Childhood Occipital Epilepsy Of Gastaut Research Articles

Sleep, cognitive functions, behavioral, and emotional disturbance in self-limited focal childhood epilepsies

BackgroundSelf-limited focal epilepsies of childhood, including self-limited epilepsy with centrotemporal spikes (SeLECTS), idiopathic childhood occipital epilepsy of Gastaut (ICOE-G), and self-limited epilepsy with autonomic seizures (SeLEAS), were considered benign conditions. However, recent research assumed potential adverse impacts on sleep, cognition, behavior, and emotional well-being. Our aim was to investigate the effects of self-limited focal epilepsies on sleep architecture, cognitive functions, behavior, and emotional disturbances in drug naive children. A cross-sectional study was conducted on 46 newly diagnosed children (ages 6–12) with SeLECTS (n = 22), ICOE-G (n = 12), SeLEAS (n = 12), and 20 healthy controls. Overnight polysomnography was performed. Cognitive assessments included intelligence scales, executive function tests, verbal fluency, visuospatial abilities, and memory. Behavioral and emotional problems were evaluated using standardized questionnaires and psychiatric interviews.ResultsChildren with epilepsy exhibited significant disturbances in sleep architecture, impairments in cognitive domains (executive functions, verbal fluency, visuospatial skills, and memory), and higher rates of internalizing/externalizing problems, social issues, attention deficit hyperactive disorder, depression, and anxiety compared to controls. No significant differences were found among the three epilepsy subtypes.ConclusionSelf-limited focal epilepsies in childhood are associated with sleep disruption, cognitive deficits, behavioral issues, and psychiatric comorbidities, challenging their traditional "benign" perception. Comprehensive management approaches addressing these multidimensional impacts are warranted.

Treatment of Classic Syndromes in Idiopathic Focal Epilepsies in Childhood

Of the classic syndromes of idiopathic (benign) focal epilepsies in childhood, more than half of children with Rolandic epilepsy and Panayiotopoulos syndrome do not need antiepileptic drug (AED) treatment. Monotherapy with carbamazepine, levetiracetam, sodium valproate, or clobazam is usually recommended for those with seizures that are unusually frequent (more than three), lengthy, distressing, or otherwise significantly interfering with the child's life. Other factors in favor of treatment include a short interval between the first three seizures, a younger age of onset (less than 4 years), or a presence of recurrent tonic–clonic seizures and diurnal seizures. In contrast to Rolandic epilepsy and Panayiotopoulos syndrome that may not need treatment, idiopathic childhood occipital epilepsy of Gastaut should be treated because seizures are frequent and secondarily generalized convulsions are probably unavoidable without medication. It is likely that all AEDs licensed as monotherapy for focal seizures and mainly carbamazepine and levetiracetam are suitable.The electroencephalogram does not predict prognosis or AED treatment needs, though there are views supporting that interictal spikes have an adverse effect on cognition.Strategies of withdrawing medication in idiopathic focal epilepsies in childhood differ among experts, although all agree that there is no need to continue medication 1 to 3 years after the last seizure, and certainly not after the age of 14 years, when most benign childhood focal seizures remit, or age 16, when they are practically nonexistent.In the acute stage of status epilepticus, control of the seizures is of paramount importance. Convulsive status epilepticus is a life threatening condition and should be terminated as soon as possible. Autonomic status epilepticus needs thorough evaluation for proper diagnosis and management. Benzodiazepines, intravenously, in rectal or buccal preparations are commonly used to terminate long lasting seizures and status epilepticus. Early parental treatment is more effective than late emergency treatment. Buccal midazolam is probably the first choice medication for out-of-hospital termination of status epilepticus.Parental education and psychological support are the cornerstone of the optimal management because despite excellent prognosis, idiopathic focal epilepsies in childhood usually have a dramatic impact to parents.

Clinical and EEG Features of Idiopathic Focal Epilepsies in Childhood

The International League Against Epilepsy (ILAE) report lists three well-defined syndromes of idiopathic focal epilepsies in childhood: benign childhood epilepsy with centrotemporal spikes (BCECTS), Panayiotopoulos syndrome (PS), and idiopathic childhood occipital epilepsy of Gastaut (ICOE-G). The concept of idiopathic and benign focal epilepsies in childhood is relevant as the term implies absence of structural brain lesions and genetic predisposition in the presence of age-dependent seizures. BCECTS is the most frequent of the benign focal epilepsies in childhood accounting for 15 to 25% of epilepsy syndromes in children below 15 years of age. It is also the most frequent epilepsy syndrome occurring at school age. The prevalence of PS was around 13% in children aged 3 to 6 years with one or more nonfebrile seizures, and 6% in the age group of 1 to 15 years. These figures may be higher if children who are currently considered to have an atypical clinical presentation are included in the syndrome. PS is the most common specific cause of nonfebrile status epilepticus in childhood. ICOE “Gastaut type” is a rare manifestation of a focal idiopathic epilepsy that has an age-related onset and is often age limited. The seizures of ICOE Gastaut type are always of occipital-lobe onset and primarily manifest with visual seizures, which are the most typical and usually the first ictal symptom, but other types of seizures may be associated. ICOE “Gastaut type” is a rare condition with a probable prevalence of 0.2 to 0.9% of all epilepsies, and accounting for 2 to 7% of benign childhood focal seizures.The recognition of these age-dependent epileptic syndromes is crucial for the adequate management of the children and their family.

Clinical dissection of childhood occipital epilepsy of Gastaut and prognostic implication.

Our aim was to describe the clinical and electrical features and the long-term evolution of childhood occipital epilepsy of Gastaut (COE-G) in a cohort of patients and to compare long-term prognosis between patients with and without other epileptic syndromes. This was a retrospective analysis of the long-term outcome of epilepsy in 129 patients with COE-G who were referred to 23 Italian epilepsy centres and one in Austria between 1991 and 2004. Patients were evaluated clinically and with electroencephalograms for 10.1-23.0 years. The following clinical characteristics were evaluated: gender, patient age at seizure onset, history of febrile seizures and migraine, family history of epilepsy, duration and seizure manifestations, circadian distribution and frequency of seizures, history of medications including the number of drugs, therapeutic response and final outcome. Visual hallucinations were the first symptom in 62% and the only manifestation in 38.8% of patients. Patients were subdivided into two groups: group A with isolated COE-G; group B with other epileptic syndromes associated with COE-G. The most significant (P < 0.05) difference concerned antiepileptic therapy: in group A, 45 children responded to monotherapy; in group B only 15 children responded to monotherapy. At the end of follow-up, the percentage of seizure-free patients was significantly higher in group A than in group B. Childhood occipital epilepsy of Gastaut has an overall favourable prognosis and a good response to antiepileptic therapy with resolution of seizures and of electroencephalogram abnormalities. The association of typical COE-G symptoms with other types of seizure could be related to a poor epilepsy outcome.

Early classification of childhood focal idiopathic epilepsies: Is it possible at the first seizure?

PurposesTo evaluate the possibility of early syndrome classification of idiopathic partial epilepsies in children at the first seizure. Patients and methodsIn 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. ResultsAfter 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. ConclusionsAt 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.

The Characterization of Childhood Occipital Epilepsy of Gastaut: A Study of Seven Patients

Characterization of the electroclinical features and evolution of childhood occipital epilepsy of Gastaut (COE-G). Seven children were retrospectively identified as having COE-G and were followed-up clinically using EEGs. Visual manifestations were the most common ictal event. Eye-associated ictal deviation was associated with ipsilateral turning of the head and migraine-like symptoms were frequent. Hemiconvulsions occurred in two children, and only one child had secondary generalized tonic-clonic seizures. In all patients, seizures occurred while awake, while two patients also had seizures while sleeping. EEG showed five patients with occipital spike-wave discharges when their eyes were closed which disappeared once their eyes were opened. Two cases continued having frequent seizures despite antiepileptic drug treatment. These patients also displayed learning difficulties and behavioral impairments after seizure onset. COE-G is a distinctive epileptic syndrome; however, the long-term prognosis for patients with the condition is unclear.

Benign occipital lobe seizures: Natural progression and atypical evolution

Benign occipital seizure syndromes are benign childhood epilepsy syndromes and are mainly of two types, Panayiotopoulos syndrome, an autonomic epilepsy and idiopathic childhood occipital epilepsy of Gastaut (ICOE-G) including the idiopathic photosensitive occipital lobe epilepsy. Although both these types are categorized as occipital seizures, they are distinct in presentation and management. They can also be tricky to diagnose as visual symptoms may not always be the presenting feature and it is also not very easy to elicit visual hallucinations during history taking. These seizures have a good response to treatment; however, there could be atypical evolution and refractoriness to treatment especially with ICOE-G. We describe three children who presented with visual and non-visual symptoms and the electroencephalography (EEG) in all the three cases showed occipital paroxysms. We have emphasized the clues in the clinical history and EEG leading to the diagnosis of these distinct epilepsy syndromes. We have also discussed the natural course of these epilepsy syndromes with some atypical evolution, which clinicians need to be aware of during treatment of these children.

Suppressors of interictal discharges in idiopathic childhood occipital epilepsy of Gastaut

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.

Idiopathic Childhood Occipital Epilepsy of Gastaut: Report of 12 Patients

This study sought to present clinical and outcome data of patients with idiopathic childhood occipital epilepsy of Gastaut, to validate previously reported characteristics of this epilepsy. The study group was comprised of 12 affected children (three boys and nine girls), with a median age of onset at 10.3 years. Common ictal manifestations included elementary visual hallucinations (75.0%), blindness or blurring of vision (50.0%), headache (50.0%), and secondarily generalized tonic-clonic seizures (58.3%). Interictal electroencephalography revealed occipital spike-wave paroxysms reactive to eye closure and opening in all patients, accompanied by spike-wave activity in the extra-occipital areas in four (33.3%), and by generalized spike-wave discharges in two (16.7%). One patient exhibited the onset of occipital lobe seizures 1 year after manifesting absence epilepsy. Seizure remission occurred in 81.8% of cases, in half of which medication was discontinued by late adolescence. This study confirmed the previously delineated electroclinical features of epilepsy syndrome, with additional aspects including the frequent association of generalized tonic-clonic seizures and atypical evolution from childhood absence epilepsy.

Idiopathic Childhood Occipital Epilepsy of Gastaut: A Review and Differentiation From Migraine and Other Epilepsies

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.

Benign childhood focal epilepsies: assessment of established and newly recognized syndromes

A big advance in epileptology has been the recognition of syndromes with distinct aetiology, clinical and EEG features, treatment and prognosis. A prime and common example of this is rolandic epilepsy that is well known by the general paediatricians for over 50 years, thus allowing a precise diagnosis that predicts an excellent prognosis. However, rolandic is not the only benign childhood epileptic syndrome. Converging evidence from multiple and independent clinical, EEG and magnetoencephalographic studies has documented Panayiotopoulos syndrome (PS) as a model of childhood autonomic epilepsy, which is also common and benign. Despite high prevalence, lengthy and dramatic features, PS as well as autonomic status epilepticus had eluded recognition because emetic and other ictal autonomic manifestations were dismissed as non-epileptic events of other diseases. Furthermore, PS because of frequent EEG occipital spikes has been erroneously considered as occipital epilepsy and thus confused with the idiopathic childhood occipital epilepsy of Gastaut (ICOE-G), which is another age-related but rarer and of unpredictable prognosis syndrome. Encephalitis is a common misdiagnosis for PS and migraine with visual aura for ICOE-G. Pathophysiologically, the symptomatogenic zone appears to correspond to the epileptogenic zone in rolandic epilepsy (sensory-motor symptomatology of the rolandic cortex) and the ICOE-G (occipital lobe symptomatology), while the autonomic clinical manifestations of PS are likely to be generated by variable and widely spread epileptogenic foci acting upon a temporarily hyperexcitable central autonomic network. Rolandic epilepsy, PS, ICOE-G and other possible clinical phenotypes of benign childhood focal seizures are likely to be linked together by a genetically determined, functional derangement of the systemic brain maturation that is age related (benign childhood seizure susceptibility syndrome). This is usually mild but exceptionally it may diverge to serious epileptic disorders such as epileptic encephalopathy with continuous spike and wave during sleep. Links with other benign and age-related seizures in early life such as febrile seizures, benign focal neonatal and infantile seizures is possible. Overlap with idiopathic generalized epilepsies is limited and of uncertain genetic significance. Taking all these into account, benign childhood focal seizures and related epileptic syndromes would need proper multi-disciplinary re-assessment in an evidence-based manner.

Childhood Occipital Epilepsy of Gastaut

The eleciroclinical features and evolution of childhood occipital epilepsy of Gastaut (COE-G) are analyzed in a study of 33 patients identified and followed between 1990 and 2007 at the Hospital National de Pediatria, Buenos Aires, Argentina.

Childhood occipital epilepsy of Gastaut: A study of 33 patients

To characterize the electroclinical features and evolution of childhood occipital epilepsy of Gastaut (COE-G). Children with electroclinical criteria of COE-G were retrospectively identified and followed-up clinically, and with sleep and awake EEGs between 1990 and 2007. We identified 33 patients with COE-G. In the same length of time, 201 children with Panayiotopoulos syndrome and 410 children with benign childhood epilepsy with centrotemporal spikes were registered. COE-G had a peak age at onset of 8.5 years. Visual manifestations were the most common ictal event. Ictal deviation of the eyes was frequent. Approximately half of the patients had migraine-like symptoms. In all patients the seizures occurred while awake, and 11 also had seizures during sleep. The majority of the patients had occipital spike-wave discharges when the eyes were closed that disappeared or attenuated when the eyes were opened. Prognosis was excellent in 80% of the cases. This study confirms the existence of COE-G, a rare but well-defined syndrome within the group of idiopathic focal epilepsies in childhood.