SUDEP and Dravet Syndrome SUDEP et Syndrome Dravet Dr. Kenneth Myers April 14, 2018

Qu’est-ce que les familles veulent? What do families want? Qu’est-ce que les familles veulent? Families of people who suffered SUDEP, have almost unanimously stated that they believe SUDEP risk should be discussed by doctors. Les familles des personnes qui ont souffrent de la SUDEP, ont presque unanimement déclaré qu’elles croient que la risque SUDEP devrait être discuté par les médecins Felt that best time for discussion was at the time of diagnosis with epilepsy. A face-to-face encounter, supplemented with written information would be ideal * Ramachandran Nair et al (2016), Bellon et al (2015)

What do doctors do? Que font les médecins? 18% of families of patients suffering SUDEP recalled a discussion of the risk with their doctor. 16% of neurologists felt SUDEP should be discussed with all epilepsy patients. 18% des familles de patients souffrant de SUDEP ont rappelé une discussion sur le risque avec leur médecin 16% des neurologues croient que SUDEP devrait être discuté avec tous les patients atteints d’épilepsie. Felt that best time for discussion was at the time of diagnosis with epilepsy. A face-to-face encounter, supplemented with written information would be ideal * Louik et al (2017), Galli et al (2017)

What is SUDEP? Qu’est-ce que SUDEP? “… a category of death in people with epilepsy occurring in the absence of a known structural cause of death …” “… une catégorie de décès chez les personnes atteintes d’épilepsie survenant en l’absence d’une cause structurelle connue de décès …” Felt that best time for discussion was at the time of diagnosis with epilepsy. A face-to-face encounter, supplemented with written information would be ideal * Nashef et al (2012)

What is SUDEP? Qu’est-ce que SUDEP? The typical story is that the person goes to bed appearing well, and is then found in the morning, having died overnight. L’histoire typique est que la personne se couche bien, et se retrouve ensuite le matin, morte pendant la nuit. Felt that best time for discussion was at the time of diagnosis with epilepsy. A face-to-face encounter, supplemented with written information would be ideal * Nashef et al (2012)

What is SUDEP? Qu’est-ce que SUDEP? In the few witnessed cases, most deaths have occurred following convulsive seizures. However, some deaths occur without a clear provoking event. Dans les quelques cas observés, la plupart des décès sont survenus à la suite de crises convulsives. Cependant, certains décès se produisent sans un événement provocateur clair. Felt that best time for discussion was at the time of diagnosis with epilepsy. A face-to-face encounter, supplemented with written information would be ideal

What is the frequency of SUDEP? Quelle est la fréquence de SUDEP? All people with epilepsy 1.2/1000 patient-years Drug-resistant epilepsy 4.2/1000 patient-years Dravet syndrome 9.3/1000 patient-years Toutes les personnes atteintes d’épilepsie 1.2/1000 années-patient Épilepsie pharmacorésistante 4.2/1000 années-patient Syndrome Dravet 9.3/1000 années-patient Felt that best time for discussion was at the time of diagnosis with epilepsy. A face-to-face encounter, supplemented with written information would be ideal * Cooper et al (2016), Devinsky et al (2016), Tomson et al (2008)

How does SUDEP contribute to Dravet mortality? Comment SUDEP contribute à la mortalité Dravet Early mortality is high in Dravet syndrome, ~15% by age 20 SUDEP is ~59% of mortality in Dravet syndrome La mortalité précoce est élevée dans le Syndrome Dravet, ~15% avant 20 ans SUDEP est ~59% des décès dans Syndrome Dravet Felt that best time for discussion was at the time of diagnosis with epilepsy. A face-to-face encounter, supplemented with written information would be ideal

What are risks for SUDEP? Quelle est les risques pour SUDEP? Uncontrolled convulsive seizures Medication noncompliance Male sex Seizures started before 16 years Prone sleeping position Seizures for more than 15 years RISQUES Convulsions incôntrolées Non-conformité aux médicaments Sexe masculin Crises commence avant 16 ans Position couchée couchée Crises pour > 15 ans Felt that best time for discussion was at the time of diagnosis with epilepsy. A face-to-face encounter, supplemented with written information would be ideal

Peut SUDEP être empêché? Can SUDEP be prevented? Peut SUDEP être empêché? No therapies or measures proven to prevent SUDEP Optimizing seizure control is likely best method to reduce risk Aucune thérapie ou mesure éprouvée pour prévenir SUDEP L’optimisation de contrôle des crises est probablement la meilleure méthode pour réduire la risque Felt that best time for discussion was at the time of diagnosis with epilepsy. A face-to-face encounter, supplemented with written information would be ideal

Les Recherches En Cours Current Research Les Recherches En Cours Felt that best time for discussion was at the time of diagnosis with epilepsy. A face-to-face encounter, supplemented with written information would be ideal

Heart Rate Variability Variabilité de la Fréquence Cardiaque Felt that best time for discussion was at the time of diagnosis with epilepsy. A face-to-face encounter, supplemented with written information would be ideal

Felt that best time for discussion was at the time of diagnosis with epilepsy. A face-to-face encounter, supplemented with written information would be ideal

Summary Résumé SUDEP is a rare event, but accounts for more than half of deaths in Dravet syndrome Controlling convulsive seizures is presently our best approach to minimizing SUDEP risk in Dravet syndrome Considerable research is presently underway to better understand SUDEP, and develop methods to prevent it SUDEP est une événement rare, mais compte pour plus de la moitié des décès dans Syndrome Dravet Le contrôle des crises convulsives est actuellement notre meilleure approche pour minimiser le risque SUDEP dans syndrome Dravet Des récherches considerable sont actuellement en cours pour mieux comprendre le SUDEP, et développer des méthodes pour le prévenir Felt that best time for discussion was at the time of diagnosis with epilepsy. A face-to-face encounter, supplemented with written information would be ideal

Questions? Felt that best time for discussion was at the time of diagnosis with epilepsy. A face-to-face encounter, supplemented with written information would be ideal