Benign myoclonic epilepsy in infancy followed by childhood absence epilepsy

Benign myoclonic epilepsy in infancy (BMEI) is a rare syndrome included among idiopathic generalized epilepsies (IGE) and syndromes with age-related onset. Recently, it has been shown that a few patients with BMEI later had other epilepsy types mainly IGE but never childhood absence epilepsy (CAE).We report a patient who at 11 months of age showed isolated myoclonic jerks occurring several times a day. The ictal video-EEG and polygraphic recording revealed generalized discharge of spike-wave (SW) lasting 1–2 s associated with isolated bilateral synchronous jerk involving mainly the upper limbs controlled by valproic acid (VPA).At 6 years and 8 months the child developed a new electroclinical feature recognized as CAE. The ictal EEG disclosed a burst of rhythmic 3 Hz generalized SW.Our case is the first patient with BMEI reported in the literature who later developed a CAE.This finding suggests a common neurobiological and genetic link between different age-related epileptic phenotypes.     

Ictal headache: Headache as first ictal symptom in focal epilepsy

Headache may be associated with seizures as a preictal, ictal, or postictal phenomenon, but it is often neglected because of the dramatic neurological manifestations of the seizure. Headache can also be the sole or predominant clinical manifestation of epileptic seizures, although this is a relatively rare condition. We describe two cases of focal symptomatic drug-resistant epilepsy with headache as the first ictal symptom. In both cases, the headache, which lasted a few seconds, was contralateral to the ictal discharge and did not have the clinical features of migraine. Ictal headache is a rare epilepsy symptom that can help to localize ictal EEG discharges. Recently, the term ictal epileptic headache has been proposed in cases in which headache is the sole ictal epileptic manifestation Diagnosis requires the simultaneous onset of headache with EEG-demonstrated ictal discharges.     

Ictal headache: headache as first ictal symptom in focal epilepsy

Headache may be associated with seizures as a preictal, ictal, or postictal phenomenon, but it is often neglected because of the dramatic neurological manifestations of the seizure. Headache can also be the sole or predominant clinical manifestation of epileptic seizures, although this is a relatively rare condition. We describe two cases of focal symptomatic drug-resistant epilepsy with headache as the first ictal symptom. In both cases, the headache, which lasted a few seconds, was contralateral to the ictal discharge and did not have the clinical features of migraine. Ictal headache is a rare epilepsy symptom that can help to localize ictal EEG discharges. Recently, the term ictal epileptic headache has been proposed in cases in which headache is the sole ictal epileptic manifestation Diagnosis requires the simultaneous onset of headache with EEG-demonstrated ictal discharges.     

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.     

Clinical and electrophysiological characteristics of startle epilepsy in childhood

ObjectiveStartle epilepsy is one syndrome of reflex epilepsies. We studied its clinical and EEG characteristics.MethodsAnalysis of the clinical and EEG characteristics of startle epilepsy.ResultsOf 11 patients, five were female. Age of onset ranged from 5 months to 7.5 years. Abnormal etiologies were found in seven patients, as a result of perinatal and postnatal factors. Neuroimaging showed abnormalities, commonly focal atrophy, in nine patients. Spontaneous seizures preceded or followed the startle seizures and were present in all patients. Startle seizures experienced included tonic, myoclonic, tonic–myoclonic, tonic–atypical absence, asymmetric tonic motor seizure and tonic–clonic seizure. Diffuse electrodecremental pattern was the most common ictal EEG pattern seen. The triggering stimuli of the startle seizures were sound in seven patients, touch in three and both sound and touch in one. Interictal EEG revealed abnormalities in 10 patients including generalized, multifocal or focal discharges. Many different anti-epileptic drugs were often unsatisfactory.ConclusionStartle epilepsies were often symptomatic reflex epilepsies and there were several types of startle seizure. The ictal EEG often showed a diffuse electrodecremental pattern. This disease has a bad prognosis.SignificanceWe delineated the clinical and EEG characteristics of startle epilepsy in childhood.     

The clinical usefulness of ictal surface EEG in neocortical epilepsy

PURPOSE: Localizable scalp EEGs, during ictal episodes, appear to be rare in neocortical epileptic syndromes. However, studies based on large numbers of patients are also rare. This study aims to identify the characteristic patterns of variable neocortical epilepsies and to evaluate their clinical usefulness in the localization of epileptogenic focuses. METHODS: We retrospectively assessed 394 noninvasive ictal recordings from 86 patients who subsequently underwent invasive study and resective surgery. Ictal EEGs were recorded using a video-EEG monitoring system with electrodes placed according to the International 10-20 system, with additional anterior temporal electrodes. The ictal recordings were analyzed according to localizing accuracy and frequency characteristics. The durations of discrete or regional ictal rhythms were also measured. RESULTS: The percentage of discrete or regional EEGs was 23% in frontal lobe epilepsy, 52% in lateral temporal lobe epilepsy, 70% in occipital lobe epilepsy, and 10% in parietal lobe epilepsy. In order of frequency, the localizable ictal rhythms were theta, beta, alpha, delta, and rhythmic spike-and-wave. The duration of discrete or regional ictal rhythms was significantly shorter in frontal lobe epilepsy and parietal lobe epilepsy than in other epilepsies. Ictal beta activity was the most common rhythm in discrete-patterned EEGs. Structural lesions found on MRI did not significantly affect the localization of epileptogenic focuses in the patients. The type of seizure was not related to the degree of localization, with the exception of simple partial seizure. CONCLUSIONS: Ictal surface EEG was clinically helpful in the localization of epileptogenic focuses in at least some neocortical epileptic syndromes.     

Pilomotor seizures: An autonomic semiology of limbic encephalitis?

PurposeIctal piloerection is an infrequent seizure semiology that is commonly overlooked as an ictal epileptic manifestation. Piloerection is considered to be principally caused by temporal lobe activity although frontal and hypothalamic seizure origins have been reported. The described etiology has shown a wide variety of structural causes such as mesial temporal sclerosis, tumors, posttraumatic, cavernomas and cryptogenic epilepsies.MethodsWe retrospectively reviewed the incidence of ictal piloerection in the clinical records of patients who underwent video-EEG monitoring (VEEGM) between 2007 and 2013 in a multicenter cooperative study. All patients presented refractory epilepsies and were evaluated with a protocol that included brain MRI, neuropsychology and VEEGM.ResultsA total of 766 patients were evaluated in four tertiary centers in Spain. Five patients showed piloerection as principal seizure semiology (prevalence 0.65%). The mean age at seizure onset was 39.6 years and the average epilepsy duration was 5.2 years (range 2–14) before diagnosis. Four patients were additionally examined with FDG-PET and/or SPECT-SISCOM. All presented temporal lobe epilepsy (TLE), three right-sided and two left-sided. A typical unilateral hippocampal sclerosis was described in 3 cases. The etiology detected in all cases was limbic encephalitis. Three had LGI1, one anti-Hu, and another Ma2 antibodies.ConclusionOur series describes a so far not well-recognized autoimmune association of pilomotor seizures to limbic encephalitis. This etiology should be ruled out through a comprehensive diagnostic work-up even in cases of long-lasting TLE with typical hippocampal atrophy on MRI.     

Hypnic Tonic Postural Seizures in Healthy Children Provide Evidence for a Partial Epileptic Syndrome of Frontal Lobe Origin

Summary: In the pediatric age period, three idiopathic partial epileptic syndromes are recognized: benign childhood epilepsy with centrotemporal spikes, childhood epilepsy with occipital paroxysms, and primary reading epilepsy. All other partial epilepsies are considered cryptogenic, and no other idiopathic partial epilepsies have been recognized. We observed 10 children with tonic partial postural seizures, mainly hypnic, coinciding with a recognizable ictal epileptiform EEG pattern. The children all had normal neuropsychological development before and after seizure onset. The seizures were tractable in all. Onset was in the early pediatric age period; no other type of seizure was recognized. The occurrence of a family history of epilepsy was high. The seizure pattern was typical of supplementary motor area origin. Because of the normal neuropsychological status, high familial incidence of epilepsy, and benign course of this partial epilepsy, we believe it should be considered idiopathic, using the criteria of the International Classification of Epilepsies and Epileptic Syndromes. We therefore suggest the existence of a partial idiopathic epileptic syndrome having onset with seizures of frontal lobe origin.     

Idiopathic Generalized Epilepsies Imitating Focal Epilepsies

Summary:  Classification of epileptic seizures and epilepsy syndromes as either focal or generalized is a fundamental and early part in the diagnostic process and is generally fairly easily accomplished. However, in patients with idiopathic generalized epilepsies, seizure and EEG features may suggest, particularly to the unwary, the occurrence of focal rather than generalized seizures. Misinterpretation of typical absence seizures as focal seizures, especially as temporal lobe seizures and of myoclonic seizures as focal clonic seizures, is a relatively common error and focal features during generalized tonic–clonic seizures may also be quite common. Sequences of seizures in idiopathic generalized epilepsies (such as absences or jerks followed by generalized tonic–clonic seizures) may also cause confusion. Versive and circling seizures are seizure types whose ictal semiology is clearly focal; nevertheless such seizures are described in idiopathic generalized epilepsies accompanied by generalized EEG discharges. The occurrence of focal EEG abnormalities in certain idiopathic generalized epilepsy syndromes is common. This is best known in juvenile myoclonic epilepsy.     

Epileptic seizures and headache/migraine: A review of types of association and terminology

PurposeThere are different possible temporal associations between epileptic seizures and headache attacks which have given rise to unclear or controversial terminologies. The classification of the International League Against Epilepsy does not refer to this type of disorder, while the International Classification of Headache Disorders (ICHD-2) defines three kinds of association: 1. migraine-triggered seizure (“migralepsy”), 2. hemicrania epileptica, and 3. post-ictal headache.MethodsWe performed an extensive review of the literature, not including “post-ictal” and “inter-ictal” headaches.ResultsOn the basis of well-documented reports, the following clinical entities may be identified: (A) “epileptic headache (EH)” or “ictal epileptic headache (IEH)”: in this condition headache (with or without migrainous features) is an epileptic manifestation per se, with onset, and cessation if isolated, coinciding with the scalp or deep EEG pattern of an epileptic seizure. EH maybe followed by other epileptic manifestations (motor/sensory/autonomic); this condition should be differentiated from “pure” or “isolated” EH, in which headache/migraine is the sole epileptic manifestation (requiring differential diagnosis from other headache forms). “Hemicrania epileptica” (if confirmed) is a very rare variant of EH, characterized by ipsilateral location of headache and ictal EEG paroxysms. (B) “Pre-ictal migraine” and “pre-ictal headache”: when a headache attack is followed during, or shortly after, by a typical epileptic seizure. The migraine attack may be with or without aura, and its seizure-triggering role (“migraine-triggered seizure”) is still a subject of debate. A differentiation from occipital epilepsy is mandatory. The term “migralepsy” has not been used uniformly, and may therefore led to misinterpretation.ConclusionsOn the basis of this review we suggest definitions and a terminology which may become the basis of a forthcoming classification of headaches associated with epileptic seizures.     

Hyperventilation revisited: physiological effects and efficacy on focal seizure activation in the era of video-EEG monitoring

PURPOSE: Hyperventilation is an activation method that provokes physiological slowing of brain rhythms, interictal discharges, and seizures, especially in generalized idiopathic epilepsies. In this study we assessed its effectiveness in inducing focal seizures during video-EEG monitoring. METHODS: We analyzed the effects of hyperventilation (HV) during video-EEG monitoring (video-EEG) of patients with medically intractable focal epilepsies. We excluded children younger than 10 years, mentally retarded patients, and individuals with frequent seizures. RESULTS: We analyzed 97 patients; 24 had positive seizure activation (PSA), and 73 had negative seizure activation (NSA). No differences were found between groups regarding sex, age, age at epilepsy onset, duration of epilepsy, frequency of seizures, and etiology. Temporal lobe epilepsies were significantly more activated than frontal lobe epilepsies. Spontaneous and activated seizures did not differ in terms of their clinical characteristics, and the activation did not affect the performance of ictal single-photon emission computed tomography (SPECT). CONCLUSIONS: HV is a safe and effective method of seizure activation during monitoring. It does not modify any of the characteristics of the seizures and allows the obtaining of valuable ictal SPECTs. This observation is clinically relevant and suggests the effectiveness and the potential of HV in shortening the presurgical evaluation, especially of temporal lobe epilepsy patients, consequently reducing its costs and increasing the number of candidates for epilepsy surgery.     

Clinical implications of scalp ictal EEG pattern in patients with temporal lobe epilepsy

ObjectiveTo determine the clinical implications of scalp ictal EEG pattern in patients with temporal lobe epilepsy (TLE).MethodsScalp EEG ictal patterns were retrospectively determined in 27 consecutive patients with medically refractory temporal lobe epilepsy who underwent phase-1 scalp video-EEG and phase-2 simultaneous scalp and intracranial video-EEG recordings for pre-surgical evaluation.ResultsOf the 192 temporal lobe seizures recorded during phase-1 and phase-2 scalp video-EEG studies, 124 (65%) seizures were associated with theta/alpha (5–9 Hz) ictal onset pattern, and 68 (35%) seizures were associated with delta (2–5 Hz) ictal onset pattern. Fourteen (52%) patients had exclusively theta/alpha ictal onset, 3 (11%) patients had exclusively delta ictal onset, and 10 (37%) patients had mixed theta/alpha and delta ictal onsets. MTLE was observed in 26 patients who had 124 seizures with theta/alpha ictal onset and 59 seizures with delta ictal onset. LTLE was observed in one patient who had 9 seizures with delta ictal onset. Scalp ictal EEG pattern was not significantly correlated with postsurgical seizure outcomes.ConclusionsBoth scalp delta and theta/alpha ictal onset patterns can be commonly found in patients with MTLE.SignificanceScalp delta ictal onset is not a unique EEG pattern for LTLE as commonly believed.     

Adult‐onset photosensitivity: clinical significance and epilepsy syndromes including idiopathic (possibly genetic) photosensitive occipital epilepsy

To evaluate the clinical associations of adult‐onset photosensitivity, we studied the clinical and EEG data of patients who were referred due to a possible first seizure and who had a photoparoxysmal response on their EEG. Patients with clinical evidence of photosensitivity before the age of 20 were excluded. Of a total of 30 patients, four had acute symptomatic seizures, two had vasovagal syncope, and 24 were diagnosed with epilepsy. Nine of the 24 patients had idiopathic (genetic) generalized epilepsies and predominantly generalized photoparoxysmal response, but also rare photically‐induced seizures, while 15 had exclusively, or almost exclusively, reflex photically‐induced occipital seizures with frequent secondary generalization and posterior photoparoxysmal response. Other important differences included a significantly older age at seizure onset and paucity of spontaneous interictal epileptic discharges in patients with photically‐induced occipital seizures; only a quarter of these had occasional occipital spikes, in contrast to the idiopathic (genetic) generalized epilepsy patients with typically generalized epileptic discharges. On the other hand, both groups shared a positive family history of epilepsy, common seizure threshold modulators (such as tiredness and sleep deprivation), normal neurological examination and MRI, a generally benign course, and good response to valproic acid. We demonstrated that photosensitivity can first occur in adult life and manifest, either as idiopathic (possibly genetic) photosensitive occipital epilepsy with secondary generalization or as an EEG, and less often, a clinical/EEG feature of idiopathic (genetic) generalized epilepsies. Identification of idiopathic photosensitive occipital epilepsy fills a diagnostic gap in adult first‐seizure epileptology and is clinically important because of its good response to antiepileptic drug treatment and fair prognosis.     

Epilepsies of neonatal onset: seizure type and evolution

Most neonatal seizures are occasional seizures and not true epilepsy. This study investigates seizure types of true neonatal epilepsies and their evolution with development. Seventy-five children with epilepsies of onset within 1 month of life, who were examined between 1970 and 1995, and whose seizure types could be confirmed with ictal EEG recordings, were studied. The patients were followed up for a minimum of 3 years and the evolution of epileptic syndromes was investigated. Sixty-three (84%) of 75 patients had partial seizures, while nine had generalized seizures, and only three had both generalized and partial seizures. Twenty-three of 24 neonates with benign familial or non-familial neonatal convulsions presented with partial seizures; these syndromes should not necessarily be categorized into generalized epilepsy as they are in the present International Classification. Age-dependent changes were a common feature of symptomatic neonatal epilepsies. Eighteen (41%) of 44 patients with symptomatic epilepsies of neonatal onset developed West syndrome in infancy. Fifteen (83%) of these 18 patients presented with symptomatic localization-related epilepsy in the neonatal period. In seven of these 15 patients, West syndrome was followed by localization-related epilepsy. Symptomatic localization-related epilepsy with transient West syndrome in infancy is another type of age-dependent epileptic syndrome.     

Diagnostic issues and treatment of cryptogenic or symptomatic generalized epilepsies

To clarify the diagnostic issues and treatment of patients with cryptogenic or symptomatic generalized epilepsies, not including West syndrome (WS), we investigated electroclinical change during the clinical course, and treatment effects in these patients. The selection criteria were minor generalized seizures as their main seizure type and diffuse epileptic discharges as their main EEG findings. Regarding EEG, we included EEGs that predominantly displayed multifocal spike-waves because of the inclusion of severe epilepsy with multiple independent spike foci (SE-MISF). We divided the subjects into two groups according to their main seizure types: Group A (54 patients) with brief tonic seizures and Group B (24 patients) with myoclonic seizures and/or atypical absences. The main epileptic syndromes were considered to be Lennox-Gastaut syndrome and SE-MISF in Group A, and epilepsy with myoclonic-astatic seizures in Group B. A history of WS was often seen in Group A, but it was exceptional in Group B. During the clinical course, seizure types did not basically change in Group A. EEG patterns were changeable in both groups. Although there was some overlap in electroclinical manifestations among epileptic syndromes, a transition between the two groups was not seen. High-dose valproate and ethosuximide were the most effective in Groups A and B, respectively. Long-term prognosis was significantly more favorable in Group B than in Group A.     

Ictal EEG patterns in band heterotopia

Band heterotopia (BH) or "double cortex" syndrome is a neuronal migration disorder resulting in a diffuse band of subcortical grey matter and variable abnormality of the overlying cortex. Patients with BH have a spectrum of psychomotor delay and seizures. Associated epileptic syndromes and interictal EEG findings have been described, but ictal EEG patterns are lacking. METHODS: We describe the clinical, interictal, and ictal EEG findings in two girls with BH and intractable seizures. RESULTS: Ictal EEG patterns correlated well with clinical seizure types, and did not have features unique to BH. Similarly, seizure behaviors and interictal EEG findings were typical of those seen in symptomatic generalized epilepsies. CONCLUSIONS: Despite evidence implicating the ectopic grey matter in seizure discharges, we conclude that seizure semiology and associated ictal EEG patterns in BH are no different from those seen in other causes of symptomatic generalized epilepsies.     

Thursday July 6, 2006Thursday July 6, 20067:30–9:00Hall 5ATeaching SessionEpilepsy surgery in infancy and childhood

¹ I. Tuxhorn (   ¹ Epilepsy Center Bethel, Germany )
Epilepsy surgery is no longer a treatment of last resort for children with drug resistant focal epilepsy. It should be considered early to reduce the negative impact of epilepsy on developmental and social outcome.
The spectrum of surgically remediable epilepsy syndromes of childhood has expanded in recent years to include 1) patients with tuberous sclerosis who have a leading resectable focus, 2) infantile spasms or Lennox-Gastaut syndrome (or other age specific epileptic encephalopathies) secondary to a resectable epileptogenic zone and 3) formerly "cryptogenic" cases with very subtle cortical dysplasias.
The goal of the presurgical evaluation is to identify patients who have surgically treatable epilepsy by assessing the risk benefit scenario of surgical intervention.
The accepted standards of evaluation include seizure analysis with video monitoring and scalp recording of the interictal and ictal EEG, high resolution MRI, neuropsychology, WADA test for assessing laterality of mnestic and language functions. Functional imaging including PET, ictal SPECT, postprocessing MRI techniques and fMRI are important techniques to evaluate difficult extratemporal cases.
The role of invasive neurophysiology with subdural, epidural and intracerebral recordings is changing since the advent of functional imaging of the epileptogenic region e.g., EEG triggered MRI and methods outlined above.
Patients with a focal electroclinical syndrome and a congruent MRI lesion are excellent candidates for selection and the seizure outcome prognosis may be considered high if the epileptogenic zone is completely resectable.
In pediatric epilepsy early patient referral for presurgical evaluation should be considered in specific syndromes—catastrophic focal epilepsy, hemispheric syndromes, Sturge Weber syndrome, and well localized symptomatic epilepsies.     

A study of 63 cases with eyelid myoclonia with or without absences: Type of seizure or an epileptic syndrome?

PurposeEyelid myoclonia and absences (EMA) induced by eye closure associated with brief, fast, and generalized paroxysms of polyspikes and waves was considered as an epileptic syndrome and a type of seizure as well. We analyzed the electroclinical features and evolution of EMA, and tried to determine if it represents a well-defined epileptic syndrome or a non-specific condition associated to other epilepsies.MethodsBetween June 1994 and June 2005, 63 patients who met diagnostic criteria of EMA were enrolled in the study and have been followed up to the present time.ResultsTwo main groups could be identified. The first group was divided into two subgroups. One subgroup of 28 patients presented EMA associated with infrequent generalized tonic–clonic seizures (GTCS), and the other 1 of 9 patients presented early-onset EMA refractory to antiepileptic drugs (AEDs), associated or not with GTCS and mental retardation. Four of them had self-induced seizures. The second group included 26 patients with EMA associated with GTCS and/or massive myoclonias, or GTCS induced by intermittent photic stimulation. All these patients had electroclinical features compatible with idiopathic generalized epilepsies.ConclusionIn the first group, EMA should be considered as a photosensitive idiopathic epileptic syndrome. A subgroup of early-onset of EMA refractory to AEDs, associated or not with GTCS and mental retardation should also be considered as a variant or a distinct photosensitive idiopathic epileptic syndrome. Finally, in the second group EMA may correspond to a type of seizures in idiopathic generalized epilepsies.     

Heart rate increase in otherwise subclinical seizures is different in temporal versus extratemporal seizure onset: support for temporal lobe autonomic influence.

Ictal heart rate was investigated in otherwise subclinical epileptic seizures to test the hypothesis as to whether ictal tachycardia is physiological and not a physical or psychological stress response. In addition, we aimed to evaluate the localizing significance of pure ictal tachycardia. We included 21 epilepsy patients, who showed an ictal EEG seizure pattern during 22, otherwise subclinical seizures. All patients underwent ictal video-EEG recordings to evaluate the possibility of resective epilepsy surgery. The changes in heart rate in these patients were investigated in order to determine their relationship to localization and duration of EEG seizure patterns. Ictal tachycardia was observed in 41% of the otherwise subclinical seizures (nine out of 22), and significantly more often in seizures arising from the temporal lobe than from extratemporal regions (62% versus 11%, p < 0.0018). The seizure duration as defined by EEG was significantly positively correlated with an increase of heart rate (p = 0.043). Ictal heart rate can increase as a result of epileptic activation of autonomic cortex, reflecting a temporal lobe autonomic influence. Thus, measurement of heart rate should be included in the evaluation of otherwise subclinical epileptic seizures, because of its localizing value.     

Benign partial epilepsy in infancy and early childhood with vertex spikes and waves during sleep: a new epileptic form

International epilepsy classification includes different epileptic syndromes with favourable outcomes in paediatric age, both partial and generalised. This is true in childhood while no partial benign forms are accepted in infancy. In 1987, Watanabe first described a new entity and he defined it as 'benign complex partial epilepsies in infancy'. In 1992, Vigevano referred similar but familial cases whose seizures had secondary generalisation. Both these forms had no interictal EEG abnormalities neither awake nor during sleep. This article presents a survey of 12 cases of partial epilepsy with favourable outcome differing from Watanabe and Vigevano's cases, both for the presence of interictal EEG abnormalities only during sleep and for seizure picture. All our patients are neurologically and neuroradiologically normal. Psychomotor development is unremarkable. Age onset range is 13-30 months. All cases present characteristic spikes and waves during slow-sleep in vertex cerebral areas. Awake EEG is always normal, at follow-up too. Our cases have such homogeneous electroclinical features as to hypothesise a new partial idiopathic epileptic syndrome with favourable outcome in infancy and early childhood. We propose to define it as 'benign partial epilepsy in infancy and early childhood with vertex spikes and waves' (BVSE).