Childhood Epilepsy With Occipital Paroxysms Research Articles

Long-Term Clinical and Electroencephalography (EEG) Consequences of Idiopathic Partial Epilepsies.

BackgroundIdiopathic partial epilepsies of childhood (IPE) affect a considerable proportion of children. Three main electroclinical syndromes of IPE are the Benign Childhood Epilepsy with Centro-temporal Spikes (BECTS), Panayiotopoulos Syndrome (PS), and Childhood Epilepsy with Occipital Paroxysms (CEOP). In this study we investigated the long-term prognosis of patients with IPE and discussed the semiological and electroencephalography (EEG) data in terms of syndromic characteristics.Material/MethodsThis study included a group of consecutive patients with IPE who had been followed since 1990. Demographic and clinical variables were investigated. Patients were divided into 3 groups – A: Cases suitable for a single IPE (BECTS, PS and CEOP); B: cases with intermediate characteristics within IPEs; and C: cases with both IPE and IGE characteristics. Long-term data regarding the individual seizure types and EEG findings were re-evaluated.ResultsA total of 61 patients were included in the study. Mean follow-up duration was 7.8±4.50 years. The mean age at onset of seizures was 7.7 years. There were 40 patients in group A 40, 14 in group B, and 7 in group C. Seizure and EEG characteristics were also explored independently from the syndromic approach. Incidence of autonomic seizures is considerably high at 2–5 years and incidence of oromotor seizures is high at age 9–11 years. The EEG is most abnormal at 6–8 years. The vast majority (86%) of epileptic activity (EA) with parietooccipital is present at 2–5 years, whereas EA with fronto-temporal or multiple sites become more abundant between ages 6 and 11.ConclusionsResults of the present study provide support for the age-related characteristics of the seizures and EEGs in IPE syndromes. Acknowledgement of those phenomena may improve the management of IPEs and give a better estimate of the future consequences.

Neurocognitive evaluation in children with occipital lobe epilepsy

PurposeThis study aimed to explore cognitive functions in patients with childhood epilepsy with occipital paroxysms (CEOP) and to compare the performance of these patients with that of patients with symptomatic occipital epilepsy (SOE) and healthy control subjects. MethodTwenty-eight patients with epilepsy (17 CEOP, 11 SOE) were enrolled. The control group had similar demographical characteristics. Cognitive functions evaluated with Wechsler Intelligence Scale for Children-revised edition (WISC-R), The Visual Aural Digit Span (VADS) and Bender Visual Motor Gestalt Test (BVMG). ResultsThe WISC-R showed lower performance IQ with WISC-R in patients with occipital epilepsy than in healthy controls. The VADS test only showed lower scores in children with symptomatic occipital epilepsy. Mean BVMG test scores were significantly abnormal in both subgroups of childhood epilepsy with occipital paroxysms (early-onset CEOP/late-onset CEOP) and the group with SOE. ConclusionPatients with CEOP, especially the late-onset form, have significant problems in the domains of visuomotor coordination, memory and attention. The academic performance of these patients should be monitored carefully in follow-up and appropriate educational support should be given as necessary.

Cognitive functions evaluated by P300 and visual and auditory number assays in children with childhood epilepsy with occipital paroxysms (CEOP)

This study was planned to evaluate cognitive functions, especially attention and immediate recall, in children with childhood epilepsy with occipital paroxysms (CEOP), by using P300 and neuropsychological tests, which included visual and auditory number assays. Thirty patients with CEOP, ages ranging from 5 to 17 years were enrolled in the study. Twenty-five healthy children were taken as the control group. Oddball paradigm was used in P300 recordings. The latency and the amplitude of the P300 wave recorded from Cz were taken into consideration. The neuropsychological test battery included visual and auditory number assays. P300 latency was significantly longer in the CEOP group (p=0.014). The results of the visual and auditory number assay test showed significant decline in the patient group when compared with the normal controls. Attention and immediate recall deficits as well as prolonged P300 latencies in children with CEOP can be due to an ongoing epileptic activity either influencing the whole brain or only the occipital lobe which can also be involved in the neuropsychological organization of the human cortex. Therefore, children with CEOP should be evaluated with more detailed neuropsychological tests for possible cognitive deficits.

Peripheral and central activation of trigeminal pain pathways in migraine: data from experimental animal models.

Animal models for migraine have provided substantial advances on the mechanisms and mediators underlying migraine attacks. The neurogenic inflammation model has helped understanding the perivascular mechanisms underlying the pathophysiology of migraine attacks, the receptors involved and the effect of specific antimigraine drugs. The model based on probing the neuronal effects of nitroglycerin--an organic nitrate known to induce spontaneous-like migraine attacks in predisposed subjects--in the rat has provided interesting insights into the neuroanatomic circuits and neuropharmacological mechanisms involved in the initiation and repetition of migraine attacks [corrected].

Electroencephalography in migraine: a review with focus on quantitative electroencephalography and the migraine vs. epilepsy relationship.

EEG-studies in migraine in the last decade has contributed modestly to the understanding of headache pathogenesis. Headache patient groups seem to have increased EEG responses to photic stimulation, but a useful biological marker for migraine in single patients has not been found. In future EEG and QEEG studies we recommend to use follow-up designs and record several EEGs across the migraine cycle. It is also important to use a blinded study design in order to avoid selection bias. A clinical EEG should be performed in patients with acute headache attacks when either epilepsy, basilar migraine, migraine with prolonged aura or alternating hemiplegia is suspected. Unequivocal epileptiform abnormalities usually suggest a diagnosis of epilepsy. In children with occipital spike-wave activity the probable diagnosis is childhood epilepsy with occipital paroxysms (CEOP). The final diagnosis of either an epilepsy syndrome or migraine must be mainly based on a clinical judgement [corrected].

Visual evoked potentials in children with occipital epilepsies

The objectives of this study are to see if any visual evoked potential (VEP) differences are present in two forms of occipital epilepsy, childhood epilepsy with occipital paroxysms (CEOP) and symptomatic occipital epilepsy (SOE) with respect to etiology, as CEOP is a benign age- and localization-related idiopathic epilepsy while SOE is a symptomatic form. Nineteen patients with CEOP and 13 patients with SOE were included in the study and P100 potential latency and amplitude values obtained from these patients were compared with the values recorded from normal controls. The amplitude values recorded from the patients with CEOP were significantly high ( P=0.033). P100 potential latency values recorded in patients with SOE were significantly long ( P=0.028). High amplitude VEP responses were mostly attributed to hyperexcitability of the occipital cortical structures whereas prolonged latency P100 responses were attributed to occipital structural changes.

Atypical evolution in childhood epilepsy with occipital paroxysms (Panayiotopoulos type)

ABSTRACT We report, on two, school‐age girls with clinical and electroencephalographic features of early onset childhood epilepsy with occipital paroxysms (CEOP) of the “Panayiotopoulos type” that showed atypical evolution. Neurological examination and brain imaging were normal in both. One child presented at age 2.5 years episodes of oculocephalic deviation, and ictal vomiting during nocturnal sleep. The EEG showed left occipital spikes during wakefulness and sleep. One year later, frequent inhibitory seizures appeared in the lower limbs causing, “pseudoataxic gait”. At the same time she presented with behavioral disturbances and aphasia. EEG showed bilateral spike‐waves while awake and continuous spike‐waves during slow sleep (CSWSS). After switching AEDs to benzodiazepines, control of seizures along with improvement of behavior, and partial restoration of cognitive functions were achieved. The CSWSS disappeared and the last EEG at age 8 years only showed only isolated right occipital spikes. The other girl had a personal and familial history of febrile seizures. At 4 years of age she presented the first non‐febrile seizures during sleep, with oculocephalic deviation and ictal vomiting, followed by a generalized tonic‐clonic seizure. Partial control of seizures was obtained with antiepileptic drugs. At age 7, the child began to have weekly episodes of oculocephalic version, occasionally with secondary generalization. Repeated inhibitory seizures and absences also appeared. EEG showed frequent bilateral spikes occupying predominantly the posterior regions while awake, and CSWSS. At 7.5 years the same electro‐clinical picture persisted. Ethosuximide was added to sodim valproate and clobazam. Fifteen days later, the seizures disappeared and the EEG showed less frequent bilateral occipital spikes. She is now 9 years old and she has been seizure‐free for 18 months. Her present neuropsychological profile shows mild mental retardation. The two children with typical electroclinical features of “Panayiotopoulos Type” CEOP developed an atypical evolution which, to our knowledge, has not been described previously.

A study on visual evoked responses in childhood epilepsy with occipital paroxysms

As some apparently idiopatic epilepsies may occasionally pose diagnostic difficulties in regard to their precise status of etiology, evoked potentials, particularly visual evoked potential (VEP), may contribute to the diagnosis of childhood epilepsy with occipital paroxysms (CEOP) as a subsidiary method of evaluation. This study includes 19 children (10 boys 52.6%; 9 girls 47.4%) ranging in age from 5 to 17 years (mean SD = 9.68 3.28) suffering from CEOP and a control group of 30 normal children, matched for chronological age and sex. Peak amplitudes and latencies of the P100 component for pattern-shift VEP (PVEP) and of major positivity for flash VEP (FVEP) are measured, respectively. The results from this study demonstrate that amplitude and latency values in patients with CEOP differs insignificantly when compared with controls. Although, non-significantly, mean values of amplitudes for both PVEP and FVEP were higher in the patients than in the normal children, whereas latencies in FVEP were somewhat longer. There may be some tendency for the amplitudes to increase and the latencies to be delayed in VEPs in patients with CEOP, when an overall interpretation of our and similar studies are considered. In certain cases of diagnostic difficulty, VEP values may provide further information for the clinician, regarding either a symptomatic or an idiopathic nature of the underlying disorder.

Benign Childhood Partial Seizures and Related Epileptic Syndromes (Current problems in epilepsy: 15)

Partial epilepsies had been considered to reflect localized organic lesions until benign childhood epilepsy with centrotemporal spikes (BCECT) was delineated. This had also been the case with partial epilepsies in infancy, and the prognosis of partial epilepsies in infancy had been considered unfavorable until we described benign partial epilepsies in infancy (BPEI). The current International Classification lists BCECT, childhood epilepsy with occipital paroxysms (CEOP) and primary reading epilepsy as idiopathic partial epileptic syndromes. Among these syndromes, BCECT, more appropriately called rolandic epilepsy or benign childhood centrogyral seizure/epilepsy after Panayiotopoulos, is the most common epileptic syndrome in children. It is only recently that the presence of early onset benign childhood occipital seizures has been recognized. This syndrome is the second most common syndrome of benign childhood partial seizures and has been delineated and extensively studied by Panayiotopoulos, the author of this extraordinary book. CEOP or late onset benign childhood occipital seizure/epilepsy is far less common than early onset type or Panayiotopoulos syndrome. These benign childhood partial seizures and related epileptic syndromes are, by virtue of high prevalence and excellent prognosis, the most commonest and most rewarding experience in the every day practice of general pediatrics and pediatric neurology, even though the importance of syndromic classification is still not widely recognized. It is important to make an accurate syndromic diagnosis and give a full explanation of their benign nature to the family and not to treat these syndromes aggressively.

Idiopathic partial epilepsy: electroclinical demonstration of a prolonged seizure with sequential rolandic and occipital involvement. Seizure spread due to regional susceptibility?

ABSTRACT Benign rolandic epilepsy (BRE) and childhood epilepsy with occipital paroxysms (CEOP) are overlapping in age range of presentation. Some children have been reported to manifest occipital and rolandic seizures, as distinct events. However, the presence during the same seizure of rolandic and occipital symptoms is exceptional. We present a 7‐year‐old boy with BRE in whom we video‐EEG recorded two seizures at 4years of age. The first episode was a classic rolandic seizure during sleep, while the second was prolonged with initial rolandic and late occipital involvement. It is possible that a rolandic seizure, in a child within the age range of both BRE and early onset CEOP, could spread to selectively involve the occipital lobe, because there is susceptibility of cortical neurons of both areas to develop seizures at this age in idiopathic partial epilepsies.

Idiopathic partial epilepsies with rolandic and occipital spikes appearing in the same children

Benign childhood epilepsy with centrotemporal or rolandic spikes (BCECTS) and childhood epilepsy with occipital paroxysms (CEOP) are the most frequent epileptic syndromes in preschool and elementary school-age children. We present 10 children with diagnostic criteria for both BCECTS and early onset of CEOP. The 10 patients (six girls and four boys) were followed from 1–7.5 years (mean: 3.5 years) with repeated electroencephalograms (EEGs) during wakefulness and sleep. All had brain computed tomography (CT) scans or magnetic resonance imaging (MRI). Age of onset, type and frequency of seizures, and family and personal histories of febrile or afebrile seizures were studied. All patients showed normal neurologic examination and brain imaging studies. Age of onset of seizures ranged from 2.5–9.5 years (mean: 5.2 years). Seizures occurred as follows: ictal vomiting in 10 cases, anarthria in 10 cases, oculocephalic deviation in nine cases, clonic partial seizures in seven cases, two of them with secondary generalization. Seizures were brief in the 10 cases, all had them during sleep, and four patients also had seizures while awake. Fits repeated each 1–5 months in four cases and 6–12 months in six cases. A personal history of febrile seizure prior to onset of BCECTS or CEOP was present in three patients. Family history of epilepsy was found in four cases, of febrile seizures in two, and of migraine in four cases. The EEGs showed occipital spikes during sleep in all cases and on wakefulness in seven patients, but none of these disappeared upon eye opening. The same EEGs showed independent centrotemporal spikes in nine cases. Two well-defined groups may be disclosed in this small series. Five children started with early onset CEOP and after certain time presented seizures of the BCECTS type. The second group comprised of three patients featuring in the same attack typical seizure of BCECTS and CEOP. Two other cases presented in different occasions independent seizures of BCECTS and CEOP. There is still room for discussion: 1) to interpret these finding as the simultaneous manifestation of two recognized idiopathic epileptic syndromes in childhood, 2) to consider these patients as support of the theory that idiopathic partial epilepsies of childhood are dynamic processes that tend to evolve into one another, and 3) to wait for more genetic information in order to demonstrate either a close genetic relationship between these syndromes or common markers with variable phenotypes.

Childhood occipital epilepsy: seizure manifestations and electroencephalographic features

Childhood epilepsy with occipital paroxysms (CEOP) is an idiopathic localization-related epilepsy. A typical seizure in CEOP begins with visual symptoms, followed by hemiclonic seizures, complex partial seizures or generalized tonic-clonic seizures. Benign nocturnal childhood occipital epilepsy (BNCOE), characterized by nocturnal seizures with tonic deviation of the eyes followed by vomiting, has the same electroencephalographic features as CEOP. In this study, we report the seizure symptoms and electroencephalographic features of 21 cases with CEOP or BNCOE. Out of these patients, nine had BNCOE, six had CEOP, four had CEOP and BNCOE and the remaining two belonged to the incomplete syndrome because of no paroxysmal discharges in EEG. When the patients with BNCOE awoke from sleep, they had tonic deviation of the eyes and could describe visual symptoms. Patients with CEOP had seizures beginning with visual symptoms followed by loss of consciousness but no generalized convulsions. In three cases, in addition to the occipital spikes, independent centro-temporal spikes were recorded and in another three cases generalized spike-wave discharges were recorded. Such a combination suggests the idiopathic nature of these epilepsies. We concluded that in the diagnosis of CEOP and BNCOE, the seizure symptomatology is important even if the EEG can be considered normal.

Early-onset benign occipital seizure susceptibility syndrome.

Childhood epilepsy with occipital paroxysms (CEOP) is characterised by ictal visual hallucinations and occipital epileptiform activity on interictal EEG. A variant has been described with nonvisual symptoms including tonic head and eye deviation, vomiting, and episodes of partial status epilepticus. We fully documented the electroclinical features of such patients to determine whether classification separate from CEOP is justified. This was a multicentre study with participating investigators submitting details of patients with idiopathic occipital seizures characterised by ictal head or eye deviation and vomiting. One hundred thirteen patients were recruited. Seizures began in early childhood (mean, 4.6 years) and occurred infrequently (mean total seizures, 3); 30% of patients had only a single seizure. Two thirds of seizures were nocturnal. Ictal eye deviation occurred in 79%, vomiting in 70%, and head deviation in 35%. Seizures were predominantly complex partial in type. Partial status epilepticus occurred in 44% of patients. Seventy-four percent of patients had occipital interictal EEG epileptiform activity, predominantly right sided, with fixation-off sensitivity. Extraoccipital EEG abnormalities occurred in 35% of patients. Prognosis was excellent: the mean duration of active seizures was 1 year. Although the two groups shared identical EEG features, the distinct clinical symptoms probably justify separate classification. Early-onset benign occipital seizure syndrome (EBOSS) is suggested as an appropriate name for the variant group.

Studies on cerebrospinal fluid kynurenic acid concentrations in epileptic children

Kynurenic acid (KYA), the only known endogenous antagonist of the excitatory amino acids, is a metabolite of kynurenine. In the present study the levels of KYA were measured in the cerebrospinal fluid (CSF) of epileptic children and age-matched controls to investigate the relationship between various forms of epilepsy and KYA levels. CSF samples from four patients with West syndrome (WS), four patients with epilepsy with grand mal seizures on awakening (EGSA), and four patients with childhood epilepsy with occipital paroxysms (CEOP) were collected by lumbar puncture before treatment. The concentration of CSF KYA was analyzed by HPLC with electrochemical detection and compared with those of age-matched controls. The levels of CSF KYA were significantly lower (P < 0.05) in patients with WS compared with controls. The levels of CSF KYA in patients with EGSA and with CEOP did not differ significantly from control levels. These results suggest that the presence of seizures in WS is associated with altered kynurenine metabolism. The possibility that seizures in WS may be related to decreased production of KYA is discussed.

Occipital paroxysmal discharges suppressed by eye opening: variability in clinical and seizure manifestations in childhood.

The EEG in childhood epilepsy with occipital paroxysms (CEOP) was termed "distinctive" by Gastaut (1985) and Talwar et al. (1992) and "characteristic" by Herranz Tanarro et al. (1984), which suggests that the EEG is specific and diagnostic for CEOP. However, this hypothesis has been challenged (Newton and Aicardi, 1983; Beaumanoir and Grandjean, 1987). To test this, we reviewed 5,291 EEG reports made in 5 1/2 years in the only tertiary pediatric center in Newfoundland and Labrador. We identified 31 children who had one or more EEGs with occipital spike/sharp waves showing suppression of discharges with eye opening and normal background activity. Six had CEOP, 17 had benign nocturnal childhood occipital epilepsy, 5 had symptomatic epilepsy, 3 had unusual complex partial seizures (CPS), 4 had only provoked seizures, and 2 had no definite seizures. Overlap between seizure types was common. The EEG criteria for CEOP are not very specific.

Paroxismos occipitais após o fechamento dos olhos: correlações clínico-eletrencefalográficas em 24 casos

Paroxismos occipitais aparecendo com o fechamento dos olhos (POFO) constituem o padrão eletrencefalográfico fundamental da epilepsia idiopática da infância com paroxismos occipitais (EIIPO). A associação de POFO e epilepsia idiopática tem sido questionada. Estudamos 24 pacientes que apresentavam POFO. A idade esteve compreendida entre 3 e 25 anos. Em 12 casos havia atividade epileptiforme de outras localizações (generalizada em 8 e rolândica em 4). Em 4 casos havia desorganização da atividade de base. Crises epilépticas espontâneas foram relatadas em 16 pacientes sendo focais em 9, generalizadas em 6 e inclassificáveis em um caso. Foi possível identificar as seguintes síndromes epilépticas: EIIPO em 7 casos; epilepsia benigna com pontas centro-temporais em um; EIIPO ou epilepsia benigna com pontas centro-temporais em um; epilepsia parcial sintomática/criptogênica em 4; idiopática generalizada em 2 e convulsões febris em 2 pacientes. Assim, concluímos que POFO foram mais comumente associados às epilepsias benignas da infância mas ocorreram em várias síndromes epilépticas ou mesmo em pacientes sem manifestações convulsivas.