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PubMed1998

Myoclonic absence-like seizures and chromosome abnormality syndromes.

PURPOSE: We explored the relationship between myoclonic absence seizures (MAS) and underlying chromosome disorders. METHODS: Among 14 patients with MAS observed in three centers, 5 had typical cryptogenic myoclonic absence epilepsy (MAE), 2 had MAS associated with other seizure types (1 with signs of a neuronal migration abnormality and 1 with signs of a metabolic disorder), and 7 had MAS, with or without other seizure types, complicating a chromosome abnormality syndrome-2 with trisomy 12p, 4 with Angelman syndrome, and 1 with inv dup (15). RESULTS: In the 7 patients with chromosomopathy, MAS appeared at a mean age of 2.9 years (range 4 months to 6 years 6 months), had a duration of 4-20 s, and were accompanied by reduced awareness and rhythmic myoclonic jerks involving proximal limb muscles. Ictal EEG showed 2- to 3-Hz generalized spike-and-wave discharges. CONCLUSIONS: In these patients, MAS differed slightly from those of typical MAE: age of onset was earlier, absences were of shorter duration, and no clear increase in muscular tone was noted. Abnormal expression of genes codifying for the subfamily of K+ channels and for gamma-aminobutyric acid-3 subunit receptors (GABRB3), both located in the chromosome segments involved in the chromosomopathies presented by our patients, could be responsible for the same generalized seizure type. Chromosome analysis should be performed in patients with mental retardation and MAS, especially when the ictal pattern does not completely overlap that observed in MAE.

Adult↗
PubMedDate not supplied

Clinical manifestations in children with occipital spike-wave paroxysms.

The pattern of occipital-posterotemporal spike-wave paroxysms (O-PT SWPs), is a distinctive EEG abnormality observed primarily with occipital epilepsy of childhood and basilar artery migraine. We studied EEG and clinical features in 30 children and young adults with this EEG pattern. Prolonged and brief O-PT SWPs were observed. Prolonged discharges (greater than 6 s) were observed only in children with seizures (p less than 0.001), and brief discharges (1-6 s) were observed immediately after eye closure. Generalized SWPs (11 patients, 37%) and background abnormalities (17 patients, 57%) were common. Photic activation of O-PT SWPs was not observed. Twenty-four patients (80%) manifested paroxysmal phenomena-seizures (20 patients, 67%) and migraine (12 patients, 40%, 4 alone and 8 with seizures). Fifteen patients (75%) had partial seizures, and 5 (25%) had absence seizures. In 7 patients with partial seizures, an etiology was evident. Neurologic examination was more often abnormal in patients with secondary partial seizures than in those with idiopathic partial seizures (p less than 0.05) and absence seizures. Conversely, migraine was more often associated with idiopathic partial seizures than with secondary partial seizures (p less than 0.05) and absence seizures. Six children (20%) had no paroxysmal events. Generalized SWPs were uncommon in patients with idiopathic partial seizures. We conclude that O-PT SWPs is a nonspecific epileptiform abnormality that may occur in children with (a) idiopathic partial, (b) symptomatic partial, and (c) absence epilepsies, but it may also occur in patients with no evidence of seizures.

Age Factors↗
PubMed2005

Management guidelines for children with idiopathic generalized epilepsy.

Most idiopathic generalized epilepsy (IGE) begins in childhood. Clinicians face many important management decisions for these children; however the existing literature provides little scientific guidance. TIME OF DIAGNOSIS: At the time of presentation, it is unclear how accurately IGE seizures and syndromes are diagnosed and how consistent are the EEG correlates. Investigations beyond EEG are of uncertain value but probably are not needed. Selection of an initial antiepileptic drug (AED) is not based on any adequately powered, blinded, randomized comparative trials; however, reasonable evidence supports the use of valproic acid (VPA), lamotrigine and ethosuccimide as initial treatment for childhood absence epilepsy (CAE). Many large case series suggest the value of VPA for juvenile myoclonic epilepsy (JME) but the relative value of other, newer AEDs has not been established. FIRST YEARS OF TREATMENT: Once AED treatment is started, it is difficult to establish that absence seizures are completely controlled and the importance of interictal spike-wave discharge remains uncertain. The value of restrictions on the child's activities has not been well studied; however serious accidents appear to be a justifiable concern in children with uncontrolled absence. Assessing the risk from photosensitivity in JME is challenging. LENGTH OF TREATMENT: The optimal length of treatment for IGE is unclear. There is a high rate of remission in CAE when AEDs are discontinued after 1-2 years of seizure freedom; however, long-term remission in CAE occurs in only 65%. It is usually assumed that treatment for JME is life long, although about 10% appear to have permanent remission in adolescence. Discontinuing AED treatment in JME requires a very individual risk assessment. PREPARATION FOR ADULT LIFE: Long-term social outcome for children with CAE is often unsatisfactory even if the epilepsy remits. The reasons are unclear and successful interventions have not been described. Long-term social outcome for JME has not been adequately described. CONCLUSIONS: Further research is needed to justify the direction of many of the necessary management decisions in the diagnosis and treatment of IGE syndromes.

Adolescent↗
PubMed1990

Potentiation of gamma-vinyl GABA (vigabatrin) effects by glycine.

Vigabatrin, because of its ability to increase brain GABA concentration, acts as an anticonvulsant on convulsive epileptic seizures and increases seizures in generalized non-convulsive epilepsy. Next to GABA, glycine is one of the most important inhibitory neurotransmitter amino acids. We studied the influence of glycine on the effects of treatment with vigabatrin in two rat models of generalized convulsive seizures and a rat model of spontaneous generalized non-convulsive seizures. Glycine (750 mg/kg i.p.) or vigabatrin (200 mg/kg i.p.), when given alone, provided partial protection against convulsive seizures, while combined treatment with the two drugs significantly suppressed the convulsive seizures in both the mercaptopropionic acid (MPA)-induced seizures and audiogenic seizures. In contrast to the response to treatment with each individual drug, the drug combination nearly abolished the appearance of isolated spikes on the EEG in MPA seizures. On the other hand, glycine also enhanced the aggravating effect of vigabatrin on spontaneous spike and wave discharges in a rat model of genetic absence epilepsy, whereas glycine or vigabatrin alone, at the above doses, produced only a slight, non-significant increase in spontaneous spike and wave discharges. The GABA-glycine interaction is the first example of a synergistic action of two inhibitory neurotransmitters on seizure-related pathological discharges.

3-Mercaptopropionic Acid↗
PubMed1989

[Epilepsy with rolandic paroxysms and migraine, a non-fortuitous association. Results of a controlled study].

The incidence of migraine was studied in 4 groups of patients: patients with centro-temporal epilepsy, patients with absence of epilepsy, patients with partial epilepsy, and non-epileptic patients with a history of cranial trauma. Migraines were present in 62% of the patients with centro-temporal epilepsy, 34% of the patients with absence of epilepsy, 8% of the patients with partial epilepsy and 6% of the patients with cranial trauma. These results suggest that the association of centro-temporal epilepsy and migraine is non-fortuitous as well as, to a lesser degree, absence of epilepsy and migraine. The authors discuss the role of serotonin in the association epilepsy-migraine and suggest that centro-temporal epilepsy might be a feature for the diagnosis of migraine.

Adolescent↗
PubMed2005

Chronic levetiracetam treatment early in life decreases epileptiform events in young GAERS, but does not prevent the expression of spike and wave discharges during adulthood.

PURPOSE: In Genetic Absence Epilepsy Rats from Strasbourg (GAERS), age-related absence seizures start to appear from postnatal day (PN) 30 concomitant with 'spike and wave discharges' (SWDs) appearing on cortical EEG recordings. The aim of this study was to investigate the effect of early chronic levetiracetam (LEV) treatment on the development of SWDs in young and adult GAERS. METHODS: From PN 23 until PN 60, LEV (54 mg/kg, i.p.) was administered once daily to GAERS (n=8), while control GAERS (n=7) received saline (0.9% NaCl, i.p.). All animals were implanted with four epidural EEG electrodes at PN 51. EEG was recorded for 3h daily, during the last 4 days of the treatment (PN 57-PN 60) and during 4 additional days after treatment had been terminated (PN 61-PN 64). The animals were monitored again at the age of 4 months (PN 120-PN 124), about 2 months after the last administration of LEV. RESULTS: During treatment, epileptiform events in the LEV group were significantly reduced (62%, P<0.05) in comparison with the control group. During the following 4 days, epileptiform events were reduced in the LEV group, with an average difference of 53% (P=0.064). Once the animals had reached adult age, there was no difference in epileptiform events between the LEV group and controls. CONCLUSION: In this study, chronic LEV administration induced a reduction in epileptiform events in young GAERS. This effect persisted to some extent after treatment cessation (PN 61-PN 64), which might indicate a slowing down of epileptogenic processes. However, at the age of 4 months all animals revealed a similar expression of epileptiform discharges.

Aging↗
PubMed1999

Is the International League against Epilepsy classification of epileptic syndromes applicable to children in Estonia?

The concept of the epileptic syndrome has had a practical and research impact on the management of patients with epilepsy. The aim of the present study was to verify the applicability of the International Classification of Epilepsies and Epileptic Syndromes in children and adolescents in Estonia. A population-based study was performed between January 1995 and December 1997 in seven counties. Only cases involving children between the ages of 1 month and 19 years with at least two unprovoked seizures were included. In all, 560 children and adolescents were referred to the Children's Hospital of the University of Tartu. A syndrome diagnosis was made in 550 (98.2%) cases: (49.4%) were localization-related (6.4% idiopathic, 18.9% symptomatic, 24.1% cryptogenic). Benign childhood epilepsy with centrotemporal spikes was present in 33 (5.9%) and childhood epilepsy with occipital paroxysms in three (0.5%); 48.4% were generalized (28.8% idiopathic, 5.7% cryptogenic or symptomatic, 14% symptomatic). Childhood absence epilepsy was present in 6.4%, juvenile absence in 2.0%, juvenile myoclonic in 0.7% and epilepsy with generalized tonic-clonic seizures on awakening in 17.7%. West syndrome was diagnosed in 1.4%, Lennox-Gastaut syndrome in 2.9% of the cases. In 0.4% of the cases it was undetermined whether seizures were focal or generalized. In 8.8% of the cases there were atypical features so they were classified as 'other symptomatic generalized epileptic syndromes not defined above' and 1.8% of the cases were unclassified. Specific neurological diseases were diagnosed in 5.0% of cases. Thus, the International Classification of Epilepsies and Epileptic Syndromes was very applicable to children and adolescents in Estonia.

Adolescent↗
PubMed2002

Clinical-etiological and therapeutic profile of 719 Mexican epileptic children.

RATIONALE: We performed this study with the intention of describing the clinical-etiological characteristics and therapeutic responses in a group of epileptic children seen at the Instituto Nacional de Pediatría (INP), a tertiary facility in Mexico City. METHODS: All patients who attended the Epileptic Clinic between March and June 1998 and fulfilled the selection criteria were enrolled in the study. Clinical and therapeutic response data were recorded. Three groups were formed by etiology. Statistical tests were two-tailed, with alpha=0.05. RESULTS: In all, 719 patients were studied. The distribution by etiology was as follows: group I, idiopathic (123 patients); group II, cryptogenic (132); and group III, symptomatic (464). In group I, 56% of the patients were female. Mean age at onset was 5 years 2 months (SD: 3 years 8 months) in group I; 1 year 11 months (SD: 2 years 5 months) in group II; and 2 years 10 months (SD: 3 years 1 month) in group III. The mean evolution time was 5 years 4 months (SD: 4 years) in all groups. The most frequent variety of epilepsy in the three groups was generalized epilepsy, followed by partial epilepsy. The following epileptic syndromes were identified: in group I, 28 patients had epilepsy with generalized tonic-clonic seizures, 15, absence epilepsy, and 6, benign rolandic seizures; in group II, all 32 patients had focal cryptogenic epilepsy; in group III, 235 had generalized symptomatic and 192, focal symptomatic epilepsy. The main etiologies were hypoxic ischemic encephalopathy (24%) and neural infections (22%). Appropriate seizure control was achieved in 108 (87%) patients in group I; 84 (64%) in group II; and 315 (68%) in group III. In group I, no patient needed more than two antiepileptic drugs and 90% had normal psychomotor development. CONCLUSION: When the three groups were compared in terms of appropriate epileptic control and normal psychomotor development, group I differed from the other groups and the difference was statistically significant.

Adolescent↗
PubMed2005

Association analysis of the Arg220His variation of the human gene encoding the GABA delta subunit with idiopathic generalized epilepsy.

PURPOSE: Mutation analysis of the gene encoding the GABA delta subunit (GABRD) identified a common missense variation (c.659G>A; Arg220His) of which the His220 allele displayed decreased GABA(A) alpha(1)beta(2)delta receptor current amplitudes. The present association study tested whether the functional GABRD His220 allele confers susceptibility to common syndromes of idiopathic generalized epilepsy (IGE). METHODS: Five hundred and sixty two unrelated German IGE patients and 664 healthy population controls were genotyped for the c.659G>A polymorphism in exon 6 of the GABRD gene. RESULTS: His220 allele frequencies did not differ significantly between IGE patients (2.3%) and the controls (2.8%; P=0.46). Likewise, no evidence for an allelic association was found with juvenile myoclonic epilepsy (n=218; 2.8%; P=0.97) or idiopathic absence epilepsy (n=260; 2.3%; P=0.56). CONCLUSION: Our results provide no evidence that the functional GABRD His220 allele mediates a substantial susceptibility effect to common IGE syndromes in the German population.

Arginine↗
PubMed1977

Nosological problems of myoclonic epilepsies in childhood and adolescence.

An attempt is made to clarify the confusion over the classification of the myoclonic epilepsies of childhood. Six types of seizure are described, the classification being based on clinical and electroencephalographic findings. The types of myoclonic epilepsy are infantile spasms, myoclonic astatic epilepsy, myoclonic absence, myoclonic epilepsy of childhood, myoclonic epilepsy of adolescence and photomyoclonic epilepsy, the last being subdivided into myoclonic jerks and eyelid myoclonia. Synonyms are given for all types, based on the major papers in the literature over the last 16 years. The similarity of infantile spasms and myoclonic astatic epilepsy is discussed, as are the so-called 'Lennox' and 'Lennox-Gastaut' syndromes.

Adolescent↗
PubMed2000

[Relapse of seizure after withdrawal of antiepileptic drug treatment in childhood epilepsy: age-dependent factors].

In 556 epileptic children, clinical and encephalographic factors concerning the discontinuation of antiepileptic drugs (AED) were studied, with emphasis on age-related factors. Seizures relapsed in 80 patients (14.4%), the incidence being high in idiopathic generalized epilepsy with onsets in adolescence or adulthood (juvenile absence epilepsy, juvenile myoclonic epilepsy, grand mal on awakening), symptomatic partial epilepsy and symptomatic generalized epilepsy. The age distribution at the relapse showed two peaks; 9 to 11 and 17 to 19 years. The first peak represented patients with school-age onset epilepsies, and the second those with the adolescent or adult onset epilepsies. The age at discontinuation of AED depended on each epileptic syndrome, and the relapse rate was significantly higher when the AED was discontinued after adolescence. In 56 patients, seizure relapsed during the withdrawal phase or less than 1 year after the discontinuation. The type of the relapsing seizure was the same as the previous ones. On the other hand, in 16 out of 24 patients whose relapse occurred 1 year or more after the discontinuation, the seizure type was different from the previous ones. These results suggest the relevance of age-dependent factors to the discontinuation of therapy.

Adolescent↗
PubMed1995

[Generalized idiopathic epilepsy with refractory absence seizures in the adult].

The prognosis for generalized epilepsy with absence seizures starting in childhood and adolescence (GEA) must be given cautiously because of the possibility that seizures may persist into adulthood and occur along with tonic-clonic seizures. Such an unfavorable course may be due to factors that are intrinsic to the various epileptic syndromes involving absences and/or to individual patient characteristics. An additional factor of particular importance in GEA is inadequate treatment. We describe 15 adult patients with GEA whose mean age when examined was 41.3 years. All had experienced absence seizures since childhood and 12 had had convulsive seizures. Most were following haphazardly combined therapies. An average of 4 years was needed to modify treatment and achieve remission of seizures in 11 of the 13 cases followed for more than 1 year. We emphasize the efficacy of valproate (in 14 of 15 patients) and its combination with ethosuximide (4 patients). GEA should not be considered refractory until appropriate use of these drugs has been tried; rather it is a syndrome that may go into remission even after decades of persistent seizures.

Adult↗
PubMed2006

The brain 5HTergic response to an acute sound stress in rats with generalized (absence and audiogenic) epilepsy.

The brain serotoninergic (5HTergic) system of epileptic subjects can influence their vulnerability to stress. We studied the putative dependency of 5HT neurotransmission parameters on emotional stress, and the presence, types and severity of seizures using rats with genetic generalized (absence and/or audiogenic) epilepsy, of WAG/Rij and Wistar strains. The animals were stressed by exposure to a short aversive noise or left without sound stimulation. Tissue concentrations of 5HT, tryptophan (TRT) and 5-hydroxyindolacetic acid (5HIAA) were assessed by HPLC. The stressor activated the 5HTergic system within thalamus (5HIAA elevated), frontal cortex (5HT, TRT elevated), hypothalamus (increased TRT) in all rats. However, the normal (non-epileptic) rats displayed the highest response in the frontal cortex and the lowest one in the thalamus, as compared to the epileptic rats. Absence-epileptic rats exhibited higher thalamic 5HIAA increase than their controls. Significant correlations existed between propensity of absence epilepsy and 5HTergic parameters measured in the cortex and hypothalamus of absence-epileptic rats. No major difference was found between groups with and without audiogenic epilepsy. The results imply that the stress response depends on the presence of epileptic pathology and the seizure type and severity. The brain 5HT may be involved in the control of the paroxysms and behaviour in absence-epileptic subjects.

Analysis of Variance↗
PubMed2004

Ketogenic diet: outpatient initiation, without fluid, or caloric restrictions.

Although the ketogenic diet has been used for more than 80 years, the optimal methods of initiating the diet and its maintenance have not been clearly defined. This retrospective study was performed to review our experience with initiation of the ketogenic diet in the outpatient and inpatient settings and maintenance of the diet without fluid or caloric restriction. We analyzed 54 patients who had medically intractable epilepsy of whom 44% manifested some degree of mental retardation, 80% had multiple seizure types, and failed on average 4.8 antiepileptic drugs. Forty-four patients underwent induction of the ketogenic diet on an outpatient basis and 21 as inpatients. Three patients in each group were fasted at the initiation of the diet. No significant differences were observed with regard to seizure control in that 62% and 71% had greater than 50% improvement in the outpatient and inpatient groups, respectively. Both groups manifested improvement in alertness and social interaction. The efficacy of a ketogenic diet in the symptomatic epilepsies was confirmed, and benefit for medically refractory childhood absence epilepsy was documented. We conclude that a prospective, randomized trial is necessary to compare outpatient vs inpatient initiation of the ketogenic diet and the utility of fluid and caloric restriction.

Ambulatory Care↗
PubMed1991

Double-blind placebo-controlled trial of flunarizine as add-on therapy in refractory childhood epilepsy.

Flunarizine (FLN) has been suggested as an add-on treatment in drug-resistant epilepsy patients. In view of the discordant experiences and of the paucity of controlled trials in children, we studied its effectiveness in 20 patients aged 6 to 18 years (10 males and 10 females), affected by drug-resistant epilepsy. 14 had symptomatic generalized epilepsy (the Lennox-Gastaut syndrome in 10; other forms in 4); 3 had cryptogenic generalized epilepsy (the Lennox-Gastaut syndrome in 2; myoclonic absences epilepsy in 1); 3 had symptomatic partial epilepsy (temporal lobe epilepsy). 7 of them were withdrawn: only 1 because of side effects. An initial four-month baseline pretrial period was followed by two four-month periods of administration of FLN or a placebo, under double blind conditions, in a randomized sequence. Preexisting antiepileptic (AEDs) medication was maintained at a constant dose throughout the study. FLN was administered as drops in a single evening dose of 5 mg (patients less than 10 years) or 10 mg. (patients greater than 10 years). During the pretrial phase, after phase 1 and phase 2, a waking EEG was recorded and blood samples were taken for hematology, hepatic-function tests, and AED serum levels. The evaluation of the activity of FLN was based on the total number of seizures. A 30-60% reduction in seizure frequency was found in 5 out of the 13 patients completing the trial (no changes occurred in the remainders). This result did not appear to be due to changes in the plasma levels of the AEDs. No significant differences were seen in the EEG paroxysmal activity in the three phases of the study. Side effects were rare. The serum FLN levels ranged between 16.4 and 109 ng/ml. It seems that the antiepileptic properties of FLN need further validation, particularly in childhood.

Adolescent↗