title: Misleading effects of clonazepam in symptomatic electrical status epilepticus during sleep syndrome.
abstract: Electrical status epilepticus during sleep syndrome and its variants are age-dependent epileptic encephalopathies associated with a sleep-related electroencephalographic pattern of continuous spike-waves, combined with motor or cognitive impairment. These epileptic encephalopathies are usually not responsive to conventional antiepileptic drugs. This report describes two children in whom clonazepam had no effect on cognitive and motor disorders but controlled spike activity, preventing a proper diagnosis. Withdrawal of clonazepam was accompanied by the recurrence of continuous spike-waves in slow sleep, permitting the diagnosis of electrical status epilepticus during sleep syndrome and appropriate therapeutic decisions. These two cases of the misleading effect of clonazepam in electrical status epilepticus during sleep syndrome illustrate the puzzling situation that can occur when therapeutic options only consider the electroencephalographic features without prior syndromic diagnosis.
authors.affiliation: Département de Pédiatrie, Service de Neuropédiatrie et Maladies Métaboliques, Hôpital Necker, Paris, France. nadia.bahi-buisson@nck.ap-hop-paris.fr
authors.forename: Nadia
authors.initials: N
authors.lastname: Bahi-Buisson
authors.forename: Rosanna
authors.initials: R
authors.lastname: Savini
authors.forename: Monica
authors.initials: M
authors.lastname: Eisermann
authors.forename: Christine
authors.initials: C
authors.lastname: Bulteau
authors.forename: Olivier
authors.initials: O
authors.lastname: Dulac
authors.forename: Lucie
authors.initials: L
authors.lastname: Hertz-Pannier
authors.forename: Catherine
authors.initials: C
authors.lastname: Chiron
mesh_terms: D000927:Anticonvulsants / Q000627:therapeutic use*; D002648:Child; D002998:Clonazepam / Q000627:therapeutic use*; D003072:Cognition Disorders / Q000209:etiology; D004569:Electroencephalography; D006801:Humans; D008297:Male; D019957:Motor Skills Disorders / Q000209:etiology; D012893:Sleep Wake Disorders / Q000175:diagnosis* / Q000188:drug therapy* / Q000523:psychology; D013226:Status Epilepticus / Q000175:diagnosis* / Q000188:drug therapy* / Q000523:psychology; D013577:Syndrome
chemical_list: D000927:Anticonvulsants; D002998:Clonazepam