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Seven patients with Rasmussen encephalitis (RE) were treated with the immunosuppressant tacrolimus and followed for a median of 22.4 months. They were compared with 12 historical untreated RE patients (median follow-up 13.9 months). The tacrolimus-treated patients had a superior outcome regarding neurologic function and progression rate of cerebral hemiatrophy but no better seizure outcome. No treated patient, but 7 of 12 control patients, became eligible for hemispherectomy. Tacrolimus did not have any major side effects.

The authors evaluated a new stereotactic radiosurgical approach in seven patients with gelastic epilepsy due to hypothalamic hamartomas. Stereotactic implantation of 125I-seeds into the hamartoma was feasible in six patients. At follow-up at least 1 year after interstitial radiotherapy, two patients had become seizure-free within 2 months, and two others had only persisting auras. There were no major perioperative or postoperative side effects.

Zusammenfassung Oxcarbazepin (OXC, Handelsname Timox ®, Trileptal ®) ist ein neues Antiepileptikum zugelassen zur Mono- und Kombinationstherapie fokaler Epilepsien mit oder ohne sekundär generalisierte tonisch-klonische Anfälle für Erwachsene und Kinder ab 6 Jahren. OXC wurde zunächst als strukturelle Variation des Carbamazepin-Moleküls zielgerichtet entwickelt, um nebenwirkungsreiche Abbauprodukte von Carbamazepin (CBZ) zu vermeiden. Dennoch bestehen erhebliche und klinisch relevante Unterschiede zwischen beiden Medikamenten. Der Hauptwirkmechanismus von OXC ist im Wesentlichen ebenfalls eine Natriumstromblockade, unterscheidet sich aber dennoch von Carbamazepin durch die zusätzliche Wirkung auf unterschiedliche Kalziumkanäle. Oxcarbazepin wird über eine Reduktion zu seiner Ketoform glukuronidiert und im Urin ausgeschieden. Die Beteiligung der hepatischen Cytochrom-P-450-abhängigen Enzymsysteme ist im Unterschied zur Metabolisierung von CBZ minimal. Daher kann OXC mit anderen Antiepileptika, wie beispielsweise Valproat, besser kombiniert werden als Carbamazepin. Aus den umfangreichen praktischen Erfahrungen von mehr als 800.000 Patientenjahren ergibt sich weiterhin ein günstiges Nutzen-Risiko-Profil von OXC. Deshalb sollte OXC wegen seiner sehr guten Wirksamkeit und des günstigeren Nebenwirkungsprofil bei Kindern, Jugendlichen und Erwachsenen mit fokalen Anfällen gegenüber alten Epilepsiemedikamenten wie Carbamazepin bevorzugt werden.

BACKGROUND: Epilepsy is commonly associated with reproductive endocrine disorders. These include polycystic ovary syndrome (PCOS), isolated components of this syndrome such as polycystic ovaries, hyperandrogenaemia, hypothalamic amenorrhoea, and functional hyperprolactinaemia. OBJECTIVE: To summarise the currently known relations between epilepsy and reproductive endocrine disorders. METHODS: A review of clinical experience and published reports. RESULTS: The most likely explanations for endocrine disorders related to epilepsy or antiepileptic drugs are: (1) a direct influence of the epileptogenic lesion, epilepsy, or antiepileptic drugs on the endocrine control centres in the brain; (2) the effects of antiepileptic drugs on peripheral endocrine glands; (3) the effects of antiepileptic drugs on the metabolism of hormones and binding proteins; and (4) secondary endocrine complications of antiepileptic drug related weight changes or changes of insulin sensitivity. Regular monitoring of reproductive function at visits is recommended, including questioning about menstrual disorders, fertility, weight, hirsutism, and galactorrhoea. Particular attention should be paid to patients on valproate and obese patients or those experiencing significant weight gain. Single abnormal laboratory or imaging findings without symptoms may not constitute a clinically relevant endocrine disorder. However, patients with these kinds of abnormalities should be monitored to detect the possible development of a symptomatic disorder associated with, for example, menstrual disorders or fertility problems. CONCLUSIONS: If a reproductive endocrine disorder is found, antiepileptic drug treatment should be reviewed to ensure that it is correct for the particular seizure type and that it is not contributing to the endocrine problem. The possible benefits of a change in treatment must be balanced against seizure control and the cumulative side effect of alternative agents.

We describe two patients with psychogenic seizures of rare semiology. Both patients (a 23-year-old man and a 26-year-old woman) attended the emergency department because status epilepticus with myoclonic seizures had been diagnosed. Seizures were documented with video-electroencephalography. Semiology of seizures were brief myoclonia of both arms resulting in a short elevation of both arms without impairment of consciousness. Ictal EEG registration was without abnormal finding. Psychiatric diagnostic assessment suggested a dissociative disorder and mild depression, respectively. During psychiatric treatment seizures occurred only rarely within a 3–5 months follow-up. One should be aware that juvenile myoclonic epilepsy may be mimicked by psychogenic seizures

For evaluation of potential functional deficits, an intraarterial amobarbital test is performed prior to neurosurgical or neuroradiological interventions. To visualize individual amobarbital perfusion patterns, simultaneous injection of 99mTc-HMPAO was performed previously. The present study describes for the first time a method of coregistration of intraarterial SPECT during selective amobarbital test to MRI. Three patients undergoing selective amobarbital test of the posterior cerebral artery were included. SATSCOM (Selective amobarbital test intraarterial SPECT coregistered to MRI) was performed by skull extraction in SPECT and MRI followed by surface matching. In all three patients, SATSCOM revealed accurate matching results. With this functional–anatomical mapping, suppression of higher cortical functions can be correlated to anatomical regions. Furthermore, a more precise mapping of amobarbital effect improves planning invasive interventions, particularly those close to eloquent areas.

ObjectivesEpilepsy surgery has proved to be a successful intervention method to achieve freedom from seizures or seizure relief in children with pharmacoresistant epilepsy. Long term studies on operated children suggest that behavioural disorders, which are often seen before surgery, improve after surgery. However, the early postoperative development of behavioural problems has not been systematically evaluated.MethodsParents of 28 children with pharmacoresistant focal epilepsies completed the child behaviour checklist (CBCL) preoperatively and 3 months after surgery. Surgeries comprised 24 focal resections (13 temporal, 11 extratemporal), two hemispherectomies, and two callosotomies. Twenty eight conservatively treated children with comparable CBCL scores served as a control group. A repeated measurement multivariate analysis of variance (MANOVA) and a regression analysis were computed to compare the development of behaviour between both groups and to identify predictors of postoperative changes in behaviour.ResultsPreoperatively 39% of the children exhibited significant behavioural problems, a further 11% were within the borderline range. The MANOVA disclosed a significant interaction between time of examination and group (F=2.23, p<0.05). The surgery group showed significant improvements on the scales "internalising problems", "externalising problems", "attention problems", and "thought problems". Behavioural problems in the control group, however, remained unchanged. No changes were seen in social problems in both groups. The significant predictor of total behavioural improvement was a good seizure outcome (R²=0.11, p<0.05). Age, sex, onset, and duration of epilepsy, the site of the focus, and changes in antiepileptic drug regimen did not influence changes in behaviour.ConclusionsThe data demonstrate an early improvement of behavioural problems after epilepsy surgery in children. The behavioural improvements can be assumed to result directly from the removal of the epileptic focus. They are not predictable on the basis of information available preoperatively, but depend on the seizure outcome.

We investigated the impact of temporal lobe epilepsy surgery on sex hormones and menstrual cycles. Sixteen female patients with temporal lobe epilepsy were investigated prior to surgery and 3, 6, and 12 months after surgery. The patients received carbamazepine (CBZ) as monotherapy (10 patients ) or in combination with other antiepileptic drugs (six patients ). Antiepileptic drugs were maintained after surgery. During the 1-year follow-up after surgery eight patients (50%) remained completely free of seizures. In another four patients (25%) only rare disabling seizures occurred. There were no significant differences between pre-surgical and post-surgical serum concentrations of testosterone, free testosterone, prolactin, dehydroepiandrosterone sulfate, growth hormone, cortisol and sex hormone binding globulin. There was, however, a significant increase in serum androstenedione concentration 6 months post-surgically (P< 0.02). Documentation of menstrual cycles in addition to laboratory parameters revealed individual post-surgical changes of the menstrual cycle in eight patients . Four patients had a change in menstrual periodicity: two patients with complete seizure control had regular cycles instead of oligomenorrhoea and two patients with incomplete seizure control had oligomenorrhoea instead of regular cycles. These data indicate that at least in some patients with temporal lobe epilepsy surgical treatment influences menstrual periodicity.

Idiopathic generalized epilepsy (IGE) comprises a heterogeneous group of disorders, in which a high genetic predisposition and a complex mode of inheritance have been suggested. However, genes, which confer liability to common IGE subtypes including juvenile myoclonic epilepsy (JME) and childhood absence epilepsy (CAE) have not been identified so far. Here, we tested the hypothesis that genetic variation in the human homolog of the ««activity-regulated cytoskeleton-associated gene»» (ARC) contributes to the etiology of common IGE disorders. The gene has recently been mapped to chromosome 8q24.3, a region which spans previously identified major IGE susceptibility loci. A systematic search for mutations was performed in 143 patients with a known family history of IGE. However, no evidence for functional variants was found in theARCcoding sequence. Nevertheless, we detected a novel common C489T single nucleotide polymorphism, which provides a useful marker in genetic linkage and association studies. By performing a population- and family-based study we however failed to show significant association between this novel single nucleotide polymorphism and IGE, a finding, which most likely rules out that genetic variation in or close to the ARCgene confers liability to common IGE subtypes.

ObjectivesExisting speculations regarding the cognitive functions of the right hippocampus suggest a special involvement in non-verbal memory functions. However, all supporting evidence in epileptic patients came from postoperative studies that did not consider the preoperative functioning level. The present study investigates in patients with right temporal lobe epilepsy (RTLE), both preoperatively and postoperatively, the role of the right hippocampus for visual memory, visuoconstruction, and spatial visualisation. Methods Two groups of patients with RTLE with and without right hippocampal damage (HC ⁺/n=15 v HC ^-/n=13) were set up retrospectively thereby taking into account the surgical treatment (selective amygdalohippocampectomy v temporal resections sparing the hippocampus), the postoperative pathological findings (hippocampal sclerosis in the HC ⁺ group) and the seizure outcome (all seizure free). The two groups were compared with regard to their neuropsychological performance preoperatively and 3 months after temporal lobe surgery. Results The preoperative results indicated impaired visual memory performance only for the HC ⁺ patients, whereas the HC ^- patients exhibited no deficiencies in any of the assessed functions. Postoperatively, both groups exhibited partially improved performances. The type of surgical treatment had no different effects on the postoperative performance changes. Conclusions The results suggest that hippocampal damage is the major cause of non-verbal memory deficiencies in preoperative patients with right temporal lobe epilepsy. Hence the results support and extend findings from previous postoperative studies. Right hippocampal resections did not increase the preoperative visual memory deficiencies, thus supporting the view that right temporal lobe resection in general bears only small risks for cognitve deteriorations.

Severe focal epilepsy is regarded as a clinical hallmark of Rasmussen encephalitis (RE). The authors report two children with progressive hemiparesis, contralateral hemispheric atrophy, and pathologic features characteristic for RE. At histologic diagnosis and over several months, neither patient experienced seizures. The report enlarges the clinical spectrum of RE and suggests that seizures are not an obligatory presenting symptom of the disorder.