Abstracts

Rationale: Sudden Unexpected Death in Epilepsy (SUDEP) is a devastating cause of mortality in patients with epilepsy. Several risk factors of SUDEP have been identified. They differ according to epilepsy syndromes. We tried to recognize risk factors of SUDEP in Unverricht–Lundborg disease (ULD) one of the most common Progressive Myoclonic Epilepsy, frequent around the Mediterranean.  Methods: A Tunisian French multicentric retrospective study was performed. Patients with a proven clinical and genetically ULD over the past 16 years prior to the study period were extracted. We assessed age, sex, severity and duration of disease, antiepileptic drugs, circumstances and presumed cause of death. Statistical analyses were performed through the parameters of demographical data clinical features, and cause of death initially for all cases and then separately for SUDEP cases.  Results: A high percentage of SUDEP cases were observed in the two centres: On a total of 19 deaths, gathering all the fatalities, six cases of SUDEP were identified (4 From Tunisia and two from France). All patients were females. Three Tunisian patients belonged to the same family (two sisters and a cousin). The median age at death was 19.5 years (12-40 years). The duration of epilepsy was 9 years (2- 26 years). The median age of seizure onset was of 10 years. One year prior to death, the median generalised tonic clonic seizures frequency was of 1 seizure per year. At the time of death, all patients were on AEDs. The median number of medications was 1 (1-4). In all patients, the state before death was known; 80% were asleep and only 20 % were awake.The following three factors significantly increased the risk of SUDEP in patients with ULD: young age (p=0.034), polytherapy (p= 95% CI: 2.2–16.6), and two specific anti-epileptic treatment (Levetiracetam and/or clonazepam (p=0.04)). No correlation was observed between the incidence of SUDEP psychiatric therapy, family history of SUDEP or seizure frequency.    Conclusions: Although ULD stands out among PMEs as a comparatively benign disorder, we identified a high risk of SUDEP. Young age and some specific AED are identified as risk factors for SUDEP.  Funding: No funding