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Treatment of Epilepsy with Electrical Status Epilepticus During Slow-Wave Sleep and Factors Related to the Response to Treatment.Purpose:To elucidate the effect of treatment of epilepsy with electrical status epilepticus during slow sleep (ESES) and factors related to the response to treatment.Methods:We studied 26 patients with ESES who were admitted to Okayama University Hospital (age range, 2y0m to 9y7m). Patients were treated with one or more of the following therapies: (a) high-dose valproate (VPA) therapy (plasma level,>100μg/ml), (b) a combination therapy of VPA and ethosuximide (ESM), (c) a short cycle of high-dose diazepam (DZP; 0.54–1 mg/kg/day, orally or intrarectally for 6–14 days), and (d) synthetic adrenocorticotropic hormone (ACTH) therapy (Cortrosyn-Z; 0.01–0.036 mg/kg/day, intramuscularly, for 11–43 days). Basically, we started treatment with high-dose VPA therapy. When this failed or yielded adverse side effects, we tried the combination therapy of VPA and ESM. When both were unsuccessful, we tried a short cycle of high-dose DZP or synthetic ACTH therapy. All patients were followed up for≥2 years. We also investigated response patterns to treatments and mental outcome in each patient.Results:Treatment effects: Regarding the initial effects, treatment was defined as effective when continuous spike–waves during slow-wave sleep (CSWSs) were suppressed (≥1 month with markedly decreased or no diffuse spike-waves during slow-wave sleep). In terms of initial effect, high-dose VPA was effective in 12 (50%) of 24 trials; combination therapy with VPA and ESM in six (43%) of 14 trials; a short cycle of high-dose DZP, in six (75%) of eight trials; and synthetic ACTH, in three (43%) of seven trials. However, CSWSs recurred within 1 year of treatment in two patients treated with high-dose VPA, in four treated with a short cycle of high-dose DZP, and in all three treated with synthetic ACTH. Response patterns and mental outcomes: Subjects were divided into two groups: 15 patients with favorable EEG response to the initial treatment maintained throughout the clinical course (group A), and 11 patients with no favorable EEG response to the initial treatment, or deterioration after an initial favorable response (group B). All patients except one in group A were treated with high-dose VPA or the combination therapy of VPA and ESM or both. All patients except two in group B were treated with a short cycle of high-dose DZP or synthetic ACTH after the failure of high-dose VPA or both or the combination therapy of VPA and ESM or both. Comparing these two groups, ages at onset of ESES were significantly lower in group B (p= 0.0009). Organic brain lesions such as brain malformations were seen more often in group B. Mental deterioration after the onset of ESES was observed significantly more often in group B (p= 0.037). Regarding mental outcome, patients with normal mentality or slight mental retardation predominated in group A, whereas those with severe mental retardation were found only in group B.Conclusions:Remission of ESES was achieved in approximately half of the patients treated with high-dose VPA therapy or combination therapy with VPA and ESM or both. Even when these treatments were ineffective, a short cycle of high-dose DZP or synthetic ACTH therapy was effective in most cases. However, the effects of the latter two treatments were only temporary. Group B was characterized by early-onset patients associated with organic brain lesions. These patients responded poorly to treatment and consequently had mental deterioration and poor mental outcomes. Thus response to ESES treatment may be related to the underlying organic brain lesion(s). To prevent poor mental outcome, we believe that it is critical to achieve remission as early as possible by using intensive treatment. An Investigation of Growth and Growth Disorders in School-Aged Patients with Post-West Syndrome.Purpose:To contribute to improved quality of life (QOL) for school-aged children with epilepsy, we investigated growth and growth disorders in patients with the post-West syndrome, who may be at greater risk for short stature.Methods:The 26 school-aged epilepsy patients with post-West syndrome (p-WS) were studied after informed consent was obtained from the parents. The patients consisted of seven who had cryptogenic WS[M/F= 3:4; mean age, 8 years; without cerebral palsy and nutritional problem (six of seven cases, 85.7%)] and 19 who had symptomatic WS[M/F= 11:8; mean age, 8.3 years; with cerebral palsy and required tube feeding (10 of 19 cases, 52.6%)]. The cross-sectional height SD score (HSDS), ratio of bone age to chronologic age (BA/CA), serum insulin-like growth factor (IGF-1) and other biochemical markers (serum ALP, Ca, IP, total protein and total albumin) were measured. The differences between the p-WS patients, divided into cryptogenic WS and symptomatic WS, and age-matched children with epilepsy were compared. The correlation between growth disorders and some clinical factors was investigated.Results:(a) The mean HSDS and the rate of short stature (HSDS≤−2.0 SD) in the p-WS patients were−1.89 SD (−5.94 to+0.63 SD) and 11 of 26 cases (42.3%), respectively. Obvious growth retardation without bone maturational delay (BA/CA: 0.88) was noticed in the p-WS patients compared with 56 age-matched epilepsy patients we reported previously (mean HSDS,−0.75 SD; HSDS≤−2.0 SD; 10 of 56 cases, 17.9%). Especially the symptomatic WS group showed markedly shorter stature (mean HSDS,−2.34 SD) and lower serum IGF-1 and ALP levels (mean, 110 ng/ml and 465 IU/L, respectively) compared with the cryptogenic WS group, who showed growth similar to that of age-matched epilepsy controls. (b) Markedly short stature (HSDS≤−2.0 SD) and abnormally low serum IGF-1 levels (≤−2.0 SD) were observed most frequently in the patients with cerebral palsy and feeding problems (eight of 11 cases, 72.7%, and five of 11 cases, 45.5%, respectively), followed by patients with prenatal etiology and those with poor seizure prognosis. However, the therapeutic regimens and the EEG findings showed no correlation with the growth disorders (3). Severely short stature (HSDS≤−3 SD, six of 26 cases, 23.1%) was noticed exclusively in the symptomatic WS group with cerebral palsy and feeding difficulties, as a result of an almost bedridden state and malnutrition.Conclusions:We have great concerns about the QOL of school-aged children with epilepsy and previously reported their growth and growth disorders as a part of the studies to improve their QOL. In this study, we investigated the growth in p-WS patients who may be at greater risk for short stature. Marked growth retardation was observed in the p-WS patients, especially in those with symptomatic WS, compared with age-matched controls and cryptogenic WS. Definite short stature (HSDS≤−2 SD) and low IGF-1 levels were observed mostly in patients with the complications of cerebral palsy requiring tube feeding, regardless of their EEGs and therapeutic regimens. It is particularly noteworthy that markedly short stature (HSDS≤−3 SD, six of 26, 23.1%) was observed exclusively in the symptomatic WS cases with cerebral palsy and feeding difficulties. The growth disorders may be a result of severe motor disturbances, leading to an almost bedridden state, and low IGF-1 levels may be due to malnutrition. Few reports exist of growth disorders in school-aged epilepsy patients, especially in the p-WS patients. As a base for comprehensive medicine for children with epilepsy to improve QOL, further accumulation of findings on growth and growth disorders is required. Long-Term Prognosis of Infantile Spasms (ISs): Late Relapses of Other Seizures After the Last IS.Purpose:The timing of discontinuation of drug treatment should vary with individual epileptic syndromes. Epilepsies with early onset probably deserve protracted periods of treatment (>5 years) because they are usually severe. In such cases, late relapses of other seizures are not uncommon, whereas precise studies are not known. In this study, we analyzed the long-term prognosis of infantile spasms (ISs) with special reference to late relapses.Methods:Between1969 to 2001, 52 children with ISs were treated at our facilities. Among them, 35 patients (19 boys and 16 girls) who had been followed up for>5 years were analyzed in this study. The follow-up period ranged from 5 to 33 years, with an average of 15.3 years. Adrenocorticotropic hormone (ACTH) therapy was used in 26 cases. Patients were divided into three groups according to the seizure status after the last IS; group I (G-I), eight seizure-free cases after the last IS; group II (G-II), eight cases with a seizure-free period of>5 years; and group III (G-III): 19 cases who continued to have seizures or relapsed within 5 years.Results:The classification of ISs and the mental status of 35 patients were analyzed in each group. Although the ratio of cryptogenic to symptomatic cases was the same in G-I (4:4) and G-II (4:4), G-III included many symptomatic cases (3:16). Mental status was assessed by the ratio of normal mentality/mild delay/moderate delay/severe delay. It was better in G-I (2:4:2:0) than in G-II (1:2:5:0), whereas G-III had many cases with severe mental delay (0:1:5:13). Next, we analyzed the forms of other seizures after the last IS in each group. The most common form of other seizures was partial seizures in G-II (seven of eight cases), often with secondary generalization (five of seven cases), and Lennox–Gastaut syndrome in G-III (nine of 19 cases). Then late relapses of other seizures were analyzed in G-II that had eight patients with seizure-free periods for>5 years (>10 years for five of eight patients, and 5–10 years for the remaining three). Ages at relapses after the last IS ranged from 5 years 11 months to 19 years 2 month, with an average of 11 years 11 months). The drug-free period of these eight cases ranged from 0 to 7 years, with an average of 2 years 6 months. Four patients were drug free for several years. For late relapses, focal paroxysmal discharges were found in the EEGs of seven patients. Focal discharges were most commonly found in the temporal region (five cases), followed by the frontal (one case) and the occipital region (one case). The prognosis of other seizures after a long seizure-free period was good in six cases, whereas in the other two patients, seizures were refractory to treatment. Refractory cases include cryptogenic as well as symptomatic cases.Discussion:Among 35 patients with ISs followed up for>5 years, eight (23%) cases had late relapses>5 years after the last IS. Five of eight cases had seizure-free periods of>10 years, and four cases had drug-free periods of several years. Four of eight cases were cryptogenic. From these observations, long-term follow-up is necessary, even in cryptogenic cases that have been seizure free and drug free for long periods. Other seizures that occurred after long seizure-free periods were mostly partial seizures with secondary generalization. These seizures, however, were fortunately not so therapy resistant. [ABSTRACT FROM AUTHOR]

The article presents information about abstracts of various papers that will be discussed at the Poster Session of the 6th European Congress on Epileptology that will be held on June 1, 2004. One of the papers that will be discussed is "Pharmacological Outcomes in People With Learning Disability and Epilepsy," by K. Kelly. Epilepsy is common in people with a learning disability. Researchers undertook a prospective outcome study on patients with a learning disability and epilepsy referred to the Epilepsy Unit in Glasgow, Scotland, between 1998 and 2001. Other papers that will be discussed are "Outcomes of Therapy in Patients With Epilepsy," by T.V. Mitrokhina, and A.V. Lebedeva and "Outcomes of Add on Treatment With New Generation Antiepileptic Drugs for Partial Epilepsy Patients," by D. Nikodijevic-Kedeva.

The article presents information about abstracts of various papers that will be discussed at the Novartis Satellite Symposium that will be held on June 1, 2004. One of the papers that will be discussed is "Laying the Foundations for Epilepsy Diagnosis and Treatment," by P. Ryvlin. The accurate diagnosis and optimal care of patients with de novo partial seizures offer many challenges to the physician. Auras, when isolated or associated with minor loss of awareness, are often misinterpreted as reflecting psychiatric or cardiovascular disorders, leading to an average delay of several years before the epilepsy can be recognized. Other papers that will be discussed are "Modern AED Monotherapy: Fulfilling the Needs of Growing Children With Partial Seizures," by B. Bourgeois and "Time for Change? Oxcarbazepine Monotherapy in the Diverse Adult Population," by D. Schmidt.

The article presents information about abstracts of various papers that will be discussed at the Merritt-Putnam Satellite Symposium that will be held on June 2, 2004. One of the papers that will be discussed is "The Diagnostic Challenges Between Refractory and Pseudo-Refractory Epilepsy," by G.L. Holmes. Refractory epilepsy is a common and serious condition. The medical, social, and economic consequences of refractory epilepsy can be enormous. Recurrent seizures are associated with significant risks for death, physical injury, cognitive impairment, and psychosocial problems. Other papers that will be discussed are "Alteration in AED Targets As a Mechanism Underlying Drug Resistance," by H. Beck and "Is Refractory Epilepsy a Disorder of Transporter Systems?" by S.M. Sisodiya.

The article presents information about abstracts of various papers that will be discussed at the Pfizer Satellite Symposium that will be held on May 31, 2004. One of the papers that will be discussed is "Is Refractory Epilepsy Preventable?" by P. Ryvlin. Although the specific criteria for the clinical definition of refractory epilepsy can be debated, it is clear that 20%-30% of patients suffer from seizures that are not adequately controlled by pharmacological treatment. Other papers that will be discussed are "Pregabalin: What Are the Prospects for This Newest AED?" by J. French and "High Dose Versus Low Dose Treatment With Antiepileptic Drugs: How Can We Define the Optimal Approach to Epilepsy Therapy?" by P. Kwan.

The article presents information about abstracts of various papers that will be discussed at the UCB Pharma Satellite Symposium that will be held on May 30, 2004. One of the papers that will be discussed is "Sequential Monotherapy, Rather Than Add-on Therapy, Should Be Our Treatment Strategy of Choice: For," by D. Schmidt. It is widely agreed that the treatment of epilepsy should be initiated with a single drug. When the initially prescribed antiepileptic drug (AED) fails to produce seizure freedom, the choice of an alternative monotherapy will still achieve seizure control in up to 30% of cases. Other papers that will be discussed are "New AEDs Have Yet to Demonstrate That They Improve Patient Outcomes: Against," by C.E. Elger and "Sequential Monotherapy, Rather Than Add-on Therapy, Should Be Our Treatment Strategy of Choice: Against," by M.J. Brodie.

The article presents information about abstracts of various papers that will be discussed at the Discussion Group of the 6th European Congress on Epileptology that will be held on June 2, 2004. One of the papers that will be discussed is "Pharmacokinetic-Based Design of New Antiepileptics and CNS Drugs," by M. Bialer. Several of the new antiepileptic drugs (AEDs) are second generation or follow-up compounds of existing AEDs that their development was pharmacokinetic- (PK)-based design in order to overcome the PK drawbacks possessed by the parent compound. Other papers that will be discussed are "How to Design New CNS Drugs That Will Possess Antiepileptogenic Activity?" by A. Nehlig and "Mechanism-Based Design of New Antiepileptics and CNS Drugs," by C.E. Elger.

The article presents information about abstracts of various papers that will be discussed at the Discussion Group of the 6th European Congress on Epileptology that will be held on June 2, 2004. One of the papers that will be discussed is "Seizure Semiology and Ictal SPECT," by S. Asenbaum. One of the main purposes of nuclear medicine in neurology is the investigation of the epileptogenic zone (EZ) in the presurgical evaluation of patients with therapy refractory focal seizures. Other papers that will be discussed are "Ictal SPECT in Extratemporal Lobe Epilepsy: Contribution or Confusion?" by K. Tatsch and "Is There a Role for Benzodiazepine Receptor SPECT in Focal Epilepsy?" by J.O. Rinne.