Primary headache types in adult epilepsy patients.

BACKGROUND: Headache is among the most common comorbidities in epilepsy. This study examined the distribution of different primary headache disorders in a large cohort of patients with diagnosed epilepsy. Headache types were analysed with regard to gender, type of epilepsy and antiepileptic drugs (AEDs). METHODS: In this prospective single-centre study, 500 patients with epilepsy (250 female, mean age: 45.52 ± 17.26 years) were evaluated with regards to primary headache types using a validated German headache questionnaire categorizing for migraine (MIG), tension-type headache (TTH) or trigeminal autonomic cephalalgias (TAC), their combinations and unclassifiable headache. Data regarding type of epilepsy, seizure-associated headache, AED treatment and seizure freedom were collected. RESULTS: Of 500 patients with epilepsy, 163 (32.6%) patients (108 female and 55 male) reported suffering from headaches at least 1 day per month. MIG (without aura, with aura) and TTH were the most frequent headache type (MIG 33.1%, TTH 33.1%). Female epilepsy patients reported headaches significantly more often than male patients (x2 = 8.20, p = 0.0042). In contrast, the type of epilepsy did not significantly affect headache distribution. Of 163 patients with headache, 66 (40.5%) patients reported seizure-associated headache and AEDs were used by 157 patients. Of importance, patients with AED monotherapy suffered from MIG less often when compared to patients on polytherapy (x2 = 4.79, p = 0.028). CONCLUSION: MIG and TTH are the most common headache types in epilepsy patients and headache is more frequent among female epilepsy patients. Monotherapy in AEDs might have a beneficial effect on the frequency of headache compared to polytherapy.

A prospective controlled study about sleep disorders in drug resistant epilepsy.

OBJECTIVE: To evaluate sleep disorders and chronotype in patients with drug resistant focal and generalised epilepsy compared to healthy controls. METHODS: Sixty four patients with focal and six with generalised, drug resistant epilepsy were included and compared to 70 age- and gender-matched healthy controls. Patients with any relevant comorbidity were excluded. Sleep disorders and chronotype were investigated by validated questionnaires. The impact of epilepsy on quality of life was also documented in patients. RESULTS: The median Pittsburgh Sleep Quality Index (PSQI) was 4 in patients and 3 in controls (median [range], IQR; patients: 4 [1-17], 3-6; controls: 3 [0-11], 2-4; p = 0.024). Self-reported confusional arousals and probable REM sleep behaviour disorder (RBD) were more frequent in patients (30.4% vs. 8.6%, p = 0.036 and 10.6% vs. 1.4%, p = 0.030, respectively). A higher risk for possible sleep apnea was identified in patients (22.9% vs. 5.7%, p = 0.042), whereas Epworth Sleepiness Score was normal in both groups (p = 1). Chronotype, assessed by the midsleep on free days, did not differ between groups (p = 0.540). Quality of life was worse in patients with PSQI scores >5 (p = 0.016). CONCLUSION: Self-reported confusional arousals, probable RBD and a high risk for sleep apnea occurred significantly more often in patients with drug resistant epilepsy. Sleep quality differed significantly between both groups. Whether these results are due to motor activity during nocturnal epileptic seizures, parasomnia episodes, or sleep-related breathing disorder, needs further evaluation via video-polysomnography. We could confirm, at least in some cases, the previously reported mutual relationship between sleep disorders and epilepsy.

The role of cortisol in trust behavior: Results from an experimental study on healthy controls and patients with juvenile myoclonic epilepsy.

Trust is one of the foundations of human society and pervades all aspects of human live. Research on humans focused primarily on identifying the biological basis of trust behavior in healthy subjects, and this evidence hints to certain brain areas, hormones, and genetic factors to be fundamentally involved. The contribution of cortisol in trust has not yet elicited much attention in research, especially when specifically examined at basal cortisol levels. Trust has been previously studied in some neurological diseases but not in patients with epilepsy, and the influence of hormones on trust in these diseases remains yet unknown. Against this background, we designed an experimental study with a group of patients with juvenile myoclonic epilepsy and a group of healthy controls to compare trust behavior and plasma cortisol levels between the two groups. This economic game is frequently used in research to operationalize trust behavior. All participants further underwent neuropsychological assessment. Our results showed that there was no significant difference in trust behavior during the trust game, but a trend toward lower trust in patients. Furthermore, there was a significant difference in cortisol levels between groups with lower levels in patients. Interestingly, cortisol levels correlated with trust only in the patient group, but not in the control group. Future studies should specifically differentiate the effect of induced cortisol increases (e.g., acute stress) versus the effect of basal cortisol levels reflecting homeostasis or chronic stress on trust behavior and leverage the potential of comparison between patients and healthy controls.

Repetitive miniature spikes - An underreported EEG pattern.

OBJECTIVES: Low-voltage repetitive spikes are mainly described with invasive recordings and considered highly suggestive for focal cortical dysplasia (FCD). This EEG pattern has received less attention in routine scalp EEG. METHODS: Prospective collection of EEGs with low-voltage (<50 µV) repetitive spikes (repetitive miniature spikes - RMS) between July 1982 and July 2017 at the EEG laboratory of the Medical University of Innsbruck. We analyzed patterns of RMS on routine scalp EEG recordings and examined the relationship to clinical and brain imaging data. RESULTS: Overall, RMS were seen in 38 patients representing zero to four observations out of 5000 records per year. RMS occurred rhythmically in 14, periodically in 17 and irregularly in seven patients. The EEG pattern appeared with a frontal and central predominance. All but five patients had epilepsies; eleven patients had non-convulsive status epilepticus. Cerebral magnetic resonance imaging (cMRI) detected malformations of cortical development in eleven patients, including six patients with focal cortical dysplasias. CONCLUSIONS: RMS are rare EEG patterns indicating focal epilepsy. Their observation on routine scalp EEGs should prompt further clinic-radiologic investigation. SIGNIFICANCE: RMS resemble a clearly recognizable pattern in routine EEG, which is highly associated with focal epilepsy. The term is descriptive and can be added to the red flags, which can be found on routine EEG indicating underlying structural brain pathology, often in form of focal cortical dysplasia.

Computer-based monitoring and evaluation of epilepsy-related health variables and their impact on treatment decision.

OBJECTIVE: This study aimed to determine the effectiveness of electronic patient-reported outcomes (ePROs) with focus on epilepsy-specific quality of life, psychiatric and psychosocial burden, drug side effects, and patient satisfaction via the Computer-based Health Evaluation System (CHES) and to evaluate their impact on treatment regimen. METHODS: Forty consecutive patients with drug-resistant focal epilepsy undergoing prolonged video-electroencephalography (EEG) monitoring at the Department of Neurology, Innsbruck Medical University were included and randomized to an intervention group (questionnaire results accessible to the physicians) and a control group (questionnaire results inaccessible to the physicians). Patients had to complete questionnaires on the day of admission (T0) and the day of discharge (T1). RESULTS: Overall, twenty-five patients (25/40, 62.5%) showed abnormal assessment results, twelve of them exclusively due to pathological scores on the Liverpool Adverse Events Profile (LAEP). Mean LAEP score was within the pathological range of 48.8 points (48.8 ±â€¯7.2). The psychosocial burden with respect to the Performance, Socio-Demographic Aspects, Subjective Evaluation (PESOS) scale "fear" (48.7 ±â€¯21.4) was also moderately affected. Moreover, mean anxiety (9.1 ±â€¯4.4) and depression (7.6 ±â€¯4.5) scores were both slightly abnormal. Quality of life (as measured using the Quality of Life Inventory in Epilepsy (QOLIE-31)) was moderately impaired (seizure worry: 46.5 ±â€¯21.3, overall quality of life: 52.6 ±â€¯18.6, well-being: 54.1 ±â€¯16.3, energy-fatigue: 39.4 ±â€¯14.7, cognitive functioning: 41.4 ±â€¯19.5, medication effects: 46.2 ±â€¯23.4, social functioning: 51.1 ±â€¯20.8, and total score: 47.2 ±â€¯12.3). Careful medical history-taking and patient-physician consultations alone failed to detect needs for psychological/psychiatric help in three out of 7 patients in the control group (42.8%). Changes over time in Hospital Anxiety and Depression Scale (HADS) and QOLIE-31 scores were not significant. CONCLUSION: The use of ePROs was feasible and well accepted in the clinical setting. Treatment-associated adverse effects were the most frequently reported health-related restrictions. In particular, psychometric evaluation by applying ePROs can detect health-related problems in patients with epilepsy.

Risky Decision Making in Juvenile Myoclonic Epilepsy.

It is not known whether patients with juvenile myoclonic epilepsy (JME) differ from healthy people in decision making under risk, i.e., when the decision-making context offers explicit information about options, probabilities, and consequences already from the beginning. In this study, we adopted the Game of Dice Task-Double to investigate decision making under risk in a group of 36 patients with JME (mean age 25.25/SD 5.29 years) and a group of 38 healthy controls (mean age 26.03/SD 4.84 years). Participants also underwent a comprehensive neuropsychological assessment focused on frontal executive functions. Significant group differences were found in tests of psychomotor speed and divided attention, with the patients scoring lower than the controls. Importantly, patients made risky decisions more frequently than controls. In the patient group, poor decision making was associated with poor executive control, poor response inhibition, and a short interval since the last seizure episode. Executive control and response inhibition could predict 42% of variance in the frequency of risky decisions. This study indicates that patients with JME with poorer executive functions are more likely to make risky decisions than healthy controls. Decision making under risk is of major importance in every-day life, especially with regard to treatment decisions and adherence to long-term medical therapy. Since even a single disadvantageous decision may have long-lasting consequences, this finding is of high relevance.

Generalized nonmotor (absence) seizures-What do absence, generalized, and nonmotor mean?

OBJECTIVE: Clinical absences are now classified as "generalized nonmotor (absence) seizures" by the International League Against Epilepsy (ILAE). The aim of this paper is to critically review the concept of absences and to put the accompanying focal and motor symptoms into the context of the emerging pathophysiological knowledge. METHODS: For this narrative review we performed an extensive literature search on the term "absence," and analyzed the plethora of symptoms observed in clinical absences. RESULTS: Arising from the localization and the involved cortical networks, motor symptoms may include bilateral mild eyelid fluttering and mild myoclonic jerks of extremities. These motor symptoms may also occur unilaterally, analogous to a focal motor seizure with Jacksonian march. Furthermore, electroencephalography (EEG) abnormalities may exhibit initial frontal focal spikes and consistent asymmetries. Electroclinical characteristics support the cortical focus theory of absence seizures. Simultaneous EEG/functional magnetic resonance imaging (fMRI) measurements document cortical deactivation and thalamic activation. Cortical deactivation is related to slow waves and disturbances of consciousness of varying degrees. Motor symptoms correspond to the spike component of the 3/s spike-and-wave-discharges. Thalamic activation can be interpreted as a response to overcome cortical deactivation. Furthermore, arousal reaction during drowsiness or sleep triggers spikes in an abnormally excitable cortex. An initial disturbance in arousal mechanisms ("dyshormia") might be responsible for the start of this abnormal sequence. SIGNIFICANCE: The classification as "generalized nonfocal and nonmotor (absence) seizure" does not covey the complex semiology of a patient's clinical events.

Personalized safety measures reduce the adverse event rate of long-term video EEG.

Objective: Safety in epilepsy monitoring units (EMUs) has become an increasing concern because adverse events occur in up to 10% of patients undergoing long-term video EEG in EMUs. The aim of this study was to assess the effectiveness of a specific safety protocol in an EMU. Methods: We retrospectively assessed the adverse event rates in a group without (group 1, 84-month period, Innsbruck, Austria) and a group with (group 2, 33-month period, Salzburg, Austria) personalized safety measures utilizing a standardized protocol for long-term epilepsy monitoring in high-risk patients. Differences in adverse event rates during and after long-term video EEG between the two groups were calculated and compared. Results: In group 1, 44/507 (9%, 95% confidence interval [CI] 6.5-11.5%) patients experienced 53 adverse events: 20/507 (4%, 95% CI 2.6-6.0%) patients had psychiatric events, 15/507 (3%, 95% CI 1.8-4.8%) patients sustained a total of 19 injuries during seizures, and 10/507 (2%, 95% CI 1.1-3.6%) patients had 13 episodes of status epilepticus; one adverse event was treatment-related (valproic acid-induced encephalopathy; 1/507, 0.2%, 95% CI 0.0-1.1%). By using the new safety protocol in group 2, the adverse event rate was only 5% (95% CI 3.4-7.6%; 30 adverse events in 26/491; 45% reduction; p = 0.036), in contrast. These events included 13 psychiatric complications in 13/491 (2%, 95% CI 1.6-4.5%, p = 0.252) patients, 12 seizure-related injuries in 9/491 (2%, 95% CI 1.0-3.4%, p = 0.250) patients, and 5 episodes of status epilepticus in 4/491 (1%, 95% CI 0.3-2.1%, p = 0.120) patients. Significance: Implementation of personalized safety measures in high-risk patients resulted in a clinically relevant reduction of adverse events in the EMU. Safety protocols are a valid tool to reduce the occurrence of adverse events in EMUs.

Corpus callosum and epilepsies.

PURPOSE: Corpus callosum (CC) is the largest forebrain commissure. This review focuses on the significance of CC for seizure disorders, the role of CC in seizure spread and the surgical disruption of callosal fibers (callosotomy) for treatment of patients with drug-resistant epilepsy. METHODS: Personal experience/extensive literature review. RESULTS: Structural CC pathologies comprise developmental abnormalities, callosal involvement in identified disorders, transient imaging findings and microstructural changes. Epilepsies are reported in up to two thirds of patients with complete or partial CC agenesis (AgCC). However, AgCC per se is not indicative for seizure disorders. Moreover, additional malformations of cortical development (MCD) are causal. Microstructural CC abnormalities are detected by advanced imaging techniques, are part of diffuse white matter disturbances and are related to cognitive deficits. The etiological significance remains unexplained. However, they are also found in non-epileptic benign and transient disorders. In drug-resistant epilepsies with violent drops to the floor ("drop seizures") callosotomy may be beneficial in seizure reduction. Since the EEG after callosotomy exhibits a single seizure focus in up to 50% of patients, consecutive resective surgical methods might be successful. CONCLUSION: CC is part of cerebral white matter and anomalies cannot act per se as seizure onset zone. Imaging techniques demonstrate additional lesions in patients with epilepsies. CC is the major pathway for seizure generalization. Therefore, callosotomy is used to prevent generalized drop seizures.

Duration of focal complex, secondarily generalized tonic-clonic, and primarily generalized tonic-clonic seizures--A video-EEG analysis.

INTRODUCTION: Identifying seizures with prolonged duration during video-electroencephalographic (EEG) monitoring is of importance to inform clinicians when to start emergency treatment of seizures to prevent status epilepticus. The aims of this study were to assess the clinical and EEG seizure duration (SD) in consecutive patients with epilepsy who underwent prolonged video-EEG monitoring and to identify a seizure type-dependent time point to start emergency treatment based on the likelihood that seizures will not stop spontaneously. Furthermore, we sought to determine predictors of SD and explored the relationship between antiepileptic drug (AED) serum levels and SD. MATERIAL AND METHODS: We retrospectively analyzed 1796 seizures in 200 patients undergoing video-EEG monitoring between January 2006 and March 2008. RESULTS: Focal simple seizures lasted significantly shorter (clinical SD: 28s, EEG SD: 42 s) compared with focal complex seizures (clinical SD: 64 s, EEG SD: 62 s), and both seizure types lasted significantly shorter compared with secondarily generalized tonic-clonic seizures (GTCSs; clinical SD: 90 s, EEG SD: 96 s). There was no difference between the duration of the convulsive phase of primary GTCSs (defined as nonfocal) and that of secondarily GTCSs (each 65 s). Cumulative clinical SD (99%) was 7 min in focal complex seizures and 11 min in focal simple seizures. Mixed linear regression model demonstrated that history of status epilepticus (P = 0.034), temporal lobe seizure onset (P = 0.040), and MRI lesions (P = 0.013) were significantly associated with logarithmic EEG SD in focal epilepsies recorded with scalp electrodes. We found significant negative correlations between the AED serum level and the EEG SD in patients treated with monotherapy: carbamazepine (P < 0.001), levetiracetam (P = 0.001), oxcarbazepine (P = 0.001), and valproic acid (P = 0.038) but not with lamotrigine monotherapy and EEG SD. DISCUSSION: Based on the results of this study, we propose 2 min of convulsive seizure activity (irrespective of focal or generalized onset) as a prolonged seizure, which could serve as a time point to consider treatment to prevent status epilepticus. In focal complex seizures, we suggest an upper limit of 7 min, and in focal simple seizures 11 min, as definition of prolonged seizures. History of status epilepticus, temporal seizure onset, and lesional MRI findings are factors associated with significantly longer SD. Negative correlations of carbamazepine, levetiracetam, oxcarbazepine, and valproic acid serum levels and SD suggest a prolonging effect on seizures during withdrawal of these AEDs during video-EEG monitoring sessions. This article is part of a Special Issue entitled "Status Epilepticus".

Seizure outcome in 175 patients with juvenile myoclonic epilepsy--a long-term observational study.

INTRODUCTION: Juvenile myoclonic epilepsy (JME) is a genetic generalized epilepsy syndrome. Under appropriate antiepileptic drugs (AED) up to 85% of patients become seizure-free, but many may have a relapse after AED withdrawal. METHODS: We retrospectively studied 242 patients with JME at the Department of Neurology, Medical University Innsbruck, Austria (1975-2006). We analyzed age at seizure onset, age at last follow up, seizure types, photosensitivity, seizure outcome and neuroimaging findings; inclusion criterion was a medical treatment period of >2 years; exclusion criteria were traumatic or infectious brain injury before the onset of JME and/or gross structural pathology on neuroimaging. RESULTS: We identified 175 patients (111 women) with a median age at seizure onset of 15 years, (range 3-46) and a median age at follow-up (FU) of 38 years (range 14-87; median FU 8 years, range 2-38). Fourteen percent showed (24/175) photosensitivity on routine EEG. Seizure outcome: 62% (109/175) were seizure-free of myoclonic seizures (MS), generalized tonic clonic seizures (GTCS) and absence seizures (AS) for >1 year, and 53% (94/175) for >2 years, including 16 patients (9%) without AEDs. Thirty-one percent (54/175) were seizure-free between 2 and 5 years, 15% (26/175) between 6 and 10, and 8% (14/175) >10 years; 38% (66/175) were not seizure-free. Not seizure-free patients had more often MS, AS and GTCS within the first year of epilepsy than those who were seizure-free at last FU (11% vs. 3%, Chi(2)=4.679, df=1, p=0.043). Seizure-free patients had more often MS and GTCS as last seizure types in the year before becoming seizure-free (37% vs. 15%, p=0.003), whereas in not seizure-free group MS only and GTCS only persisted. CONCLUSIONS: JME does not always need lifelong treatment, as a substantial minority of patients remain seizure-free without AEDs. AS, MS and GTCS at onset of the disease are indicators of poor long-term seizure control.

Selective serotonin reuptake inhibitors prolong seizures - preliminary results from an observational study.

OBJECTIVE: Selective serotonin reuptake inhibitors (SSRIs) are often used in the treatment of depressive disorders in patients with epilepsy. Pro- and anti-convulsive effects of SSRIs are discussed controversially. The aim of this study was to investigate a possible impact of SSRIs-treatment on duration of EEG and clinical features in epilepsy patients. METHODS: We studied video-EEG data from 162 patients with focal epilepsies between January 2006 and March 2008 using a case-control study design. Eleven patients with 19 complex focal seizures (CFSs) and 16 secondary generalized tonic-clonic seizures (sGTCSs) treated with SSRIs (SSRIs+) were matched to 13 patients without SSRIs-treatment (SSRIs-). We compared duration of ictal EEG in CFSs and sGTCSs, duration of convulsions in sGTCSs and duration of postictal EEG suppression after sGTCSs in SSRIs+ and SSRIs- patients. RESULTS: Ictal EEG duration of both, CFSs and sGTCSs, was significantly longer in SSRIs+ patients than in SSRIs- patients (p=0.004 and p=0.015, respectively). No significant difference was found between convulsive phase duration of sGTCSs as well as duration of postictal EEG suppression after sGTCSs in both groups. CONCLUSION: Seizures last significantly longer in patients with epilepsy and SSRIs as co-medication. A causative role of SSRIs in ictal activity has to be explored in prospective studies.

Midbrain-hindbrain malformations in patients with malformations of cortical development and epilepsy: a series of 220 patients.

Midbrain-hindbrain malformations (MHM) may coexist with malformations of cortical development (MCD). This study represents a first attempt to investigate the spectrum of MHM in a large series of patients with MCD and epilepsy. We aimed to explore specific associations between MCD and MHM and to compare two groups of patients: MCD with MHM (wMHM) and MCD without MHM (w/oMHM) with regard to clinical and imaging features. Two hundred and twenty patients (116 women/104 men, median age 28 years, interquartile range 20-44 years at the time of assessment) with MCD and epilepsy were identified at the Departments of Neurology and Pediatrics, Innsbruck Medical University, Austria. All underwent high-resolution MRIs (1.5-T) between 01.01.2002 and 31.12.2011. Midbrain-hindbrain structures were visually assessed by three independent raters. MHM were seen in 17% (38/220) of patients. The rate of patients wMHM and w/oMHM differed significantly (p=0.004) in three categories of MCD (category I - to abnormal neuronal proliferation; category II - to abnormal neuronal migration; and category III - due to abnormal neuronal late migration/organization): MCD due to abnormal neuronal migration (31%) and organization (23%) were more commonly associated with MHM compared to those with MCD due to abnormal neuronal proliferation (9%). Extensive bilateral MCD were seen more often in patients wMHM compared to those w/oMHM (63% vs. 36%; p=0.004). In wMHM group compared to w/oMHM group there were higher rates of callosal dysgenesis (26% vs. 4%; p<0.001) and hippocampal abnormalities (52% vs. 27%; p<0.001). Patients wMHM were younger (median 25 years vs. 30 years; p=0.010) at the time of assessment and had seizure onset at an earlier age (median 5 years vs. 12 years; p=0.043) compared to those w/oMHM. Patients wMHM had higher rates of learning disability (71% vs. 38%; p<0.001), delayed developmental milestones (68% vs. 35%; p<0.001) and neurological deficits (66% vs. 47%; p=0.049) compared to those w/oMHM. The groups (wMHM and w/oMHM) did not differ in their response to antiepileptic treatment, seizure outcome, seizure types, EEG abnormalities and rate of status epilepticus. Presence of MHM in patients with MCD and epilepsy is associated with severe morphological and clinical phenotypes.

Proper name retrieval in temporal lobe epilepsy: naming of famous faces and landmarks.

The objective of this study was to further explore proper name (PN) retrieval and conceptual knowledge in patients with left and right temporal lobe epilepsy (69 patients with LTLE and 62 patients with RTLE) using a refined assessment procedure. Based on the performance of a large group of age- and education-matched normals, a new test of famous faces and famous landmarks was designed. Recognition, naming, and semantic knowledge were assessed consecutively, allowing for a better characterization of deficient levels in the naming system. Impairment in PN retrieval was common in the cohort with TLE. Furthermore, side of seizure onset impaired stages of name retrieval differently: LTLE impaired the lexico-phonological processing, whereas RTLE mainly impaired the perceptual-semantic stage of object recognition. In addition to deficient PN retrieval, patients with TLE had reduced conceptual knowledge regarding famous persons and landmarks.

Generalized tonic-clonic seizures and antiepileptic drugs during pregnancy--a matter of importance for the baby?

This study investigates the impact of generalized tonic-clonic seizures (GTCS) and antiepileptic drugs (AED) during pregnancy on gestational age (GA) and anthropometric data of newborns. One hundred twenty-nine singleton pregnancies resulting in live births from September 1999 to October 2010 in 106 women with epilepsy on AED therapy, recorded within the framework of the EURAP (International Registry of Antiepileptic Drugs and Pregnancy) program at the Department of Neurology, Medical University Innsbruck, Austria, were studied. Occurrence of ≥ 1 GTCS during pregnancy was associated with a shorter GA [median (range) 37.5 [35.1-41.6] vs. 39.7 [29.1-46.3] weeks; p ≤ 0.001], an overall five times higher preterm risk (p = 0.042) and a reduced birth weight in boys (2,900 [2,050-3,870] vs. 3,205 [1,575-4,355] g; p = 0.040). In primipara, when compared to multipara, GTCS ≥ 1 significantly reduced the GA (37.9 [35.1-41.6] vs. 39.7 [29.4-44.9] weeks; p = 0.020) and raised the incidence of low birth weight (LBW) (p = 0.022) in neonates. Antiepileptic drug polytherapy significantly increased the risk for small-for-gestational-age regarding weight (SGA(W); p = 0.035) and regarding weight and/or length (SGA(W/L); p = 0.046) when compared to monotherapy. GTCS during pregnancy was associated with diverse negative effects comprising shorter GA, an increased incidence of prematurity and LBW in primiparous women. Furthermore, AED polytherapy was correlated with an enhanced risk for SGA delivery. Re-evaluating the need for drug therapy (in particular polytherapy), maintaining seizure control for a given period before pregnancy and counseling about the importance of preventing GTCS might improve pregnancy outcome in women with epilepsy.

Intravenous lacosamide in status epilepticus and seizure clusters.

Status epilepticus (SE) and seizure clusters (SC) represent neurologic emergencies with a case fatality rate up to 34%, depending on cause and comorbidity. As SE becomes more refractory to treatment over time, appropriate medication is important. This study aimed to investigate efficacy and tolerability of intravenous (IV) lacosamide (LCM) in treatment of SC and SE. Data of patients with SE or SC who were treated with IV LCM between December 2009 and February 2011 in two Austrian centers were analyzed retrospectively. Clinical information was extracted from patients' charts. Forty-eight patients (26f/22m) aged median 62 years (range 17-95 years) were identified. Thirty-five percent of patients (17 of 48) had SC and 65% (31 of 48) had SE. SE was nonconvulsive in 10 (32%), convulsive in 11 (36%), and focal in 10 (32%) patients. SE was acute symptomatic in six (20%) and remote symptomatic in 11 (35%) patients. Fourteen (45%) had preexisting epilepsy. Median initial bolus dose was 200 mg (range 200-400 mg) in patients with SE and 200 mg in patients with SC. Maximum infusion rate was 60 mg/min. Cessation was observed in 42 patients (88%). Success rate in patients with SE receiving LCM as first or second drug was 100% (8 of 8), as third drug 81% (11 of 15), and as fourth or later drug 75% (6 of 8). There were no side effects observed except for pruritus and skin rash in two patients. These data support use of IV LCM as a potential alternative to standard antiepileptic drugs for acute treatment of seizure emergency situations, although randomized controlled studies are needed.

Cranial nerve monitoring during subpial dissection in temporomesial surgery.

OBJECTIVE: Cranial nerves (CNs) crossing between the brainstem and skull base at the level of the tentorial hiatus may be at risk in temporomesial surgery involving subpial dissection and/or tumorous growth leading to distorted anatomy. We aimed to identify the surgical steps most likely to result in CN damage in this type of surgery. METHODS: Electromyographic responses obtained with standard neuromonitoring techniques and a continuous free-running EMG were graded as either contact activity or pathological spontaneous activity (PSA) during subpial resection of temporomesial structures in 16 selective amygdalohippocampectomy cases. Integrity of peripheral motor axons was tested by transpial/transarachnoidal electrical stimulation while recording compound muscle action potentials from distal muscle(s). RESULTS: Continuous EMG showed pathological activity in five (31.2%) patients. Nine events with PSA (slight activity, n = 8; strong temporary activity, n = 1) were recorded. The oculomotor nerve was involved three times, the trochlear nerve twice, the facial nerve once, and all monitored nerves on three occasions. Surgical maneuvers associated with PSA were the resection of deep parts of the hippocampus and parahippocampal gyrus (CN IV, twice; CN III, once), lining with or removing cotton patties from the resection cavity (III, twice; all channels, once) and indirect exertion of tension on the intact pia/arachnoid of the uncal region while mobilizing the hippocampus and parahippocampal gyrus en bloc (all channels, once; III, once). CMAPs were observed at 0.3 mA in two patients and at 0.6 mA in one patient, and without registering the exact amount of intensity in three patients. CONCLUSION: The most dangerous steps leading to cranial nerve damage during mesial temporal lobe surgery are the final stages of the intervention while the resection is being completed in the deep posterior part and the resection cavity is being lined with patties. Distant traction may act on nerves crossing the tentorial hiatus via the intact arachnoid.

Video-EEG monitoring: safety and adverse events in 507 consecutive patients.

PURPOSE: Video-electroencephalography (EEG) monitoring plays a central role in the presurgical evaluation of medically refractory epilepsies and the diagnosis of nonepileptic attack disorders (NEADs). The aim of this study was to analyze safety and adverse events (AEs) during video-EEG monitoring. METHODS: We retrospectively evaluated 596 video-EEG sessions in 507 patients (233 men, mean age 36 years, standard deviation = 14, range 9-80 years) within a 6-year period. AEs were examined in detail and their risk factors were assessed using multiple logistic regression analysis. KEY FINDINGS: Forty-four patients (9%) experienced 53 AEs: 20 had psychiatric events (17 postictal psychosis, 2 panic attacks, 1 interictal psychosis), 15 had injuries (14 falls with minor injuries, 2 falls with fractures, 2 fractures without fall, 1 fall with epidural hematoma), 10 patients had 13 episodes of status epilepticus (SE), and one AE was treatment-related (valproic acid--induced encephalopathy). Patients with AEs were older (p = 0.036) and had a longer duration of epilepsy (p = 0.019). All AEs resulted in a prolonged hospital stay (p < 0.001). Ninety-one percent of the AEs occurred within the first 4 days of monitoring. Independent risk factors were duration of epilepsy >17 years [odds ratio (OR) 3.096; 95% confidence interval (CI) 1.548-6.189], a previous history of psychiatric illness (OR 16.882; 95% CI 5.469-52.110), a history of seizure-related injuries (OR 3.542; 95% CI 1.069-11.739), or a history of SE (OR 3.334; 95% CI 1.297-8.565). SIGNIFICANCE: The most common AEs were postictal psychosis, falls, and SE. Patients with an older age, long disease duration, psychiatric comorbidity, history of injuries, and SE have a higher risk.

Asymmetric seizure termination in primary and secondary generalized tonic-clonic seizures.

PURPOSE: In temporal lobe epilepsies an asymmetric termination (AST) of the clonic phase of secondary generalized tonic-clonic seizures (sGTCS) reliably lateralizes the side of seizure onset. The last clonic activity occurs ipsilateral to the side of the seizure onset zone. We compared the prevalence and lateralizing value of AST in sGTCS of frontal and temporal lobe origin as well as in primary generalized tonic-clonic seizures (pGTCS). METHODS: We analyzed 177 seizures in 84 consecutive patients. Forty-one patients had temporal lobe epilepsy (TLE), 24 frontal lobe epilepsy (FLE), and 19 had nonfocal (primary) generalized epilepsies (GE). All patients underwent intensive video-EEG (electroencephalography) monitoring, high-resolution magnetic resonance imaging (MRI), neuropsychological testing, and single photon emission computed tomography/positron emission tomography (SPECT/PET) when feasible. Two investigators blinded for diagnosis, EEG, and imaging data assessed frequency and side of the last clonic jerk. RESULTS: AST occurred in 63% of patients with TLE (47% of seizures), in 71% with FLE (60% of seizures), and in 42% with GE (21% of seizures). These results were not significant for patients, but significant for seizures in TLE versus GE and in FLE versus GE (p < 0.001). The positive predictive value (PPV) for the side of seizure onset was 74% (p = 0.003) in TLE and 75% (p = 0.008) in FLE. DISCUSSION: AST in sGTCS lateralizes the side of seizure onset in TLE and in FLE to the ipsilateral hemisphere with a high PPV. However, AST was also observed in GE. Therefore, asymmetric clinical signs should not inevitably lead to the assumption of focal epilepsy syndromes.

Successful surgery in late onset epilepsy with tuberous sclerosis complex.

[Case records of Epileptic Disorders. Anatomo-electro clinical correlations. Case 01-2009]. Tuberous sclerosis complex (TSC) is a multisystem genetic disorder with variable phenotypic expression, caused by mutations in one of the two tumor suppressor genes, TSC1 or TSC2. Epilepsy is the most common neurological presentation and seizures are often medically intractable. Definition of the epileptogenic zone during presurgical evaluation is challenging given the multiple potentially epileptogenic lesions visible on MRI. However, TSC patients may nevertheless achieve seizure freedom, when preoperative evaluation yields concordant results. The strategies used in these patients vary substantially among different epilepsy surgery centres. We present a 21-year-old right-handed, intellectually not impaired woman with TSC and medically intractable seizures since the age of 15 years. Careful multi-stage presurgical evaluation, including prolonged video-EEG-monitoring, cerebral high resolution MRI, ictal and interictal [99m Tc]HMPAO-SPECT, [18 F]FDG-PET and further invasive recordings with subdural and depth electrodes led to the identification of an epileptogenic tuber with concordant seizure onset zone in the right neocortical temporal lobe. A tailored resection was performed leading to excellent surgical outcome (follow-up 12 months, Engel class I).