title: Unusual consequences of status epilepticus in Dravet syndrome.
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.
authors.affiliation: Service de neuropédiatrie, Centre de référence épilepsies rares, AP-HP, Hôpital Necker Enfants Malades, Paris, France. mathilde.chipaux@nck.aphp.fr
authors.forename: M
authors.initials: M
authors.lastname: Chipaux
authors.forename: N
authors.initials: N
authors.lastname: Villeneuve
authors.forename: P
authors.initials: P
authors.lastname: Sabouraud
authors.forename: I
authors.initials: I
authors.lastname: Desguerre
authors.forename: N
authors.initials: N
authors.lastname: Boddaert
authors.forename: C
authors.initials: C
authors.lastname: Depienne
authors.forename: C
authors.initials: C
authors.lastname: Chiron
authors.forename: O
authors.initials: O
authors.lastname: Dulac
authors.forename: R
authors.initials: R
authors.lastname: Nabbout
mesh_terms: D000927:Anticonvulsants / Q000009:adverse effects; D001463:Barbiturates / Q000009:adverse effects; D001921:Brain / Q000098:blood supply / Q000473:pathology*; D002675:Child, Preschool; D002658:Developmental Disabilities / Q000209:etiology; D004827:Epilepsy / Q000188:drug therapy / Q000235:genetics / Q000473:pathology*; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D009154:Mutation; D062550:NAV1.1 Voltage-Gated Sodium Channel; D009419:Nerve Tissue Proteins / Q000235:genetics; D015222:Sodium Channels / Q000235:genetics; D013226:Status Epilepticus / Q000188:drug therapy / Q000235:genetics / Q000473:pathology*; D014057:Tomography, X-Ray Computed
chemical_list: D000927:Anticonvulsants; D001463:Barbiturates; D062550:NAV1.1 Voltage-Gated Sodium Channel; D009419:Nerve Tissue Proteins; C568242:SCN1A protein, human; D015222:Sodium Channels