Photosensitivity in epileptic syndromes of childhood and adolescence.

PURPOSE: Photosensitivity, a reaction of the brain to external photic stimulation, can be graded from 1 to 4, and is most frequently seen in the first decades of life. This study investigated photosensitivity in children with epilepsy. METHODS: A retrospective study performed in the neuropaediatric department of the largest paediatric hospital in Kiel, treating patients at all medical care levels. The clinical data and EEG records of 566 patients with the most common epileptic syndromes were analyzed, in particular regarding photosensitivity. Their EEGs included application of intermittent light stimulation using standard techniques at twice the minimum. RESULTS: The proportion of photosensitive patients was significantly higher in the paediatric cohort than in adult patients, as published in the literature: 46% of patients with generalized epilepsies showed photosensitivity as compared to 20% with focal epilepsies. Photosensitivity was more common in idiopathic generalized epilepsy (IGE), (epilepsy with grand mal on awakening, 74%; juvenile absence epilepsy, 56%; juvenile myoclonic epilepsy, 50%; childhood absence epilepsy, 44%) than in focal types (idiopathic partial - Rolandic epilepsy, 23%; symptomatic/cryptogenic type of epilepsy, 16%), while in patients who experienced occasional seizures (neonatal/febrile seizures), this ranged between 40% and 23%, respectively. The generalized photoparoxysmal response, (PPR), grades 3 and 4 were found significantly more often in patients with IGE (92%) than in patients with focal epilepsies. Finally, the female preponderance was confirmed (37% to 27% of all epilepsies). CONCLUSIONS: Photosensitivity can be detected both in patients with IGE, with idiopathic and symptomatic/cryptogenic types of focal epilepsies, and with epileptic (occasional) seizures. PPR grades 3 and 4 are the most common in IGE.     

A comparison of adult onset and "classical" idiopathic generalised epilepsy

OBJECTIVES: To report the characteristics of a population of patients with idiopathic generalised epilepsy (IGE) with age of onset over 20 years, and compare them with patients with "classical" IGE. METHODS: Data were collected from a computerised database of all patients with IGE attending a regional adult epilepsy clinic. Demographic data, epilepsy characteristics, and treatment outcomes were recorded. RESULTS: 72 patients with IGE of a total population of 844 had an age of onset over 20 years (8.5%). There was similar incidence of family history of epilepsy, EEG findings, and remission rates between those with a younger and older age of onset of IGE. There was a lower incidence of previous febrile convulsions in patients with adult onset. There were fewer patients with absence seizures in the adult onset group (15.3% v 46.4% in the "classical" group). CONCLUSIONS: IGE with onset later than the third decade was rare in the population studied. Prolonged EEG in selected patients may be helpful in diagnosing adult onset IGE, but the diagnosis of epilepsy remains clinical. Adult onset IGE shares many features with "classical" IGE, including EEG findings and prognosis, and is likely to represent a genetic epilepsy.     

24小时动态脑电图癔病诱发试验对癔病与癫痫的鉴别诊断价值

目的寻找鉴别癔病性发作与癫痫发作的有效方法。方法对９６例发作性疾病患者进行２４小时动态脑电图监测及癔病诱发试验（ＡＥＥＧ－ＨＰＴ）研究。结果难治性癫痫疑伴癔病组（１８例）、癔病或癫痫发作诊断不定组（３６例）、癫痫组（１８例）、癔病组（２４例）４组中癔病诱发试验阳性率分别为７７．８％、６６．７％、５．６％、９１．７％；记录到非诱发下自发发作（癫痫或癔病）分别为３、７、３、１例；记录到发作间期癫痫性放电分别为１６、１８、１７、２例。９６．０％的患者明确诊断。结论ＡＥＥＧ－ＨＰＴ是鉴别癔病与癫痫发作的有效方法之一。     

Sleep spindle asymmetry in epileptic patients.

OBJECTIVE: To investigate sleep spindle asymmetry in patients with idiopathic generalized epilepsy (IGE), cryptogenic partial epilepsy (CPE), and symptomatic partial epilepsy (SPE). METHODS: Post-sleep deprivation EEG records of the patients were investigated. Fast Fourier transform was applied to 200 s of stage-2 activity (without spikes, spike-waves and artefacts). Individual spindle frequency and topography was identified in each patient. Spindle intensity was computed and expressed in microvolts. In order to calculate spindle asymmetry the site of maximum spindle intensity within one hemisphere was compared to the mirror site in the other hemisphere. Asymmetry was expressed by the asymmetry index (AI). In the PE group AI was defined as [AI=(P-N)/(P+N)x100], where P and N were spindle intensity in the pathological and normal hemisphere, respectively. In the IGE group P and N were substituted for L and R (spindle intensity in the left and right hemisphere, respectively). RESULTS: In IGE patients spindle asymmetry was in the range of -13 to +12%. No significant lateralization to the left or right hemisphere was observed. In PE patients, spindle asymmetry ranged from -23 to+29%. As compared to spindle intensity in the unaffected hemisphere, spindling was enhanced in the 'epileptic' hemisphere in most PE patients (15/20 in the CPE and 5/7 in the SPE group). CONCLUSION: In IGEs (where the epileptic condition involved both hemispheres to the same extent) spindle intensity did not show significant asymmetry. In PE patients (where the epileptic process was clearly lateralized to one hemisphere while the other hemisphere was unaffected) enhanced spindling usually was related to the side of the epileptic process. The generally held notion that the pathological hemisphere displays less spindling than the healthy one might be misleading in cases of focal epilepsy. The results suggest that epileptogenic cortex slightly facilitates spindle generation.     

The prevalence of seizures in comatose children in the pediatric intensive care unit: A prospective video‐EEG study

Purpose: Studies in adult and neonatal intensive care units (ICUs) report a high prevalence of epileptic seizures in comatose patients. The prevalence of seizures in pediatric ICUs is variably reported in a few retrospective studies using different electroencephalography (EEG) methods. We aimed to determine prospectively the prevalence of epileptic seizures (clinical and subclinical) in comatose children in the pediatric ICU using continuous video‐EEG (v‐EEG) monitoring. Methods: We performed v‐EEG in consecutive children aged 2 months to 17 years admitted to the pediatric ICU with sustained depressed consciousness over a period of 15 months. Results: We monitored 100 comatose children, 69% within 24 h of ICU admission. Median length of ICU stay was 5 days. Median duration of v‐EEG was 20 h. Epileptic seizures were identified in only seven patients, of whom six had a history of epilepsy with witnessed seizures immediately prior to v‐EEG. All epileptic seizures were recorded in the first 3 h of v‐EEG. Seizures were suspected by ICU staff in 18 monitored patients, only four of whom had confirmed epileptic seizures. Discussion: The lower prevalence of epileptic seizures and the shorter length of ICU stay in children compared to adults and neonates suggest a different spectrum of disease and neurologic response. Short‐duration v‐EEG in patients with a history of prior seizures, epilepsy, or clinical events suspected to be seizures seems more appropriate than routine v‐EEG in all comatose children in the pediatric ICU.     

The diagnostic aid of routine EEG findings in patients presenting with a presumed first-ever unprovoked seizure

Data are available on the yield of a single EEG recording in patients with epilepsy but there is little information on EEG findings as an aid in supporting the diagnosis of an epileptic event in patients presenting with a first-ever event suspected of being an unprovoked seizure. We retrieved files of patients above the age of 15 years admitted through the emergency room during 1991-1995 with presumed first-ever unprovoked seizure. There were 91 patients (age 50+/-24; 52 males), of whom 66% had a presumed seizure of unknown origin and 34% had presumed remote symptomatic seizures. About 80% had generalized seizures (primarily or secondarily). In all the patients an EEG had been performed within 48 h of the event. Abnormal EEGs were obtained in 69%, with epileptiform activity in 21% (10% focal, 9% generalized and 2% focal and generalized), slowing in 58% (21% focal, 31% generalized and 7% focal and generalized), and both epileptiform activity and slowing in 10%. Epileptiform activity was most common in younger patients with seizures of unknown origin, compared with older individuals with symptomatic seizures (34, 38 vs. 27%, 7%, P=0.001). We conclude that following a single unprovoked presumed seizure, adults commonly exhibit abnormalities in an EEG recorded close in time to the event. The EEG is particularly helpful in supporting the epileptic nature of the event in younger patients and in those with seizures of unknown origin.     

Idiopathic generalized epilepsies with versive or circling seizures

OBJECTIVES: To describe the electroclinical features of the idiopathic generalized epilepsies (IGEs) with versive or circling seizures. METHODS: Sixteen patients with versive or circling seizures and interictal electroclinical features of IGE were studied. Patients with insufficient clinical or imaging data, with a follow-up period less than 1 year or with partial seizures in addition to the versive or circling ones were excluded from the study. All patients underwent full interictal clinical and neurophysiological studies. The EEG patterns of 13 versive or circling seizures from 4 patients were also analyzed. RESULTS: A specific IGE syndrome was recognized in 9 out of the 16 patients (56%). More specific, 1 patient had childhood absence epilepsy (CAE), 4 had juvenile absence epilepsy (JAE), and 4 had juvenile myoclonic epilepsy (JME). No specific IGE syndrome was recognizable in the remaining 7 patients (44%). These 7 patients had a juvenile epileptic syndrome (mean age at onset of seizures was 15.7 years) characterized by versive or circling seizures followed or not by generalized tonic-clonic fits. Three main EEG patterns were identified during versive or circling seizures: 1) generalized spike-and-wave discharges at 3-4 cps; 2) generalized polyspike-and-wave discharges at 1 to 2.5 cps beginning with generalized fast activity at 12-14 cps, and 3) generalized spike-and-wave discharges at 3-4 cps intermingled with fast activity at 12-14 cps. Most patients had good response to treatment on a single drug regimen (mainly valproic acid). CONCLUSIONS: Versive or circling seizures may occur in the context of an IGE. Although many individuals share the features of different IGE syndromes including CAE, JAE and JME, a consistent number of patients, who show circling or versive seizures solely, remain without a specific syndromic diagnosis. When occurring in the context of IGE, circling or versive seizures do not worsen the prognosis.     

Outcome after extended callosal section in patients with primary idiopathic generalized epilepsy

Purpose:   We report the outcome of patients with refractory idiopathic generalized epilepsy (IGE) who were submitted to extended one-stage callosal section.
Methods:   Eleven patients with IGE who were submitted to extended one-stage callosal section were studied. Preoperative workup included history and neurologic examination, interictal, and ictal electroencephalography (EEG) recording, high resolution 1.5T magnetic resonance imaging (MRI) and intelligence quotient (IQ) testing. All patients were submitted to extended one-stage microsurgical callosal section, leaving only the splenium intact.
Results:   Preoperative ictal patterns included repetitive spike and wave or polyspike and wave discharges or fast epileptic recruiting rhythm. MRI showed no focal lesions. Preoperatively, mean general IQ was 85. Postoperatively, at least a 75% reduction in the frequency of generalized tonic–clonic seizures was noted in all patients. In three patients absences disappeared completely, and the others had at least 90% reduction in seizure frequency. Only one patient had myoclonic seizures preoperatively, and these seizures disappeared after callosal section. After surgery, mean general IQ was 89. A very clear increase in attention level was noted in all patients. Postoperative interictal EEG recordings showed rupture of bilateral synchrony in all patients.
Discussion:   This article reports on a large and homogeneous series of patients with refractory IGE submitted for callosal section. There was a marked decrease in generalized seizure frequency and increase in the attention level in this patient population. Our results suggest that corticocortical interaction might have a role in IGE pathogenesis. Callosotomy is a safe, effective, and underused palliative procedure in these well-selected patients with refractory IGE.     

Clinical application of neuroimaging in epilepsy

OBJECTIVE: To evaluate the use of neuroimaging in clinical practice and to assess the prevalence of detected structural abnormalities in epilepsy patients in a clinical set up. METHODS: 919 outpatients were identified and the scan results reviewed. A total of 677 patients had chronic active epilepsy (88 had idiopathic generalised epilepsy (IGE), 588 had localisation related epilepsy, one had symptomatic generalised epilepsy), 57 had a single epileptic seizure, 46 were in remission, and 139 had non-epileptic attacks. RESULTS: 391 patients had no scan (53 patients in this group had IGE, 182 had localisation related epilepsy, one had generalised symptomatic epilepsy, 18 had single epileptic attacks, 21 were in remission, 116 had non-epileptic attacks). Altogether 528 patients had a scan, the results were not available in 33, 163 had x ray computed tomography (CT) only, 178 had standard magnetic resonance imaging (MRI) (slice thickness 5 mm), and 154 had high resolution MRI (including a T1 weighted sequence with 1.5 mm thick slices). Some 252 of 495 scans (51%) were abnormal. Abnormalities were hippocampal sclerosis (n=128), atrophy or non-specific white matter lesions (n=35), vascular abnormalities (n=27), tumours (n=25), brain damage (n=24), malformations of cortical development (n=13). Excluding atrophy and non-specific white matter lesions the prevalence of detected abnormalities was 54% in localisation related epilepsy, 18% in single seizure patients, 16% in epilepsy in remission, and 0% in IGE and non-epileptic attacks. CONCLUSIONS: Abnormalities were detected in more than half of all patients with localisation related epilepsy, and in about one in five patients with single seizures or epilepsy in remission. Many patients had no scan or only CT or standard MRI. The true prevalence of structural abnormalities may be have been higher. Scanning did not add any information in patients with IGE or non-epileptic attacks.     

De novo late‐onset absence status epilepticus or late‐onset idiopathic generalized epilepsy? A case report and systematic review of the literature

Aim. Idiopathic (genetic) generalized epilepsies (IGEs) are age‐related epileptic syndromes with typical age onset in childhood or adolescence. We report a patient with de novo late‐onset absence status epilepticus (ASE) occurring at the age of 64 years, with clinical and EEG features suggestive of late‐onset IGE. We also discuss the relationship between de novo late‐onset ASE and late‐onset IGE, and provide a comprehensive and critical review of the available literature on late‐onset (i.e. onset ≥60 years) IGE. Methods. MEDLINE (1966–2016 [23^th April]) was systematically searched in order to identify reports of patients with late‐onset IGE. Grey literature was also comprehensively searched. Results. We identified nine patients with electroclinical features suggestive of late‐onset IGE. Median age at seizure onset was 71 years (range: 60–80), with a female prevalence (67%). A family history of epilepsy was reported in 67% of cases. All patients had generalized tonic‐clonic seizures, and 44% also had myoclonic seizures. Treatment and outcome were reported for six patients; all of whom reached seizure freedom under monotherapy with valproic acid (83%) or lamotrigine (17%) (range of follow‐up: 3 to 24 months). Conclusion. Late‐onset IGE are entities with unknown prevalence and incidence, and should be differentiated on the basis of late‐onset reactivation of previous IGE. Late‐onset IGEs are probably unrecognized or misdiagnosed, based on a common misconception that all elderly individuals with first‐ever seizures have focal symptomatic epilepsy. Late‐onset IGE should be actively investigated by accurate history taking aimed at identifying seizures, which may have been unnoticed, and familial antecedents of epilepsy. In elderly patients presenting with de novo late‐onset ASE, a diagnosis of late‐onset IGE should be considered in the differential diagnosis, particularly in atypical cases (e.g. absence of triggering factors, coexistence of generalized tonic‐clonic or myoclonic seizures, and interictal generalized epileptiform discharges).     

PLED pattern and its clinical significance in stroke patients

The pathophysiological connection between periodic lateralized epileptiform discharges (PLED) and epileptic seizures is still not clear. In the study clinical data and EEG findings were analysed in 22 patients aged 43-90 years with a history of stroke in whom EEG disclosed PLED. Eleven patients were studied in the acute phase of stroke and 11 were studied years after stroke when the diagnosis was established of poststroke epilepsy. In 2 patients in acute stroke group single epileptic seizures occurred and 5 had partial status epilepticus. In the group with poststroke epilepsy 4 had single seizures and 4 had epileptic status with partial epilepsy seizures. Thus, in 15 out of 22 patients PLEDs were noted after epileptic seizures. In all cases PLED appearance was connected with consciousness disturbances, lasting 1 to 17 days. In 6 cases PLED pattern was interrupted by seizure activity over one hemisphere, in 3 of them partial epileptic seizures were associated with it. In acute phase of stroke neuroimaging demonstrated the presence of fresh ischaemic foci, but in cases of poststroke epilepsy no such fresh foci were observed. These results suggest that PLED frequently can be associated with epilepsy, and in some patients it can be a bioelectrical manifestation of partial status epileptic.     

Épilepsies généralisées idiopathiques chez le sujet âgé : le point de vue du gériatre

Introduction

Long-term follow-up studies indicate a low remission rate in idiopathic generalised epilepsies (IGE) (Martinez-Juarez et al., 2006), suggesting they may persist to an advanced age. However there are few estimates of IGE frequency in the elderly.

Methods

EEGs of 700 patients aged over 70 years, recorded between January 2006 and March 2007, were reviewed for anomalies consistent with IGE. We then examined the clinical history of patients with these anomalies.

Results

A persistent IGE was identified in four female patients (mean age: 79 years); in two cases it was a juvenile myoclonic epilepsy (JME) and in two an epilepsy with grand mal seizures. Seizures in three patients had begun in childhood or adolescence and in one at 40 years. Before hospitalization, few or no seizures were reported and IGE had not been diagnosed. IGE was revealed in each patient by a relatively severe event: an absence status (AS), subcontinuous myoclonic seizures or repeated convulsive generalised seizures (CGS). These events were not situation-related but in one patient the relapse of simple convulsive seizures, may have been related to the withdrawal of anti-epileptic drugs (AED) several months previously. EEG records showed generalised spikes or polyspikes and waves organised in a status epilepticus or in interictal rhythmic discharges. In one case they were evident only from a 24 hours recording. Clonazepam injection was used to suppress the AS episode and the subintrant myoclonia. After the AS, interictal generalised epileptic discharges persisted. Two of the four patients had familial history of epilepsy or febrile seizures but in no case was an epileptogenic lesion evident in brain CT scan or MRI. Clinical exams and biologic parameters were normal. All of the patients had worked and were married with children. Appropriate therapies were followed after the diagnosis of IGE. One patient with JME had been treated by Valproate which was discontinued by the general practitioner because of lethargy and replaced by Carbamazepine; seizures were aggravated under both Carbamazepine and then Lamotrigine and until the patient became seizure-free on Levetiracetam. The antiepiletic treatment was also modified in a second patient, while the two others responded well to Valproate.

Conclusions

IGE can exacerbate in the elderly, as different types of seizures including AS, subintrant myoclonia or repeated CGS. Our data suggest persistent IGE are quite frequent in an aged population and may be underestimated due to difficulties in diagnosis. Correctly diagnosed, adjustment of AED may offer substantial clinical improvements in IGE of the elderly.     

Outpatient Video-EEG Monitoring in Children

Summary: Video-EEG monitoring enables correlation of behavioral activity with EEG activity, which is useful in recognition of pseudoepileptic seizures and in investigation of patients for epilepsy surgery. Because most patients are monitored for a prolonged time as in-patients, the cost of the procedure is high. We investigated the value of brief (2–3 h) outpatient video-EEG monitoring in 43 children with frequent seizures, most of whom had symptomatic generalized epilepsy. Indications for monitoring included differentiation of epileptic from nonepileptic behavior, seizure classification, and determination of seizure frequency. Clinical episodes were recorded in 36 of 43 children (83%). A definite diagnosis was established in 9 of the 17 patients investigated to determine the nature of the clinical behavior. Seizures were classified in 1.5 of the 25 patients investigated to determine seizure type, and classification was different from the original in 9 of the 15 children. A change in epilepsy syndrome classification was made in 9 children. The video-EEG allowed diagnosis in 25 of the 43 children (59.5%). Video-EEG appears to be an effective method for outpatient investigation of children with frequent seizures, particularly those with symptomatic géneralizéd epilepsy.     

Prevalence of epilepsy in rural Bolivia: a door-to-door survey

OBJECTIVE: To carry out a door-to-door survey in rural areas of the Cordillera Province, Santa Cruz Department, Bolivia, to determine the prevalence of neurologic diseases (epilepsy, stroke, parkinsonism, and peripheral neuropathy) in a sample of approximately 10,000 inhabitants. METHODS: A team of nondoctor health workers administered a standard screening instrument for neurologic diseases-a slightly modified version of the World Health Organization protocol. All subjects found positive during the screening underwent a neurologic examination. RESULTS: On screening, the authors found 1,130 positive subjects, of whom 1,027 were then investigated by neurologists. On the basis of the definition proposed by the International League Against Epilepsy, we detected 124 epileptic patients (prevalence, 12.3/1,000), 112 of whom had active epilepsy (prevalence, 11.1/1,000) on the prevalence day (November 1, 1994). Peak age-specific prevalence occurred in the 15 to 24-year age group (20.4/1,000). Sex-specific prevalence was higher in women (13.1/1,000) than men (11.4/1,000). Eighty-nine patients (71.8%) underwent a standard EEG recording. Considering both EEG and clinical data, partial seizures were the most common type (53.2%) based on the classification of the International League Against Epilepsy. The mean age at onset was 20.7 years for partial seizures and 13.6 years for generalized seizures. Only 10.5% of patients had received specific treatment for more than 2 months of their life. CONCLUSION: This report on epilepsy prevalence in Bolivia confirms that epilepsy is a major health problem in rural areas of developing countries.     

Clinical-etiological and therapeutic profile of 719 Mexican epileptic children

RATIONALE: We performed this study with the intention of describing the clinical-etiological characteristics and therapeutic responses in a group of epileptic children seen at the Instituto Nacional de Pediatría (INP), a tertiary facility in Mexico City. METHODS: All patients who attended the Epileptic Clinic between March and June 1998 and fulfilled the selection criteria were enrolled in the study. Clinical and therapeutic response data were recorded. Three groups were formed by etiology. Statistical tests were two-tailed, with alpha=0.05. RESULTS: In all, 719 patients were studied. The distribution by etiology was as follows: group I, idiopathic (123 patients); group II, cryptogenic (132); and group III, symptomatic (464). In group I, 56% of the patients were female. Mean age at onset was 5 years 2 months (SD: 3 years 8 months) in group I; 1 year 11 months (SD: 2 years 5 months) in group II; and 2 years 10 months (SD: 3 years 1 month) in group III. The mean evolution time was 5 years 4 months (SD: 4 years) in all groups. The most frequent variety of epilepsy in the three groups was generalized epilepsy, followed by partial epilepsy. The following epileptic syndromes were identified: in group I, 28 patients had epilepsy with generalized tonic-clonic seizures, 15, absence epilepsy, and 6, benign rolandic seizures; in group II, all 32 patients had focal cryptogenic epilepsy; in group III, 235 had generalized symptomatic and 192, focal symptomatic epilepsy. The main etiologies were hypoxic ischemic encephalopathy (24%) and neural infections (22%). Appropriate seizure control was achieved in 108 (87%) patients in group I; 84 (64%) in group II; and 315 (68%) in group III. In group I, no patient needed more than two antiepileptic drugs and 90% had normal psychomotor development. CONCLUSION: When the three groups were compared in terms of appropriate epileptic control and normal psychomotor development, group I differed from the other groups and the difference was statistically significant.     

Latency to first spike in the EEG of epilepsy patients.

BACKGROUND: Routine EEGs in individuals with epilepsy have interictal spikes in 56% of cases. The availability of prolonged EEG has changed the use of EEG in the assessment of epilepsy. OBJECTIVE: To determine the time to first epileptiform activity on EEG in patients with epilepsy. This data will help optimize the duration of electrographic assessment for interictal activity in epileptic individuals. METHODS: 46 consecutive patients aged 10 years or older with epilepsy were evaluated. Individuals with seizures in the prior 24h or with acute symptomatic seizures were excluded. Continuous EEG (for 1-7 days) was analyzed to find the first definite epileptiform activity and the latency assessed. RESULTS: 37% of the patients had epileptiform activity in the first 20min of the continuous recording (duration of a routine EEG). 89% had epileptiform activity within 24h. The yield drops beyond 24h. 8% of the individuals had no epileptiform activity even after 72h. CONCLUSIONS: The study suggests the need to consider a change in EEG strategy to assess interictal epileptiform activity. The greatest probability of capturing an interictal abnormality within 20min was in individuals with generalized epilepsy. In individuals with suspected epilepsy in whom electrographic interictal spike confirmation is deemed necessary, after a first nonspecific or normal routine EEG, a 24h EEG should be the next step in the electrographic assessment. This study suggests that there may not be much benefit in monitoring for durations longer than 24h, unless capturing a seizure is the intent.     

EEG features in idiopathic generalized epilepsy: clues to diagnosis

PURPOSE: To investigate the EEG profile and its contribution for diagnosis and management in a group of patients with a clinical diagnosis of idiopathic generalized epilepsy (IGE) who were referred to a tertiary hospital. METHODS: We retrospectively studied clinical and EEG features of 180 consecutive patients with IGE. Eighty patients were diagnosed with juvenile myoclonic epilepsy (JME), 35 had absence epilepsy (AE), 13 had generalized tonic-clonic seizures on awakening (GTCS-A), 28 had generalized tonic-clonic seizures only (TCS), and 24 had adult-onset idiopathic generalized epilepsy (AIGE). The EEGs were classified in typical (synchronous generalized spike or polyspikes-and-wave discharges with normal background), atypical (with clear focalities or asymmetries), and normal. RESULTS: The 493 EEG exams were analyzed. The first EEG was normal in 45% of the 180 patients, and only 33% had typical abnormalities. AE had a higher proportion of typical examinations and needed fewer sequential examinations to register a typical abnormality compared with the other groups. By contrast, the serial EEG profile of TCS and AIGE showed a higher proportion of normal and atypical EEG findings. CONCLUSIONS: These findings support previous recommendations that IGE patients should be treated with appropriate therapy based on clinical history. Waiting for a typical abnormal EEG pattern can generate an unacceptable delay in the correct diagnosis and treatment of these patients. In patients with long-term epilepsy, the diagnosis may be difficult. Furthermore, serial EEGs can help to elucidate the syndromic diagnosis, especially in patients with TCS and AIGE.     

Eyelid myoclonia with absences: an overlooked epileptic syndrome?

AIM: To identify, among patients referred to our Epilepsy Center, those fulfilling eyelid myoclonia with absences (EMA) criteria and to evaluate their semiological, electroclinical and evolutive features. In addition, to examine some possible causes of underdiagnosis and to stress the role of video-EEG (VEEG) recording. MATERIALS AND METHODS: Retrospective analysis of 2780 epileptic patients. INCLUSION CRITERIA: Eyelid myoclonia and brief absences, related to EEG generalized paroxysmal activity and triggered by eye closure and/or by intermittent photic stimulation. RESULTS: 7.46% of our patients with idiopathic generalized epilepsy (IGE) could be classified as EMA. Female/male ratio was 1.7:1. Familial history of epilepsy was present in about half of the patients, with two pairs of identical twins in the sample. Rare generalized tonic-clonic seizures occurred in most cases. CONCLUSIONS: EMA is a not infrequent condition among IGEs. It is likely to be underdiagnosed due to the subtle clinical semiology and to masking of EEG changes by the effects of age and anti-epileptic drugs. VEEG analysis is often needed for diagnosis of EMA. Most likely, only genetic research will be able to clarify whether EMA is a distinct epileptic syndrome.     

Induction of partial epileptic seizures by flumazenil

PURPOSE: This study addressed the efficacy of flumazenil (FMZ) to induce or activate interictal or ictal epileptic discharges in patients with medically intractable partial epilepsies. METHODS: Flumazenil, 1 mg, was injected intravenously in 67 patients undergoing presurgical monitoring for epilepsy surgery, 49 of whom had been treated with benzodiazepines (BZDs) before flumazenil was given. Continuous video electroencephalogram (EEG) monitoring with surface or intracranial electrodes was used to evaluate interictal EEG activity, ictal discharges, and the occurrence and semiology of clinically manifest epileptic seizures. RESULTS: Interictal epileptiform potentials did not change in frequency or distribution after FMZ. In patients not pretreated with BZDs, epileptic seizures could not be provoked. In eight of the 49 patients pretreated with BZDs, epileptic seizures occurred within 30 min of FMZ application. Seizure semiology and regional EEG onset were identical to seizures recorded without FMZ. Patients operated on according to seizure-onset localization with FMZ had a >75% reduction in seizure frequency or became seizure free. CONCLUSIONS: Seizure induction by FMZ seems to be a valid method for evaluating seizure semiology and localization of the seizure-onset zone during presurgical monitoring of patients with medically intractable localization-related epilepsies.