Overlap cases of eyelid myoclonia with absences and juvenile myoclonic epilepsy.

Eyelid myoclonia with absences (EMA) and juvenile myoclonic epilepsy (JME) are two separate epileptic syndromes included in the new classification of epilepsies and epileptic syndromes by ILAE in 2001. Both are idiopathic generalized epilepsies with their clinical onset in the first two decades. EMA is characterized by eyelid myoclonia associated with absences and photosensitivity. Self-induced seizures are frequently seen in EMA. It can be associated with mildly mental retardation and resistance to treatment. JME includes three types of generalized seizures: typical absences, myoclonic jerks and generalized tonic-clonic seizures. The myoclonic jerks occur almost exclusively on awakening, involve preferently the upper extremities, may rarely affect the lower extremities or the entire body. More severe attacks may be accompanied by a fall. The myoclonic jerks occur rarely in EMA. They are usually mild and are freqently restricted to the upper extremities. Generalized tonic-clonic seizures, photosensitivity and generalized polyspike-wave discharges provoked by eye closure are features of both epileptic syndromes. In this study, we describe four female patients with eyelid myoclonia associated with absences, myoclonic jerks causing falling down and rare generalized tonic-clonic seizures. All patients had good school performance and total seizure control under sodium valproate treatment. Their EEGs show generalized polyspike-wave discharges with a frequency of 3.5-6Hz always appearing a few seconds after eye closure and photoparoxysmal response. These patients show the characterictics of both epileptic syndromes. It is clinically important to make a syndromic diagnosis for an optimum advise on treatment, lifestyle restrictions and prognosis. In this study, we have gathered evidence that EMA and JME are dynamic syndromes that tend to evolve into one another.     

Epileptic seizures difficult to differentiate from alternating hemiplegia in infants: a case report

A child with epileptic seizures had a clinical course similar to that of alternating hemiplegia of infancy (AHI). Tonic hemiplegia began at 2 months of age, and atonic alternating hemiplegia and tetraplegic attacks began at 2 years of age. Clinical findings were paroxysmal ocular movement abnormalities, choreoathetotic involuntary movements, and severe developmental retardation. An interictal EEG at 6 years of age showed multiple independent spike discharges. An ictal EEG showed diffuse irregular spike-wave and slow wave bursts following focal spikes. The present case suggests that a long-term extensive follow-up is necessary to differentiate epileptic seizures from AHI.     

Epileptic spasms without hypsarrhythmia showing astatic seizures in clusters

It is difficult to differentiate astatic seizures among atonic, myoclonic and tonic seizures without the help of ictal polygraphic recordings. We described a girl with epilepsy presenting periodic astasia caused by epileptic spasms. Her seizures had occurred in clusters since 1-year and 7-month of age. Her interictal electroencephalogram (EEG) showed intermittent diffuse (poly) spike (s) and wave discharges, without a trace of hypsarrhythmia. She had a diagnosis of epilepsy with myoclonic-astatic seizures at a previous hospital, and her seizures had been resistant to multiple antiepileptic agents. After she was referred to our hospital, the ictal video-EEG recordings were made before and after ACTH administration, which revealed that her astatic seizures were epileptic spasms with presumed cortical origin. We should carefully evaluate the astatic seizures during early childhood which may be an atypical presentation of epileptic spasms.     

Landau–Kleffner syndrome: A study of 29 patients

PurposeThe aim of the study was to retrospectively analyze the electroclinical features, etiology, treatment, and prognosis of 29 patients with Landau–Kleffner syndrome (LKS) with a long-term follow-up.MethodsInclusion criteria were a diagnosis of LKS with: (1) acquired aphasia or verbal auditory aphasia; (2) with or without focal seizures, secondarily generalized tonic-clonic seizures, absences, or atonic seizures.ResultsMean follow-up was 12 years. All cases except six had seizures. Before the onset of aphasia, developmental language and behavioral disturbances were present in 19 and 14 patients, respectively. All patients had verbal auditory agnosia. Aphasia was severe in 24 patients and moderate in five. Nonlinguistic cognitive dysfunctions were moderate in 14 patients. Behavioral disturbances were found in 16 patients. During the continuous spike-and-wave discharges during slow sleep phase, the spike-wave index was >85% in 15, 50–85% in eight, and 30–50% in four. In two patients, the EEG recording showed occasional bilateral spikes, without continuous spike-and-wave discharges during slow sleep. In this phase, the awake EEG recording showed more frequent interictal abnormalities, predominantly in the temporal regions. Eight patients recovered language completely, but the remaining patients continue to have language deficits of different degrees.ConclusionLandau–Kleffner syndrome is an epileptic encephalopathy characterized by acquired verbal auditory aphasia and seizures in most of the patients associated with continuous or almost continuous spike-and-wave discharges during slow wave sleep. The most commonly used treatments were clobazam, ethosuximide, sulthiame. High-dose steroids were also administered. Adequate and early management may avoid language and cognitive deterioration.     

Seizure outcome after corpus callosotomy: the Taiwan experience

From September 1989 to August 1996, we performed anterior corpus callosotomy in 83 patients. Unfortunately, 9 patients were lost to follow-up. Among the remaining 74 patients, 59 had Lennox-Gastaut syndrome (evolved from infantile spasms in 22), 9 had complex partial seizures with or without secondary generalized seizures, 1 had multifocal independent epileptogenic foci (MISF) syndrome, 3 had hemiconvulsion-hemiplegia-epilepsy (HHE), and 2 had infantile spasms. All cases were followed up for at least 2 years after surgery. The highest rate of significant improvement (more than 50% reduction in seizure frequency) was noted in the patients with generalized tonic-clonic seizures, 82.1% of whom experienced significant improvement, followed by those with generalized tonic seizures (76.7%), atonic seizures (72.7%), myoclonic seizures (64.9%), atypical absences (58.6%), and complex partial seizure with or without secondary generalization (61.5%). Complete freedom from seizures was noted in 14 cases (18.9%). One patient had the anterior half of his right palm amputated following radial artery thrombosis complicated by insertion of an arterial line during anesthesia. Otherwise, there were no major postoperative complications except for brief mutism and multifocal jerks in some patients during the 1st postoperative week. Thus, we conclude that corpus callosotomy is a safe alternative treatment for all kinds of medically intractable seizures, especially generalized epilepsy. Received: 23 June 1999     

Epileptiform EEG Activity of the Centromedian Thalamic Nuclei in Children with Intractable Generalized Seizures of the Lennox-Gastaut Syndrome

Centromedian thalamic nuclei (CM) epileptiform EEG activities were recorded in children with intractable generalized seizures of Lennox-Gastaut syndrome (LGS) through implanted recording-stimulating electrodes used for seizure control. Ictal CM epileptiform activities were consistently correlated to widespread surface cortical EEG activities and symptoms in all patients and all types of generalized seizures; i.e., fast spike discharges at CM correlated at onset of tonic and tonic-clonic generalized seizures; slow (1-2 Hz) spike-wave complex discharges at CM correlated for atypical absence seizures; slow polyspike-wave complex discharges correlated for myoclonic seizures; and spike bursts and suppression patterns correlated for combined tonic-atonic-myoclonic seizures. Ictal EEG activities occurred simultaneously at right and left CM and surface at onset of all seizure types, with the exception of myoclonic seizures where CM complete discharges and individual spike-wave complexes significantly lead those of the surface. Brief tonic-atonic spasms clinically undistinguishable from "real" epileptic seizures showed no EEG counterparts at CM and surface. Interictal CM spike-wave complete discharges and individual spike-wave complexes showed variable amplitude-temporal patterns. Amplitude emphasis on CM and frontopolar regions was observed in most of complete discharges, however, and phase shifts between CM and frontopolar regions were observed in individual spike-wave complexes.     

The New Classification of Seizures by the International League Against Epilepsy 2017

Purpose of Review

This review presents the newly developed International League Against Epilepsy (ILAE) 2017 classification of seizure types.

Recent Findings

The fundamental distinction is between seizures that begin focally in one hemisphere of the brain, generalized onset seizures that apparently originate in both hemispheres, and seizures of unknown onset. Focal seizures optionally can be subclassified according to whether awareness (a surrogate marker for consciousness) is intact or impaired. The next level of classification for focal seizures is motor (with subgroups automatisms, atonic, clonic, epileptic spasms, hyperkinetic, myoclonic, tonic), non-motor (with subgroups autonomic, behavior arrest, cognitive, emotional, sensory), and focal to bilateral tonic-clonic. Generalized seizures are categorized as motor (tonic-clonic, clonic, tonic, myoclonic, myoclonic-tonic-clonic, myoclonic-atonic, atonic, epileptic spasms) and non-motor/absence (typical, atypical, myoclonic, eyelid myoclonia).

Summary

The classification allows new types of focal seizures and a few new generalized seizures, and clarifies terms used to name seizures.

     

Delineation of cryptogenic Lennox-Gastaut syndrome and myoclonic astatic epilepsy using multiple correspondence analysis.

PURPOSE: To distinguish various types of childhood severe cryptogenic/idiopathic generalised epilepsy on the basis of reproducible diagnostic criteria, using multiple correspondence analysis (MCA). METHODS: We applied MCA to a series of 72 children with no evidence of brain damage, starting epilepsy between 1 and 10 years, with two or more types of generalised seizures. We excluded patients with infantile spasms or typical absences. MCA was performed on all clinical and EEG parameters, first throughout follow-up, then restricted to the first year of the disease. RESULTS: When including all follow-up variables, there were three groups: (1) Thirty-seven children with male predominance, familial history of epilepsy, simple febrile convulsions, massive myoclonus, tonic-clonic fits. Outcome was favourable, with no seizures and mildly affected cognitive functions. Interictal EEG showed short sequences of irregular 3-Hz spike-waves. (2) In 18 children, clinical characteristics were similar to those of the first group at the early stage, but 95% exhibited myoclonic status and vibratory tonic seizures, with persisting seizures on follow-up. EEG showed long sequences of generalised irregular spike and slow waves. Those two groups meet the characteristics of childhood onset myoclonic-astatic epilepsy (MAE) with respectively, favourable and unfavourable outcome. (3) Eleven children had later onset, atypical absences, tonic and partial seizures, and no myoclonus, or vibratory tonic seizures. All had mental retardation and persisting seizures. EEG showed long sequences of slow spike-wave activity and half the patients had spike and slow wave foci. These patients met the major characteristics of Lennox-Gastaut syndrome. Initial parameters failed to distinguish the first two groups, but Lennox-Gastaut syndrome (the third group) was distinct from both groups of myoclonic astatic epilepsy from the onset. Within MAE groups combined, clinical and EEG risk factors for mental retardation could be identified. CONCLUSION: It is possible to validate statistically the distinction between discrete epileptic syndromes. Myoclonic astatic epilepsy is therefore distinct from Lennox-Gastaut syndrome, and the distinction appears from the first year of the disorder.     

Childhood absence epilepsy: Elctroclinical features and diagnostic criteria

Objective: To analyze the electroclinical features of children with childhood absence epilepsy (CAE) and discuss the diagnostic criteria for CAE. Methods: The video-electroencephalogram (VEEG) database in our hospital was searched using “absence seizures” and “3-Hz generalized spike and waves (GSW)” as key-words. Other epileptic syndromes with typical absence seizures were carefully excluded. Children meeting the CAE diagnostic criteria of the International League Against Epilepsy (ILAE) in 1989 were further evaluated with the diagnostic criteria proposed by Panayiotopoulos in 2005. Results: Totally 37 children met the 1989 ILAE criteria of CAE. The onset age of absence seizures ranged from 3 to 11 years. All patients had frequent absence seizures (5-60 times per day). Two patients (5.4%) had generalized tonic-clonic seizures. Hyperventilation induced absences in all patients. VEEG confirmed that 7 patients (18.9%) had only simple absences, 25 patients (67.6%) had only complex absences, and 5 patients (13.5%) had both simple and complex absences. Ictal EEG showed 3 Hz GSW discharges in all patients. The seizure duration ranged from 3 to 40 s. Four patients (10.8%) had two spikes per wave in ictal EEG. GSW fragments were found in 29 patients (78.4%) during sleep. Interictal polyspikes and waves were present in 17 patients (45.9%). Focal discharges predominantly in the anterior regions, were found in 22 patients (56.8%). Only 7 patients (18.9%) met the diagnostic criteria proposed by Panayiotopoulos in 2005. Conclusions: Few patients meeting the 1989 ILAE diagnostic criteria for CAE meet the new diagnostic criteria proposed by Panayiotopoulos in 2005. The new criteria for CAE are too strict to appropriately classify some patients.     

Clinical and electrographic features of epileptic spasms persisting beyond the second year of life

PURPOSE: Few reports detailing the electroclinical features of epileptic spasms persisting beyond infancy have been published. We sought to characterize this unique population further. METHODS: We retrospectively reviewed the clinical and video-EEG data on 26 patients (4-17 years; mean, 93 months) with a confirmed diagnosis of epileptic spasms and who were evaluated at our tertiary referral center between 1993 and 2000. RESULTS: In half of our cases, epileptic spasms were associated with disorders of neuronal migration, severe perinatal asphyxia, and genetic anomalies. Interictal EEGs showed generalized slowing in the majority of patients, and a slow-wave transient followed by an attenuation of the background amplitude was the most common ictal EEG pattern associated with an epileptic spasm (19 cases). Other seizure types (number of cases in parentheses) included tonic seizures with or without a preceding spasm (13), partial seizures (11), myoclonic seizures (11), generalized tonic-clonic seizures (six), atypical absence seizures (two), and atonic seizures (one). Cases with a more organized EEG background (especially with frequencies > or =7 Hz) were more likely to have better cognition. Continued disorganization of the EEG background and persistence of hypsarrhythmia were associated with poor developmental outcome. CONCLUSIONS: Patients with epileptic spasms persisting beyond age 2 years constitute a truly refractory population, one that should be better recognized by clinicians. Interestingly, although many therapies resulted in a >50% reduction in seizures, neither neurocognitive function nor quality of life was substantially improved with intervention. The interictal EEG background is the most helpful in predicting neurologic outcome.     

Absences in juvenile myoclonic epilepsy: a clinical and video-electroencephalographic study

We report a prospective clinical and electroencephalographic study of 19 patients with juvenile myoclonic epilepsy and absence seizures. Absences began 1 to 9 (4.5 +/- 2.5) years before myoclonic jerks and generalized tonic-clonic seizures. Clinical manifestations during the absence ictus showed great variation, ranging from subtle or no overt features to severe impairment of consciousness, and severity was age related. Simple and complex absence seizures can occur in the same patient. The electroencephalographic features were distinct, with many interictal discharges, fragmentation of the paroxysms, and frequent polyspikes of varying numbers and amplitude for each spike-slow wave component. The combined clinical-electroencephalographic manifestations were characteristic and allow differentiation of absences in juvenile myoclonic epilepsy from typical absence seizures in other epileptic syndromes.     

Epilepsy With Myoclonic Atonic Seizures: An Electroclinical Study of 69 Patients

Epilepsy with myoclonic-atonic seizures is characterized by myoclonic-atonic, absence, tonic-clonic, and eventually tonic seizures, appearing in previously normal children at ages 18-60 months. We analyzed the electroclinical features, treatment, and outcome of 69 patients with myoclonic-atonic seizures; these patients were followed between 1990 and 2012 at the Juan P. Garrahan Pediatric Hospital, Buenos Aires, Argentina. No structural or metabolic etiology was identified. Based on the electroclinical features and evolution, two groups could be distinguished. The first group of 39 patients with myoclonic and myoclonic-atonic seizures with or without generalized tonic-clonic seizures and absences associated with generalized spike- and polyspike-and-wave paroxysms had excellent prognoses. The second group of 30 patients had myoclonic jerks and myoclonic-atonic seizures associated with other seizure types including tonic seizures; some had myoclonic status epilepticus and cognitive deterioration. The interictal EEG showed frequent generalized spike- and polyspike-and-wave paroxysms. In 16 patients, the seizures remitted within 3.6 years. The two groups were distinguished in retrospect, when enough time had elapsed to evaluate cognitive deterioration and different seizure types. In conclusion, epilepsy with myoclonic atonic seizures is an epileptic syndrome with a broad clinical spectrum and variable prognosis.     

Case of subacute sclerosing panencephalitis with atypical absences and myoclonic-atonic seizures as a first symptom

The 2-year history of a 10-year-old boy with subacute sclerosing panencephalitis is presented. After 6 months of epilepsy manifested by atypical absences and myoclonic-atonic seizures with an electroencephalographic (EEG) correlation of generalized spike-and polyspike-wave discharges, the child developed chorioretinitis and demonstrated a dramatic intellectual decline during corticosteroid treatment. Neuroimaging investigations did not demonstrate any pathologic changes, including at the time of fully developed disease, with neurologic deficits, periodic spasms, polymorphic epileptic seizures, and dementia. The typical generalized periodic complexes and general slowing replaced the epileptic abnormalities in the EEG. The initial anticonvulsant treatment was temporarily effective, and the purposeful isoprinosine therapy had no significant beneficial effect. The subacute sclerosing panencephalitis rapidly developed to stages III to IV, and only during the vegetative state did computed tomography show marked brain atrophy. This case is among the few described in the literature with atypical absences and myoclonic-atonic seizures as the first manifestation of subacute sclerosing panencephalitis.     

The Axial Spasm—The Predominant Type of Drop Seizure in Patients with Secondary Generalized Epilepsy

In 45 patients suffering from secondary generalized epilepsy, we have recorded 239 drop seizures both by radio-telemetered EEG/split-screen video taping and polygraphically. Twenty of these patients had pure tonic drop seizures. Nine patients each had myoclonic-atonic or pure atonic seizures, whereas seven had slow falls that occurred during gradually developing tonic or akinetic seizures. We term the pure tonic drop seizure "axial spasm." It consists of a uniform pattern of movement lasting from 0.5 to 0.8 s and leading to a specific bearing characterized by a moderate flexion of the hips, the upper trunk, and the head. The arms are almost always involved being abducted, elevated and in a semiflexed position. A fall is provoked by the rapidity and violence of the flexion in the hips. The spasms can occur independently (pure axial spasm) or in connection with other seizure manifestations (combined axial spasm). In pure spasms, no disturbance of consciousness and no significant EEG changes--apart from a slight attentuation--are seen. In the combined axial spasms, distinct epileptic phenomena, in close connection with the axial spasms, can be observed, appearing either in the postspasm phase or in both the pre- and the afterphases. The prephase consists always of an absence and spike-and-wave activity. The onset of the spasm coincides with the last generalized spike, which is followed by a biphasic high-voltage slow wave. In the afterphases, various seizure patterns can be seen; quite characteristic are arrest states during which the patients are completely immobile and take up a postural-like or athetoid position. Almost as frequent are absences or absence-like states and tonic seizures. The afterphase can be compounded by two or three of these seizure patterns, the most common sequence of events in these cases being tonic--arrest state--absence. From the clinical and neurophysiological points of view, axial spasms can be regarded as a more mature form of infantile spasms. Clinical and experimental findings suppose a brainstem origin.     

A case of Panayiotopoulos syndrome showing an atypical course

We describe herein a patient with Panayiotopoulos syndrome (PS) showing an atypical course. The patient initially had seizures typical of this syndrome from 3 to 5 years of age. EEG showed right occipital high-amplitude sharp and slow-wave complexes followed by brief generalized discharges of slow waves. Sequential EEGs obtained from 5 to 11 years of age showed both multifocal discharges and generalized spike and wave complexes. With these changes in EEG findings, the patient experienced various types of seizures. The seizures were frequent and showed oculocephalic deviation followed by absence, atonic seizures, generalized tonic clonic convulsions and clonic seizures of the eyelids, which were observed between 7 and 10 years of age. Antiepileptic drugs were only partially effective for these seizures. Ictal EEG recorded at 8 years of age revealed high-voltage slow waves from the bilateral frontal and occipital regions prior to diffuse high-amplitude spike-wave bursts. At 9 years of age, magnetoencephalography (MEG) revealed the calculated dipoles of the preceding bifrontal spike-wave discharges to be in the frontal areas, while those of the following generalized spike-wave bursts were in the bilateral mid-temporal areas. In PS, reportedly, dipoles of multifocal epileptic discharges are usually located in the occipital and Rolandic areas. The unique clinical evolution in our case may be associated with the unusual frontal localization of dipoles detected by MEG.     

Eight years follow-up of a generalized epilepsy patient with eating-induced late-onset epileptic spasms and atypical absence with myoclonic jerks

PurposeEating epilepsy was previously known as a kind of focal reflex epilepsy. However, the development of eating-induced multiple generalized seizures and the associated EEG changes were rarely reported. Herein, we present a 13-year-old generalized epilepsy patient with eating-induced generalized seizures since the age of 5.Case presentationThe 13-year-old male patient had suffered from late-onset eating-induced epileptic spasms during the meal since the age of 5. Meanwhile, he also experienced spontaneous epileptic spasms during the period of sleep. The seizure frequency and type gradually increased from 7 years of age. In addition to epileptic spasms, he started experiencing atypical absence with myoclonic jerks during the meal. Ictal EEG presented as the appearance of an irregular slow-wave mixed with generalized polyspike wave with the intake of food, and gradually evolved to bursts of generalized polyspike wave complexes. At the end of the meal, the EEG returned to normal. Nevertheless, at the age of 13, his seizure frequency increased and appeared new seizure type, and besides epileptic spasm and atypical absence, he began to experience myoclonic seizure during sleep and awaking-generalized tonic-clonic seizure in the morning. In this period he started taking valproic acid, topiramate and clonazepam, and his seizure frequency was reduced.ConclusionIn conclusion, this case demonstrated the variability of eating induced multiple generalized seizure types, and eight years follow-up also indicates that generalized epilepsy progressed with age. The EEG and clinical changes of our patient contribute to a better understanding of the electro-clinical features of eating-induced multiple generalized seizures and the course of generalized epilepsy with such seizures.     

Video-EEG Analysis of Drop Seizures in Myoclonic Astatic Epilepsy of Early Childhood (Doose Syndrome)

We studied 36 drop seizures in 5 patients with myoclonic astatic epilepsy of early childhood (MAEE) with simultaneous split-screen video recording and polygraph. Sixteen were falling attacks and 20 were either less severe attacks exhibiting only deep head nodding or seizures equivalent to drop attacks in terms of ictal pattern but recorded in the supine position. All seizures except those that occurred in patients in the supine position showed sudden momentary head dropping or collapse of the whole body downward. Recovery to the preictal position was observed in 0.3-1 s. As a result of carefully repeated observations, the 36 seizures were classified as myoclonic flexor type in 9, myoclonic atonic type in 2, and atonic type, with and without transient preceding symptoms in the remaining 25. The MF seizure was characterized by sudden forward flexion of the head and trunk as well as both arms, which caused the patient to fall. In the myoclonic atonic seizure, patients showed brief myoclonic flexor spasms, immediately followed by atonic falling. The AT seizure showed abrupt atonic falling, with and without transient preceding facial expression change and/or twitching of extremities. The ictal EEGs of all 36 seizures exhibited generalized bilaterally synchronous single or multiple spike(s) and wave discharges. Atonic drop attacks appear to be a common cause of ictal epileptic falling in MAEE.     

Generalized epilepsy with febrile seizures plus: further heterogeneity in a large family

BACKGROUND: Generalized epilepsy with febrile seizures plus (GEFS(+)) is a recently described benign childhood-onset epileptic syndrome with autosomal dominant inheritance. The most common phenotypes are febrile seizures (FS) often with accessory afebrile generalized tonic-clonic seizures (GTCS, FS(+)). In about one third, additional seizure types occur, such as absences, myoclonic, or atonic seizures. So far, three mutations within genes encoding subunits of neuronal voltage-gated Na(+) channels have been found in GEFS(+) families, one in SCN1B (beta(1)-subunit) and two in SCN1A (alpha-subunit). METHODS: The authors examined the phenotypic variability of GEFS(+) in a five-generation German family with 18 affected individuals. Genetic linkage analysis was performed to exclude candidate loci. RESULTS: Inheritance was autosomal dominant with a penetrance of about 80%. A variety of epilepsy phenotypes occurred predominantly during childhood. Only four individuals showed the FS or FS(+) phenotype. The others presented with different combinations of GTCS, tonic seizures, atonic seizures, and absences, only in part associated with fever. The age at onset was 2.8 +/- 1.3 years. Interictal EEG recordings showed rare, 1- to 2-second-long generalized, irregular spike-and-wave discharges of 2.5 to 5 Hz in eight cases and additional focal parietal discharges in one case. Linkage analysis excluded the previously described loci on chromosomes 2q21-33 and 19q13. All other chromosomal regions containing known genes encoding neuronal Na(+) channel subunits on chromosomes 3p21-24, 11q23, and 12q13 and described loci for febrile convulsions on chromosomes 5q14-15, 8q13-21, and 19p13.3 were also excluded. CONCLUSION: These results indicate further clinical and genetic heterogeneity in GEFS(+).     

Long-term prognosis in two forms of childhood epilepsy: typical absence seizures and epilepsy with rolandic (centrotemporal) EEG foci

Findings in children seen between 1955 and 1965 during the year of onset of typical absence seizures (90 patients) or rolandic epilepsy (79 patients) were analyzed by actuarial methods. One hundred and eighteen patients were followed for more than 15 years. Rolandic epilepsy is a true benign epilepsy ending with puberty. Although school and family problems are common during the acute stage of the disease, the social adaptability of such patients is excellent. We considered only typical absences occurring as a first epileptic sign in normal children. Myoclonic or atonic absences have a poor prognosis. Many patients with simple and automatic absences experience remission 15 years after withdrawal of medication. The overall cessation rate in those experiencing absences was only 57.5%, however, and 36% of patients developed tonic-clonic seizures. Social adaptability was often inadequate. Simple and automatic absences (constituting a homogeneous group) are not truly a benign form of epilepsy, even though prognosis for those afflicted is better than that for those with other forms of primary generalized epilepsy.