The benign partial nonrolandic epilepsies

Although rolandic epilepsy is the only epileptic syndrome that, at present, fully meets all the criteria of benignity and is widely recognized as such, there are also other forms of partial epilepsy that are benign. These are also genetically determined and age-dependent, occurring in neurologically intact children older than 18 months of age. As a rule, the seizures are brief and infrequent, usually responding well to antiepileptic drugs and remitting spontaneously before adulthood. The EEG background is normal, the morphology of the spikes is typical, and they are activated by sleep; generalized spike-wave discharges may occur. During the past decade, there have been reports of a number of additional syndromes of benign partial epilepsy. These include benign occipital epilepsy (the most common in this group), benign frontal epilepsy, benign epilepsy with affective symptomatology (or benign psychomotor epilepsy), benign epilepsy with extreme somatosensory evoked potentials, benign partial epilepsy of adolescence, and benign epilepsy associated with multiple spike foci. The Landau-Kleffner syndrome is often included among the benign epilepsies. These nonrolandic benign epilepsies are described in detail, and the relevant literature is reviewed.     

The localization-related epilepsies: some problems with subclassification

One of the nosological problems of childhood epilepsies is the evolutionary change of foci with age. Benign childhood epilepsy with centro-temporal spike may evolve to show EEG features of childhood epilepsy with occipital paroxysms and vice versa. West syndrome is generally accepted as generalized epilepsy but is often preceded or followed by partial seizures and may have both partial seizures and tonic spasms in a single attack. Among epilepsies with both generalized and focal seizures, severe myoclonic epilepsy in infancy is more linked to generalized epilepsy whereas epilepsy with continuous spike-waves during slow wave sleep is more linked to localization-related epilepsy. Any type of partial epilepsy may develop nonconvulsive generalized seizures transiently with generalization of paroxysmal discharges during the clinical course. Moreover, nonconvulsive generalized seizures may occur even in localization-related epilepsy with spike-wave index during NREM sleep in less than 85%.     

Differentiating between benign and less benign: epilepsy surgery in symptomatic frontal lobe epilepsy associated with benign focal epileptiform discharges of childhood

Benign focal epileptiform discharges of childhood are a genetically determined electroencephalographic trait. Assessment of their clinical relevance in children with epilepsy may be difficult if imaging reveals a lesion congruent or incongruous with the focus of the benign focal epileptiform discharges of childhood. This article reports a boy with parietooccipital benign focal epileptiform discharges of childhood in whom videoelectroencephalography and magnetic resonance imaging disclosed symptomatic frontal lobe epilepsy. Surgical removal of a focal cortical dysplasia in the left frontal lobe yielded freedom from seizures and positive behavioral and cognitive development. Nocturnal benign focal epileptiform discharges of childhood persisted until puberty (follow-up, 50 months). Early diagnostic differentiation of idiopathic syndromes such as idiopathic benign focal epilepsy of childhood from symptomatic focal epilepsies with a potentially less benign course is important. In symptomatic frontal lobe epilepsy, epilepsy surgery may yield an excellent outcome despite the presence of concurrent benign focal epileptiform discharges of childhood.     

The Localization-Related Epilepsies: Some Problems with Subclassification

Abstract: One of the nosological problems of childhood epilepsies is the evolutionary change of foci with age. Benign childhood epilepsy with centro-temporal spike may evolve to show EEG features of childhood epilepsy with occipital paroxysms and vice versa. West syndrome is generally accepted as generalized epilepsy but is often preceded or followed by partial seizures and may have both partial seizures and tonic spasms in a single attack. Among epilepsies with both generalized and focal seizures, severe myoclonic epilepsy in infancy is more linked to generalized epilepsy whereas epilepsy with continuous spike-waves during slow wave sleep is more linked to localization-related epilepsy. Any type of partial epilepsy may develop nonconvulsive generalized seizures transiently with generalization of paroxysmal discharges during the clinical course. Moreover, nonconvulsive generalized seizures may occur even in localization-related epilepsy with spike-wave index during NREM sleep in less than 85%.     

A magnetoencephalographic study of patients with Panayiotopoulos syndrome

Summary:  Purpose: Panayiotopoulos syndrome (PS) is a newly identified type of benign childhood epilepsy characterized by ictal vomiting and eye deviation. It is usually accompanied by occipital spike discharges; however, its classification as an early-onset benign childhood occipital epilepsy is controversial. To characterize this condition further, we examined the localization of equivalent current dipoles (ECDs) of spike discharges by magnetoencephalography (MEG) in patients with PS.
Methods: We studied 13 patients with a mean age at time of examination of 5 years (range, 3–14 years). MEG was measured by using a whole-head 204-channel neuromagnetometer with simultaneous EEG recordings. The estimated locations of ECDs of each peak of the spike discharges were overlaid on magnetic resonance images of the brain.
Results: Eleven (84.6%) patients showed clustered ECDs in the areas alongside the parietooccipital sulcus (eight of 13; 61.5%) and/or the calcarine sulcus (four of 13; 30.8%). Despite Fp-O synchronization of the spike discharges in the scalp EEG of five patients, no frontal locations of ECDs were found. All five (38.5%) boys with sylvian seizures, who also showed clustered ECDs in rolandic areas, had an earlier age at onset and higher seizure frequency than did other patients. ECD orientations were regular in all but one patient, who showed irregular and dispersed ECDs alongside bilateral calcarine sulci.
Conclusions: Our results demonstrate localized cortical hyperexcitability in the areas alongside major cortical sulci in PS and indicate that PS is closely related to benign childhood epilepsy with centrotemporal spikes.     

Gastaut type-idiopathic childhood occipital epilepsy and childhood absence epilepsy: A clinically significant association?

We report an unusual association between idiopathic occipital epilepsy and childhood absence epilepsy in 2 pediatric patients. At first clinical and electroencephalographic evaluation, the patients presented the peculiar signs of idiopathic occipital epilepsy Gastaut type: focal sensory visual seizures, migraine-like symptoms (only in one patient) and unilateral spike–wave discharges over occipital regions. Both children were treated with valproic acid and their seizures were rapidly controlled. After a seizure-free period, the patients presented typical absence with ictal electroencephalographies showing 3 cycles/s generalized and symmetrical spike–wave complexes. We discuss the possible association between these two epileptic syndromes and its common pathophysiological mechanisms.     

Longitudinal study of epileptiform EEG patterns in normal children

EEG were recorded in 3,726 children, from 6 to 13 years of age who were neurologically normal and had no history of epileptic seizures. The records were taken during wakefulness, at rest, and during hyperventilation. In 131 cases (3.54%) epileptiform patterns were found. They consisted of 3 count/sec spike and slow waves discharges (4 cases), multiple spike and slow wave complexes (37 cases), midtemporal spikes (50 cases), rolandic or parietal spikes (27 cases), occipital spikes (2 cases), and multifocal spikes (11 cases). Half of the subjects with EEG abnormalities had behavior problems and/or slight psychomotor ability disturbances. Follow-up studies over an 8 to 9 year period were performed. These demonstrated the spontaneous disappearance of the EEG abnormalities, usually within school age or, at the latest, during adolesence. Only seven individuals developed epileptic seizures of the primary generalized type which responded well to anticonvulsant drug treatment. From this study we can deduce that the epileptiform EEG patterns that often are found in children during school age have no clinical relationship to epilepsy in the great majority of cases. The relationship with epilepsy exists probably on a genetic level for the generalized discharges. The spike foci are non-epileptic in nature in all probability, especially if they emerge from a fairly normal background activity and their duration is very similar to that of the constituents of the background activity, as found in the majority of these subjects. On the contrary, it is probable that these alterations express difficulties in affective or motor adaptation during childhood.     

EEG dipole characteristics in Panayiotopoulos syndrome

PURPOSE: Panayiotopoulos syndrome (PS) is a type of benign childhood partial epilepsy that is frequently associated with abundant multifocal spikes other than main occipital spikes on the EEG. In this study, we investigated the characteristic features of dipoles in PS. METHODS: We performed dipole analysis of the interictal occipital spike discharges seen in 10 children with PS (group A) and in 10 children with other types of symptomatic localization-related epilepsy (group B). We analyzed the dipoles of the averaged spike in each patient. RESULTS: In group A, the averaged occipital spikes in each patient showed dense dipole locations in the mesial occipital area; in group B, widely scattered dipole locations were observed. In Group A, the geometric centers of the dipoles at each time point (such as at the main negative peak and before or after the main peak) were estimated in the neighboring locations. In contrast, they tended to be scattered in group B. CONCLUSIONS: Our study reveals that PS has high dipole stability, similar to that of rolandic epilepsy. From the electroencephalographic view, this seems to indicate a close link between these two syndromes.     

Abstracts of the 38th Congress of the Japan Epilepsy Society, Shizuoka, Japan, September 30-October 1, 2004

Diagnosis and Treatment of Idiopathic Focal Epilepsies (Benign Partial Epilepsies) in Infancy and Childhood. ¹ Tamiko Negoro ( ¹ Department of Laboratory Medicine, Nagoya University Hospital, Nagoya, Japan ). Introduction: According to the revised classification of epilepsies and epileptic syndromes proposed by the Commission on Classification and Terminology of the International League Against Epilepsy (1989), benign childhood epilepsy with centrotemporal spikes (BCECT), childhood epilepsy with occipital paroxysms (Gastaut‐type late‐onset CEOP), and primary reading epilepsy were included in the idiopathic localization‐related epilepsies (ILRE). Since then, new epileptic syndromes such as Panayiotopoulos‐type early‐onset benign childhood occipital epilepsy (also known as benign childhood epilepsy with occipital paroxysms or BCEOP) and benign partial epilepsies in infancy (BPEI) have been additionally included as ILRE. The diagnostic criteria for benign partial epilepsies include (1) normal neurological examination; (2) normal intelligence; (3) normal neuroimaging; (4) a family history of benign‐type seizures; (5) brief stereotyped seizures; (6) frequent nocturnal occurrence; (7) easy control with antiepileptic drugs (AEDs), except ethosuximide; and (8) remission before adolescence. The EEG features include (1) normal background activity; (2) spikes with a characteristic morphology and location; (3) sleep activation; and (4) occasional generalized paroxysms. In our 114 cases of childhood onset localization‐related epilepsies (LRE) diagnosed between 1997 and 2000, 48 cases (42%) were diagnosed as ILRE and 66 were cryptogenic or symptomatic LRE. Among the 48 ILRE cases, 28 (58%) were classified as BCECT, six (13%) as BPEI, five (10%) as BCEOP, two (4%) as atypical benign partial epilepsy, and seven (15%) as unclassified. Carbamazepine (CBZ), valproate (VPA) and clonazepam (CZP) were equally effective in controlling the seizures in our ILRE patients. This review describes briefly the diagnosis and treatment of BCECT, BCEOP, and BPEI. Benign Childhood Epilepsy with Centrotemporal Spikes: BCECT is the most common ILRE (also known as idiopathic focal epilepsies) in infancy and childhood. Sylvian seizures (simple partial seizures or focal motor or sensory seizures beginning unilaterally at the lower part of the face) and EEG findings (central‐midtemporal high‐amplitude, repetitive sharp waves) are quite stereotyped, and diagnosis of typical cases is relatively easy. Frontal lobe seizures with bilateral perioral twitches or unilateral eyelid twitches may sometimes be misdiagnosed as Sylvian seizures. Since status epilepticus is very rare and many patients only have occasional seizures, many physicians choose not to treat the disorder. If treatment is necessary, the seizures are usually easily controlled with carbamazepine, valproic acid, or clonazepam. Continuation of AEDs is not necessary after the EEG has normalized around puberty. The prognosis is excellent. However, a small percentage of patients may show atypical evolutions such as atypical benign partial epilepsy, epilepsy with continuous spike‐and‐waves in slow wave sleep, or epilepsy with centrotemporal spikes and oromotor deficit. Seizure and EEG exacerbations by AEDs, especially carbamazepine, have to be considered. Early recognition and proper treatment are necessary for these conditions. Benign Childhood Epilepsy with Occipital Paroxysms: BCEOP is also known as Panayiotopoulos‐type early‐onset benign childhood occipital epilepsy. The mean age at first seizure is around 5 years. Seizures comprise an unusual constellation of autonomic, mainly emetic, syndromes with unilateral deviation of the eyes and impairment of consciousness. Visual symptoms, which are the main symptoms in Gastaut‐type, late‐onset CEOP, are exceptions in BCEOP. According to our 41 cases examined between 1984 and 1993, two‐thirds of the patients show hemi‐ or generalized convulsions and one‐fourth experience prolonged seizures for longer than 30 minutes. EEG findings (bilateral occipital paroxysms) consist of some extraoccipital abnormalities especially in the frontal area (48% of our cases) or generalized paroxysmal discharges (32% of ours cases), together with various migrations of spike foci. EEG findings seem to normalize around the age of 10 years. Many patients only have occasional seizures and these seizures are usually easy to control with carbamazepine or valproic acid. The prognosis is excellent. Prevention of status epilepticus is the major problem in this disorder. Recently, new concepts including early‐onset benign occipital seizure susceptibility syndrome (EBOSS) and Panayiotopoulos syndrome (idiopathic susceptibility to early‐onset benign childhood seizures with mainly autonomic symptoms), have been introduced for BCEOP. Benign Partial Epilepsies in Infancy: BPEI comprise two forms. One is partial epilepsy with complex partial seizures (CPS) and the other is partial epilepsy with secondarily generalized seizures (SGS). Onset is mostly during the first year of life. Seizures often occur in clusters and are characterized by motion arrest, decreased responsiveness, staring or blank eyes often with automatisms, and mild convulsive movements in the CPS form; and motion arrest or opening of eyes with staring or blank eyes followed by generalized tonic–clonic seizures in the SGS form. Interictal EEGs mostly show no abnormal findings. According to the ictal EEGs, the most frequent site of seizure origin is in the frontal or temporal area in the CPS form; and central, parietal, or occipital area in the SGS form. Treatment with carbamazepine, phenobarbital, zonisamide or valproic acid immediately stops the cluster of seizures. The prognosis is excellent. Recently, sleep‐related and low‐voltage Rolandic and vertex spikes have been reported as an EEG marker of benignity in this disorder. Most of benign infantile convulsions may belong to partial epilepsy with SGS, although confirmation with ictal EEG recording is necessary for accurate diagnosis.     

A follow-up study of healthy children with epileptiform EEG discharges

Fifty-two healthy elementary schoolchildren with epileptiform EEGs who were not taking anticonvulsants were followed for an average period of 3 years and 3 months. Thirty-seven had centrotemporal spikes, three had occipital spikes, one had frontal spikes, nine had generalized spike-and-wave complexes, and two had a combination of multiple spike-and-wave complexes and focal spikes. Disappearance of epileptiform discharges was observed in 65% on waking EEGs and in 45% on sleep EEGs. After 5 years, the rate of disappearance was over 80% in waking and sleep EEGs. This study confirms that most epileptiform discharges in healthy children disappear spontaneously during childhood and that age-related development and disappearance occur in healthy children who would otherwise never be subjected to EEG examination. Epileptic seizures occurred in three cases (6%). This low percentage is not considered to be an indication for treating children with epileptiform discharges with anticonvulsants. However, the risk for seizure development may be higher than in the general population. According to the literature, children with multiple spike-wave complexes have a greater tendency to develop seizures. One girl with multiple spike-wave complexes and focal spikes developed generalized tonic-clonic convulsions. Two boys with centrotemporal spikes developed benign childhood epilepsy with centrotemporal spikes (BCECS). This study confirms that some (5%) of healthy children with centrotemporal spikes, the most common epileptiform pattern in healthy children, develop BCECS.     

Electroencephalographic generalized features in idiopathic childhood focal epilepsies

PurposeIdiopathic focal epilepsies in childhood including benign childhood epilepsy with occipital paroxysms (BEOP) or benign childhood epilepsy with centro-temporal spikes (BCECTS) are characterized by specific focal electrographic patterns as the name indicates. Generalized spike-wave discharges in children with idiopathic focal epilepsy can suggest a neurobiological continuum with the idiopathic generalized epilepsies. We assessed the prevalence of generalized epileptiform discharges and generalized seizures in BEOP/BCECTS patients.MethodsBetween August 2005 and November 2008, we identified 220 cases with electroclinical features typical of idiopathic focal epilepsies, 172 patients with BCECTS and 48 patients with BEOP, excluding patients whose neurological examinations or brain MRI were abnormal. We analyzed gender, age at onset, manifestation of generalized seizures, and serial EEG records to detect generalized abnormalities.ResultsOf our population, 42 patients (19.1%, 22 boys), 30 (17.4%) of 172 BCECTS patients and 12 (25.0%) of 48 BEOP patients, showed generalized spike-wave discharges once or more during follow-up. The typical 3-Hz generalized spike wave discharge was noticed in 7 patients and concurrence with clinical generalized seizure was observed in 11.ConclusionA relatively high incidence of generalized spike-wave discharge and concurrence with generalized seizure were observed in patients with BEOP/BCECTS, with the incidence being higher in BEOP patients than in those with BCECTS. It may be inferred that idiopathic focal epilepsy is not a fixed syndrome but is a part of a broad, age-related, benign, seizure susceptibility syndrome.     

Dipole analysis in panayiotopoulos syndrome

Panayiotopoulos syndrome (PS) is a type of benign childhood partial epilepsy, which has a good prognosis despite the fact that it is frequently associated with abundant multifocal spikes on the electroencephalography (EEG). We investigated whether stable dipoles, as seen in rolandic epilepsy, were also present in PS. We performed dipole analysis of the interictal spike discharges seen in the interictal EEGs of eight children with PS. We chose more than 10 spikes for each kind of spike, and investigated whether or not more than three of these spikes showed consistently stable dipole locations. (1) We observed 15 different kinds of spikes in various regions in the EEGs of the eight children. (2) Twelve of the 15 kinds of spikes had dipoles with a high goodness of fit. Furthermore, 14 of the 15 spikes had stable dipoles with similar locations for more than three individual spikes. (3) Fourteen of the 15 spikes, including frontal spikes, showed dense dipole locations in the mesial occipital area. Thirteen of these 14 spikes also showed other dipole locations in the rolandic area and/or the vertex (Cz). Our study revealed that the various types of spikes observed in PS have similar and stable dipole locations. The dipoles showing high stability, were located in the mesial occipital area, and were accompanied by dipoles located in the rolandic area. The stability and location of these dipoles indicate that there may be a pathogenetic link between PS and rolandic epilepsy.     

伴强直性发作癫痫患儿的临床表现和脑电图特征

目的通过对67例伴强直性发作癫痫患儿的临床表现及视频脑电图(VEEG)特点分析,提高对该发作类型的诊断水平。结果收集河北省儿童医院神经内科67例伴强直性发作的癫痫患儿的病例资料,分析其临床表现和VEEG特征。结果 67例患儿均监测到明确的临床发作,其中清醒期发作19例(28%),睡眠期发作30例(45%),且容易出现在睡眠I期、II期。发作间期脑电图表现:①背景活动正常37例,慢化者15例;②广泛性棘波节律阵发,易出现在非快速眼动期(NREM期);③广泛性及多灶性慢波、棘慢波或多棘慢波阵发;④一侧或双侧前头部棘波、棘慢波或θ活动发放;⑤单侧或双侧Rolandic区棘慢波发放;⑥高度失律。发作期脑电图表现:①局灶起始的棘波节律发放;②广泛性棘波节律发放;③广泛性慢波阵发,其上复合或其后跟随棘波节律;④广泛性4~6Hz棘慢波发放→广泛性棘波节律阵发;⑤广泛性低波幅棘波节律发放→广泛性高波幅棘慢波阵发。以上表现形式有时会组合出现于同一例患者中。发作持续时间与背景活动的关系:发作持续约1~8s者39例(39/67,58.2%),背景活动慢化者4例(4/39,10.3%);发作持续8~15s,甚者更长者(15s)28例(28/67,41.7%),背景活动慢化者11例(11/28,39.3%)。67例患者随访研究1年,最终诊断为:8例(11.9%)诊断为婴儿痉挛征,7例(10.4%)诊断为Lennox-Gastaut综合征(LGS),3例(4.4%)诊断为额叶癫痫,15例(22.3%)诊断为伴有中央颞区棘波的儿童良性癫痫(BECT),34例(50.7%)仅停留在发作类型的诊断层面。结论强直性发作可单独出现,也可出现在多种癫痫综合征中;VEEG可监测患儿发作期临床表现及脑电图异常波形,为临床诊断及鉴别诊断提供理论依据。     

Benign focal epilepsy of childhood (BFEC)]

Thirty cases of benign focal epilepsy of childhood were reported. The seizures were partial or generalized motor ones in all cases. One patient had episodes of visual hallucination with motor seizures. No objective examination has demonstrated cerebral lesions in all cases. The most characteristic in the present study was that the attacks were in relation to the sleep in 90% of cases, 56.7% of all patients had nocturnal seizure only. The characteristic EEG patterns were the spike or sharp discharges in Rolandic area in 29 cases, and occipital sharps or sharp wave complexes in one patient on normal background activities. The discharge rate of Rolandic spikes or sharps were significantly higher during sleep than during the awake stage, and 12 cases had Rolandic discharges only during sleep. Sleep EEG recordings is suggested when children were suspected of having such kind of seizure type but having a normal EEG pattern when awake. Brief induced sleep is usually adequate.     

Autism and autistic epileptiform regression with occipital spikes

The electroencephalographs abnormalities seen in Landau-Kleffner syndrome (LKS) (language deterioration) are non-specific, and consist of a variety of epileptiform discharge patterns including continuous slow spike-wave discharges during sleep, focal sharp waves with spikes, and centrotemporal (rolandic) spikes. Similarly, the EEG abnormalities seen in autistic epileptiform regression (language and social/behavioral deterioration) are nonspecific and overlap with those seen in LKS. By contrast, distinct epilepsy syndromes in otherwise normal children occur in the EEG-deflned benign focal epilepsies of childhood. Occipital spikes or spike-wave present either in the older child with visual symptoms and headache or in the younger child with autonomic symptoms followed by brief or prolonged partial motor seizures. Seven young children (five from a consecutive series of 42) presenting clinically with autism or autistic regression and possible or definite seizures, whose EEGs revealed occipital spikes or spike-wave characteristic of the benign epilepsies, are reported. Although occipital spikes are commonly seen in young children as an age-dependent EEG-defined benign focal epilepsy, their high frequency in this population with cognitive difficulties suggests a possible causal relation. The effects of the epileptiform discharge on cognitive functioning presumably reflect extension into temporal and parietal lobes, rather than occipital disturbances per se.     

Panayiotopoulos-type benign childhood occipital epilepsy: a prospective study

OBJECTIVE: To characterize the clinical and EEG features of the syndrome of benign childhood partial seizures with ictal vomiting and EEG occipital spikes (Panayiotopoulos syndrome [PS]). METHODS: Prospective study of children with normal general and neurologic examinations who had seizures with ictal vomiting and EEG with occipital spikes. RESULTS: From February 1990 to 1997, the authors found 66 patients with PS and 145 children with benign childhood epilepsy with centrotemporal spikes. Peak age at onset of PS was 5 years. Ictal deviation of the eyes and progression to generalized seizures were common. One-third had partial status epilepticus. During sleep, all had seizures. While awake, one-third also had seizures. Five children with PS had concurrent symptoms of rolandic epilepsy and another five developed rolandic seizures after remission of PS. Prognosis was excellent: one-third had a single seizure, one-half had two to five seizures, and only 4.5% had frequent seizures. CONCLUSIONS: Panayiotopoulos-type benign childhood occipital epilepsy is less common than benign childhood epilepsy with centrotemporal spikes but is well defined and recognizable by clinical and EEG features.     

Occipital Paroxysmal Discharges Suppressed By Eye Opening: Variability in Clinical and Seizure Manifestations in Childhood

Summary: The EEG in childhood epilepsy with occipital paroxysms (CEOP) was termed "distinctive" by Gastaut (1985) and Talwar et al. (1992) and "characteristic" by Herranz Tanarro et al. (1984), which suggests that the EEG is specific and diagnostic for CEOP. However, this hypothesis has been challenged (Newton and Aicardi, 1983; Beaumanoir and Grandjean, 1987). To test this, we reviewed 5,291 EEG reports made in 51/2 years in the only tertiary pediatric center in Newfoundland and Labrador. We identified 31 children who had one or more EEGs with occipital spike/sharp waves showing suppression of discharges with eye opening and normal background activity. Six had CEOP, 17 had benign nocturnal childhood occipital epilepsy, 5 had symptomatic epilepsy, 3 had unusual complex partial seizures (CPS), 4 had only provoked seizures, and 2 had no definite seizures. Overlap between seizure types was common. The EEG criteria for CEOP are not very specific.     

Benign childhood epilepsy with occipital paroxysms: a 15-year prospective study

Eighteen of 418 children who had onset of epilepsy before the age of 13 years showed clinical and electroencephalographic evidence of benign childhood epilepsy with occipital paroxysms. They represented one-fifth of all benign age- and localization-related idiopathic epilepsies seen. Some patients were followed as long as 15 years. There was a preponderance in females and peak age at onset of epilepsy was 5 years. In 16 children, the seizures were infrequent and sometimes prolonged and consisted mainly of tonic deviation of the eyes and vomiting, often with evolution to unilateral or generalized convulsions. Seizures were only nocturnal in 11 and nocturnal and diurnal in another 5 children. Prognosis was excellent; 5 children had only one fit. Remission usually occurred 1 to 2 years after onset and no seizures occurred after the age of 12 years. The remaining 2 children had frequent diurnal episodes consisting of visual hallucinations, postictal headache, and occasional nocturnal hemiconvulsions. Their prognosis was less favorable. Electroencephalographic abnormalities in all 18 patients consisted of repetitive spike and slow-wave discharges confined to the occipital regions and attenuated when the eyes were open. These outlasted clinical remission for many years, sometimes up to the age of 16. Fixation-off sensitivity was demonstrated frequently. Based on these findings, a unifying definition for benign childhood epilepsy with occipital paroxysms is proposed.     

Benign childhood epileptic syndromes with occipital spikes: new classification proposed by the International League Against Epilepsy

In a recent proposal, the Commission on Classification and Terminology of the International League Against Epilepsy recognized early-onset childhood epilepsy with occipital spikes (Panayiotopoulos type), differentiating it from the only other type of childhood epilepsy with occipital spikes previously accepted: late-onset childhood epilepsy with occipital spikes (Gastaut type). The importance of this newly recognized syndrome of benign childhood partial seizures is that it is very common-only 2.4 times less frequent than benign rolandic epilepsy, and of equally excellent prognosis. It is characterized by a unique seizure type comprising a combination of autonomic and behavioral disturbances, vomiting, deviation of the eyes, and often with impairment of consciousness that can progress to convulsions. These commonly last for more than 3 minutes and in one quarter of cases for hours. One or more of these symptoms can predominate or be absent. Eyes can remain open without deviation, ictal vomiting might not occur, and autonomic and behavioral disturbances can predominate, particularly in the early stages of the ictus, and be missed in nocturnal seizures. Age at onset is 5 years, with a singular or a median of three seizures, which are predominantly nocturnal. Interictal electroencephalography (EEG) frequently shows occipital paroxysms or occipital spikes but one-fifth of the cases have only extraoccipital spikes on normal EEG. Treatment might not be needed. Panayiotopoulos syndrome, like rolandic epilepsy, needs recognition by the general pediatrician because of the invariably excellent prognosis and also because it can be misdiagnosed as an acute cerebral insult.     

Clinical correlations of occipital epileptiform discharges in children.

OBJECTIVES: To determine the frequency of different causes of occipital epileptiform discharges (OEDs) in children and to analyze the EEG features that help predict epilepsy type and prognosis. METHODS: We identified children with OEDs in the absence of other focal discharges using an EEG database at our center; the presence of generalized spike-wave discharges (GSW) or slowing was not an exclusion criterion. Diagnosis, neurologic status, seizure semiology, and seizure remission status were recorded. RESULTS: Of 90 patients with OEDs, 50 (56%) had symptomatic seizures (18 with cerebral palsy, 11 with cerebral dysgenesis, 8 with genetic abnormalities); 31 (34%) had idiopathic seizures, including 6 with benign childhood epilepsy with occipital paroxysms (BCEOP), 8 (9%) had no seizures; and 1 (1%) had febrile seizures. Only two reported ictal visual symptoms. Eighty-seven percent with background slowing had symptomatic seizures, and 87% with normal backgrounds had idiopathic seizures (p < 0.001). Of 72 children with seizures and adequate follow-up, 28 of 45 (62%) with a normal background experienced seizure remission compared with 10 of 27 (37%) with background slowing (p = 0.04). Twenty of 81 patients with epilepsy had GSW. Twelve (60%) of the 20 GSW-positive patients had idiopathic epilepsy compared with 19 of 61 (31%) without GSW (p = 0.02). CONCLUSIONS: Most epilepsy in referred children with OEDs is symptomatic; syndromes such as BCEOP are rare. Visual ictal symptoms are rare. The presence of GSW or a normal background rhythm correlates with idiopathic seizures and a better prognosis.