Unusual consequences of status epilepticus in Dravet syndrome

Chipaux, M.; Villeneuve, N.; Sabouraud, P.; Desguerre, I.; Boddaert, N.; Depienne, C.; Chiron, C.; Dulac, O.; Nabbout, R.

doi:10.1016/j.seizure.2010.01.007

« Previous Next »Seizure: European Journal of Epilepsy
Volume 19, Issue 3 , Pages 190-194, April 2010

Unusual consequences of status epilepticus in Dravet syndrome

M. Chipaux
- Service de neuropédiatrie, Centre de référence épilepsies rares, AP-HP, Hôpital Necker Enfants Malades, Paris, France
- Inserm, U663, Paris, F-75015 France; University Paris Descartes, Paris, F-75015, France
- Corresponding author at: Service de neuropédiatrie, Centre de référence épilepsies rares, AP-HP, Hôpital Necker Enfants Malades, Paris, France. Tel.: +33 144481536; fax: +33 142192692.
N. Villeneuve
- Centre Saint-Paul, Hôpital Henri Gastaut, Marseille, France
P. Sabouraud
- Hôpital Américain, Service de Pédiatrie A, CHU de Reims, Reims, France
I. Desguerre
- Service de neuropédiatrie, Centre de référence épilepsies rares, AP-HP, Hôpital Necker Enfants Malades, Paris, France
N. Boddaert
- Service de radiologie pédiatrique, AP-HP, Hôpital Necker Enfants Malades, Paris, France
C. Depienne
- UF de neurogénétique moléculaire et cellulaire, Hôpital Pitié-Salpétrière, Paris, France
C. Chiron
- Service de neuropédiatrie, Centre de référence épilepsies rares, AP-HP, Hôpital Necker Enfants Malades, Paris, France
- Inserm, U663, Paris, F-75015 France; University Paris Descartes, Paris, F-75015, France
O. Dulac
- Service de neuropédiatrie, Centre de référence épilepsies rares, AP-HP, Hôpital Necker Enfants Malades, Paris, France
- Inserm, U663, Paris, F-75015 France; University Paris Descartes, Paris, F-75015, France
R. Nabbout
- Service de neuropédiatrie, Centre de référence épilepsies rares, AP-HP, Hôpital Necker Enfants Malades, Paris, France
- Inserm, U663, Paris, F-75015 France; University Paris Descartes, Paris, F-75015, France

Received 30 June 2009; received in revised form 7 January 2010; accepted 15 January 2010. published online 22 February 2010.

Abstract

Although status epilepticus (SE) affects the course of Dravet syndrome (DS), it rarely alters dramatically psychomotor outcome. We report an unusual pattern in 3 patients who following refractory SE lasting respectively 2, 7 and 12h experienced persistent and severe cognitive and motor deterioration. We compared these patients to published data and to personal experience in Necker hospital, to find links between severe outcome and clinical features such as treatment or duration of refractory SE. The key point was that anoxoischemic-like lesions appeared on MRI although cardiovascular function had remained stable. Therefore, neither hemodynamic failure, nor abnormalities of cardiac rhythm could explain the lesions and neurological worsening. For theoretical reasons the responsibility of therapy common for the 3 patients, e.g., barbiturates was suspected.