Gudrun Gröppel

Health-related quality of life (HRQOL), activity of daily living (ADL) and depressive mood disorder in temporal lobe epilepsy patients

We determined the interrelations of chronological age, age at seizure onset, duration of seizure disorder, cognitive functioning (IQ), scales of activities of daily living, depressive mood disorder and measures of health-related quality of life (HRQOL). Furthermore, we investigated the association of the laterality of seizure onset zone and absence/presence of hippocampal atrophy and/or sclerosis (HA/HS) with measures of HRQOL, activities of daily living (ADL) and depressive mood disorder. In the setting of pre-surgical epilepsy evaluation, a sample of 56 patients with temporal lobe epilepsy (TLE) was studied using the Bonner Skalen für Epilepsie (BPSE) and the depression inventory D-S of von Zerssen. Patients reported high levels of dependency on others and poor coping capabilities. Our data also showed specific ADL-behaviour suggesting social withdrawal and isolation. Our results indicate emotional impairment as a major problem in TLE, because 45% of our patients scored in the depressive range of the D-S depression scale. Depression score was found to be a powerful predictor of self-reported quality of life after adjusting for seizure-related variables, demographic variables and cognitive functioning (IQ). The only scale showing a significant laterality effect was ADL-home. No relationship between the dependent measures of HRQOL, ADL-social, ADL-cultural, depressive mood disorder and laterality of the epileptogenic zone or absence/presence of HA/HS was found. HRQOL and depressive mood disorder are strongly interrelated indicating that patients with depressive symptoms report lower quality of life and specific patterns of ADL. HRQOL, ADL and depressive mood disorder are largely independent of biological markers such as laterality of seizure onset zone and absence/presence of HA/HS in TLE.

Epilepsy surgery in children and adolescents with malformations of cortical development—-Outcome and impact of the new ILAE classification on focal cortical dysplasia

UNLABELLED To determine long-term efficacy and safety of epilepsy surgery in children and adolescents with malformations of cortical development (MCD) and to identify differences in seizure outcome of the various MCD subgroups. Special focus was set on the newly introduced International League Against Epilepsy (ILAE) classification of focal cortical dysplasia (FCD). STUDY DESIGN This is a single center retrospective cross-sectional analysis of prospectively collected data. INCLUSION CRITERIA age at surgery <18 years, pre-surgical evaluation and epilepsy surgery performed at the Vienna pediatric epilepsy center, histologically proven MCD, complete follow-up data for at least 12 months. Clinical variables evaluated: type and localization of MCD, type of surgery and a variety of clinical characteristics reported to be associated with (un-)favorable outcomes. MCD were classified following the existing classification schemes (Barkovich et al., 2012. Brain. 135, 1348-1369; Palmini et al., 2004. Neurology. 62, S2-S8) and the ILAE classification for FCD recently proposed by Blümcke in 2011. Seizure outcome was classified using the ILAE classification proposed by Wieser in 2001. RESULTS 60 Patients (51.7% male) were included. Follow up was up to 14 (mean 4.4 ± 3.2) years. Mean age at surgery was 8.0 ± 6.0 (median 6.0) years; mean age at epilepsy onset was 2.9 ± 3.2 (median 2.0) years; duration of epilepsy before surgery was 4.8 ± 4.4 (median 3.0) years. 80% of the patients were seizure free at last follow-up. AEDs were successfully withdrawn in 56.7% of all patients. Extended surgery, lesion localization in the temporal lobes and absence of inter-ictal spikes in postsurgical EEG recordings were predictive of favorable seizure outcomes after surgery. However, no association was found between outcome and MCD sub-types. Epilepsy surgery is highly effective in carefully selected drug-resistant children with MCD. Surrogate markers for complete resection of the epileptogenic zone remain the only significant predictors for seizure freedom after surgery.

Vertical perithalamic hemispherotomy: A single‐center experience in 40 pediatric patients with epilepsy

The current concept for hemispherotomy includes various lateral techniques and the vertical perithalamic hemispherotomy introduced by Delalande in 1992. We have chosen the vertical approach because of advantages that possibly influence outcome: the possibility to completely disconnect the hemisphere at the level of the thalamus obviating both the need to resect the insula and the need to open and dissect the subarachnoid space of the Sylvian fissure.

A case of startle epilepsy and SSMA seizures documented with subdural recordings.

Summary: Purpose: To study the mechanisms of startle‐induced supplementary sensorimotor area (SSMA) seizures.

Regional Cerebral Blood Flow During Temporal Lobe Seizures Associated with Ictal Vomiting: An Ictal SPECT Study in Two Patients

Summary: Purpose: Ictal vomiting represents a rare clinical manifestation during seizures originating from the temporal lobes of the nondominant hemisphere. The precise anatomic structures responsible for generation of ictal vomiting remain to be clarified. Ictal single photon emission computed tomography (SPECT), which allows one to visualize the three‐dimensional dynamic changes of regional cerebral blood flow (rCBF) associated with the ongoing epileptic activity, should be useful to study the brain areas activated during ictal vomiting.

Impaired oligodendroglial turnover is associated with myelin pathology in Focal Cortical Dysplasia and Tuberous Sclerosis Complex

Conventional antiepileptic drugs suppress the excessive firing of neurons during seizures. In drug‐resistant patients, treatment failure indicates an alternative important epileptogenic trigger. Two epilepsy‐associated pathologies show myelin deficiencies in seizure‐related brain regions: Focal Cortical Dysplasia IIB (FCD) and cortical tubers in Tuberous Sclerosis Complex (TSC). Studies uncovering white matter‐pathology mechanisms are therefore urgently needed to gain more insight into epileptogenesis, the propensity to maintain seizures, and their associated comorbidities such as cognitive defects. We analyzed epilepsy surgery specimens of FCD IIB (n = 22), TSC (n = 8), and other malformations of cortical development MCD (n = 12), and compared them to autopsy and biopsy cases (n = 15). The entire lesional pathology was assessed using digital immunohistochemistry, immunofluorescence and western blotting for oligodendroglial lineage, myelin and mTOR markers, and findings were correlated to clinical parameters. White matter pathology with depleted myelin and oligodendroglia were found in 50% of FCD IIB and 62% of TSC cases. Other MCDs had either a normal content or even showed reactive oligodendrolial hyperplasia. Furthermore, myelin deficiency was associated with increased mTOR expression and the lower amount of oligodendroglia was linked with their precursor cells (PDGFRa). The relative duration of epilepsy (normalized to age) also correlated positively to mTOR activation and negatively to myelination. Decreased content of oligodendroglia and missing precursor cells indicated insufficient oligodendroglial development, probably mediated by mTOR, which may ultimately lead to severe myelin loss. In terms of disease management, an early and targeted treatment could restore normal myelin development and, therefore, alter seizure threshold and improve cognitive outcome.

The ketogenic diet in infants – Advantages of early use

OBJECTIVE To evaluate the efficacy and safety of the ketogenic diet (KD) in infants (< 1.5 years of age) compared with older children. METHODS Patients with complete follow-up data of ≥ 3 months after initiation of the KD were analyzed retrospectively. Infants < 1.5 years at initiation of the KD (Group A) were compared with children > 1.5 years (Group B). RESULTS 127 children were screened, 115 (Group A: 58/Group B: 57) were included. There were no significant differences between groups with respect to responder rates (63.8% vs. 57.9% at 3 months), but more infants became seizure free (34.5% vs. 19% at 3 months; 32.7% vs. 17.5% at 6 and 12 months). This result remained stable also after termination of the KD (30.6% vs. 3.9% at last follow-up) (p = 0.000). Looking at infants < 9 months of age separately (n = 42), this result was even stronger with significantly more infants being seizure free at 6 and at 12 months (p = 0.005, p = 0.014, respectively). In addition, a significantly higher number of infants remained seizure free in the long-term (p = 0.001). No group differences between infants and children with respect to safety were observed. Overall 52/115 patients (45.21%) reported side effects, but withdrawal of the KD was only necessary in one infant. Acceptance of the KD was better in infants compared with children at 3 months (0 vs. 14, p = 0.000), but became difficult when solid food was introduced (16 vs. 14; n.s.). SIGNIFICANCE According to our results, the KD is highly effective and well tolerated in infants with epilepsy. Seizure freedom is more often achieved and maintained in infants. Acceptance of the diet is better before the introduction of solid food. Therefore, we recommend the early use of the KD during the course of epilepsy.

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

Summary 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.

Improvement of language development after successful hemispherotomy

PURPOSE To investigate language development after functional hemispherotomy and to evaluate prognostic factors for (un-)favourable outcomes. METHODS Children and adolescents who had vertical perithalamic hemispherotomy at the Medical University Wien (MUW) paediatric epilepsy centre were identified from a prospectively maintained database. Inclusion criteria were: complete clinical, neurophysiological and neuropsychological data, seizure freedom and a minimum follow-up of 12 months after surgery. The language quotients (LQ) prior to surgery and at last follow-up were calculated for each child. In addition, associations between pre- to post-surgical changes in LQ and the following variables were examined: age at epilepsy-onset, age at surgery and duration of epilepsy prior to surgery, aetiology, side of surgery, interictal EEG including sleep organization before and 12 months after surgery and antiepileptic-drug (AED) withdrawal state at last follow-up. Analyses were carried out in SPSS version 20.0 (SPSS Inc., Chicago, IL, USA). Nonparametric Wilcoxon and chi-square tests were applied, as required. RESULTS Data from 28 children (14 female) were analyzed. The median age at epilepsy surgery was 64.5 months. The median follow-up after surgery was 3.0 years (±2.6 years, range 12 months to 12 years). Significant gains in LQs at last follow-up were found in 31% of the children (p=0.008). Short disease duration prior to surgery, acquired pathology, lack of epileptiform EEG discharges in the contralateral hemisphere and/or normalization of EEG sleep patterns after surgery, and successful AED withdrawal were linked to favourable language outcomes. CONCLUSION Successful and early hemispherotomy results in improvement of language function in the intact hemisphere.

Immediate termination of electrical status epilepticus in sleep after hemispherotomy is associated with significant progress in language development.

To study the effect of hemispherotomy on electrical status epilepticus in sleep (ESES) and language development.