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Introduction: The electrical status epilepticus in sleep (ESES) accompanies a wide spectrum of focal and generalized epilepsies, which manifest with cognitive-linguistic regression. Both ESES and language impairment can be seen in self-limited focal epileptic syndromes of childhood (SFEC). The association between the presence of ESES pattern on the EEG and the severity of the language impairment has not been adequately clarified. Methods: Twenty-eight SFEC cases without intellectual and motor disabilities and 32 healthy children were recruited. Cases with active ESES (A-ESES, n=6) and without ESES pattern on EEG (non-ESES, n=22) were compared in terms of clinical features and linguistic parameters by both standard and descriptive assessment tools. Results: The only significantly different clinical feature in the A-ESES group was the increased prevalence of polytherapy. While most of the linguistic parameters were impaired in A-ESES and non-ESES groups compared to healthy controls, A-ESES patients differed from non-ESES patients only in terms of decreased complex sentence production, which was assessed by narrative analysis. A-ESES patients also showed trends toward producing lower numbers of words, nouns, verbs, and adverbs during narrative analysis. There were no differences among patients under polytherapy and monotherapy in terms of these language parameters. Conclusion: Our results show that ESES increases the negative effect of chronic epilepsy on complex sentence and word production. Linguistic distortions that are not reflected in objective tests can be detected by narrative tools. Complex syntactic production obtained by narrative analysis is an important parameter that extensively characterizes language skills in school-age children with epilepsy. [ABSTRACT FROM AUTHOR]

Objectives:Posterior cortex epilepsies (PCE) are characterized by seizures originating from the occipital, parietal, or occipital border of the temporal lobe. It is very important to analyze the patient series and bring them to the literature in the evaluation of the clinical features of this rare seizure type. In our study, the patients with PCE were retrospectively examined and the cognitive functions of the patients were evaluated and the results were compared with those of temporal lobe epilepsy (TLE).Methods:Patients who fulfilled the inclusion criteria among epilepsy patients treated in Cerrahpaşa Faculty of Medicine, Department of Neurology were retrospectively screened and included in this study. As a result of the described findings, cases where localization could be made to the posterior or temporal lobe were included in the study and two separate study groups were formed: Group-1; Posterior cortex patients, Group-2; Temporal cortex patients. A control group without any neurological or psychiatric disease was also formed. Detailed neurocognitive tests were applied to all 3 patient groups.Results:There was no significant difference between the demographic data of the control and patient groups. In all the parameters studied, the test results of Group-1 were found to be lower than the control group. The variables that best differentiated the control group from Group-1 and 2 were determined as ‘Rey–Osterrieth complex figure test (ROCF)’, ‘Trail Making Test (TMT) Form A’, ‘The Judgment of Line Orientation (JLO) test and California Verbal Learning Test (CVLT) delayed recall’ scores.Conclusion:Our findings suggest that both posterior epilepsies and TLE are associated with impairments in visual configuration, verbal skills, and executive functions. From these results, deterioration in visual configuration is an expected result in PCE, whereas deterioration in verbal skills and executive functions is considered an unexpected result in PCE cases since it is typically controlled by the fronto-temporal regions. It was suggested that it would be useful to follow up the patients with a comprehensive cognitive assessment.

Objectives: Posterior cortex epilepsies (PCE) are characterized by seizures originating from the occipital, parietal, or occipital border of the temporal lobe. It is very important to analyze the patient series and bring them to the literature in the evaluation of the clinical features of this rare seizure type. In our study, the patients with PCE were retrospectively examined and the cognitive functions of the patients were evaluated and the results were compared with those of temporal lobe epilepsy (TLE). Methods: Patients who fulfilled the inclusion criteria among epilepsy patients treated in Cerrahpaşa Faculty of Medicine, Department of Neurology were retrospectively screened and included in this study. As a result of the described findings, cases where localization could be made to the posterior or temporal lobe were included in the study and two separate study groups were formed: Group-1; Posterior cortex patients, Group-2; Temporal cortex patients. A control group without any neurological or psychiatric disease was also formed. Detailed neurocognitive tests were applied to all 3 patient groups. Results: There was no significant difference between the demographic data of the control and patient groups. In all the parameters studied, the test results of Group-1 were found to be lower than the control group. The variables that best differentiated the control group from Group-1 and 2 were determined as 'Rey-Osterrieth complex figure test (ROCF)', 'Trail Making Test (TMT) Form A', 'The Judgment of Line Orientation (JLO) test and California Verbal Learning Test (CVLT) delayed recall' scores. Conclusion: Our findings suggest that both posterior epilepsies and TLE are associated with impairments in visual configuration, verbal skills, and executive functions. From these results, deterioration in visual configuration is an expected result in PCE, whereas deterioration in verbal skills and executive functions is considered an unexpected result in PCE cases since it is typically controlled by the fronto-temporal regions. It was suggested that it would be useful to follow up the patients with a comprehensive cognitive assessment. [ABSTRACT FROM AUTHOR]

Objective: Occipital lobe epilepsies (OLE) comprise 5–10% of focal epilepsies in surgical and paediatric series; with little data from adult medical cohorts. This longitudinal study examined OLE patients, to characterise prevalence, semiology, co-morbidity and prognosis in a neurology outpatient setting. Methods: 24 adult OLE patients identified over 12 months from 1548 patients in a neurologist's service were followed over 12 years. Results: 92% of these OLE patients had simple visual hallucinations, misdiagnosed in 40% of cases. 75% had co-morbid interictal migraine and 38% had visual field defects. Only 33% achieved long-term remission, and only 2 /10 (20%) of OLE patients with a structural aetiology were seizure-free. The two patients with migralepsy achieved remission. Conclusion: Adult OLE accounted for 7.7% of focal epilepsies in this cohort, misdiagnosed or misclassified in 40%. Most patients had co-existing migraine. A minority had migralepsy characterised by a longer aura and good prognosis. Remission rates were lower than that of childhood OLE and general adult epilepsy populations, strengthening the argument for considering epilepsy surgery in drug-resistant OLE patients with a structural cause. Precision medicine will potentially refine diagnosis and management in those OLE patients without an identified cause but is predicated on accurate clinical phenotyping. [ABSTRACT FROM AUTHOR]

In this case series, we have identified an atypical pattern of OIRDA (Occipital intermittent rhythmic delta activity) on the electroencephalograms (EEGs) of three pediatric patients with self-limited focal epilepsies, including Childhood Epilepsy with Centrotemporal Spikes (CECTS), and Panayiotopoulos syndrome (PS). Previously, OIRDA was described as a symmetric sinusoidal occipital-maximal activity, often associated with childhood idiopathic generalized epilepsies, although it was also reported among other physiologic or pathological entities including focal epilepsy. We have observed in our case series that OIRDA, without prominent field effect, is lateralized or maximal on the hemispheric side ipsilateral to the more defining epileptiform discharges in these focal epilepsies. They also exhibit a notched morphology due to the intermixed sharp wave activities, although the sharp waves are not occurring repetitively. This report provides additional evidence that OIRDA can be associated with a spectrum of idiopathic focal epilepsies and may suggest a cortical origin of OIRDA in these patients as opposed to a hypothesized subcortical generator in patients with primary generalized absence epilepsy, even though further investigation is warranted for either hypothesis.

Sturge-Weber syndrome is a rare sporadic, neurocutaneous syndrome. In most instances, the patients present with seizures other than the port-wine stain and ipsilateral leptomeningeal angiomata. The documentation of seizures' semiology in the current literature is scarce. Here we reported two cases who presented with visual hallucination as an ictal phenomenon and associated ictal headache and vomiting. The phenomenology was very close to migraine with aura. A careful history, associated seizure, and EEG findings helped in differentiation. Both the cases responded well with carbamazepine. [ABSTRACT FROM AUTHOR]

Infants with West Syndrome and underlying structural pathology typically experience persistent symptomatic focal seizures and intellectual disability. We performed a retrospective case review of 84 patients with West Syndrome evaluated at one institution between 1990 and 2013. From this group we identified three patients with West syndrome and congenital hemiplegia who later developed genetic epilepsy features and had normal intellectual development. This outcome is highly unusual and raises important questions about the relationship and possible influence of genetic epilepsy in patients with pre-existent West Syndrome and brain injury.

Introduction: Occipital lobe epilepsies are rarely met in clinical practice, but when they occur, they can be misdiagnosed as migraine-like headache. Their prevalence ranges from 5%to 10% of all epilepsies. Seizures can occur at any age; etiologically speaking they can be symptomatic, cryptogenic and idiopathic (most often onsetis in childhood). Clinical symptomatology is manifested by partial epileptic seizures in the sense of visual elementary and/or complex manifestations, palinopsia, amaurosis, tonic head deviation, bulbus, nistagmus and headache. Propagation discharge to neighbour areas (temporal, parietal and frontal) is a frequent occurrence appearing with complex partial seizures frequently finishing with secondary generalized tonic-clonic (GTC) seizures. Case report: We are presenting a17-year-old male patient who has suffered from attacks of visual problemswith headache since 10 years of age. All the time it is treated as a migraine headache. During the last attack of headache the patient also had a loss of consciousness, EEG that was performed for the first time evidenced epileptic discharges of the occipital area. The therapy also included treatment with antiepileptic drug pregabalin resulting in seizure withdrawal. Conclusion: The appearance of visual symptoms followed by headache is most frequently qualified as migraine triggered headache. However, when antimigraine therapy does not give favorable results epileptic headache should be suspected, with obligatory performance of EEG recording. Occipital lobe epilepsy often presents diagnostic dilemmas due to clinical manifestations that are similar to that of non-migraine headache.

Abstract: idiopathic occipital epilepsy , not uncommon in practice, pediatric neurologist . According to Panayiotopoulos , they are found in 30% of cases of idiopathic epilepsy (25 % – Panayiotopoulosao syndrome, 4% – Gastaut type ). Particular difficulty is the abundance of autonomous symptoms, the presence of transitional forms of epilepsy, the combination of migraine , which has age-appropriate. In addition, the child s brain is different excitability and registration abnormalities in the EEG is not casuistry . We have provided supervision for children with occipital epilepsy and EEG changes in the occipital region, clinical and medical history characteristics of this group and outcome monitoring for 5-7 years.

The childhood epilepsy with occipital paroxysms and early onset or Panayiotopoulos syndrome is a benign focal epileptic syndrome,a benign disease, developing at infants. The disease has been described in 1989 by S. Panayiotopoulos on the basis of results of own observations, who has called this syndrome “benign night childhood occipital lobe epilepsy”. In 1996 N. Fejerman et al. offered the new name of the syndrome – benign childhood occipital lobe epilepsy, with earlier onset (Panayiotopoulos type), versus the childhood occipital lobe epilepsy, starting later in childhood (Gastaut type). The Panayiotopoulos syndrome occurs only at children, develops at healthy children without neurological disorders, the pick of disease falls on 4–5 years. The morbidity of boys and girls is almost the same. In most cases of the syndrome seizures take place during the sleep, 2 / 3 of patients report seizures only during sleep. As a rule, seizures last for more than 5 min, in 40 % of cases it last for more than 30 min, what corresponds (according to the consciousness level) to focal or generalized epileptic status criteria. To typical clinical syndromes at Panayiotopoulos syndrome are referred the ictal vomiting, as well as different vegetative symptoms. So, theictal vomiting, which, generally, is not typical for epilepsy, takes place in about 80 % of cases at Panaytopululos syndrome. Authors describe their own observation of the Panayiotopoulos syndrome at 5 years old girl.

Panayiotopoulos syndrome is a common idiopathic benign epilepsy that has a peak age of onset in early childhood. The syndrome is multifocal and shows significant electroencephalogram (EEG) variability, with occipital predominance. Although a benign syndrome often refers to the absence of neurological and neuropsychological deficits, the syndrome has recently been associated with cognitive impairments. Also, despite frequent occipital EEG abnormalities, research regarding the visual functioning of patients is less reported and often contradictory. The purpose of this study was to gain additional knowledge regarding the neurocognitive functioning of patients with Panayiotopoulos syndrome and specifically to address any visual processing deficits associated with the syndrome. Following diagnosis of the syndrome based on typical clinical and electrophysiological criteria, three patients, aged 5, 8, and 10 years were referred by epileptologists for neuropsychological evaluation. Neuropsychological findings suggest that the patients had notable impairments on visual memory tasks, especially in comparison with verbal memory. Further, they demonstrated increased difficulty on picture memory suggesting difficulty retaining information from a crowded visual field. Two of the three patients showed weakness in visual processing speed, which may account for weaker retention of complex visual stimuli. Abilities involving attention were normal for all patients, suggesting that inattention is not responsible for these visual deficits. Academically, the patients were weak in numerical operations and spelling, which both rely partially on visual memory and may affect achievement in these areas. Overall, the results suggest that patients with Panayiotopoulos syndrome may have visual processing and visual memory problems that could potentially affect their academic capabilities. Identifying such difficulties may be helpful in creating educational and remedial assistance programs for children with this syndrome, as well as developing appropriate presentation of information to these children in school. [ABSTRACT FROM AUTHOR]

Foram estudadas as características evolutivas clínico-eletrencefalográficas de 14 crianças com epilepsia occipital benigna da infância de início precoce (tipo Panayiotopoulos). O tempo médio de segmento foi 50,5 meses. A idade média na primeira crise foi 3,7 anos. O número total de crises foi até 3 crises em 11 casos, numerosas em 3; o período médio entre a primeira e a última crise foi 14,5 meses. Em 4 casos as crises tiveram duração prolongada, constituindo estado de mal parcial. Atividade epileptiforme (AE) occipital foi observada em todos os casos no primeiro EEG e, foi também, extra-occipital, em 3 casos. Houve bloqueio da AE occipital, pela abertura dos olhos, em 4 casos; em 3 casos foram observadas, também, pontas evocadas. O EEG normalizou-se em 9 casos, em um período médio de 29 meses.We studied clinical and electroencephalographic features of 14 children, age range of 2 -8 years, with no neurological or neuroradiological evidence of brain damage and with occipital epileptiform activity in the EEG. Seizures were numerous in 3 cases. Age at onset was between 1-7 years. In 4 cases the seizures last for more than 20 min. Spikes were observed in 6 cases and spike and slow-wave complex in 8. Discharges blocking by eyes opening were confirmed in 4 cases. Somatosensory evoked spikes by foot stimulation were observed in 2 cases. Autonomic and versive seizures are the main clinical manifestations of Panayiotopoulos syndrome. Discharges blocking by eyes opening are a less frequent feature.

Foram estudadas as características clínico-eletrencefalográficas de 63 crianças com crises epilépticas, atividade epileptiforme occipital ao EEG e sem elementos sugestivos de lesão cerebral. As crises foram únicas em 16 casos e numerosas em 16. A idade na primeira crise variou de 1-12 anos. As crises foram focais em 49 casos, com manifestações autonômicas em 25, versivas em 24, visuais em 10 e exclusivamente tônico-clônicas generalizadas em 14 casos. Ocorreram durante o sono em 37 e foram prolongadas em 15 crianças. O EEG evidenciou pontas em 27 casos e complexos de ponta-onda lenta em 36; houve bloqueio pela abertura dos olhos em 15. Pontas evocadas foram observadas em 9,5% dos casos. Os diagnósticos de epilepsias idiopáticas foram: occipital precoce - tipo-Panayiotopoulos (EIOP), 32 casos; occipital tardia - tipo-Gastaut (EIOT), 8; rolândica em 7. A EIOP foi mais freqüente que a EIOT e em 63,4% dos casos foi possível a classificação nas síndromes occipitais reconhecidas.We studied clinical-EEG features of 63 children, age range of 2-14 years, with occipital epileptiform activity in the EEG, with epileptic seizures and no evidences of brain damage. Age at onset was between 1-12 years. In 15 cases the seizures last for more than 30 min up to 6 h. Seizures occurred during sleep in 28 children. Autonomic symptoms were apparent in 25, head deviation in 24 and hemiconvulsion or generalization in 17 cases. Ten children reported visual symptoms. In 14 children the seizures were exclusively generalised convulsions. Spikes were observed in 27 cases and spike and slow-wave complex in 36. Discharges blocking by eyes opening were confirmed in 15 cases. Somatosensory evoked spikes by foot stimulation were observed in 9.5% cases. The cases were classified in the following idiopathic epileptic syndromes: early-onset occipital - Panayiotopoulos-type (EOO), 32 cases; late-onset occipital - Gastaut type (LOO), 8; rolandic epilepsy in 7. Conclusion: EOO occurred more frequently than LOO. Idiopathic occipital syndromes diagnosis was possible in 63.4% of the cases.

Posterior cortex epilepsies (PCE) are characterized by less satisfying postoperative results than temporal lobe epilepsies and are thus challenging for non-invasive presurgical investigations. The objective of this study was to evaluate the performance of magnetic source imaging (MSI) in PCE, validating the results by the SEEG (stereoelectroencephalography) definition of irritative and epileptogenic zones (IZ and EZ). Fourteen PCE surgery candidates were investigated using MSI and SEEG. LCMV (Linearly Constrained Minimum Variance) and MUSIC algorithms were used. IZ was quantified using a semi-automatic detection of interictal spikes. EZ was quantified using the epileptogenicity index (EI) method that accounts for both the propensity of a brain area to generate rapid discharges and the time for this area to get involved in the seizure. EI values range from 0 (no epileptogenicity) to 1 (maximal epileptogenicity). Levels of concordance between MSI and IZ, MSI and EZ were determined as follows: A = localized on MSI and SEEG for the site of value 1 (IZ and EZ quantification), B = localized on MSI and SEEG for a part of the IZ or a structure involved in the EZ (without the maximal value 1), C = localized on MSI and not SEEG, D = localized on SEEG and not MSI, E = localized on MSI and SEEG, discordant for site. Five PCE cases were characterized by focal IZ, nine by distributed IZ between several distant brain areas. MSI allowed to determinate IZ in 4/5 focal IZ cases. In case of distributed IZ, levels of concordance were A (2 cases), B (4 cases) and D (3 cases). In most distributed cases, MSI allowed to localize only a part of the IZ. Medial temporal involvement in the IZ was frequent (9/12 cases) and not evidenced by MSI. The brain area that demonstrated the maximal value of EI was shown by MSI in four out of five (80 %) focal IZ cases, in two out of nine (22 %) distributed cases. MSI results depend on IZ characteristics. A distributed IZ organization presents difficulties for MSI and highlights the need for further methodological approaches. [ABSTRACT FROM AUTHOR]

Idiopathic photosensitive occipital lobe epilepsy is a reflex, age- and localization-related syndrome. We describe the clinical and electroencephalographic features, therapy, and outcome of 16 children/adolescents with this syndrome. The cohort included 2 sets of siblings and 7 patients with other first- or second-degree relatives with a seizure history. All patients had occipital onset seizures and 15 had secondarily generalized tonic-clonic seizures. Seizure frequency was relatively low in all patients but one. Myoclonic seizures later developed in 2 patients with juvenile myoclonic epilepsy. Eight patients achieved full seizure control with monotherapy, and 5 required a second drug; 3 patients had rare seizures and were not treated with antiepileptics. Seven patients required special education or developmental assistance. This interesting syndrome sheds light on the pathophysiology and genetic etiology of common phenomena such as photosensitivity and headache. Further large prospective studies are required to better define this unique syndrome and its implications. [ABSTRACT FROM PUBLISHER]

The idiopathic focal epilepsies comprise a group of syndromes characterized by focal-onset seizures for which there is no detectable structural brain abnormality and for which there is a proposed functional mechanism for the epilepsy and electroencephalography (EEG) abnormalities. This group includes benign rolandic epilepsy (BRE), benign epilepsy with occipital paroxysms (both early onset and late-onset types), idiopathic photosensitive occipital lobe epilepsy, and some less well-defined syndromes. The limits of the early onset idiopathic occipital epilepsy syndrome are not clear, and perhaps this entity represents part of a larger syndrome group of 'autonomic' age-related epilepsies. The term 'idiopathic' implies absence of a structural brain lesion and a genetic propensity to seizures. The term 'benign' implies that the epileptic seizures are easily treated or require no treatment, show remission without sequelae with ultimate and definitive remission before adulthood, do not have severe or exceedingly disturbing seizures, and have no associated serious intellectual or behavioral disturbances. It may be that a syndrome is benign only when it can be recognized early with reasonable certainty, thereby avoiding unnecessary investigations, overtreatment, and lifestyle restrictions. Although BRE has such characteristic clinical and EEG features to make early recognition possible, this is less constantly so in the other focal idiopathic epilepsy syndromes, where the term 'benign' may be inappropriate. Mild and selective neuropsychological impairment may occur even in those with typical syndromes but it is unclear whether such selective deficits outlast the active phase of epilepsy. Sometimes the clinical course may be complicated by obvious cognitive and language impairments. In such cases, the term benign is obviously inappropriate, even when seizures are rare. In most patients with the typical focal idiopathic epilepsy syndromes, medication is not necessary. [ABSTRACT FROM AUTHOR]

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Abstract: Purpose: The objective of this retrospective study was to identify clinical and EEG features in children with occipital epilepsy that predict MR imaging abnormalities and seizure outcome. Methods: Patients with clinical and/or EEG features indicating occipital lobe involvement were identified from the epilepsy database at a tertiary children’s hospital. The clinical and EEG features were analyzed to identify the most important predictors of abnormal MR imaging and seizure outcome. Results: Sixty-six patients were identified: 21 had symptomatic epilepsy with abnormal MR imaging; 12 patients had probable symptomatic epilepsy based on an abnormal neurological exam; 33 patients had normal neurological development, normal neurological examination and normal neuroimaging. Twenty-two of these 33 patients satisfied criteria for idiopathic occipital syndromes: Panayiotopoulos syndrome (9 patients), Gastaut type idiopathic childhood occipital epilepsy (12 patients) and idiopathic photosensitive occipital epilepsy (1 patient). Eleven patients could not be classified. Early age of seizure onset and an abnormal neurological examination were significant predictors of abnormal MR imaging. None of the variables examined were strong predictors of seizure outcome. Conclusion: Early age of seizure onset and an abnormal neurological examination are the best predictors of abnormal MR imaging in children with epilepsy with occipital features. [ABSTRACT FROM AUTHOR]

Abstract: Panayiotopoulos syndrome (PS) is a common childhood susceptibility to autonomic seizures and status epilepticus. Despite its high prevalence, PS has been a source of significant debate. We present ictal EEG documentation of autonomic seizures and autonomic status epilepticus in six cases of PS and a review of 14 reported cases. Interictal EEGs showed spikes of variable locations that often changed with time. Ictal EEG onsets were also variable, starting from wide anterior or posterior regions usually with theta waves intermixed with small spikes and fast rhythms. Ictal vomiting and other autonomic manifestations, as well as deviation of the eyes, did not appear to relate to any specific region of EEG activation. These data document that PS is a multifocal autonomic epilepsy and support the view that the clinical manifestations are likely to be generated by variable and widely spread epileptogenic foci acting on a temporarily hyperexcitable central autonomic network. [ABSTRACT FROM AUTHOR]

Panayiotopoulos syndrome (PS) is a benign childhood epilepsy with predominant autonomic symptoms. The syndrome can have varied presentations resulting in diagnostic dilemma. We herein describe a 3-year-old boy with PS, who had manifestations similar to septic shock. His investigations were normal and had a complete recovery. Through this case, we wish to highlight the unusual presentation of PS as septic shock. Physicians should be aware of the different ways in which this syndrome can present to ensure its early diagnosis and treatment. [ABSTRACT FROM AUTHOR]

In its first description (1989), Panayiotopoulos syndrome was defined as an idiopathic epilepsy syndrome with an excellent prognosis, characterized by a clinical ictal triad of nocturnal seizures, tonic deviation of the eyes, and vomiting. The electroencephalographic and clinical features of this condition were highly suggestive of occipital lobe involvement. Subsequently, the concept of this benign age-related focal epilepsy has been expanded over the years, including a wider and larger spectrum of seizure manifestations far beyond the occipital manifestations, and for which the eponym of Panayiotopoulos syndrome (PS) has been adopted. However, many theoretical and practical points, including diagnostic, genetic, and pathophysiologic issues remain still unresolved for PS. [ABSTRACT FROM AUTHOR]

Migrainous headache is reported by patients with photosensitive epilepsy, whereas their relatives complain more often about headache than the relatives of patients with other types of epilepsy. We therefore investigated whether headache itself could be an epileptic symptom related to photosensitivity. Four probands with headache and photosensitive epilepsy were selected. Their first-degree family members were studied using video-EEG with extensive intermittent photic stimulation and pattern stimulation. Nine of the 12 subjects (10 female and two male, mean age 30 years, range 14–46 years) proved to be photosensitive with either focal ( n = 5) or generalized ( n = 4) epileptiform discharges. In two subjects an ictal recording of headache occurred after visual stimulation. We found evidence that, in specific patients, headache could be an ictal sign of epilepsy. Photic stimulation during EEG recording can contribute to correct diagnosis and lead to the best care and management of the patient. [ABSTRACT FROM AUTHOR]

To determine the validity of scalp-electroencephalography (EEG)-interictal spike (IIS) source localization in posterior cortex epilepsies (PCE). Eleven patients with drug-resistant PCE were studied with high-resolution EEG (HR-EEG) and stereoelectroencephalography (SEEG). Sixty-four scalp channels, a realistic head model, and different algorithms [multiple signal classification (MUSIC) and equivalent current dipoles] were used. Results were compared to intracerebral SEEG recordings. For SEEG, a semiautomatic detection of intracerebral IIS was used, allowing a classification of intracerebral IIS into one of three groups: Medial, lateral, and mediolateral. In the medial group (two patients), scalp-EEG IIS were usually absent for one patient whereas for the other, scalp-EEG was misleading. Indeed, scalp-EEG IIS had a posterior projection, predominantly contralateral to the source. In the lateral group (two patients), scalp-EEG IIS were subcontinuous and accurately localized. In the mediolateral group (seven patients), intracerebral interictal distribution was complex and bilateral for four of seven patients. Source localizations were able to determine only a part, whether lateral or medial, of the intracerebral interictal distribution. The accuracy of scalp-EEG IIS source localization is dependant on the type of intracerebral interictal distribution. In the most frequent type of PCE, patients proved to have a complex interictal distribution between both medial and lateral cortices, and source localizations always  underestimated intracerebral IIS. In cases where intracranial sources were quite focal, surface EEG sources were localized with accuracy, even in medial occipital lobe structures. [ABSTRACT FROM AUTHOR]

We report the history of a 14-year-old girl with atypical childhood occipital epilepsy “Gastaut type” whose first generalized tonic–clonic seizure was preceded by migraine without aura and followed by a status migrainosus. This status lasted for 3 days despite standard analgesic therapy. An EEG recording revealed an occipital status epilepticus during her migraine complaints. Seven minutes after intravenous administration of 10 mg diazepam under continuous EEG recording, a suppression of the epileptiform discharges over the right occipital was seen, while the headache subsided 3 min later. After precise questioning about the circumstances that possibly could have led to these events, it appeared that she had played for hours with a play station on the new color TV and she had visited an exhibition of Matisse and Bonnard with bright colors and contrast-rich text. Standardized extensive intermittent photic stimulation (IPS), 2 days after the status migrainosus, evoked besides asymmetrical right-sided driving, green spots in her left visual field, while in the EEG sharp waves were recorded over the right parietotemporal region. After further IPS with 20 Hz (eye closure), she started complaining of a light pulsating headache right occipitally and in the EEG right parietotemporal sharp-waves were seen. This lasted for about 10 min. Later, an interictal routine EEG was normal except for some theta over the right temporooccipital area. The most likely diagnosis is an atypical form of occipital epilepsy “Gastaut type.” We would therefore advocate recording EEGs with photic stimulation in patients with atypical migraneous features. [ABSTRACT FROM AUTHOR]

Purpose: Posterior cortex epilepsies (PCEs) encompass a group of epilepsies originating from the occipital, parietal, or occipital border of the temporal lobe, or from any combination of these regions. When their seizures are refractory to pharmacologic treatment, these patients are usually referred for surgery. The aim of our study was to analyze clinical characteristics of all PCE patients referred for surgery from 1994 to 2003, and to search for predictors of surgical outcome. Methods: We performed a retrospective analysis of clinical and laboratory data from 81 consecutive refractory PCE patients referred for surgery. Surgical and nonsurgical groups of patients were compared, and detailed analyses of all variables of the surgical cases were performed in the search for predictors of seizure outcome. Results: Risk factors for PCEs included gliosis (34.56%), malformations of cortical development (33.33%), tumors (8.64%), brain trauma (3.70%), Sturge–Weber disease (4.93%), vascular malformations (3.70%), family history of epilepsy (3.70%), history of CNS infections (2.46%), and low IQ (2.46%). Of the 81 patients, 44 were submitted to surgery at the time of the completion of this study. Surgical treatment was highly effective in improving seizures (p < 0.001) when compared with previous pharmacologic treatment alone. Twenty-eight (65.11%) patients became seizure free after surgery versus none in the nonsurgical group. Regarding outcome predictors, patients with shorter duration of epilepsy and those without neurologic abnormalities on clinical examination had higher chances of favorable evolution. Conclusions: Surgical treatment is effective for the treatment of PCEs and superior to pharmacologic therapy alone. In our series, shorter duration of epilepsy and normal neurologic examination were the only independent variables that predicted better surgical outcome. [ABSTRACT FROM AUTHOR]

Coeliac disease, epilepsy and cerebral calcifications (CEC) syndrome is a rare clinical condition. One hundred and seventy-one patients have been reported in the literature. Patients are mostly from Italy, Spain, and Argentina, suggesting a geographically restricted condition. Epilepsy is more frequently characterized by occipital seizures. It may be benign or drug-resistant, sometime evolving into severe epileptic encephalopathy. Gluten free diet (GFD) efficacy seems to be inversely related to the duration of epilepsy and the young age of the patient. Patients with cerebral calcifications (CC) and coeliac disease (CD) without epilepsy are considered as having an incomplete form of CEC syndrome. Some patients with epilepsy and CC without CD are supposed to have a CEC syndrome with silent or latent CD.Whether CEC syndrome is a genetic condition, or whether epilepsy and/or CC are a consequence of an untreated CD is unknown yet. Since histopathological findings seem to be the expression of vascular calcifyied malformation, CEC syndrome may be considered a genetically determined entity, such as a type of Sturge-Weber-like phacomatosis. Moreover, CEC, as well as CD, is associated with HLA-DQ2 and HLA-DQ8 phenotype and genotype. The progressive growth and late occurrence of CC before beginning a GFD, the demonstration of anti-gliadin antibodies in the cerebro-spinal fluid and the association with HLA class II genes, suggest that an immune reaction originating from the jejunal mucosa, triggered by gliadin in gluten intolerance predisposed subjects (HLA phenotype) may be responsible for seizures and CC. Moreover, a long-lasting untreated CD folic acid deficiency may cause calcifications.Probably, CEC is considered a genetic, non-inherited, ethnically and geographically restricted syndrome associated with environmental factors. [Copyright &y& Elsevier]

In order to gain new insight into the pathogenesis and nosography of benign partial epilepsy with occipital paroxysms (BEOP) we retrospectively analysed cases with benign focal sharp waves (SHW) of different localisations, in which analogous EEG changes had been found in at least one first degree relative. Fifty-six families were evaluated. Nineteen cases with occipital SHW (group A) were compared with 37 cases without (group B). There was a broad spectrum of symptomatology with large overlaps between the two groups. However, a number of striking differences, yielding a characteristic picture of early childhood epilepsy with occipital foci were identified: (1) Age of onset under 5 years; (2) a larger proportion of febrile convulsions (FC 47 vs. 19%); (3) a trend towards a higher rate of typical early childhood occipital seizures (26 vs. 5%); (4) a higher rate of frontal and generalizing SHW foci (32 vs. 5%; 37 vs. 11%); (5) a higher rate of generalized spikes and waves (SW) (46 vs. 14%); (6) a trend towards a higher rate of photoparoxysmal response (PPR) (57 vs. 32%). The high prevalence of independent genetic traits favours a multifactorial pathogenesis. The predisposition to FC with characteristic early seizure onset and varying patterns of generalized genetic EEG traits plays a crucial role within the complex pathogenetic network. The early-onset benign childhood occipital seizure susceptibility syndrome of Panayiotopoulos (Benign Childhood Partial Seizures and Related Epileptic Syndromes, John Libbey & Company Ltd., London (1999)) cannot be regarded as the sole representative of occipital spikes in early childhood but as an important even though rare form of occipital epilepsy. [Copyright &y& Elsevier]

Summary : Serial-ictal single-photon-emission computed to mography (SPECT) examinations are presented in two infants (ages 1 and 2 years), with early ictal and ictal in one, and ictal and late ictal images in the other. Both had pharmacoresistant occipital epilepsy, due to focal cortical dysplasia. In the first case, size of ictal hyperperfusion increased in the course of the seizure from early ictal to ictal state. A concomitant ictal hypoperfusion was observed around the hyperperfused area. In the second patient, there was a dramatic difference between ictal and late ictal images. In the late ictal state, the previous occipital ictal hyperperfusion and extraoccipital ictal hypoperfusion disappeared, together with homolateral posterotemporal and contralateral occipital hyperperfusion, corresponding to seizure propagation. Ictal extratemporal blood-flow changes are therefore highly dynamic, particularly in very young children. [ABSTRACT FROM AUTHOR]

• Pharmaco-resistant epilepsy is common sequelae of perinatal insults. • Imaging mostly reveals bilateral parieto-occipital gliosis/ulegyria pattern. • Electrophysiology localizes to posterior head region but mostly non-lateralizing. • Despite deterrents, curative intent surgeries lead to excellent seizure outcomes. • Complete corpus callosotomy offers excellent palliation in one third of the cohort. To study surgical outcomes in pharmaco-resistant epilepsy associated with posterior cortex ulegyria secondary to perinatal insults. A cohort was analysed for clinico-radiological charectaristics, surgical interventions and seizure outcomes. A total of 38 patients underwent surgery, divided as group A - curative surgeries (n = 20) and group B - palliative surgeries (n = 18). Mean age of onset of epilepsy in group A was 5.2 ± 3.4 years against 2.7 ± 2.4 years in group B (p < 0.01). Electroclinical Lennox Gastaut Syndrome was encountered in 9/20 patients in group A, against all 18 patients in group B. Disabling reflex epilepsy was seen in 10 (26 %) patients. Interictal electrophysiology localized in the posterior cortex in all patients in group A, but ictal onsets contributed in only 7/20 patients. Nine patients from group A had unilateral parieto-occipital ulegyria while bilateral in 11/20 patients, and 16/18 from group B. Group A patients underwent parieto-occipital resection (n = 10) and temporo-parieto-occipital disconnection (n = 10) while group B underwent complete corpus callosotomy (n = 18). In group A, Engel Ia outcome was achieved in 15/20 patients (75 %) at mean follow up of 23.5 ± 7.9 months. Group B patients experienced cessation of head drops in all 18 patients, with two-third reduction in seizure frequency at 29.2± 12.4 months of mean follow up. Reflex seizures responded completely in both groups. Epilepsy surgeries for posterior cortex ulegyria results in excellent seizure outcomes. Corpus callosotomy appears highly effective as a palliation for head drop as well as disabling reflex seizures in a well selected cohort. [ABSTRACT FROM AUTHOR]