as well as both arms, which caused the patient to fall. In the myoclonic atonic seizure, patients showed brief myoclonic flexor spasms, immediately followed by atonic falling. The AT seizure showed abrupt atonic falling, with and without transient preceding facial expression change and/or twitching of extremities. The ictal EEGs of all 36 seizures exhibited generalized bilaterally synchronous single or multiple spike(s) and wave discharges. Atonic drop attacks appear to be a common cause of ictal epileptic falling in MAEE.     

Non-convulsive status epilepticus associated with tiagabine therapy in children

A case of a 7-year-old male with epilepsy who developed non-convulsive status epilepticus (NCSE) with electroclinical features consistent with those of atypical absence seizures after adjunctive antiepileptic therapy of tiagabine (TGB) is reported. The patient had frequent generalised and rare partial seizures with generalised epileptic discharges on prior electroencephalogram (EEG) recordings. NCSE was developed when rapid dosage increase and high dose of TGB was given. This case emphasises the need for close monitoring of children with epilepsy taking TGB for exacerbation of seizures or development of NCSE.