Precipitating factors and therapeutic outcome in epilepsy with generalized tonic-clonic seizures

OBJECTIVES: The aim of the study was to evaluate the influence of precipitating factors and therapy on the outcome of epilepsy with generalized tonic-clonic seizures. PATIENTS AND METHODS: Retrospective analysis of data from 34 patients (mean age at seizure onset 19 years; mean duration of follow-up 9.2 years) suffering from epilepsy of either cryptogenic or remote symptomatic (n = 19), or idiopathic (n = 15) etiology. The total number of seizures in all patients was 146. RESULTS: Without treatment 97 seizures manifested during 90.5 years without treatment (1.07 seizures/year), during treatment with carbamazepine or valproate 49 seizures occurred within 224 years (0.2 seizures/year). The frequency of seizures was significantly lower during treatment. Precipitating factors were found in relation to 31% of seizures in patients with remote symptomatic or cryptogenic epilepsy, and for 51% of seizures in patients with idiopathic epilepsy. CONCLUSIONS: There was a low frequency of seizures in patients with generalized tonic-clonic seizures. Precipitating factors are common. Antiepileptic drug treatment is effective.     

Clinical analysis of childhood occipital lobe epilepsy in 43 Taiwanese patients

Occipital lobe epilepsy in children can present as an idiopathic form (i.e., childhood epilepsy with occipital paroxysms) or as a symptomatic form. Forty-three children (18 boys, 25 girls) were divided into the idiopathic group or symptomatic group, according to the classification for epileptic seizures of the International League Against Epilepsy. Patients in the idiopathic group were further subdivided into the Panayiotopoulos or Gastaut type, according to clinical presentation. The idiopathic group consisted of 15 children (5 boys, 10 girls), of whom 11 were of the Panayiotopoulos type and 4 of the Gastaut type. The symptomatic group consisted of 28 children (13 boys, 15 girls). The average age of seizure onset in the idiopathic group was younger than in the symptomatic group (6.5 +/- 2.4 vs 8.5 +/- 3.0 years). Ictal vomiting was more common in the idiopathic group, and positive visual symptoms were more common in the symptomatic group. Mean epilepsy duration in the idiopathic group was shorter (5.7 +/- 5.3 vs 20.1 +/- 16.0 months), and the response to treatment was better. The average age of seizure onset was much younger in the Panayiotopoulos than in the Gastaut type (5.4 +/- 1.5 vs 9.5 +/- 1.5 years), and mean epilepsy duration was also shorter (3.9 +/- 4.2 vs 10.5 +/- 4.9 months). Seizure semiology can distinguish between idiopathic occipital lobe epilepsy and the symptomatic form with ictal vomiting and positive visual symptoms. In idiopathic occipital lobe epilepsy, the Panayiotopoulos type has better prognosis than the Gastaut type.     

Seizure control and educational outcome in childhood-onset epilepsy.

Patients with epilepsy are more prone to have learning disabilities. This study investigated the therapeutic and educational outcome of 102 epileptic children. Analyzed data included age at onset, etiology, presence of underlying brain lesions, seizure type, and electroencephalographic (EEG) patterns. Cryptogenic seizures, remote symptomatic seizures, and underlying brain lesions were found in 29, 26, and 14 patients, respectively, whereas 47 patients had idiopathic seizures. Eighty-three patients achieved seizure control (46 remained seizure free), and 19 patients remained poorly controlled. Sixty-five patients were in regular schools, and 37 required special education (17 with mental retardation). Predictors for poor seizure control were remote symptomatic seizures, underlying brain lesions, and (when grouped together) hypsarrhythmia and mixed EEG patterns (P < .001). Predictors for special education needs were young age at onset, remote symptomatic seizures, underlying brain lesions, hypsarrhythmia and mixed EEG patterns, and poor seizure control (P < .001). We conclude that in childhood epilepsy, the need for special education is substantial and more common than treatment failure.     

Discontinuing antiepileptic drugs in children with epilepsy: A prospective study

In a prospective study, antiepileptic drugs were discontinued in 264 children with epilepsy after a mean seizure-free interval of 2.9 years. They were then followed for a mean of 58 months to ascertain whether seizures recurred. Seizures recurred in 95 (36%) of the children. Etiology was a significant predictor of outcome (relative risk [RR] = 1.81). On multivariable analysis, significant factors in the idiopathic group included age at onset above 12 years (RR = 5.4), a family history of seizures (RR = 3.1), the presence of slowing on the electroencephalogram prior to medication withdrawal (RR = 2.4), and a history of atypical febrile seizures (RR = 2.8). Specific epileptic syndromes such as juvenile myoclonic epilepsy and benign rolandic epilepsy were also significant predictors of outcome. In the remote symptomatic group, significant predictors of outcome included age at onset older than 12 years (RR = 3.6), moderate to severe mental retardation (IQ < 50) (RR = 2.8), a history of atypical febrile seizures (RR = 2.0), and a history of absence seizures (RR = 0.4). The majority of children with epilepsy in remission while on antiepileptic drug therapy will remain seizure free when medications are withdrawn. A few readily available parameters distinguish those with a good prognosis from those in whom seizures are likely to recur. These data provide the framework for the clinical decision making for withdrawal of medications in these children.     

Epilepsy in the Elderly

Summary: Purpose : The aim of this study was to evaluate the clinical characteristics of elderly patients with epilepsy.
Methods : We retrospectively reviewed the clinical records of 190 patients (104 males and 86 females) aged 60 years or older at the time of study.
Results : Epilepsies were classified as generalized in 33 patients (17.4%), partial in 145 (76.3%), and undetermined in 12 (6.3%). Twenty-nine of 33 patients with generalized epilepsy were idiopathic, whereas all patients with partial epilepsy were symptomatic. Symptomatic partial epilepsy (SPE) began at all ages (2 to 81 years). Patients with early onset (< 20 years) showed the most unfavorable course in both seizure control and social adaptability. Patients with late onset (50 years or older) had no family history of epilepsy, and half of them had a past history of ccrebrovascular disease or head injury as a presumed etiology. In patients with idiopathie generalized epilepsy (IGE), 25 of 29 had early onset, and a family history of epilepsy was found in 31%. Nineteen patients continued to have seizures after 50 years of age, albeit infrequently. Furthermore, 10 of them showed exacerbation around the age of 50.
Conclusions : Most of the late onset epilepsies were SPE with a relatively good prognosis. General belief has held that seizure outcome in IGE is favorable, but some IGE patients show an increased seizure propensity in old age.     

Autism and epilepsy: a retrospective follow-up study

So-called "idiopathic" autism, which exhibited no major complications before diagnosis is well-known as one of the risk factors for epilepsy. This retrospective follow-up study aimed to clarify the characteristics of epilepsy in the autism; onset of seizure, seizure types, EEG findings and epilepsy outcome and the differences as a group between the autism with epilepsy and those without epilepsy. One hundred thirty individuals with autistic disorder or atypical autism diagnosed in childhood were followed up over 10 years and were evaluated almost every year up to 18-35 years of age. Their medical records related to perinatal conditions, IQ, social maturity scores and several factors of epilepsy were reviewed in October 2005. Thirty-three of the follow-up group (25%) exhibited epileptic seizures. The onset of epilepsy was distributed from 8 to 26 years of age. Two types of seizure were observed; partial seizure with secondarily generalized seizure and generalized seizure. Twenty of the epileptics (61%) showed the partial seizure. Although 18% of the non-epileptic group exhibited epileptic discharges on EEG, 68% of the epileptic group revealed epileptiform EEG findings before the onset of epilepsy. No differences were observed concerning the sex ratio, autistic disorder/atypical autism and past history of febrile seizures between the epileptic and non-epileptic groups. Lower IQ, lower social maturity score and higher frequency of prescribed psychotropics were observed in the epileptic group compared to the non-epileptics. Idiopathic autism was confirmed as the high risk factor for epilepsy. Epileptiform EEG findings predict subsequent onset of epileptic seizures in adolescence. Epilepsy is one of negative factors on cognitive, adaptive and behavioral/emotional outcomes for individuals with autism.     

Possibilities of non-pharmacological conservative treatment of epilepsy.

This study set out to assess the effect of non-pharmacological conservative (NPC) interventions as alternatives to antiepileptic pharmacotherapy. A prospective follow-up cohort study was conducted in an outpatient seizure clinic of a referral center for epilepsy. Twenty-five patients (nine males, 16 females) aged 16-45, with at least two well-described epileptic seizures, were included who had rejected antiepileptic pharmacotherapy. Twelve had idiopathic generalized epilepsy, 11 had symptomatic or cryptogenic localization-related epilepsy, and two had epilepsy with generalized and focal signs. Twenty-three of the patients were followed for more than 2 years. The patients were treated with arrest after focal seizure onset (2 cases), sensory protection against reflex seizures (3 cases), avoidance of non-specific seizure-precipitating factors ('life hygiene', 16 cases), and/or miscellaneous interventions (8 cases). The main outcome measures were complete seizure control (more than 2 years) or sufficient improvement to continue with NPC treatment alone. Eight of the 23 patients were completely seizure free for more than 4 years, and three were sufficiently improved to continue NPC treatment without drugs. Trends were observed for patients with idiopathic generalized epilepsies with less than seven convulsive seizures, and with only one seizure type to respond better to NPC treatment. The duration of epilepsy, and the finding of generalized epileptiform discharge in the EEG had no influence on the outcome. Rational NPC treatments which are aimed at specific factors in the precipitation and development of epileptic seizures can be useful therapeutic alternatives for patients with milder forms of epilepsy. Apart from photosensitive patients, those most likely to profit are patients with idiopathic generalized epilepsy, a maximum of six generalized tonic-clonic seizures which were precipitated by lack of sleep or excessive alcohol intake, and with no or rare concomitant absences. In such cases, NPC treatment may be as effective as pharmacotherapy and gives the patient a positive experience of regained self-control.     

Partial Epilepsies in Infancy: A Study of 40 Cases

Forty patients with partial epilepsy that began before they were aged 3 years were recorded at the Centre Saint-Paul between 1981 and 1986 with a follow-up ranging from 1 year 9 months to 20 years. We analyzed the following data: age at onset, clinical features of seizures at onset and during the follow-up period, ictal and interictal EEG features, etiologic circumstances, evolution of the epilepsy, and psychomotor development. The age of onset was mostly between 2 months and 2 years (more than two thirds of cases). Most had partial symptomatic epilepsy. In nine cases, epilepsy was preceded by febrile convulsions. Seizures at onset were of the following type (in order of decreasing occurrence): unilateral seizures, complex partial seizures, elementary partial seizures, and other seizures, often difficult to classify. A few patients with infantile spasms associated with focal or multifocal EEG abnormalities, differing from West's syndrome, were included in this study. We discuss the problem arising from the classification of infantile seizures and epilepsies.     

Characteristics of epilepsy occurring in the first four months

Introduction: Epilepsies with an onset during the early infantile period are relatively rare and their characteristics are not well recognized. The aim of this study was to determine the clinical characteristics of epilepsies with an onset during the early infantile period. Methods: Clinical information on 73 patients with the onset of epilepsy within the first four months was collected from hospitals affiliated with Nagoya University. Patients were categorized into three groups: the idiopathic (20 patients), cryptogenic (19 patients), and symptomatic groups (34 patients). Results: Fourteen (70%) of the 20 patients in the idiopathic group, nine (47%) of the 19 patients in the cryptogenic group, and 10 (29%) of the 34 patients in the symptomatic group had their first seizure within the first month of life. All patients in the idiopathic group, 12 patients (63%) in the cryptogenic group, and 18 patients (53%) in the symptomatic group had partial seizures (PS) alone throughout their clinical course. Four patients in the cryptogenic group and nine in the symptomatic group had PS at the onset, but evolved into spasms later. All patients in the idiopathic group, 13 patients (68%) in the cryptogenic group, and 13 patients (38%) in symptomatic group had experienced no seizures for at least one year at the time of the last follow-up. Conclusions: In patients with non-idiopathic epilepsy, an age-dependent evolution of seizure types was often observed. Recognition of this subgroup of patients could be important for the identification of appropriate candidates for early epilepsy surgery.     

Refractory epilepsy in a Chinese population

OBJECTIVES: To investigate the proportion of Chinese patients with intractable seizures and the risk factors leading to refractory epilepsy. METHODS: Consecutive patients over 14 years of age attending a Neurology clinic were evaluated. Patients with epilepsy were classified into two groups according to their seizure control: refractory or seizure-free. Epilepsy was classified as idiopathic as defined by age-related onset and typical electroclinical characteristics, symptomatic if secondary to a structural abnormality and cryptogenic if the cause was unknown. Age, sex, epilepsy syndrome classification, aetiology, presence of mental retardation and the number of drugs used were compared between patients with refractory epilepsy and those in remission. RESULTS: Among 260 adolescent and adult patients with a mean age of 34 years (range 15-79), complete seizure control was achieved in 157 (60%) cases. Multivariate binomial logistic regression analysis showed that patients with mesial temporal sclerosis (OR=7.6, 95% CI 3.53-16.4, p<0.01) and the presence of mental retardation (OR=9.39, 95% CI 3.98-22.12, p<0.01) were more likely to develop pharmacoresistant epilepsy. CONCLUSION: In adults the underlying aetiology is an important factor as to whether patients develop intractable seizures. Poor control was also associated with the presence of mesial temporal sclerosis and mental retardation.     

Two-year remission and subsequent relapse in children with newly diagnosed epilepsy

PURPOSE: Although remission is the ultimate measure of seizure control in epilepsy, and epilepsy syndrome should largely determine this outcome, little is known about the relative importance of syndrome versus other factors traditionally examined as predictors of remission or of relapse after remission. The purpose of this study was to examine remission and relapse with respect to the epilepsy syndrome and other factors traditionally considered with respect to seizure outcome. METHODS: A prospectively identified cohort of 613 children with newly diagnosed epilepsy was assembled and is actively being followed to determine seizure outcomes. Epilepsy syndrome and etiology were classified at diagnosis and again 2 years later. Remission was defined as 2 years completely seizure-free, and relapse as the recurrence of seizures after remission. Multivariable analysis was performed with the Cox proportional hazards model. RESULTS: Five hundred ninety-four of the original 613 children were followed > or = 2 years (median follow-up, 5 years). Remission occurred in 442 (74%), of whom 107 (24%) relapsed. On multivariable analysis, idiopathic generalized syndromes and age at onset between 5 and 9 years were associated with a substantially increased remission rate, whereas remote symptomatic etiology, family history of epilepsy, seizure frequency, and slowing on the initial EEG were associated with a decreased likelihood of attaining remission. Young onset age (<1 year) and seizure type were not important after adjustment for these predictors. Relapses occurred more often in association with focal slowing on the initial EEG and with juvenile myoclonic epilepsy. Benign rolandic epilepsy and age at onset <1 year were associated with markedly lower risks of relapse. About one fourth of relapses were apparently spontaneous while the child was taking medication with good compliance, and more than half occurred in children who were tapering or had fully stopped medication. CONCLUSIONS: A large proportion of children with epilepsy remit. Symptomatic etiology, family history, EEG slowing, and initial seizure frequency negatively influence, and age 5-9 years and idiopathic generalized epilepsy positively influence the probability of entering remission. Factors that most influence relapse tend to be different from those that influence remission.     

Special education needs of children with newly diagnosed epilepsy

Children with epilepsy often experience poor social and educational outcomes. This study aimed to determine the timing of services with respect to the onset of seizures. It also aimed to identify the aspects of childhood epilepsy (type of epilepsy, etiology, seizure control, and treatment) that are associated with the use of special education services. As part of a prospective community-based study, 613 children were recruited when first diagnosed with epilepsy. Mean age at first seizure was 5 years 11 months (SD 4, range 1mo to 15y 8mo). Parents were interviewed 5 years after children were first diagnosed with epilepsy (n=542; 276 [51%] males). Children's mean age at time of interview was 11 years 10 months (SD 4y 1mo, range 5y 8mo to 21y 8mo). Etiology was classified as idiopathic (n=181, 33.4%), cryptogenic (n=261, 48.2%), and remote symptomatic (n=100, 18.5%). Service use was reported in 315 (58%) children. Compared with neurologically intact children (i.e. cryptogenic and idiopathic etiology; n=415, 77%), children with a remote symptomatic etiology and/or an epileptic encephalopathy (n=127, 23%) received services more frequently (88% vs 49%, p<0.001). In the former group, services were initiated for 66 (15%) children before their first seizure; according to age at onset, services were initiated before the first seizures in 12/164 (7.3%) if <5 years, 34/171 (19.9%) if 5-9 years, and 20/80 (25%) if >10 years. A large proportion of children with epilepsy, even if neurologically otherwise normal, receive special education services. Initiation of services often precedes onset of seizures even in neurologically intact children. This suggests that behavioral and cognitive abnormalities may predate the onset of epilepsy and are not necessarily the direct consequences of epilepsy.     

Generalized spike-and-wave patterns in children: clinical correlates

All electroencephalograms performed in our institution between 1980 and 1990 were reviewed. The clinical characteristics of children with epilepsy and generalized spike-and-wave (SW) patterns were analyzed. The SW patterns were classified according to their frequency. Electroencephalograms of 154 children with epilepsy revealed SW patterns. Absence seizures were the most common first seizure, but partial seizures were frequent. More than 40% had several types of seizures. Sixty percent of the epileptic syndromes were generalized, but almost 25% were partial. The typical SW pattern was associated with absence seizures, a normal examination and computed tomographic scan, idiopathic generalized epilepsies, monotherapy, freedom from seizures, and lack of recurrence. The slow SW pattern was associated with West syndrome; a younger age at seizure onset; atonic, myoclonic, tonic, and partial simple seizures; an abnormal examination and computed tomographic scan; cryptogenic or symptomatic generalized epilepsy or symptomatic partial epilepsy; polytherapy; and poor seizure control. The fast SW pattern was associated with secondary generalized, partial, tonic-clonic, and complex partial seizures; a normal computed tomographic scan; cryptogenic partial epilepsy; isolated seizures; and seizure recurrence. Epilepsy with a typical SW pattern should be considered benign, epilepsy with a slow SW pattern malignant, and epilepsy with a fast SW pattern treacherous.     

Epilepsy in hemiplegic cerebral palsy due to perinatal arterial ischaemic stroke

Aim The aim of this study was to describe the frequency, risk factors, manifestations, and outcome of epilepsy in children with hemiplegic cerebral palsy (CP) due to perinatal arterial ischaemic stroke (AIS). Method The study group comprised 63 participants (41 males, 22 females) from a population‐based CP register whose brain imaging showed perinatal AIS. Information collected included occurrence of neonatal seizures, family history of epilepsy, motor function and epilepsy onset, treatment, and outcome. Electroclinical findings were classified according to seizure semiology, seizure type, and epilepsy syndrome. Results Mean age of participants at the time of study was 10 years 6 months (SD 4y 7mo, range 4–20y). Gross Motor Function Classification System levels I and II were reported in 96% of participants, and Manual Ability Classification System levels I and II were reported in 79% of children. Thirty‐four children (54%) developed epilepsy. Term delivery and more severe motor impairment were associated with epilepsy, but neonatal seizures and family history of epilepsy were not. Initial seizures were epileptic spasms, focal seizures, or myoclonic seizures. Focal seizure semiology suggested Rolandic or occipital seizure origin in the majority of children. Focal epileptic discharges in children with focal seizures had features of idiopathic partial epilepsy. Only 15% of children had active epilepsy 10 years after onset. Interpretation Despite a high incidence of epilepsy in children with hemiplegic CP due to AIS, the prognosis for seizure remission is good. Many children have clinical features, electroencephalography findings, and remission typical of idiopathic partial epilepsy.     

Clinical features and treatment of refractory epilepsy in children]

Clinical features of refractory epilepsy in children are symptomatic localization-related epilepsy, especially frontal lobe epilepsy, the onset in young age less than 3 years-old, and complication of developmental retardation. The treatments usually start with one drug of choice for specific seizure type. In the idiopathic epilepsy group, valproic acid was effective in 82% of the patients with generalized epilepsy and in 45% of localization-related epilepsy while carbamazepine was effective in 71% and 67%, respectively. However, in the refractory group which did not react to the drugs of choice at the initial treatment and continued to have seizures, no specific drugs were effective. Therefore, various kind of drugs, new or old, should be tried in sequence irrespective of the type of seizures. The seizure control was attained only in 10% (in the cases of CZP), and 17% (CLB) in localization-related epilepsy and in 9% (VPA), 12% (NZP) and 20% (ZNS) in generalized one in the refractory group. Although new drugs developed, the patients with refractory epilepsy do not tend to decrease in frequency and overall management including daily life or surgical therapy is mandatory for the children with refractory epilepsy.     

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.     

Is There a Partial Benign Epilepsy in Infancy?

Benign idiopathic neonatal convulsions and partial benign childhood epilepsy, the most frequent types of childhood benign epilepsy, are both characterized by partial seizures. This study was performed to determine whether any kind of benign epilepsy with partial seizures occurs in the age range between these two syndromes. We identified 17 cases of benign epilepsy with partial seizures from a review of 442 records collected in a 5-year period in which seizures occurred between 8 days and 3 years of age. Six patients with onset before 6 weeks of life had clinical features characteristic of benign idiopathic neonatal convulsions, and appeared to be a delayed type of this syndrome. Eleven patients had clinical features characteristic of partial benign childhood epilepsy. In the age range between occurrence of these two recognized epileptic syndromes, we identified no case of partial epilepsy with favorable outcome.     

Long-Term Outcome of Pattern-Sensitive Epilepsy

Summary:  Purpose: To evaluate the long-term outcome of patients with pattern-sensitive epilepsy.
Methods: We prospectively studied 35 patients (21 females and 14 males) with pattern-sensitive epilepsy (follow up ≥5 years; mean 13.9; range 6.4 – 27.6). All cases had regular clinical examinations and serial electroencephalographic (EEG) recordings. Photosensitivity and pattern sensitivity were ascertained neurophysiologically in all cases. Outcome was evaluated according to the following variables: duration of photosensitivity, rate of remission (seizure-free period ≥ 2 years), withdrawal of therapy, and recurrence after drug discontinuation.
Results: The epilepsy was generalized in 18 cases (17 idiopathic, one symptomatic) and partial in 17 (13 idiopathic, four symptomatic). Sixteen patients (46%) had a family history of seizures. The mean age at the last examination was 21.4 years (range 11.2–35.5 years). Five patients (14%) had only reflex seizures. The most common type of spontaneous seizures was generalized (60%), whereas reflex seizures were more frequently partial (74%). Mean epilepsy duration was 8.7 ± 6.3 years. Patients with only reflex seizures were instructed to avoid precipitating stimuli and were not treated with antiepileptic drugs. Treatment was gradually withdrawn in 10 out of 30 treated patients, with relapse in only two cases. At the end of follow up, 28 patients (80%) were seizure-free.
Conclusion: The long-term outcome of patients with pattern-sensitive epilepsy indicates a good prognosis with a favorable course for both spontaneous and reflex seizures.     

Clinical Indicators of Genetic Susceptibility to Epilepsy

Summary: We evaluated clinical indicators of genetic susceptibility to epilepsy in the families of 1,957 adults with epilepsy (probands) ascertained from voluntary organizations. Very few of the probands in this series had idiopathic epilepsy syndromes. Among relatives of probands with postnatal CNS insults, risks of epilepsy were no higher than in the general population. Risk was increased in relatives of probands without identified CNS insults (i.e., those with idiopathic/cryptogenic epilepsy) or with neurological deficit presumed present at birth, compared with relatives of probands with postnatal CNS insults. Among relatives of probands with idiopathic/cryptogenic epilepsy, risks were higher in parents and siblings, but not in offspring, of probands with generalized onset as compared with partial onset seizures. Risks in offspring were higher if the probands had onset of idiopathic/cryptogenic epilepsy before age 10 as compared with age 10 years, but risks in parents and siblings were not associated with the proband's age at onset. These results suggest that genetic susceptibility increases risk of some forms of cryptogenic epilepsy and of epilepsy associated with neurological deficit presumed present at birth, but not of postnatal symptomatic epilepsy. The influences on risk in offspring may differ from those in parents and siblings.     

Lennox-Gastaut syndrome in the adult

The authors used the definition of the Lennox-Gastaut syndrome (LGS) adopted by the Commission on Classification and Terminology of the International League against Epilepsy. Three hundred and thirty eight patients with childhood LGS have been followed until adulthood; 62.4 p. 100 had an unfavourable outcome. All began their LGS in childhood or infancy (between the ages of 1 and 8.80 p. 100 before the age of 4). In 46.9 p. 100 of cases, complete LGS persisted in the adult. Most cases were apparently primitive. In 15.6 p. 100 of cases, generally symptomatic, the LGS disappeared but an often severe, mostly multifocal epilepsy persisted. 37.6 p. 100 of cases had a more or less favourable outcome. In these cases, the LGS had often begun later (between 7 and 11 years of age) and lasted for less (between 2 and 6 years). Some patients (20.4 p. 100) still had fairly rare partial seizures and neurological or psychiatric symptoms. These cases were mostly symptomatic, 17.4 p. 100 of cases seemed to have been nearly completely cured. These were cases where the LGS had followed another type of epilepsy, mostly of the idiopathic generalized type. Fourty four patients with no prior epilepsy presented with LGS appearing between the ages of 13 and 23. They were divided into 2 groups: in 31 patients, the LGS was associated with focal signs. In all these cases, the evolution was for the worse, whatever the age at onset. In 13 cases, there were no focal signs. In these, there was a different prognosis between those with an onset between the ages of 13 and 15, where the entire syndrome persisted and the evolution is for the worse, and those with an onset after the age of 15, where the outcome was better.