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Lamberts R.J.,Stichting Epilepsie Instellingen Nederland SEIN
Nederlands tijdschrift voor geneeskunde | Year: 2013

Sudden unexpected death in epilepsy (SUDEP) is the most important direct epilepsy-related cause of death. Patients with refractory epilepsy are at especially high risk of SUDEP. SUDEP occurs predominantly at night and unwitnessed, and can best be regarded as a fatal tonic-clonic seizure. While its pathophysiology is incompletely understood, SUDEP is most probably triggered by a number of predisposing and precipitating factors, including seizure-induced respiratory depression and cardiac arrhythmia. Achieving seizure freedom is the best way to prevent SUDEP. Nocturnal supervision may be another alternative preventive strategy, but this requires further research.

Van Sonderen A.,Erasmus Medical Center | Schreurs M.W.J.,Erasmus Medical Center | De Bruijn M.A.A.M.,Erasmus Medical Center | Boukhrissi S.,Erasmus Medical Center | And 7 more authors.
Neurology | Year: 2016

Objective: To assess the clinical relevance of a positive voltage-gated potassium channel (VGKC) test in patients lacking antibodies to LGI1 and Caspr2. Methods: VGKC-positive patients were tested for LGI1 and Caspr2 antibodies. Patients lacking both antibodies were matched (1:2) to VGKC-negative patients. Clinical and paraclinical criteria were used to blindly determine evidence for autoimmune inflammation in both groups. Patients with an inconclusive VGKC titer were analyzed in the same way. Results: A total of 1,455 patients were tested by VGKC radioimmunoassay. Fifty-six patients tested positive, 50 of whom were available to be included. Twenty-five patients had antibodies to LGI1 (n 19) or Caspr2 (n 6) and 25 patients lacked both antibodies. Evidence for autoimmune inflammation was present in 7 (28%) of the VGKC-positive patients lacking LGI1 and Caspr2, compared to 9 (18%) of the VGKC-negative controls (p 0.38). Evidence for autoimmune inflammation was mainly found in patients with limbic encephalitis/encephalomyelitis (57%), but not in other clinical phenotypes (5%, p < 0.01). VGKC titers were significantly higher in patients with antibodies to LGI1 or Caspr2 (p < 0.001). However, antibodies to Caspr2 could also be detected in patients with inconclusive low VGKC titer, while many VGKC-positive patients had no evidence for autoimmune inflammation. Conclusions: VGKC positivity in the absence of antibodies to LGI1 and Caspr2 is not a clear marker for autoimmune inflammation and seems not to contribute in clinical practice. No cutoff value for the VGKC titer was appropriate to discriminate between patients with and without autoimmune inflammation. © 2016 American Academy of Neurology.

Keezer M.R.,University College London | Bauer P.R.,Stichting Epilepsie Instellingen Nederland SEIN | Ferrari M.D.,Leiden University | Sander J.W.,University College London
European Journal of Neurology | Year: 2015

A number of studies have suggested a pathophysiologic link between migraine and epilepsy. Our aim was to examine the relative lifetime prevalence of migraine in people with epilepsy (PWE) as well that of epilepsy in migraineurs. We carried out a systematic review, searching five electronic databases, specified bibliographies and conference abstracts in order to identify population-based studies that measured the lifetime co-prevalence of migraine and epilepsy. Two reviewers independently screened all titles and abstracts, carried out a risk of bias assessment and extracted the data. Meta-analyses were carried out using random effects models. Of the 3640 abstracts and titles screened, we identified 10 eligible studies encompassing a total of 1 548 967 subjects. Few of the studies used validated case ascertainment tools and there were inconsistent attempts to control for confounding. There was an overall 52% increase in the prevalence of migraine among PWE versus those without epilepsy [PR: 1.52 (95% CI: 1.29, 1.79)]. There was an overall 79% increase in the prevalence of epilepsy among migraineurs versus those without migraine [PR: 1.79 (95% CI: 1.43, 2.25)]. Subgroup analyses revealed that the method of ascertaining the epilepsy or migraine status of subjects was an important source of inter-study heterogeneity. Additional high quality primary studies are required, ones that use validated and accurate methods of case ascertainment as well as control for potential confounders. © 2014 EAN.

Giussani G.,Istituto di Ricerche Farmacologiche Mario Negri | Canelli V.,Istituto di Ricerche Farmacologiche Mario Negri | Bianchi E.,Istituto di Ricerche Farmacologiche Mario Negri | Erba G.,University of Rochester | And 5 more authors.
European Journal of Neurology | Year: 2016

Background and purpose: Seizures in most people with epilepsy remit but prognostic markers are poorly understood. There is also little information on the long-term outcome of people who fail to achieve seizure control despite the use of two antiepileptic drugs (drug resistance). Methods: People with a validated diagnosis of epilepsy in whom two antiepileptic drugs had failed were identified from primary care records. All were registered with one of 123 family physicians in an area of northern Italy. Remission (uninterrupted seizure freedom lasting 2 years or longer) and prognostic patterns (early remission, late remission, remission followed by relapse, no remission) were determined. Results: In all, 747 individuals (381 men), aged 11 months to 94 years, were followed for 11 045.5 person-years. 428 (59%) were seizure-free. The probability of achieving 2-year remission was 18% at treatment start, 34% at 2 years, 45% at 5, 52% at 10 and 67% at 20 years (terminal remission, 60%). Epilepsy syndrome and drug resistance were the only independent predictors of 2- and 5-year remission. Early remission was seen in 101 people (19%), late remission in 175 (33%), remission followed by relapse in 85 (16%) and no remission in 166 (32%). Treatment response was the only variable associated with differing prognostic patterns. Conclusion: The long-term prognosis of epilepsy is favourable in most cases. Early seizure remission is not invariably followed by terminal remission and seizure outcome varies according to well-defined patterns. Prolonged seizure remission and prognostic patterns can be predicted by broad syndromic categories and the failure of two antiepileptic drugs. © 2016 EAN

van Andel J.,University Utrecht | Thijs R.D.,Stichting Epilepsie Instellingen Nederland SEIN | Thijs R.D.,Leiden University | de Weerd A.,Stichting Epilepsie Instellingen Nederland SEIN | And 2 more authors.
Epilepsy and Behavior | Year: 2016

Objective: This study aimed to (1) evaluate available systems and algorithms for ambulatory automatic seizure detection and (2) discuss benefits and disadvantages of seizure detection in epilepsy care. Methods: PubMed and EMBASE were searched up to November 2014, using variations and synonyms of search terms "seizure prediction" OR "seizure detection" OR "seizures" AND "alarm". Results: Seventeen studies evaluated performance of devices and algorithms to detect seizures in a clinical setting. Algorithms detecting generalized tonic-clonic seizures (GTCSs) had varying sensitivities (11% to 100%) and false alarm rates (0.2-4/24 h). For other seizure types, detection rates were low, or devices produced many false alarms. Five studies externally validated the performance of four different devices for the detection of GTCSs. Two devices were promising in both children and adults: a mattress-based nocturnal seizure detector (sensitivity: 84.6% and 100%; false alarm rate: not reported) and a wrist-based detector (sensitivity: 89.7%; false alarm rate: 0.2/24 h). Significance: Detection of seizure types other than GTCSs is currently unreliable. Two detection devices for GTCSs provided promising results when externally validated in a clinical setting. However, these devices need to be evaluated in the home setting in order to establish their true value. Automatic seizure detection may help prevent sudden unexpected death in epilepsy or status epilepticus, provided the alarm is followed by an effective intervention. Accurate seizure detection may improve the quality of life (QoL) of subjects and caregivers by decreasing burden of seizure monitoring and may facilitate diagnostic monitoring in the home setting. Possible risks are occurrence of alarm fatigue and invasion of privacy. Moreover, an unexpectedly high seizure frequency might be detected for which there are no treatment options. We propose that future studies monitor benefits and disadvantages of seizure detection systems with particular emphasis on QoL, comfort, and privacy of subjects and impact of false alarms. © 2016.

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