Purpose: To determine hippocampal expression of neuronal GABA-transporter (GAT-1) and glial GABAtransporter (GAT-3) in patients with temporal lobe epilepsy (TLE) and hippocampal sclerosis (HS).Methods: Hippocampal sections were immunohistochemically stained for GABA-transporter 1 and GABA-transporter-3, followed by quantification of the immunoreactivity in the hilus by optical density measurements. GABA-transporter 3 positive hilar cells were counted and GABA-transporter protein expression in sections that included all hippocampal subfields was quantified by Western blot.Results: The hilar GABA-transporter 1 expression of patients with severe hippocampal sclerosis was about 7% lower compared to that in the mild hippocampal sclerosis/control group (p < 0.001). The hilar GABA-transporter 3 expression was about 5% lower in the severe hippocampal sclerosis group than in the mild hippocampal sclerosis/control group (non-significant). Also, severe hippocampal sclerosis samples contained 34% less (non-significant) GABA-transporter 3 positive cells compared to that of controls.Protein expression as assessed by Western blot showed that GABA-transporter 1 was equally expressed in mild and severe bippocampal sclerosis samples, whereas GABA-transporter 3 was reduced by about 62% in severe hippocampal sclerosis samples (p < 0.0001).Conclusion: These data confirm that GABA-transporter expression is spatially and isoform-specific reduced and GABA-transporter 3 positive cell numbers are unchanged in hippocampal sclerosis. Implications for the use of GABAergic antiepileptic therapies in hippocampal sclerosis vs non-hippocampal sclerosis patients remain to be studied. (C) 2015 Elsevier B.V. All rights reserved.

Background and purpose In the epilepsy community, there is talk that the number of classical patients with early onset temporal lobe epilepsy ( TLE ) and A mmon's horn sclerosis ( AHS ) is decreasing. This is counterintuitive, considering the success story of epilepsy surgery, improved diagnostic methods and the current recommendation of early admission to surgery. In order to recognize trends, the development of temporal lobe surgery over 20 years in three major G erman epilepsy centers was reviewed. Methods Age at surgery and duration of epilepsy, which was differentiated according to histopathology ( AHS , developmental, tumor, vascular), year of surgery and center, were evaluated in a cohort of 2812 patients from three German epilepsy centers who underwent temporal lobe surgery between 1988 and 2008. The analysis was carried out for the pooled cohort as well as for each center separately. Results Of all patients, 52% showed AHS . Compared with other pathologies, the AHS group had the earliest epilepsy onset and the longest duration of epilepsy. Across five time epochs, the diagnosis of AHS increased in the first epoch, remaining constant thereafter. Contrary to the trends in other pathologies, in the AHS group the mean age of patients at surgery increased by 7 years and the duration of epilepsy until surgery increased by 5 years. This trend could be replicated in all three centers. As initially hypothesized for all groups, age and duration of epilepsy in other pathology groups remained constant or indicated earlier submission to surgery. Conclusions During the first few years studied, most probably due to progress in brain imaging, the proportion of patients with AHS increased. However, despite stable numbers over time, and contrary to the trends in other pathology groups, age and duration of epilepsy in mesial TLE with AHS ( mTLE + AHS ) increased over time. This supports the hypothesis of a decreasing incidence of AHS . This trend is discussed with respect to disease‐modifying factors which have changed the incidence of classical mTLE + AHS or, alternatively, to recent developments in antiepileptic drug treatment, the appraisal of surgery and economic incentives for treatment options other than surgery.

Introduction: After a first epilepsy surgery, that did not reach the attempted success, the outcome of a reoperation is unclear. The aim of the study is to give an idea of risks and benefits of epilepsy reoperations to the parents and the referring doctors.

Es wird über 2 Patienten berichtet, die im Rahmen einer Epilepsie eine „out-of-body experience“ (OBE) erlebten. Die autoskopische Illusion wurde konstant lateralisiert wahrgenommen. Eine Patientin hatte eine kontralateral zur Lokalisation der autoskopischen Wahrnehmung gelegene Läsion; bei dem anderen Patienten bestand der Verdacht darauf. Bei Patienten mit autoskopischer Wahrnehmung sollte in jedem Fall nach einer Lateralisation gefragt werden, da dies ein Hinweis auf eine kontralaterale gelegene Störung sein könnte. Generell sollten Patienten explizit nach autoskopischen Wahrnehmungen gefragt werden, da sie hierüber vermutlich selten spontan berichten werden. Limitiert wird die Aussagekraft der Hypothese der kontralateral zur autoskopischen Illusion gelegenen zerebralen Läsion durch den retrospektiven Charakter der Datenerhebung und durch die geringe Zahl der in der Literatur veröffentlichten Fälle über eine Lateralisation des Doppelgängers im Gesichtsfeld oder zu einer Körperseite. Ob dies daran liegt, dass eine Lateralisation nicht ermittelt worden ist, oder ob tatsächlich selten eine Lateralisation vorgelegen hat, kann nur durch weiterführende prospektive Untersuchungen geklärt werden.

The efficacy of Levetiracetam (LEV) in postoperative, nonseizure-free patients has been shown in previous studies. In the following article, seizure outcome after re-administrating LEV in 10 patients who had not become seizure-free after surgery and who had not responded to LEV before surgical intervention is reviewed. Seven patients became seizure-free using lower doses of LEV compared to the dose administered prior to surgery. (Five of 7 patients became completely free of auras and seizures and 2 patients had only auras.) In two further patients, seizure frequency decreased by >50%. Only in 1 patient was no improvement observed. Levetiracetam is effective in patients who have undergone surgery but did not become seizure-free after the operation. Furthermore, the majority of patients who did not respond to LEV before surgery responded after surgery.

Objective Limbic encephalitis is rare in people <18 years of age and rarely given a formal diagnosis.Design Retrospective study on presentation and outcome of children and adolescents with the clinico-radiological syndrome of limbic encephalitis tested for specific neuronal autoantibodies (Abs) over 3.5 years.Setting Assessment, diagnosis, treatment and follow-up at 12 neuropaediatric and neurological departments in Europe, with Abs determined in Bonn, Germany and Oxford, UK.Patients Ten patients <18 years of age who presented with a disorder mainly affecting the limbic areas of <5 years' duration with MRI evidence of mediotemporal encephalitis (hyperintense T2/FLAIR signal, resolving over time).Results Median age at disease onset was 14 years (range 3-17). Eight patients had defined Abs: one each with Hu or Ma1/2 Abs, four with high titre glutamic acid decarboxylase (GAD) Abs, two of whom had low voltage-gated potassium channel (VGKC) Abs and two with only low titre VGKC Abs. A tumour was only found in the patient with Hu Abs (a neuroblastoma). After a median follow-up of 15 months with corticosteroid or intravenous immunoglobulin treatment, starting after a median of 4 months, two patients recovered, eight remained impaired and one died.Conclusions Limbic encephalitis is a disease that can occur in childhood or adolescence with many of the hallmarks of the adult disorder, suggesting that both result from similar pathogenic processes. Since most of the cases were non-paraneoplastic, as now also recognised in adults, more systematic and aggressive immunotherapies should be evaluated in order to improve outcomes.

Objective: Psychiatric disorders, particularly psychosis, depression, personality and behavioral disorders, mental retardation and anxiety disorders, are frequent comorbidities in patients with epilepsy. According to our own recent data, 19.6% of patients with refractory focal epilepsy also have a diagnosis of anxiety disorder. The purpose of the study reported here was to evaluate the efficacy and safety of pregabalin (PGB) in patients with comorbid epilepsy and anxiety disorder.

Background:One of the possible pathomechanisms of sudden death in epilepsy (SUDEP) is a postictal dysregulation of autonomic nervous system. We performed a heart rate variability (HRV) analysis of the periictal state to analyze whether a cardiac autonomic disturbance exists after an epileptic seizure.Methods:We included 31 periictal video-EEG-ECG recordings of 31 patients with epilepsy who had consecutively undergone pre-surgical evaluation. Nine generalized tonic-clonic (GTCS), 15 complex partial, and seven simple motor seizures were included. HRV was evaluated by analyzing 5-min-long ECG epochs, sampling from baseline, direct preictal, early-postictal (< 15 min after the seizure), and late-postictal (5-6 h after the seizure) periods.Results:The heart rate was elevated immediately after the seizures, but 5-6 h postictally returned to the baseline level. Time-domain components of HRV decreased after the seizure and this decrease lasted for 5-6 h. Low-frequency power decreased in the early-postictal phase and high-frequency power of HRV dropped in the late-postictal phase. GTCS had an impact on short-term but not on long-term postictal HRV decrease.Conclusions:We found decreased HRV immediately after the seizures, which lasted at least 5-6 h postictally, indicating a long-term postictal disturbance of the autonomous nervous system. GTCS were accompanied by a more decreased HRV than other seizures. Our results may have relevance in explaining pathomechanism of SUDEP.

Ziele: Die hochauflösende MRT ist das wichtigste bildgebende Verfahren zur Fokussuche bei Patienten mit fokaler Epilepsie. Das häufigste pathologische Substrat einer fokalen Epilepsie temporaler Semiologie ist die einseitige Hippocampussklerose. Ziel dieser Untersuchung ist die Implementierung und Evaluation eines dedizierten MR-Epilepsieprotokols von 1,5 Tesla bei 7 Tesla mit möglichst hoher örtlicher Auflösung bei Patienten mit bekannter Hippocampussklerose. Methode: Sieben Patienten mit fokaler Epilepsie und bei 1,5 Tesla diagnostizierter Hippocampussklerose wurden bei 7 Tesla (Magnetom 7T, Siemens, Erlangen, 8-Kanal-Sende-/Empfangs-Kopfspule, Rapid Biomedical, Würzburg) mit PD-/T2-, T2*-, T1- und FLAIR-gewichteten Sequenzen untersucht. Die Gesamtmesszeit betrug maximal 90 Minuten pro Patient. Ergebnis: Die maximale, nicht-interpolierte örtliche Auflösung erreichte 0,2×0,2×3 mm3 in T2*-gewichteten bzw. 0,5×0,5×3 mm3 in den übrigen Sequenzen. Zusammen mit „neuen“ suszeptibilitätsbedingten Kontrasten ermöglichte die hohe räumliche Auflösung eine gegenüber 1,5T deutlich verbesserte in vivo Abgrenzbarkeit der hippocampalen Binnenstrukturen. Allerdings verstärkten sich auch störende Suszeptibilitätsartefakte an der Schädelbasis bei einzelnen Patienten deutlich. Bedingt durch das hohe B0-Feld ist zudem das in einer Sequenz zu untersuchende Volumen durch SAR-Limitationen eingeschränkt. Schlussfolgerung: Fokale Epilepsien sind prinzipiell neurochirurgisch behandelbar, wenn ein operabler epileptogener Focus lokalisiert werden kann. Daher sollten Fortschritte in der hochauflösenden MR-Bildgebung die diagnostische Wertigkeit erhöhen. Die Möglichkeit höchstauflösender in vivo MR-Bildgebung beim Menschen und „neuer“ Kontraste führt zu einer deutlich verbesserten Darstellung hippocampaler Binnenstrukturen und sollte daher zu einer verbesserten Detektion kleinster kortikaler –auch intrahippocampaler-epileptogener Läsionen und damit zu einer Verringerung der „MR-negativen“ Patienten mit fokaler Epilepsie führen.

Bei einem eineiigen Zwilling mit prolongiertem Fieberkrampf im Alter von 8 Monaten begann eine linksseitige mesiale Temporallappenepilepsie im Alter von 2 Jahren, im MRT mit Darstellung einer linksseitigen mesialen temporalen Sklerose. Der zweite Zwilling hatte zunächst häufige einfache Fieberkrämpfe, im Alter von 4 Jahren im Rahmen einer Meningitis nach kurzen Fieberkrämpfen eine längere Bewusstlosigkeit ohne folgende Epilepsie und mit normalem Befund im MRT, einschließlich T2-Relaxometrie. Entsprechend auch weiterer Literatur zeigt diese Falldarstellung einen genetischen Faktor für Fieberkrämpfe und spricht für das Risiko der Entwicklung einer mesialen Temporallappenepilepsie nach prolongiertem Fieberkrampf bei Kindern, die jünger als 4 Jahre sind.