SUMMARYObjective: In 2014 the European Union-funded E-PILEPSY project was launched to improve awareness of, and accessibility to, epilepsy surgery across Europe. We aimed to investigate the current use of neuroimaging, electromagnetic source localization, and imaging postprocessing procedures in participating centers. Methods: A survey on the clinical use of imaging, electromagnetic source localization, and postprocessing methods in epilepsy surgery candidates was distributed among the 25 centers of the consortium. A descriptive analysis was performed, and results were compared to existing guidelines and recommendations. Results: Response rate was 96%. Standard epilepsy magnetic resonance imaging (MRI) protocols are acquired at 3 Tesla by 15 centers and at 1.5 Tesla by 9 centers. Three centers perform 3T MRI only if indicated. Twenty-six different MRI sequences were reported. Six centers follow all guideline-recommended MRI sequences with the proposed slice orientation and slice thickness or voxel size. Additional sequences are used by 22 centers. MRI postprocessing methods are used in 16 centers. Interictal positron emission tomography (PET) is available in 22 centers; all using 18F-fluorodeoxyglucose (FDG). Seventeen centers perform PET postprocessing. Single-photon emission computed tomography (SPECT) is used by 19 centers, of which 15 perform postprocessing. Four centers perform neither PET nor SPECT in children. Seven centers apply magnetoencephalography (MEG) source localization, and nine apply electroencephalography (EEG) source localization. Fourteen combinations of inverse methods and volume conduction models are used. Significance: We report a large variation in the presurgical diagnostic workup among epilepsy surgery centers across Europe. This diversity underscores the need for highquality systematic reviews, evidence-based recommendations, and harmonization of available diagnostic presurgical methods.

Epilepsy surgery can be a successful treatment option for temporal lobe epilepsy but there are concerns about accelerated memory decline in long-term follow-up. Method: 161 adult operated (77 right, 84 left temporal resections) versus a heterogeneous group of 208 nonoperated patients with focal epilepsies were consecutively recruited and re-evaluated focusing on memory, executive functions, and vocational outcome after follow-up intervals of > 5 years (5-22 years, mean 8 ± 3 years). Results: Major losses in the operated group manifest early, at one-year follow-up. Few patients declined further. Long-term changes after surgery did not differ from those observed without surgery. The factor "surgery" caused verbal memory decline, "seizure freedom" (operated 48%, non-operated 17%) was associated with recovery of verbal memory, and "drug reduction" positively affected the course of executive functions. In terms of the critical function of verbal memory, only 3-17% of seizure-free patients showed long-term decline whereas 16-20% showed improvement (operated and non-operated). Persistent seizures were associated with decline in 12-37% and improvement in 4-12% of patients. Improvement was related to longer retest intervals and was stronger in younger patients. Independent of surgery or seizure outcome, the vocational status remained unchanged or change was negative (22%) rather than positive (3%). Conclusions: Patients' cognitive course 5-22 years after surgery is stable and may even be positive if epilepsy is controlled and drug load reduced. Depending on seizure outcome, recovery is more frequently observed than continuing decline. Recovery, however, takes time and age is a limiting factor.

We explored the current practice with respect to the neuropsychological assessment of surgical epilepsy patients in European epilepsy centers, with the aim of harmonizing and establishing common standards. Twenty-six epilepsy centers and members of "E-PILEPSY" (a European pilot network of reference centers in refractory epilepsy and epilepsy surgery), were asked to report the status of neuropsychological assessment in adults and children via two different surveys. There was a consensus among these centers regarding the role of neuropsychology in the presurgical workup. Strong agreement was found on indications (localization, epileptic dysfunctions, adverse drugs effects, and postoperative monitoring) and the domains to be evaluated (memory, attention, executive functions, language, visuospatial skills, intelligence, depression, anxiety, and quality of life). Although 186 different tests are in use throughout these European centers, a core group of tests reflecting a moderate level of agreement could be discerned. Variability exists with regard to indications, protocols, and paradigms for the assessment of hemispheric language dominance. For the tests in use, little published evidence of clinical validity in epilepsy was provided. Participants in the survey reported a need for improvement concerning the validity of the tests, tools for the assessment of everyday functioning and accelerated forgetting, national norms, and test co-normalization. Based on the present survey, we documented a consensus regarding the indications and principles of neuropsychological testing. Despite the variety of tests in use, the survey indicated that there may be a core set of tests chosen based on experience, as well as on published evidence. By combining these findings with the results of an ongoing systematic literature review, we aim for a battery that can be recommended for the use across epilepsy surgical centers in Europe.

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We describe a 35-year-old male patient presenting with depressed mood and emotional instability, who complained about severe anterograde and retrograde memory deficits characterized by accelerated long-term forgetting and loss of autonoetic awareness regarding autobiographical memories of the last 3 years. Months before he had experienced two breakdowns of unknown etiology giving rise to the differential diagnosis of epileptic seizures after various practitioners and clinics had suggested different etiologies such as a psychosomatic condition, burnout, depression, or dissociative amnesia. Neuropsychological assessment indicated selectively impaired figural memory performance. Extended diagnostics confirmed accelerated forgetting of previously learned and retrievable verbal material. Structural imaging showed bilateral swelling and signal alterations of temporomesial structures (left >right). Video-EEG monitoring revealed a left temporal epileptic focus and subclincal seizure, but no overt seizures. Antibody tests in serum and liquor were positive for glutamic acid decarboxylase antibodies. These findings led to the diagnosis of glutamic acid decarboxylase antibody related limbic encephalitis. Monthly steroid pulses over 6 months led to recovery of subjective memory and to intermediate improvement but subsequent worsening of objective memory performance. During the course of treatment, the patient reported de novo paroxysmal non-responsive states. Thus, antiepileptic treatment was started and the patient finally became seizure free. At the last visit, vocational reintegration was successfully in progress. In conclusion, amygdala swelling, retrograde biographic memory impairment, accelerated long-term forgetting, and emotional instability may serve as indicators of limbic encephalitis, even in the absence of overt epileptic seizures. The monitoring of such patients calls for a standardized and concerted multilevel diagnostic approach with repeated assessments.

Object. The purpose of this study was to retrospectively assess the objective and subjective neuropsychological outcome after epilepsy surgery in patients with bilateral Ammon's horn sclerosis (AHS).Methods. Memory and executive functions were evaluated at baseline and at follow-up in 11 surgically treated patients and compared with 8 pharmacologically treated patients with temporal lobe epilepsy and bilateral AHS. The median follow-up duration was 16 months in the surgically treated patients and 80.5 months in the pharmacologically treated group. Subjective outcome was evaluated by questionnaires and included mood, quality of life, subjective memory, and activities of daily living.Results. At the follow-up assessment, 82% of the surgically treated patients as opposed to 0% of the nonsurgery patients were seizure free. In the surgical group, nonverbal memory performance did not change significantly in any patient after surgery, but there was a floor effect in 55% of the surgical patients. Regarding verbal memory, 9% of the surgical patients improved while 73% declined, despite severe impairments already evident at baseline. In the nonsurgery control group, 13% of the patients declined in nonverbal memory (floor effect in 63%) and 25% declined in verbal memory (floor effect in 25%) at follow-up. None of the controls improved at follow-up. Executive functions remained unchanged on an impaired level in both groups. At follow-up, the patient groups did not differ significantly with respect to mood, quality of life, subjective memory, or activities of daily living. However, in most aspects, surgically treated patients reported a slightly better subjective outcome than pharmacologically treated patients and a significantly improved quality of life.Conclusions. These results suggest that beyond benefits concerning seizure control, surgically treated patients with bilateral AHS, despite already poor baseline performance, are still at risk for severe postoperative decline in memory. In the light of predominantly minor benefits on a subjective level, the findings put the overall outcome of epilepsy surgery in bilateral AHS patients into perspective. (http://thejns.org/doi/abs/10.3171/2014.7.JNS132037) key WordS • neuropsychological outcome • temporal lobe epilepsy surgery • memory • bilateral Ammon's horn sclerosis Abbreviations used in this paper: AHS = Ammon's horn sclerosis; BDI = Beck Depression Inventory; QOLIE = Quality of Life in Epilepsy Inventory; RCI = reliable change index; TLE = temporal lobe epilepsy; VLMT = Verbaler Lern-und Merkfähigkeitstest.

The main analysis did not confirm the effects of approach on memory outcome seen in our previous study. Post hoc testing, however, showed greater memory losses with the subtemporal approach. Previous findings were replicated for semantic fluency. The discrepant results are discussed on the background of the different study designs.