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PubMed1994

Personal continuity and the care of patients with epilepsy in general practice.

BACKGROUND: So far no study has shown that patients with a chronic illness benefit from seeing the same doctor in general practice although many believe this to be so. AIM: Epilepsy was chosen as an example to test the hypothesis that if patients see the same doctor more often in general practice they are more likely to discuss personally important aspects of their illness. METHODS: In this cross-sectional survey 99 patients aged 15-84 years with active epilepsy were interviewed at home and then their records were reviewed. The patients came from four large Southampton group practices, one with a strict personal list system and three with combined lists. Outcome measures included reported discussion of feelings about stopping medication, stigma and concealment and the patient's relationship with practice doctors. Continuity was assessed from the records. RESULTS: Discussion of epilepsy was not significantly associated with continuity of doctor but was significantly associated with ease of talking to one or more doctors. CONCLUSION: Encouraging patients with epilepsy to see the same doctor may be less important than improving doctors' communication skills and paying specific attention to the psychosocial aspects of epilepsy as well as to seizure control. It is recommended that a simple checklist including these items is used when a patient's care is reviewed.

Adolescent↗

PubMed2003

Treatment of epilepsy in general hospitals: do patients and neurologists agree on success or failure?

OBJECTIVE: Opinions of patients and neurologists about aspects of their epilepsy and their treatment were compared. METHOD: Thirty-two neurologists, working in general hospitals, included 198 patients aged 16 years or more. Both neurologist and patient independently completed a questionnaire consisting of simple open questions about the epilepsy and the drug treatment. The average characteristics for this group are very similar to characteristics of the general population: age and gender distribution, highest completed educational level, occupational participation and family circumstances are not statistically significant from the general population. RESULTS: Neurologists and patients appear to agree about most areas that we assessed: seizure count, severity of epilepsy, efficacy and tolerability of the treatment and impact of the epilepsy and treatment on daily life (as expressed in the 'Quality of Life ratings'). Detailed analysis showed that this agreement is partly artificially increased by the group with good outcome and less strong or even absent for the more severe epilepsies. For seizure count, we see 96.4% agreement for the group with low seizure frequency and 73.1% agreement in case of high seizure frequency. For the evaluation of severity of the epilepsy, 73.2% agreement is found for mild classification and 16.6% agreement for the severe classification. Agreement on Quality of Life (QOL) is almost complete in patients with excellent QOL (91% agreement) and almost absent for a low QOL (17% agreement). Finally, the same pattern is found for reports on side-effects of the medication. Both neurologists and patients report side-effects in about 40% of the cases. This suggests excellent agreement but individual data show that agreement is only satisfactory for the milder epilepsies (16.2% vs. 16.2%). For the more severe agreement is almost absent (4.1% of the neurologists vs. 13.4% of the patients). DISCUSSION: Possibly, it is more difficult for the neurologist to perceive and appraise all relevant factors in the case of complex epilepsies. Alternatively, subjective definitions and assumptions of patients differ from the standards set by the neurologists in the case of continuing seizures. The clinical relevance of our findings is that, especially in the cases of more severe and refractory epilepsies, patients' opinions are of utmost importance and we cannot take our own opinions and evaluations at face value.

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↗

PubMedDate not supplied

Disabling erratic myoclonus during lamotrigine therapy with high serum level--report of two cases.

We present two patients with epilepsy who experienced disabling myoclonic jerks during lamotrigine treatment. Both were young males who had intractable cryptogenic generalized epilepsy since childhood. They received a lamotrigine-valproate combination resulting in an excellent improvement; however, after 2-3 years of therapy, both patients were hospitalized because of continuous disabling myoclonic jerks. The dosage of lamotrigine was the same before and at the onset of myoclonus. When the severe myoclonus started, both patients had a higher serum lamotrigine level (16.5 and 17.7 mg/L, respectively) than in previous findings. Disabling myoclonus was also present during lamotrigine monotherapy with 15 mg/L serum level. Lamotrigine may severely worsen myoclonic phenomena in generalized epilepsies, in which adverse events may be dependent on drug serum level.

Adolescent↗

PubMed2004

Effect of localization of missense mutations in SCN1A on epilepsy phenotype severity.

BACKGROUND AND METHODS: Many missense mutations in the voltage-gated sodium channel subunit gene SCN1A were identified in patients with generalized epilepsy with febrile seizures plus (GEFS+) and severe myoclonic epilepsy of infancy (SMEI), although GEFS+ is distinct from SMEI in terms of clinical symptoms, severity, prognosis, and responses to antiepileptic drugs. The authors analyzed the localization of missense mutations in SCN1A identified in patients with GEFS+ and SMEI to clarify the phenotype-genotype relationships. RESULTS: Mutations in SMEI occurred more frequently in the "pore" regions of SCN1A than did those in GEFS+. These SMEI mutations in the "pore" regions were more strongly associated than mutations in other regions with the presence of ataxia and tendency to early onset of disease. The possibility of participation of ion selectivity dysfunction of the channel in the pathogenesis of SMEI was suggested by a mutation in the pore region (R946C) identified in a SMEI patient. CONCLUSIONS: There was a significant phenotype-genotype relationship in generalized epilepsy with febrile seizures plus and severe myoclonic epilepsy of infancy with SCN1A missense mutations. More severe sodium channel dysfunctions including abnormal ion selectivity that are caused by mutations in the pore regions may be involved in the pathogenesis of SMEI.

Age of Onset↗

PubMedDate not supplied

Lamotrigine in treatment of 120 children with epilepsy.

One hundred twenty children aged 10 months to 16 years 9 months were included in three studies with lamotrigine (LTG): a single-blind study (n = 60), a pharmacokinetic study (n = 23), and a compassionate group (n = 37). At 3 months, 11 patients had become seizure-free and 34 had > 50% decrease in seizure frequency. The best results involved absence epilepsy, Lennox-Gastaut syndrome (LGS), and other symptomatic generalized epilepsy. Forty-two patients were followed > 1 year, 22 for a mean of 2.2 years, and there was no significant increase in seizure frequency as compared with 3-month follow-up. Fourteen patients became seizure-free for > 6 months; all except 1 had generalized epilepsy. For 12 patients, treatment could be reduced to monotherapy, but for those with valproate (VPA) comedication LTG dosage had to be increased; 25% of patients with VPA monotherapy exhibited skin rash, appearing 3-18 days after starting LTG. For 4 patients, LTG could be reintroduced after VPA was withdrawn. Ten patients had ataxia and/or drowsiness and 2 had vomiting. For all other patients, tolerance was excellent.

Adolescent↗

PubMed2005

Treatment of epilepsy in adults: expert opinion, 2005.

RATIONALE: Over the past decade, there has been a proliferation of new therapies for the treatment of epilepsy. Faced with this growing list of options, clinicians must decide what therapy, or combination of therapies, is best for a given individual. Although controlled clinical trials exist for each treatment option, the answer to these questions may remain unclear. In 2000, a survey of expert opinion was done to address questions concerning which treatment options might be best in a number of clinical situations. We surveyed a group of US epileptologists again in 2004 and compared the results of the two surveys. METHODS: We sent a questionnaire on the treatment of adolescent and adult epilepsy syndromes to a group of opinion leaders in the field of epilepsy. The questions were formatted to simulate real-world clinical situations in the treatment of symptomatic localization related epilepsy (SLRE) and idiopathic generalized epilepsy (IGE). The experts were asked to rate treatment options based on a modified RAND 9-point scale (with "9" most appropriate and "1" least appropriate). Statistical analysis of data was performed as defined by the expert consensus method. The results were used to develop user-friendly recommendations concerning overall treatment strategies and choice of specific medications. RESULTS: Of the 48 experts to whom the survey was sent, 43 (90%) responded; 29 (67%) of the respondents had also participated in the first survey. For initial monotherapy for IGE (generalized tonic-clonic [GTC], absence, and myoclonic seizures), valproate was rated as treatment of choice. For IGE-GTC seizures, lamotrigine and topiramate were also identified as usually appropriate for initial monotherapy. For IGE-absence seizures, ethosuximide was also a treatment of choice, and lamotrigine was usually appropriate. For SLRE, the experts were again asked to rate treatment options based on seizure type: simple partial seizures (SPS), complex partial seizures (CPS), and secondarily generalized tonic-clonic seizures (SGTC). In SLRE-SPS and SLRE-SGTC, carbamazepine and oxcarbazepine were treatments of choice, with lamotrigine and levetiracetam also usually appropriate. In SLRE-CPS, carbamazepine, lamotrigine. and oxcarbazepine were treatments of choice, while levetiracetam was also usually appropriate. For women who are pregnant or trying to conceive, lamotrigine was treatment of choice for both syndrome types. In the elderly, whether medically stable or ill, the treatment of choice was lamotrigine, while levetiracetam was also usually appropriate (along with gabapentin for persons with comorbid medical illness). In persons with HIV and epilepsy, lamotrigine and levetiracetam were usually appropriate. In people with both epilepsy syndromes who have depression, lamotrigine was treatment of choice. In a person with seizures and renal disease, lamotrigine was usually appropriate for both syndromes, with valproate also usually appropriate for IGE. In patients with hepatic disease, levetiracetam and lamotrigine were usually appropriate for IGE; in SLRE, levetiracetam was treatment of choice, with gabapentin also usually appropriate. CONCLUSIONS: Although the panel of experts reached consensus on many treatment options, there are limitations to these types of data. Despite this, the expert consensus method concisely summarizes expert opinion, and this opinion may be helpful in situations in which the medical literature is scant or lacking. The information in this report should be evaluated in conjunction with evidence-based findings.

Adult↗

PubMed2006

Reproductive endocrine function in women with epilepsy: the role of epilepsy type and medication.

The purpose of the analysis described here was to assess reproductive endocrine disorders in 148 women with epilepsy (WWE) by epilepsy type and antiepileptic drug use. Women with idiopathic generalized epilepsy had a higher prevalence of reproductive endocrine disorders than control subjects. In addition, hyperandrogenism, polycystic ovaries, and polycystic ovary syndrome were more prevalent in WWE on valproate than in WWE taking other drugs or control women. The use of VPA was a predictor of the development of polycystic ovaries and polycystic ovary syndrome, and the use of valproate and younger age predicted the development of hyperandrogenism. In conclusion, both idiopathic generalized epilepsy and valproate were associated with an increased risk of reproductive endocrine disorders in WWE in this post hoc reanalysis of data on a large number of WWE. This was especially evident if the epilepsy was active and required treatment early in life.

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↗

PubMed2004

Efficacy and tolerability of the new antiepileptic drugs, I: Treatment of new-onset epilepsy: report of the TTA and QSS Subcommittees of the American Academy of Neurology and the American Epilepsy Society.

PURPOSE: To assess the evidence demonstrating efficacy, tolerability, and safety of seven new antiepileptic drugs [AEDs; gabapentin (GBP), lamotrigine (LTG), topiramate (TPM), tiagabine (TGB), oxcarbazepine (OXC), levetiracetam (LEV), and zonisamide (ZNS), reviewed in the order in which these agents received approval by the U.S. Food and Drug Administration] in the treatment of children and adults with newly diagnosed partial and generalized epilepsies. METHODS: A 23-member committee, including general neurologists, pediatric neurologists, epileptologists, and doctors in pharmacy, evaluated the available evidence based on a structured literature review including MEDLINE, Current Contents, and Cochrane Library for relevant articles from 1987 until September 2002, with selected manual searches up to 2003. RESULTS: Evidence exists, either from comparative or dose-controlled trials, that GBP, LTG, TPM, and OXC have efficacy as monotherapy in newly diagnosed adolescents and adults with either partial or mixed seizure disorders. Evidence also shows that LTG is effective for newly diagnosed absence seizures in children. Evidence for effectiveness of the new AEDs in newly diagnosed patients with other generalized epilepsy syndromes is lacking. CONCLUSIONS: The results of this evidence-based assessment provide guidelines for the prescription of AEDs for patients with newly diagnosed epilepsy and identify those seizure types and syndromes for which more evidence is necessary.

Acetates↗

PubMed1980

Late results of stereotactic radiofrequency lesions in epilepsy.

Thirty-five patients with partial complex seizures and two patients with generalized epilepsy were treated by stereotactic radiofrequency lesions. Follow-up from 2 to 13 years was available in 30 patients. The stereotactic targets in patients with partial complex seizures were: a) Amygdala; b) Ammon's horns; c) parahippocampal gyrus; d) Fornix. Depending on scalp EEG and depth electrode studies, each patient had one or more target coagulated, unilaterally or bilaterally. In the 2 cases of generalized epilepsy, bilateral Forel field lesions were performed. Late surgical results are discussed in relation to the depth EEG studies and the number and site of stereotactic lesions.

Adolescent↗

PubMed1995

Neuroepidemiological study of childhood epilepsy by application of international classification of epilepsies and epileptic syndromes (ILAE, 1989).

A population-based survey of childhood epilepsy was made in 1975 on the total population of children aged < 10 years living in Okayama Prefecture (n = 2,378 patients). Using the data obtained, we attempted to reclassify the various types of epilepsy according to the international classification (ILAE, 1989). Reclassification was possible in 1,872 (78.7%) of the 2,378 cases. The 1,872 cases consisted of 1,045 (55.8%) with localization-related epilepsies, 824 (44.0%) with generalized epilepsies, and 3 (0.2%) with epilepsies undetermined whether focal or generalized. Classification of the epilepsies in a population-based survey using the international classification involves difficulties, because both clinical and EEG findings are essential. However, if an appropriate area is selected, classification of epilepsies and epileptic syndromes in a population-based survey is possible by referring to all medical records stored at every hospital and practitioner's clinic that administers treatment to patients with epilepsy in the area.

Age Factors↗

PubMed2006

The natural history of myoclonic astatic epilepsy (Doose syndrome) and Lennox-Gastaut syndrome.

The purpose of this article is to present a short review of the natural history of myoclonic astatic epilepsy (MAE; Doose syndrome) and the Lennox-Gastaut syndrome (LGS). In the 1989 classification of the International League Against Epilepsy (ILAE, 1989), MAE and LGS were initially included in group 2.2: "Cryptogenic or symptomatic generalized epilepsies and syndromes." The subsequent classification of the Proposed Diagnostic Scheme for People with Epileptic Seizures and with Epilepsy (see Ref. 8) placed MAE in axis 3 in the "generalized epilepsy" group and LGS, severe myoclonic epilepsy of infancy (SMEI or Dravet syndrome) and atypical benign partial epilepsy/pseudo-Lennox syndrome (ABPE/PLS) in the "epileptic encephalopathy" group. The semiology of MAE and LGS and their differential diagnosis from SMEI and ABPE/PLS are described. Before the onset of SMEI, MAE, and ABPE/PLS, the development of the child is usually normal. In contrast, in LGS, development is frequently retarded at the onset, depending on the etiopathogenesis of the underlying brain disease. The course of MAE is highly variable with regard to seizure outcome (complete remission in some cases, persistent epilepsy in others) and cognitive development (normal or delayed). The course of LGS and SMEI is generally poor, both with regard to the epilepsy and to the cognitive development whereas the course and seizure outcome of ABPE/PLS is favorable; the patients will be seizure-free at puberty. However, the neuropsychological outcome is less favorable; most patients remain mentally retarded.

Age of Onset↗

PubMed1979

Clinical biochemistry of epilepsy. I. Nature of the disease and a review of the chemical findings in epilepsy.

In idiopathic or generalized epilepsy, serum glucose and cholesterol concentrations tend to be low, especially just before the seizure. Glucose tolerance curves are abnormal and variable. The electrolyte balance is disturbed, and epileptics tend to go readily into alkalosis. Serum [Na+] is usually unaffected, but [K+] is normal to low between attacks and increases during and after the seizure. Serum [Cl-] is usually high just before the seizure. Epileptics are generally mildly hypocalcemic, especially in the period before the seizure. Serum urea and nonprotein nitrogen values are low between paroxysms but increase after the seizure. Serum protein concentration is usually normal. Stress, which releases epinephrine and corticotropin, results in high serum citrate concentration, which probably contributes to decreased serum [Ca2+] just before a seizure. In the healthy individual, any increase in serum citrate is accompanied by increasing [Ca2+]. In the rabbit, convulsions can be induced with corticotropin, a result of increased serum citrate concentration coupled with a decrease in [Ca2+]. The net result is severe hypo-ionic-calcemia. A similar phenomenon has been reported in a few humans. Administration of insulin causes serum citrate concentrations to decrease. Apparently, the dynamic system that controls glucose and lipid metabolism, and thus electrolyte balance, through the hormones epinephrine, corticotropin, insulin, glucagon, calcitonin, and parathormone, is abnormal in the epileptic.

Adrenocorticotropic Hormone↗

PubMed1990

Brainstem auditory evoked responses in partial and generalized seizures.

This study presents clinical neurophysiologic evidence of altered upper brainstem function in patients with generalized epilepsy who do not otherwise differ clinically from the general population. While the differences in absolute latencies are not great enough to support the use of BAERs in routine evaluation, this data does support experimental studies implicating brainstem structures in the pathophysiology of primary generalized epilepsy. The lack of evidence of brainstem involvement in complex partial seizures may suggest a mechanism of seizure spread that is not dependent upon primary brainstem pathology, or may indicate that any brainstem abnormality in epilepsy may involve areas not mediating transmission of auditory stimuli and therefore not assessed by BAERs.

Adult↗

PubMed1985

Myoclonus: relation to epilepsy.

Myoclonus can be divided into those types which are fragments of epilepsy and those which are nonepileptic. Epileptic myoclonus is viewed as the effect of an isolated spike in neurons of the motor system. Cortical reflex myoclonus is a fragment of partial epilepsy and represents hyperactivity of a focal area of cerebral cortex. Reticular reflex myoclonus is a fragment of generalized epilepsy with hyperactivity of medullary brainstem reticular formation. Primary generalized epileptic myoclonus is a fragment of primary generalized epilepsy and may represent a generalized hyperactive response of cortex to subcortical input.

Brain↗

PubMed1993

The chromosome 6p epilepsy locus: exploring mode of inheritance and heterogeneity through linkage analysis.

Juvenile myoclonic epilepsy (JME) is a common form of adolescent-onset, generalized epilepsy. JME is genetically linked to the HLA locus on chromosome 6. Families of JME patients also have a significant recurrence of other forms of generalized epilepsy. We used the linkage data to investigate the mode of inheritance of JME and the associated electroencephalographic (EEG) traits at the HLA-linked locus. We investigated how robust the linkage results were when we changed the assumptions of mode of inheritance and penetrance and whether absence and clonic-tonic-clonic in JME families are influenced by the same gene locus as JME. Our results show that the finding of linkage is stable within a wide range of assumptions of penetrance and mode of inheritance, and that the EEG traits seen in unaffected family members reflect the actions of the same gene that is involved in the expression of JME. Our data also suggest that the same locus is responsible for non-JME forms of epilepsy seen in JME families, and that either different doses of the disease allele at the JME locus may lead to different epilepsy phenotypes or that another locus influences the final disease phenotype.

Adolescent↗

PubMed1990

Reflex seizures induced by calculation, card or board games, and spatial tasks: a review of 25 patients and delineation of the epileptic syndrome.

Nine patients had reflex activation of seizures by calculation, card and board games, or spatial tasks. The common denominator for these and the 16 others reported in the literature appears to be activity related to function of the parietal lobe. The clinical and EEG findings in all 25 patients support the diagnosis of primary generalized epilepsy. Seizures usually start during adolescence and consist of myoclonus, absences, and generalized convulsions. Specific inquiry about reflex activation should be carried out in patients with generalized epilepsy since this is rarely provided spontaneously. Attacks could be controlled satisfactorily in 89% of our patients. The genetic features are those of a primary generalized epileptic disorder without evidence for a specific inheritance of reflex sensitivity. Neuropsychological analysis of the stimuli points to parietal cortical dysfunction. These stimuli lead to activation of a generalized epileptic process analogous to the occipital cortical participation in the activation of generalized epileptic abnormality occurring in patients with photosensitive epilepsy.

Adolescent↗