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Posterior reversible encephalopathy syndrome (PRES) can develop in association with a vast array of conditions including hypertensive encephalopathy and exposure to toxic agents. Clinical manifestations include seizures, headache, focal neurological deficits, visual disturbances and altered mental status and reversible radiological findings such as grey and white matter abnormalities on magnetic resonance imaging. Recurrent PRES in conjunction with periodic lateralized epileptiform discharges on electroencephalogram has been associated with new-onset epilepsy. Here, we report a pediatric case with systemic juvenile idiopathic arthritis with recurrent episodes of PRES and focal occipital epilepsy. The reason for presenting this case is to show an atypical evolution of this disease, rarely described in children.

Structural (or symptomatic) epilepsy is any type of epilepsy caused by damage of brain structure for example injury, infection, tumours, anoxia, hereditary or metabolic diseases. Lesions may be the remaining of an old problem (residual) while others may get worse in time (progressive). The symptoms of epileptic seizures depend on the brain site of the lesion (frontal, parietal, temporal, occipital). Mesial temporal lobe epilepsy is the commoner type of structural epilepsy. The outcome depends on the underlying cause and the extent of the lesion. The needs of patients with structural epilepsy vary significant depending on the severity of epilepsy and the severity of brain damage.

Benign (or self-limited) childhood focal epilepsy affects otherwise normal children and includes three epileptic syndromes: rolandic epilepsy (or epilepsy with centrotemporal spikes), Panayiotopoulos syndrome and childhood occipital epilepsy of Gastaut. These syndromes affect 25% of children with non-febrile seizures. Epileptic seizures are focal with sensory-motor symptoms in rolandic epilepsy, autonomic symptoms in Panayiotopoulos syndrome, and visual/occipital lobe symptoms in childhood occipital epilepsy of Gastaut. EEG is very useful. Prophylactic treatment may not be needed. Parental education and psychological support is the most important aspect of management. All seizures stop usually in the mid-teens.

Gastaut type idiopathic childhood occipital epilepsy is an uncommon epileptic syndrome characterised by frequent seizures, most commonly presenting as elementary visual hallucinations or blindness. Other occipital (non-visual) symptoms may also occur. Interictal EEG typically shows occipital paroxysms, often with fixation-off sensitivity. Ictal EEG is usually characterised by interruption by paroxysms and sudden appearance of low-voltage, occipital, fast rhythm and/or spikes. Despite well described clinical and EEG patterns, to our knowledge, there are very few reports in the literature with video-EEG recording of either seizure semiology or fixation-off phenomena. We present a video-EEG recording of a 12-year-old girl with Gastaut type epilepsy, illustrating the interictal and ictal aspects of this syndrome. Our aim was to demonstrate the clinical and neurophysiological pattern of a typical seizure of Gastaut type epilepsy, as well as the fixation-off phenomena, in order to further clarify the typical presentation of this syndrome. [Published with video sequences].

One in four children with migraine may report an aura during some or all migraine attacks. Visual disturbances are the most common forms of migraine aura. Intense visual disturbances during migraine attacks can be frightening to young children and may cause concerns to parents, especially if they were not followed by headache, and may lead physicians to carry out several investigations. Alice in wonderland syndrome describes a phenomenon when patients with migraine report a variety of image distortions either separately or in combinations. Differential diagnoses include occipital epilepsy and transient ischemic attacks. Early recognition and reassurance are necessary in order to avoid unnecessary investigations and anxiety.

To explore the neuropsychological and neurobehavioral profile in adult patients affected by nonsymptomatic (cryptogenic and idiopathic) occipital lobe epilepsy (OLE), with normal intelligence, we enrolled 20 adult patients with nonsymptomatic OLE and 20 age-, sex-, and education-matched healthy subjects. All participants underwent neuropsychiatric assessment scales, and standardized neuropsychological tests tapping memory, executive functions, constructional, visuospatial and visuoperceptual skills. After Bonferroni correction for multiple comparisons, patients performed significantly worse than controls on several tests tapping complex visuospatial skills and frontal lobe functions. The analysis of single patients' performance revealed that a significantly higher number of OLE patients achieved age- and education-adjusted pathological scores on three tests (Benton Judgment of Line Orientation Test, Freehand Copying of Drawings Test, color-word interference task of Stroop test) with respect to controls. Patients did not differ from control subjects on neuropsychiatric aspects. The direct comparison between OLE subtypes showed that cryptogenetic OLE patients tended to achieve lower scores than idiopathic OLE patients on most tests, but no difference between the two groups was fully significant. In summary, patients with nonsymptomatic OLE can be affected by clinically relevant impairments in selected neuropsychological domains: complex visuospatial skills and executive functions. It could be speculated that frontal and visuospatial cognitive deficits might be the result of epileptic activity spreading within a neural network that includes structures far beyond the occipital lobe.

The fixation-off sensitivity (FOS), an epileptic phenomenon induced by elimination of central vision and fixation, is rarely seen in symptomatic occipital lobe epilepsy. The cerebral mechanisms and the structural correlate underlying FOS remain unclear. We describe a 19-year-old male with persistent left sided FOS following perinatal insult. MRI revealed asymmetric changes with more gliosis and ulegyria over the left posterior occipital cortex corresponding to the topographic representation of the macula. We suggest that the extensive denervation of the area representing macula along with the presence of hyperexitable ulegyric cortex is responsible for the phenomenon of FOS.

INTRODUCTION: The new proposed classification of ILAE Task Force (2001) proposes that the occipital epilepsies should be split into two subtypes: an early-onset benign childhood occipital epilepsy (or Panayiotopoulos type) and late-onset childhood occipital epilepsy (or Gastaut type). Migraine with visual aura must be considered as a differential diagnosis in childhood and adolescents with occipital epilepsy without motor phenomena. OBJECTIVE: The goal of our paper is to report the case a 16-year-old female, with normal psychomotor development, that during the lunch time presented an event characterized by the vision of multiple colored spots which were moving horizontally and vertically and also in circles through the visual field. Minutes after the visual event, the patient referred to a severe diffuse throbbing headache with frontal predominance. During the clinical investigation was submitted to a video-electroencephalogram exam for 12 hours with, reveling occipital sharp-waves discharges in occipital right region as well as in occipital left region. CONCLUSION: We reported of such classic type of epileptic syndrome in a patient in the unusual age of onset, the end of adolescence, considering the differential diagnosis with migraine with visual aura. INTRODUÇÃO: A nova proposta de classificação da ILAE (2001) propõe que as epilepsias occipitais sejam classificadas em dois subtipos: epilepsia occipital benigna da infância de início precoce (ou tipo Panayiotopoulos) e epilepsia occipital benigna da infância de início tardio (ou tipo Gastaut). A migrânia com aura visual deve ser considerada como um diagnóstico diferencial nas crianças e nos adolescentes com epilepsia occiptal sem fenômenos motores associados. OBJETIVO: relatar o caso de uma paciente do sexo feminino de 16 anos, com desenvolvimento neuropsicomotor normal, que durante o almoço apresentou um evento caracterizado pela visão de múltiplas manchas coloridas que se movimentavam no sentido horizontal e vertical e também em círculos no seu campo visual. Minutos após este evento visual, a paciente passou a referir cefaléia difusa com predomínio frontal. Durante a investigação clínica foi submetida à realização de vídeo-eletrencefalograma com 12 horas de duração revelando descargas de ondas agudas ora na região occipital direita ora na região occipital esquerda. CONCLUSÃO: apresentamos um tipo clássico de síndrome epiléptica iniciando em uma idade pouco usual, o final da adolescência, considerando o diagnóstico diferencial com a migrânia com aura visual.

Serial-ictal single-photon-emission computed tomography (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.

To evaluate epileptic children with occipital lobe epilepsy (OLE) in the light of the characteristics of Panayiotopoulos syndrome and late-onset occipital lobe epilepsy of Gastaut (OLE-G).Patients were categorized into six groups: primary OLE with autonomic symptoms (Panayiotopoulos syndrome), primary OLE with visual symptoms (OLE-G), secondary OLE with autonomic symptoms (P-type sOLE), secondary OLE with visual symptoms (G-type sOLE), and non-categorized primary OLE and non-categorized secondary OLE according to characteristic ictal symptoms of both Panayiotopoulos syndrome and OLE-G, as well as aetiology (primary or secondary). Patients were compared with regards to seizure symptoms, aetiology, cranial imaging, EEG, treatment and outcome.Of 108 patients with OLE (6.4±3.9 years of age), 60 patients constituted primary groups (32 with Panayiotopoulos syndrome, 11 with OLE-G, and 17 with non-categorized primary OLE); the other 48 patients constituted secondary groups (eight with P-type sOLE, three with G-type sOLE, and 37 with non-categorized sOLE). Epileptiform activity was restricted to the occipital area in half of the patients. Generalized epileptiform activity was observed in three patients, including a patient with Panayiotopoulos syndrome (PS). Only one patient had refractory epilepsy in the primary groups while such patients made up 29% in the secondary groups.In OLE, typical autonomic or visual ictal symptoms of Panayiotopoulos syndrome and OLE-G do not necessarily indicate primary (i.e. genetic or idiopathic) aetiology. Moreover, primary OLE may not present with these symptoms. Since there are many patients with OLE who do not exhibit the characteristics of Panayiotopoulos syndrome or OLE-G, additional definitions and terminology appear to be necessary to differentiate between such patients in both clinical practice and studies.

To investigate the hemifield dependence of visually evoked responses to colour in the human fusiform gyrus we recorded evoked potentials from subdural electrodes in a patient suffering from occipital epilepsy. The responses in the fusiform gyrus show a strong hemifield dependence and discriminate the onset from the offset of the stimulus. Additionally, we found responses to squares made of random dots, whereas no responses were found to squares with a homogeneous bright surface. Our findings further support the idea that the fusiform gyrus is related to colour and pattern perception. However, the hemifield dependence we found may indicate that further processing is required in order to combine information from both visual hemifields.

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.

A 7-year-old boy presented with episodic blindness for the last 2 months with occipital paroxysms and fixation-off sensitivity on electroencephalography (EEG). The clinico-EEG features were suggestive of idiopathic childhood occipital epilepsy of Gastaut. The interesting phenomenon of fixation-off sensitivity is discussed.

Introduction. Carbamazepine can lead to aggravation of epileptic seizures in generalized epilepsies (primary or secondary) with clinical manifestations of absence (typical or atypical) and/or myoclonic seizures. However, some focal epilepsies can be also aggravated by the introduction of carbamazepine. Case report. We presented a 10-year-old boy born after a complicated and prolonged delivery completed by vacuum extraction, of early psychomotor development within normal limits. At the age of 8 years he had the first epileptic seizure of simple occipital type with generalization and urination. Brain magnetic resonance imaging (MRI) showed focal cortical reductions in the left parietal and occipital regions. Interictal EEG recorded slowed basic activities above the posterior regions of the left hemisphere, with intermittent occurrence of occipital sharp waves and bioccipital sharp and slow-wave complexes. Initially, treatment with valproate was administered; however, the addition of carbamazepine into therapy induced aggravation of seizures and EEG findings, changed behavior and poor performance at school. By withdrawal of carbamazepine the condition improved both clinically and in EEG findings. Conclusion. Childhood occipital epilepsy lesions show deterioration due to carbamazepine, which if administered induces aggravation of seizures, behavior changes, cognition with occurrence of long-term bilateral discharges, and posterior sharp and slow wave high amplitude complexes recorded by EEG.

Past surgical series have emphasized the diagnostic complexity of posterior cortex epilepsy. Available data are sparse, especially in children, and most published series report a high number of surgical failures and post-operative neurological deficits. In this article, we present a paediatric cohort of 62 children who underwent surgery for drug resistant posterior cortex epilepsy before the age of 16 years with a mean post-operative follow-up of 6.94 years (range: 2-16). Mean age at epilepsy onset was 3.2 years and 28 children (45%) had onset before 1 year of age. The mean age at surgery was 7.9 years (range: 1-16). Daily seizures were present in 63% of children. MRI was positive in 58 cases (93.5%) and invasive stereo-EEG was judged mandatory in 24/62 (39%) of patients. Surgery was confined to the parietal lobe in 11 children, the occipital lobe in 8, the occipito-parietal region in four, the occipito-temporal region in 18, and involved both the temporal and parietal lobes in the remaining 21. Following surgery, 53 subjects (85.5%) remained seizure-free and among those who underwent a SEEG procedure, 75% achieved seizure freedom. Focal cortical dysplasia was the most frequent histopathological diagnosis (50%), followed by tumoural (24%) and gliotic lesions (14.5%). An older age at epilepsy onset, the presence of a rather restricted epileptogenic area, and a complete resection of the epileptogenic zone were predictive of a favourable surgical outcome. These results demonstrate that a good surgical outcome is possible in children with drug resistant posterior cortex epilepsy. Accurate analysis of the chronology of ictal semiology and electrophysiological features, viewed in the context of the complete electroclinical pattern, provides a topographical orientation for posterior cortex epilepsy and, together with the presence of a lesion detectable on imaging, may improve the rate of surgical success of posterior cortex epilepsy at paediatric age.

Children with occipital seizures often have ictal autonomic symptoms such as pallor and vomiting and lack motor manifestations. This has lead to misdiagnosis of occipital seizures in children. The following case report highlights the clinical features of a child with occipital epilepsy misdiagnosed as having migraine. The aetiology of symptomatic occipital epilepsy will be discussed.

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

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.

Purposes To evaluate the possibility of early syndrome classification of idiopathic partial epilepsies in children at the first seizure. Patients and methods In this observational study we prospectively evaluated 298 patients, aged between 1 month and 17 years and consecutively referred for the first unprovoked focal seizure. The whole cohort included 133 patients; the final analysis was carried out on 107 (59 males) individuals. Age at the first seizure ranged between 2.3 and 13.0 years. Clinical and EEG data of all patients were independently reviewed by two medical doctors. Patients were followed-up for at least 5 years, with a mean period of follow-up of 6.9 years. Results After the first seizure, a specific syndrome could be diagnosed in eighty (74.7%) children. In particular, Childhood Epilepsy with Centro-Temporal Spikes (CECTS) 42.9% of cases, Panayiotopoulos Syndrome (PS) 28.9%, idiopathic childhood occipital epilepsy of Gastaut (ICOE-G) 2.8%. Unclassified cases were 25.4%. At the end of the follow-up, the diagnosis was confirmed in 72 of 80 children (90%): BCECTS 89% of patients, PS 90% and ICOE-G 100%: among the unclassified cases, in 11 patients (40.7%) the diagnosis did not change, whereas 16 patients (59.3%) evolved into other syndromes or into atypical forms. Conclusions At the onset an initial diagnosis is possible in the majority of cases; epilepsy syndromes can be identified at the time of the initial diagnosis and at follow up this diagnosis has not to be revised in 90% of the cases.

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.

Idiopathic childhood occipital epilepsy of Gastaut (ICOE-G) is a rare form of epilepsy, characterized by visual hallucinations, periods of blindness, motor seizures, and migraine‐like symptoms. A characteristic EEG feature is fixation-off sensitivity: epileptiform discharges are suppressed by visual input. Here, we present an 11-year‐old girl suffering from ICOE-G, who was studied to identify potential additional suppressors of the epileptiform discharges.

Benign childhood focal seizures represent the mostcommonepilepticmanifestationsinchildhood and affect approximately 22% of children. Three identifiable electro clinical syndromes are coded by the International League against Epilepsy (ILAE) 1 :benignepilepsywithcentrotemporalspikes (BECTS), Panayiotopoulos syndrome (PS), and the idiopathic childhood occipital epilepsy of Gastaut (ICOE-G). Theterm“benign”referstothepositiveprognosis of these disorders in regard to the EEG pattern and the seizures. However, a significant number of children with BECTS present various cognitive deficits affecting language and memory functions the severity of which associated with the intensity and the duration of interictal epileptic discharges (IED) and resolve with EEG normalization. 2–6 Since the publication of the ILAE classification of epileptic syndromes, 7 the group of focal idiopathic epilepsies has been enlarged to a subgroup of epileptic encephalopathies (EE) with continuous spike-and-waves during slow-wave sleep (CSWS) in which the appearance and persistence of IED are associated with cognitive regression. BECTS and EE with CSWS represents opposite ends of a spectrum, behavioral and cognitive deficits are often milder in BECTS and severe in the CSWS epileptic encephalopathy. 8–14

This study intended to investigate the clinical and electroencephalographic benign occipital epilepsy of childhood (BOEC) characteristics in a population sample of patients from two tertiary Brazilian hospitals. We analyzed retrospectively 4912 electroencephalograms (EEGs) records, and the included patients were submitted to a new clinical and EEG evaluation. Were included 12 (0.92%) patients; 4 (33.3%) with criteria for early BOEC; 6 (50%) for late form and 2 (16.7%) with superimposed early and late onset forms. After new investigation, 2 (16.7%) had normal EEG; 4 (33.3%) had paroxysms over the occipital region; 3 (25%) over the temporal posterior regions and 3 (25%) over the posterior regions. Sharp waves were the predominant change, occurring in 8 (66.6%); spike and slow wave complexes in 1 (8.3%) and sharp and slow wave complexes in 1 (8.3%). Vomiting, headache and visual hallucinations were the most common ictal manifestations, presented in 100% of patients with superimposed forms. Vomiting were absent in the late form and headache was present in all forms of BOEC. Este estudo teve como objetivo investigar as características clínicas e eletrencefalográficas da epilepsia occipital benigna da infância (EOBI) em uma amostra populacional de pacientes de dois hospitais terciários brasileiros. Foram analisados retrospectivamente 4912 registros de eletrencefalograma (EEG). Os pacientes incluídos foram submetidos a nova avaliação clínica e eletrencefalográfica. Foram incluídos 12 (0,92%) pacientes; 4 (33,3%), com critérios para EOBI de início precoce; 6 (50%) para a forma tardia e 2 (16,7%), com superimposição de formas de início precoce e tardio. Após nova investigação, 2 (16,7%) apresentaram EEG normal; 4 (33,3%) paroxismos sobre a região occipital; 3 (25%) sobre a região temporal posterior e 3 (25%) sobre as regiões posteriores. Ondas agudas foram a alteração predominante, ocorrendo em 8 (66,6%); complexos espícula e onda lenta em 1 (8,3%) e complexos onda aguda e onda lenta em 1 (8,3%). Vômitos, cefaléia e alucinações visuais foram as manifestações ictais mais comuns, estando presentes em 100% dos pacientes com formas superimpostas de EOBI. Vômitos não foram relatados na forma tardia e cefaléia esteve presente em todas as formas de EOBI.

We describe a unique patient with cerebral calcifications of unknown origin presenting with pharmacoresistant occipital lobe epilepsy and fixation-off sensitivity. Our report further expands the spectrum of seizure disorders associated with fixation-off sensitivity.

The purpose of this review is to provide guidance for appropriate diagnosis and management of idiopathic childhood occipital epilepsy of Gastaut. The typical clinical features are visual seizures that typically consist of brief elementary visual hallucinations, which are mainly multicolored and circular. Ictal blindness and deviation of the eyes are also common symptoms. The seizures are usually frequent and diurnal. The electroencephalography is the only investigation with abnormal results, showing occipital spikes and often occipital paroxysms demonstrating fixation-off sensitivity. Brain magnetic resonance imaging is used to exclude symptomatic occipital epilepsy. Patients usually respond well to antiepileptic medication and about two-thirds remit by the age of 16 years. Idiopathic childhood occipital epilepsy of Gastaut is frequently misdiagnosed as migraine with visual aura, acephalgic, or basilar migraine. Differentiation from symptomatic occipital epilepsy, particularly when children are otherwise normal, can be difficult. Most children need prophylactic antiepileptic medication.

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.

Panayiotopoulos syndrome is a common and benign childhood autonomic epilepsy of debated localization. Although officially considered as occipital epilepsy, this is most likely of multifocal origin. Ictal electroencephalography is the gold standard of seizure localization, but in Panayiotopoulos syndrome, because patients have single or rare seizures, only 7 cases with ictal electroencephalography have been reported. Ictal onsets show variable anterior and more often posterior locations. We describe an 8-year-old girl with 5 nocturnal autonomic seizures typical of Panayiotopoulos syndrome from age 4. The last seizure was captured with electroencephalography and showed a diffuse onset of the ictal discharge, whereas various interictal electroencephalography had infrequent multifocal spikes. This case contributes to the understanding of the pathophysiology of Panayiotopoulos syndrome in favor of a diffuse and multifocal cortical epileptogenicity that triggers an unstable central autonomic nervous system solely or prior to the focal cortical symptoms

Two cases of focal occipital epilepsy with cerebral calcifications poorly responsive to antiepileptic treatment are described. In both cases coeliac disease was diagnosed and folic acid deficiency documented. A gluten-free diet and a brief supplementation with folic acid lead to a complete EEG and clinical normalization in one case and to a significant improvement of EEG and seizure control in the other.

Fixation-off sensitivity is an electroencephalographic phenomenon characterized by spike-and-wave discharges that only occur when central vision and fixation are eliminated. It is especially seen in children with Panayiotopoulos-type, early-onset, benign childhood occipital epilepsy or Gastaut type, late-onset, childhood occipital epilepsy. It can also be seen in eyelid myoclonia with absences, in other idiopathic generalized epilepsies, and in asymptomatic children without epilepsy. We describe a boy with atypical, benign partial epilepsy of childhood who exhibited the reverse: epileptiform activity that was suppressed by the absence of central vision or fixation, and activated by central vision or fixation.

Late onset childhood occipital epilepsy–Gastaut type (LOCOE) is a rare idiopathic epilepsy syndrome with an uncertain long-term prognosis. Elementary visual hallucinations and interictal spike-and-wave complexes in the occipital areas represent the main electroclinical findings of the syndrome. The functional nature of LOCOE has been emphasized together with the presence of genetic predisposition in the affected patients. Here, we report on two families in which two patients, respectively, showed electroclinical features compatible with LOCOE. Although further studies are needed to validate our observations, the involvement of two generations in one of the families we studied may corroborate the previously formulated hypothesis of an autosomal dominant model of inheritance in LOCOE. Of course, the identification of larger families is propaedeutic to linkage analysis studies.

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.

The aim of this paper is to promote the correct classification of, and provide guidelines on, the diagnosis and management of panayiotopoulos syndrome (ps). an international consortium of established researchers in the field collaborated to produce a consensus document. the resulting document defines ps, characterizes its electro-clinical features, considers its likely pathogenesis, and provides guidance on appropriate management. we conclude that ps is a common idiopathic, benign seizure disorder of childhood, which should be classified as an autonomic epilepsy, rather than an occipital epilepsy.

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.

Occipital lobe seizures, as defined by subjective symptoms and objective signs, can be recognized by clinical seizure characteristics in most cases. Visual symptoms such as hallucinations and amaurosis are the most common occipital lobe seizure symptoms. The patients must be classified in order to be able to define the prognosis. In this study, we classified patients with occipital epilepsy as childhood epilepsy with occipital paroxysms (19 patients), idiopathic photosensitive occipital epilepsy (10 patients) and symptomatic occipital epilepsy (25 patients). They were evaluated according to clinical, electrographic and neuroimaging characteristics.

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.