Seizure-related complication rate in a residential population with epilepsy and intellectual disability (ECOMRAID-trial).

INTRODUCTION: The aim of the ECOMRAID trial (Epileptic seizure related Complication RAte in residential population of persons with epilepsy and Intellectual Disability) was to study seizure-related complications (status epilepticus, respiratory complications, or other severe complications) in people with epilepsy and intellectual disability living in a residential setting. The results of the present study are a prerequisite for performing a prospective study into the effectiveness of nocturnal surveillance patients with high risk for Sudden unexpected death in epilepsy (SUDEP). MATERIAL AND METHODS: A retrospective study was conducted in three general residential care institutions and one residential specialized epilepsy clinic. In this 5-year cohort, we collected the following data: age (at inclusion and in case of death), sex, type of residential care, different types of complications, rescue/emergency medication administration, transfers to another department (internal midcare / monitoring unit or general hospital) and a self-designed SUDEP risk score. Our primary research questions were to assess the number of patients who experienced seizure-related complications and their individual complication rates. The secondary research questions were to document the relationship of these complications with the SUDEP risk score, with the type of residential living, and with the frequency of interventions by caregivers. RESULTS: We included 370 patients (1790 patient-years) and in 135 of them, we found 717 seizure-related complications. The following complication rates were found: all complications: at 36%, status epilepticus: at 13%, respiratory complications: at 5%, and other complications at 26%. In residential care institutions, we found fewer patients with complications compared to the specialized epilepsy clinic (all complications 24% vs 42%, OR 0.44, p < 0.01; status epilepticus 5% vs 17%, OR 0.27, p < 0.01; other: complications 19% vs 30%, OR 0.56, p < 0.05). In residential care institutions, we found more "other complications" than in the specialized epilepsy clinic (89% vs 71%, OR 3.13, p < 0.0001). The annual frequency of all complications together was higher in residential care institutions (range 0 to 21 vs 0 to 10, p < 0.05). Rescue medication was given to 75% of the patients, but more often in the specialized epilepsy clinic (median 2.6 vs 0.5 times/patient/year, p < 0.001). In the specialized epilepsy clinic, more patients were transferred to a midcare / monitoring unit or general hospital (56% vs 9%, OR 13.44, p < 0.0001) with higher yearly frequencies (median 0.2 vs 0.0, p < 0.001). There were no reported cases of SUDEP. The median SUDEP risk score was higher in the specialized epilepsy clinic (5 vs 4, p < 0.05) and was weakly correlated with the status epilepticus (ρ = 0.20, p < 0.001) and (total) complication rate (ρ = 0.18, p < 0.001). CONCLUSION: We found seizure-related complications in more than one-third of the patients with epilepsy and intellectual disability living in a residential setting over a period of 5 years. The data also quantify seizure-related complications in patients with epilepsy and intellectual disability.

Changes in the frequency of benign focal spikes accompany changes in central information processing speed: a prospective 2-year follow-up study.

We prospectively examined whether changes in the frequency of benign focal spikes accompany changes in cognition. Twenty-six children with benign focal spikes (19 with Rolandic epilepsy) and learning difficulties were examined with repeated 24-hour EEG recordings, three cognitive tests on central information processing speed (CIPS), and questionnaires on cognition and behavior at baseline, 6months, and 2years. Antiepileptic drug changes were allowed when estimated necessary by the treating physician. At baseline, a lower CIPS was correlated with a higher frequency of diurnal interictal epileptiform discharges (IEDs) and with worse academic achievement. At follow-up, there was a significant correlation between changes in CIPS and EEG changes in wakefulness (in the same direction) when the EEG outcome was dichotomized in IED frequency "increased" or "not increased". Behavioral problems were more often observed in patients with higher frequency of IEDs in sleep at baseline and in those with ongoing IEDs compared with those with EEG remission (without or with sporadic IEDs in the recording) at the end of the study period. No changes were observed in the results of the questionnaires. A lower diurnal IED frequency at baseline, lack of serial IEDs, and occurrence of only unilateral IEDs were correlated with a higher chance of EEG remission at 2-year follow-up. Electroencephalography remission could not be predicted from other epilepsy variables except from seizure freedom in the last six months. Our results confirm the nonbenign character of 'benign' focal spikes. Whether an early and stable EEG remission can be achieved through antiepileptic treatment and whether this is of benefit for cognitive development should be examined in prospective placebo-controlled randomized trials.

Reading performance in children with rolandic epilepsy correlates with nocturnal epileptiform activity, but not with epileptiform activity while awake.

OBJECTIVE: An association between language impairment and rolandic epilepsy is frequently reported. This impairment could be correlated with the amount of nocturnal epileptiform activity. METHODS: We retrospectively analyzed 26 children with rolandic epilepsy and/or rolandic spikes. All had undergone a 24-hour EEG and neuropsychological assessment within 2 weeks. Reading performance (reading words and sentences) and intelligence were measured. RESULTS: There was a significant negative correlation between amount of nocturnal epileptiform activity and reading sentences R=-0.525 (P=0.008). There was a trend in this correlation for reading words R=-0.398 (P=0.054). We found a negative correlation between amount of nocturnal epileptiform activity and Verbal IQ (R=-0.51 P=0.08). No correlation was found between reading performance or Verbal IQ and amount of diurnal epileptiform activity. CONCLUSIONS: Reading performance is impaired in children with rolandic epilepsy and is correlated with the amount of nocturnal epileptiform activity.

The intracarotid amobarbital or Wada test: unilateral or bilateral?

OBJECTIVE: In the Netherlands, presurgical screening for temporal lobe epilepsy (TLE) includes the intracarotid amobarbital procedure (IAP), consisting of two consecutive injections of amobarbital, ipsilateral and contralateral to the epileptic focus. We studied whether a bilateral IAP has added value to a unilateral, ipsilateral IAP. METHODS: This population-based study included 183 consecutive patients referred for screening for TLE surgery who underwent bilateral IAP. Using multivariable modeling, we assessed the added value of bilateral IAP on the decision for surgery, resection size, amygdalohippocampectomy, post-operative seizure freedom, memory performance, and IQ change. RESULTS: Given the results from the unilateral IAP, the bilateral IAP had added prognostic value for postoperative change in verbal memory (P < 0.01) and verbal IQ (P < 0.01), especially if patients had a left-sided focus. In contrast, information provided by the contralateral IAP was not associated with decision-making or surgical strategy. CONCLUSIONS: A bilateral IAP has added value in predicting post-operative verbal memory and IQ. A bilateral IAP is currently not used to guide surgical strategy, but may be used for this purpose when verbal capacity is of particular concern in patients with a left-sided focus. In other cases, IAP is best performed unilaterally.

Sudden unexpected death in epilepsy patients: Risk factors. A systematic review.

INTRODUCTION: Several risk factors for sudden unexplained death in epilepsy patients (SUDEP) have been proposed, but subsequent work has yielded conflicting data. The relative importance of various risk factors for SUDEP was never explored. The aim of this study is to review systematically risk factors for SUDEP and also to determine their relevance for SUDEP by calculating relative risk factor ratios. METHODS AND MATERIALS: Authors performed a literature-search on "SUDEP" in Medline, the Cochrane Library and EMBASE. Studies with unknown number of SUDEP cases or with less than five SUDEP cases and reviews were excluded from further analysis. The value of each paper was assessed, based on the quality of the study and the reliability of the diagnosis of SUDEP. This value ranged from 1 (low quality) to 10 (high quality). Papers with a value below 7 were eliminated for further analysis. For each analysed factor, a risk factor ratio was determined, with a higher ratio for a stronger risk factor. RESULTS: A number of strong risk factors for SUDEP: young age, early onset of seizures, the presence of generalized tonic clonic seizures, male sex and being in bed. Weak risk factors for SUDEP: prone position, one or more subtherapeutic bloodlevels, being in the bedroom, a strucural brain lesion and sleeping. CONCLUSIONS: In this study, authors have designed a quality scale to select papers. The relative importance of risk factors for SUDEP is demonstrated.

Can spikes predict seizure frequency? Results of a pilot study in severe childhood epilepsies treated with vagus nerve stimulation.

We evaluated whether spike-rates are useful as an outcome parameter following vagus nerve stimulation (VNS). Spikes/minute and spikebursts/minute were counted in serial electroencephalograms before and after implantation of a vagus nerve stimulator in n = 19 patients with severe childhood epilepsies. In the period of 2 years post VNS, spike-rate and reported seizure frequency were significantly correlated (Spearman's R = 0.61); spikebursts and seizures were correlated with R = 0.74. The response rate, counted after 6 months, was too small to detect differences in responders and non-responders as to spike-reduction. Larger samples and effect sizes are necessary to prove the hypothesis that spike reduction is useful as outcome parameter after VNS or other interventions.