A Homozygous PTRHD1 Missense Variant (p.Arg122Gln) in an Individual with Intellectual Disability, Generalized Epilepsy, and Juvenile Parkinsonism.

Biallelic variants in PTRHD1 have been associated with autosomal recessive intellectual disability, spasticity, and juvenile parkinsonism, with few reported cases. Here, we present the clinical and genetic findings of a female of Austrian origin exhibiting infantile neurodevelopmental abnormalities, intellectual disability, and childhood-onset parkinsonian features, consistent with the established phenotypic spectrum. Notably, she developed genetic generalized epilepsy at age 4, persisting into adulthood. Using diagnostic exome sequencing, we identified a homozygous missense variant (c.365G > A, p.(Arg122Gln)) in PTRHD1 (NM_001013663). In summary, our findings not only support the existing link between biallelic PTRHD1 variants and parkinsonism with neurodevelopmental abnormalities but also suggest a potential extension of the phenotypic spectrum to include generalized epilepsy.

Visual outcomes after anterior temporal lobectomy and transsylvian selective amygdalohippocampectomy: A quantitative comparison of clinical and diffusion data.

OBJECTIVE: Anterior temporal lobectomy (ATL) and transsylvian selective amygdalohippocampectomy (tsSAHE) are effective treatment strategies for intractable temporal lobe epilepsy but may cause visual field deficits (VFDs) by damaging the optic radiation (OpR). Due to the OpR's considerable variability and because it is indistinguishable from surrounding tissue without further technical guidance, it is highly vulnerable to iatrogenic injury. This imaging study uses a multimodal approach to assess visual outcomes after epilepsy surgery. METHODS: We studied 62 patients who underwent ATL (n = 32) or tsSAHE (n = 30). Analysis of visual outcomes was conducted in four steps, including the assessment of (1) perimetry outcome (VFD incidence/extent, n = 44/40), (2) volumetric OpR tractography damage (n = 55), and the (3) relation of volumetric OpR tractography damage and perimetry outcome (n = 35). Furthermore, (4) fixel-based analysis (FBA) was performed to assess micro- and macrostructural changes within the OpR following surgery (n = 36). RESULTS: Altogether, 56% of all patients had postoperative VFDs (78.9% after ATL, 36.36% after tsSAHE, p = .011). VFDs and OpR tractography damage tended to be more severe within the ATL group (ATL vs. tsSAHE, integrity of contralateral upper quadrant: 65% vs. 97%, p = .002; OpR tractography damage: 69.2 mm3 vs. 3.8 mm3 , p = .002). Volumetric OpR tractography damage could reliably predict VFD incidence (86% sensitivity, 78% specificity) and could significantly explain VFD extent (R2  = .47, p = .0001). FBA revealed a more widespread decline of fibre cross-section within the ATL group. SIGNIFICANCE: In the context of controversial visual outcomes following epilepsy surgery, this study provides clinical as well as neuroimaging evidence for a higher risk and greater severity of postoperative VFDs after ATL compared to tsSAHE. Volumetric OpR tractography damage is a feasible parameter to reliably predict this morbidity in both treatment groups and may ultimately support personalized planning of surgical candidates. Advanced diffusion analysis tools such as FBA offer a structural explanation of surgically induced visual pathway damage, allowing noninvasive quantification and visualization of micro- and macrostructural tract affection.

Diagnostic exome sequencing in non-acquired focal epilepsies highlights a major role of GATOR1 complex genes.

BACKGROUND: The genetic architecture of non-acquired focal epilepsies (NAFEs) becomes increasingly unravelled using genome-wide sequencing datasets. However, it remains to be determined how this emerging knowledge can be translated into a diagnostic setting. To bridge this gap, we assessed the diagnostic outcomes of exome sequencing (ES) in NAFE. METHODS: 112 deeply phenotyped patients with NAFE were included in the study. Diagnostic ES was performed, followed by a screen to detect variants of uncertain significance (VUSs) in 15 well-established focal epilepsy genes. Explorative gene prioritisation was used to identify possible novel candidate aetiologies with so far limited evidence for NAFE. RESULTS: ES identified pathogenic or likely pathogenic (ie, diagnostic) variants in 13/112 patients (12%) in the genes DEPDC5, NPRL3, GABRG2, SCN1A, PCDH19 and STX1B. Two pathogenic variants were microdeletions involving NPRL3 and PCDH19. Nine of the 13 diagnostic variants (69%) were found in genes of the GATOR1 complex, a potentially druggable target involved in the mammalian target of rapamycin (mTOR) signalling pathway. In addition, 17 VUSs in focal epilepsy genes and 6 rare variants in candidate genes (MTOR, KCNA2, RBFOX1 and SCN3A) were detected. Five patients with reported variants had double hits in different genes, suggesting a possible (oligogenic) role of multiple rare variants. CONCLUSION: This study underscores the molecular heterogeneity of NAFE with GATOR1 complex genes representing the by far most relevant genetic aetiology known to date. Although the diagnostic yield is lower compared with severe early-onset epilepsies, the high rate of VUSs and candidate variants suggests a further increase in future years.

Field testing of ICHD-3 beta criteria of periictal headaches in patients with focal epilepsy - a prospective diary study.

Background To date we are lacking prospective data for field testing of ICHD-3 beta criteria for periictal headache (PIH). Methods Patients with focal epilepsy diagnosed by means of prolonged video-EEG monitoring completed a paper-pencil diary for three months and recorded seizures and headaches on a daily basis. According to ICHD-3 beta, we classified PIH, defined as headache present on a day with at least one seizure, as "7.6 headache related to epileptic seizure", "7.6.1 hemicrania epileptica" or "7.6.2 postictal headache". In addition, we compared the ICHD-3 beta diagnoses to the diagnoses according to ICHD-2. Results Thirty two patients completed the diary. Data analysis included 2,668 patient days, 300 seizures and 37 episodes of PIH. Two of these episodes (5.4%) were classified as headache related to seizure, three (8.1%) fulfilled both the criteria of headache related to seizure and hemicrania epileptica and four (10.8%) were postictal headaches. Twenty eight episodes (75.7%) did not fulfil any of the ICHD-3 beta criteria of seizure-related headaches, mostly because headache onset was before seizure onset. Applying ICHD-2 criteria allowed only one single episode of PIH to be classified as postictal headache. Discussion Our study is the first to present prospective field testing data of the ICHD-3 beta criteria for three types of seizure-related headaches. The majority of PIH episodes do not fulfil any of these criteria. One quarter can be classified according to ICHD-3 beta, whereas purely clinical diagnosis of PIH is markedly restricted in ICHD-2 because of mandatory electroencephalographic evidence.

Seizures After Adult Cardiac Surgery and Interventional Cardiac Procedures.

The aim of this narrative review is to evaluate the incidence of seizures after adult cardiac surgery or cardiac interventions, to describe risk factors, and to provide suggestions regarding diagnostic measures and proper management. Based on published peer-reviewed articles, the authors demonstrate specific procedure-related risks for seizures. Early diagnosis, the identification of underlying causes, and avoidance of amenable risk factors are crucial to reduce associated long-term morbidity and mortality. Methods of early recognition of seizures, particularly focusing on the initiation of appropriate diagnostic measures, their management, and their timely treatment, are presented in the article.

Psychoses in epilepsy: A comparison of postictal and interictal psychoses.

We retrospectively analyzed data of patients with epilepsy (n=1434) evaluated with prolonged EEG monitoring in order to estimate the prevalence of postictal psychosis (PP) and interictal psychosis (IP), to investigate a potential association of psychosis subtype with epilepsy type, and to assess differences between PP and IP. The overall prevalence of psychosis was 5.9% (N=85); prevalence of PP (N=53) and IP (N=32) was 3.7% and 2.2%, respectively. Of patients with psychosis, 97.6% had localization-related epilepsy (LRE). Prevalence of psychosis was highest (9.3%) in patients with temporal lobe epilepsy (TLE). When comparing PP with IP groups on demographic, clinical, and psychopathological variables, patients with IP were younger at occurrence of first psychosis (P=0.048), had a shorter interval between epilepsy onset and first psychosis (P=0.002), and more frequently exhibited schizophreniform traits (conceptual disorganization: P=0.008; negative symptoms: P=0.017) than those with PP. Postictal psychosis was significantly associated with a temporal seizure onset on ictal EEG (P=0.000) and a higher incidence of violent behavior during psychosis (P=0.047). To conclude, our results support the presumption of a preponderance of LRE in patients with psychosis and that of a specific association of TLE with psychosis, in particular with PP. Given the significant differences between groups, PP and IP may represent distinct clinical entities potentially with a different neurobiological background.

Depressive symptoms predict the quality of sleep in patients with partial epilepsy--A combined retrospective and prospective study.

INTRODUCTION: Epilepsy is one of the most common neurological diseases and has many detrimental effects on the patients' well-being as well as sleep quality. The aim of this study was to assess the subjective quality of sleep and influencing factors on subjective sleep quality in patients with partial epilepsy using a combined retrospective and prospective study design. METHODS: We conducted a combined retrospective and prospective study in patients with partial epilepsy and analyzed subjective ratings of sleep quality in 32 patients (17 female, 15 male; mean age: 40.41 ± 12.67 years, range: 20-64) with partial epilepsy (mean duration of epilepsy diagnosis: 18.31 ± 13.26 years) and 32 healthy gender-matched and age-matched controls. All patients filled out a seizure diary for 90 days, which included the number, duration, and type (partial vs. secondary generalized) of epileptic seizures and intake of antiepileptic and sleep medications. At baseline, all participants completed the Pittsburgh Sleep Quality Index (PSQI), Epworth Sleepiness Scale (ESS), Beck's Depression Inventory (BDI), and Beck Anxiety Inventory (BAI). Poor sleepers were defined by a PSQI score of ≥ 5. RESULTS: Twenty-three patients (72%) reported 15.17 ± 25.54 seizures in the previous three months, and nine (28%) patients reported being seizure-free. During the 90-day diary period, twenty-two patients (69%) documented a total of 319 epileptic seizures, while ten patients (31%) reported that they were seizure-free. The mean PSQI score of all patients was 4.88 ± 2.92 (range: 1-14) and the mean ESS score was 5.25 ± 2.98 (range: 0-10). The mean PSQI score of the control group was 3.25 ± 1.57 (range: 1-6), and their mean ESS score was 6.72 ± 3.48 (range: 0-14). The comparison of the two groups showed a significantly higher PSQI score in the patient group (t = 2.778, p = 0.008), but no statistically significant difference regarding their ESS score (t = -1.811, p = 0.075). Sixteen (50%) patients were poor sleepers. Good sleepers showed a significantly lower PSQI (2.69 ± 1.08 vs. 7.06 ± 2.49; p < 0.001) and BDI scores (2.38 ± 2.50 vs. 9.63 ± 7.63; p < 0.002) than poor sleepers. Linear regression analysis showed that the BDI score was the significant predictor for the PSQI score (estimate: 0.2019; p = 0.00819) and for the ESS score (estimate: 0.2251; p = 0.0321). CONCLUSION: In patients with partial epilepsy, a higher depression score was the best predictor for a poor subjective sleep quality and increased daytime sleepiness.

Which parameters influence cognitive, psychiatric and long-term seizure outcome in mesial temporal lobe epilepsy after selective amygdalohippocampectomy?

BACKGROUND: We aimed to analyze potentially prognostic factors which could have influence on postoperative seizure, neuropsychological and psychiatric outcome in a cohort of patients with mesial temporal lobe epilepsy (MTLE) due to hippocampal sclerosis (HS) after selective amygdalohippocampectomy (SAHE) via transsylvian approach. METHODS: Clinical variables of 171 patients with drug-resistant MTLE with HS (88 females) who underwent SAHE between 1994 and 2019 were evaluated using univariable and multivariable logistic regression models, to investigate which of the explanatory parameters can best predict the outcome. RESULTS: At the last available follow-up visit 12.3 ± 6.3 years after surgery 114 patients (67.9%) were seizure-free. Left hemispheric MTLE was associated with worse postoperative seizure outcome at first year after surgery (OR = 0.54, p = 0.01), female sex-with seizure recurrence at years 2 (OR = 0.52, p = 0.01) and 5 (OR = 0.53, p = 0.025) and higher number of preoperative antiseizure medication trials-with seizure recurrence at year 2 (OR = 0.77, p = 0.0064), whereas patients without history of traumatic brain injury had better postoperative seizure outcome at first year (OR = 2.08, p = 0.0091). All predictors lost their predictive value in long-term course. HS types had no prognostic influence on outcome. Patients operated on right side performed better in verbal memory compared to left (VLMT 1-5 p < 0.001, VLMT 7 p = 0.001). Depression occurred less frequently in seizure-free patients compared to non-seizure-free patients (BDI-II Z = - 2.341, p = 0.019). CONCLUSIONS: SAHE gives an improved chance of achieving good postoperative seizure, psychiatric and neuropsychological outcome in patients with in MTLE due to HS. Predictors of short-term outcome don't predict long-term outcome.

Implementation of a 7T Epilepsy Task Force consensus imaging protocol for routine presurgical epilepsy work-up: effect on diagnostic yield and lesion delineation.

OBJECTIVE: Recently, the 7 Tesla (7 T) Epilepsy Task Force published recommendations for 7 T magnetic resonance imaging (MRI) in patients with pharmaco-resistant focal epilepsy in pre-surgical evaluation. The objective of this study was to implement and evaluate this consensus protocol with respect to both its practicability and its diagnostic value/potential lesion delineation surplus effect over 3 T MRI in the pre-surgical work-up of patients with pharmaco-resistant focal onset epilepsy. METHODS: The 7 T MRI protocol consisted of T1-weighted, T2-weighted, high-resolution-coronal T2-weighted, fluid-suppressed, fluid-and-white-matter-suppressed, and susceptibility-weighted imaging, with an overall duration of 50 min. Two neuroradiologists independently evaluated the ability of lesion identification, the detection confidence for these identified lesions, and the lesion border delineation at 7 T compared to 3 T MRI. RESULTS: Of 41 recruited patients > 12 years of age, 38 were successfully measured and analyzed. Mean detection confidence scores were non-significantly higher at 7 T (1.95 ± 0.84 out of 3 versus 1.64 ± 1.19 out of 3 at 3 T, p = 0.050). In 50% of epilepsy patients measured at 7 T, additional findings compared to 3 T MRI were observed. Furthermore, we found improved border delineation at 7 T in 88% of patients with 3 T-visible lesions. In 19% of 3 T MR-negative cases a new potential epileptogenic lesion was detected at 7 T. CONCLUSIONS: The diagnostic yield was beneficial, but with 19% new 7 T over 3 T findings, not major. Our evaluation revealed epilepsy outcomes worse than ILAE Class 1 in two out of the four operated cases with new 7 T findings.

Psychiatric symptoms and comorbidities in patients with drug-resistant epilepsy in presurgical assessment-A prospective explorative single center study.

Introduction: People with epilepsy (PWE) have a higher prevalence of psychiatric disorders. Some individuals with drug-resistant epilepsy might benefit from surgical interventions. The aim of this study was to perform an assessment of psychiatric comorbidities with a follow-up period of 12 months in patients with drug-resistant epilepsy, comparing those who underwent surgery to those who did not. Material and methods: We assessed psychiatric comorbidities at baseline, after 4 months and after 12 months. Psychiatric symptoms and diagnoses were assessed using SCID-Interview, Hamilton Rating Scale for Depression, Beck-Depression Inventory, Hamilton Anxiety Rating Scale, Prodromal-Questionnaire and the Global Assessment of Functioning Scale. Results: Twenty-five patients were included in the study, 12 underwent surgery, 11 were esteemed as being neurologically unqualified for surgery and two refused surgery. Patients in the no-surgery group were significantly older, reported more substance use, had significantly higher levels of anxiety and were more often diagnosed with a personality disorder. Age and levels of anxiety were significant predictors of being in the surgery or the no-surgery group. The described differences between surgery and no-surgery patients did not change significantly over the follow-up period. Discussion: These data point toward a higher expression of baseline psychiatric symptoms in drug-resistant PWE without surgery. Further studies are warranted to further elucidate these findings and to clarify potential psychotropic effects of epilepsy itself, drug-resistant epilepsy and of epilepsy surgery and their impact on psychopathology. Clinically, it seems highly relevant to include psychiatrists in an interdisciplinary state-of-the-art perioperative management of drug-resistant PWE.

The Impact of Nonconvulsive Status Epilepticus after Cardiac Surgery on Outcome.

Neurological complications after heart surgery are associated with tremendous morbidity and mortality. Nonconvulsive status epilepticus (NCSE), which can only be verified by EEG, may cause secondary brain damage. Its frequency and its impact on outcomes after cardiac surgery is still unclear. We collected the neurological files and clinical data of all our patients after heart surgery who, in the course of their ICU stay, had been seen by a neurologist who ordered an EEG. Within 18 months, 1457 patients had cardiac surgery on cardiopulmonary bypass. EEG was requested for 89 patients. Seizures were detected in 39 patients and NCSE was detected in 11 patients. Open heart surgery was performed in all 11 NSCE patients, of whom eight showed concomitant brain insults. None had a history of epilepsy. Despite the inhibition of seizure activity with antiseizure medication, clinical improvement was only noted in seven NCSE patients, three of whom were in cerebral performance category 2 and four in category 3 at hospital discharge. The four patients without neurological benefit subsequently died in the ICU. The occurrence of NCSE after open cardiac surgery is significant and frequently associated with brain injury. It seems prudent to perform EEG studies early to interrupt seizure activity and mitigate secondary cerebral injury.

Utility of Absolute Quantification in Non-lesional Extratemporal Lobe Epilepsy Using FDG PET/MR Imaging.

The purpose of this study was to establish a non-invasive clinical PET/MR protocol using [18F]-labeled deoxyglucose (FDG) that provides physicians with regional metabolic rate of glucose (MRGlc) values and to clarify the contribution of absolute quantification to clinical management of patients with non-lesional extratemporal lobe epilepsy (ETLE). The study included a group of 15 patients with non-lesional ETLE who underwent a dynamic FDG PET study using a fully-integrated PET/MRI system (Siemens Biograph). FDG tracer uptake images were converted to MRGlc (µmol/100 g/min) maps using an image derived input function that was extracted based on the combined analysis of PET and MRI data. In addition, the same protocol was applied to a group of healthy controls, yielding a normative database. Abnormality maps for ETLE patients were created with respect to the normative database, defining significant hypo- or hyper-metabolic regions that exceeded ±2 SD of normal regional mean MRGlc values. Abnormality maps derived from MRGlc images of ETLE patients contributed to the localization of hypo-metabolic areas against visual readings in 53% and increased the confidence in the original clinical readings in 33% of all cases. Moreover, quantification allowed identification of hyper-metabolic areas that are associated with frequently spiking cortex, rarely acknowledged in clinical readings. Overall, besides providing some confirmatory information to visual readings, quantitative PET imaging demonstrated only a moderate impact on clinical management of patients with complex pathology that leads to epileptic seizures, failing to provide new decisive information that would have changed classification of patients from being rejected to being considered for surgical intervention.

Phenotypic variability of GABRA1-related epilepsy in monozygotic twins.

Variants in GABRA1 have been associated with different epilepsies ranging from mild generalized forms to epileptic encephalopathies. Despite the broad clinical spectrum, phenotypes were found to be largely concordant within families. Contrary to this observation, we report monozygotic twin sisters with generalized epilepsy due to the c.541C>T; p.(Pro181Ser) de novo variant in GABRA1. One experienced juvenile absence seizures promptly responding to first-line medication, whereas the second developed severe treatment-refractory epilepsy with febrile, absence, atonic, and tonic-clonic seizures indicating marked intrafamilial variability in GABRA1-related epilepsy. Moreover, we provide a molecular characterization of the novel variant based on recently published structural data.

Outcome predictors for surgical treatment of temporal lobe epilepsy with hippocampal sclerosis.

PURPOSE: To study long-term postoperative course and identify predictors for postoperative seizure control in patients with medically intractable temporal lobe epilepsy (TLE) associated with hippocampal sclerosis (HS), diagnosed by magnetic resonance imaging (MRI), and ascertained histopathologically. To compare patients becoming seizure-free (i.e., cured from epilepsy) and patients experiencing prolonged seizure-free periods interposed with recurring seizures. METHODS: One hundred thirty-five patients (74 women) underwent complete evaluation for epilepsy surgery. The predictive value of duration of epilepsy, age at onset, age at surgery, gender, febrile convulsion history, ictal dystonic posturing, unilateral interictal electroencephalography (EEG) discharges (IED), preoperative secondarily generalized tonic-clonic seizures (SGTCS), and preoperative seizure frequency for short- and long-term postoperative seizure control were evaluated with two classification systems: Classification 1 (seizure-freedom with or without auras during 12-months before observation points) and the stringent classification 2 [International League Against Epilepsy (ILAE) Ia; absolute absence of seizures and auras after operation]. RESULTS: Unilateral IED at year 1 and 2 (p = 0.037 and p = 0.034), male gender and low seizure frequency at year 2 (p = 0.013 and p = 0.046) were significant predictors for seizure freedom using classification 1. All variables (except male gender at year 2; p = 0.035) lost their predictive power, applying classification 2. The proportion of seizure-free patients remained stable between 70% to 79% with classification 1, but decreased from 64.4% at year 1 to 45.8% at year 5 with classification 2. DISCUSSION: Positive predictors of short-term outcome do not predict long-term outcome in patients with TLE associated with HS. Absolute freedom of seizures and auras cannot be predicted by conventional preoperative variables.

Pre-transplant predictors of cerebrovascular events after kidney transplantation.

BACKGROUND: We evaluated cerebrovascular events (CVE) after kidney transplantation (KTx) and sought to identify pre-transplant predictors of transient ischaemic attacks (TIA) and strokes post-transplantation. METHODS: A total of 1617 consecutive kidney and 16 kidney-pancreas recipients transplanted between 1995 and 2005 were analysed in this retrospective single-centre study. Risk factors for CVE, e.g. recipient and donor age and gender, diagnosis of chronic kidney disease, end-stage renal disease (ESRD) duration, histories of hypertension, hyperlipidaemia, smoking, atrial fibrillation (AF), diabetes mellitus (DM), ischaemic heart, peripheral- and cerebro-vascular disease, as well as pre-transplant myocardial infarction or CVE (i.e. TIA/strokes) were analysed. Furthermore, the predictive value of pre-transplant screening tests, i.e. echocardiography (n = 1184) and carotid ultrasound (n = 922), was investigated. RESULTS: During a median follow-up of 4 years, 64 CVE (54 strokes and 10 TIA) were observed. Nineteen (5.1%) of 373 deceased patients died from fatal stroke. Recipient age, history of AF and hyperlipidaemia (P = 0.00, respectively), reduced left ventricular function (LVF) (P = 0.01) and the degree of stenosis by carotid ultrasound (P = 0.002), duration of ESRD (P = 0.03) and interstitial nephritis as renal disease cause (P = 0.04) evolved as predictors of TIA/stroke post-transplant in univariate analysis. In multivariable analysis, AF (P = 0.001) and DM (P = 0.037) were significant predictors for post-transplant CVE. CONCLUSIONS: AF and DM are independent predictors of CVE after KTx. Beyond their general ability to detect sev- erely comorbid patients, pre-transplant screening tests (e.g. carotid ultrasound or echocardiography) were not able to identify renal transplant candidates at risk for CVE after transplantation.

Combined electroencephalography and magnetoencephalography of interictal spikes in benign rolandic epilepsy of childhood.

OBJECTIVE: The neurogenesis and functional organization of the interictal spikes in benign rolandic epilepsy of childhood (BREC) still remains controversial. METHODS: We performed a combined neuroelectric and neuromagnetic study in 24 consecutive patients with BREC using a 143-channel whole-head magnetoencephalography (MEG) system simultaneously with electroencephalography (EEG) recorded from 40 closely spaced scalp-EEG electrodes. Isopotential and isofield maps were calculated over the time window from 250ms before to 250ms after the maximum of the negative peak of the spike. We then performed principal component analysis (PCA) and spatio-temporal dipole modeling in order to estimate the number, location and temporal activity of sources. RESULTS: EEG and MEG spikes were characterized by a stereotypical appearance both within and across patients showing a stable dipolar field distribution over the entire time window. The spikes were generated by a single tangential dipolar source located in the precentral gyrus with the positive pole directed frontally and the negative pole directed centro-temporally. CONCLUSIONS: One source located in the precentral gyrus can adequately explain the spike complex in BREC. SIGNIFICANCE: Simultaneous EEG and MEG provide comprehensive information on functional organization of spikes in BREC.

Mesial temporal lobe epilepsy: long-term seizure outcome of patients primarily treated with transsylvian selective amygdalohippocampectomy.

OBJECTIVE The aim of this study was to present long-term seizure outcome data in a consecutive series of patients with refractory mesial temporal lobe epilepsy primarily treated with transsylvian selective amygdalohippocampectomy (SAHE). METHODS The authors retrospectively analyzed prospectively collected data for all patients who had undergone resective surgery for medically refractory epilepsy at their institution between July 1994 and December 2014. Seizure outcome was assessed according to the International League Against Epilepsy (ILAE) and the Engel classifications. RESULTS The authors performed an SAHE in 158 patients (78 males, 80 females; 73 right side, 85 left side) with a mean age of 37.1 ± 10.0 years at surgery. Four patients lost to follow-up and 1 patient who committed suicide were excluded from analysis. The mean follow-up period was 9.7 years. At the last available follow-up (or before reoperation), 68 patients (44.4%) had achieved an outcome classified as ILAE Class 1a, 46 patients (30.1%) Class 1, 6 patients (3.9%) Class 2, 16 patients (10.4%) Class 3, 15 patients (9.8%) Class 4, and 2 patients (1.3%) Class 5. These outcomes correspond to Engel Class I in 78.4% of the patients, Engel Class II in 10.5%, Engel Class III in 8.5%, and Engel Class IV in 2.0%. Eleven patients underwent a second surgery (anterior temporal lobectomy) after a mean of 4.4 years from the SAHE (left side in 6 patients, right side in 5). Eight (72.7%) of these 11 patients achieved seizure freedom. The overall ILEA seizure outcome since (re)operation after a mean follow-up of 10.0 years was Class 1a in 72 patients (47.0%), Class 1 in 50 patients (32.6%), Class 2 in 7 patients (4.6%), Class 3 in 15 patients (9.8%), Class 4 in 8 patients (5.2%), and Class 5 in 1 patient (0.6%). These outcomes correspond to an Engel Class I outcome in 84.3% of the patients. CONCLUSIONS A satisfactory long-term seizure outcome following transsylvian SAHE was demonstrated in a selected group of patients with refractory temporal lobe epilepsy.

Incidental epileptiform discharges in patients of a tertiary centre.

OBJECTIVE: This controlled study set out to assess the rate of incidental epileptiform discharges (ED) during routine EEG and the incidence of epilepsy within a 4-year follow-up period in patients without a history of epilepsy. METHODS: We retrospectively analyzed electroencephalography (EEG) recordings of 1750 consecutive patients referred to the Department of Neurology at the Vienna General Hospital between January 1 and December 31, 2009. The incidence of epilepsy in patients with ED and no prior history of epilepsy was compared with a disease control group matched for gender and neuroimaging findings. RESULTS: ED were identified in 26 (4%) of 629 patients without a history of epilepsy. Sixteen (62%) of these patients developed epilepsy during follow-up compared with five (19%) of the disease controls (p=0.01), yielding an adjusted odds ratio of 8.8 (95% CI 2.1-37.7) for developing epilepsy in patients with ED and no prior history of epilepsy. CONCLUSIONS: Incidental ED during routine EEG significantly increase the likelihood of developing epilepsy. SIGNIFICANCE: Patients with incidental ED should be specifically asked for any signs and symptoms suggestive of seizures, since they appear more prone to develop epilepsy.

Temporal evolution and prognostic significance of postoperative spikes after selective amygdala-hippocampectomy.

The purpose of this study was to quantify the temporal evolution and to determine the prognostic significance of spikes on serial postoperative EEGs after selective amygdala-hippocampectomy. The authors performed postoperative EEGs 4 months, 1 year, and 2 years after surgery in 31 patients with unilateral mesial temporal lobe epilepsy. Spike frequency was determined ipsilateral to the resection (group I, no spikes; group II, 1 to 10 spikes; group III, 11 to 20 spikes; group IV, more than 21 spikes during the 30-minute recording). The temporal evolution of postoperative spike frequency was assessed, and these parameters were correlated with surgical outcome. Twenty-two patients showed spikes on postoperative EEG. Spike frequency decreased over time in 14 patients, while no changes or minimal changes occurred in seven patients, and spike frequency increased in one patient. Nine patients had no spikes. There was no correlation between occurrence, frequency, and temporal evolution of spikes with postoperative seizure control. The authors' results demonstrate a progressive decrease of spike frequency during the postoperative period after selective amygdala-hippocampectomy, although occurrence, frequency, and temporal evolution of postoperative spikes had no implications on surgical outcome.