Autosomal dominant nocturnal frontal lobe epilepsy

Research output: Chapter in Book/Report/Conference proceedingChapter


The causal disease In 1981, Lugaresi and Cirignotta described five patients with frequent episodes characterized by violent limb movements and dystonic–tonic posturing during light sleep. The unusual motor behavior, uniform in all patients with stereotyped dystonic-dyskinetic aspects, the lack of clear-cut ictal and interictal abnormalities on electroencephalogram (EEG) and the response to carbamazepine led the authors to label this condition “hypnogenic paroxysmal dystonia.” Some years later, the authors better defined this condition, renaming it “nocturnal paroxysmal dystonia” (NPD) (Lugaresi et al.1986). Since the first descriptions, the epileptic or non-epileptic origin of short-lasting NPD attacks has been debated. However, the similarity of NPD attacks with complex, often bizarre motor behavior, bipedal and bimanual activity, and violent rocking axial and pelvic movements characteristic of frontal seizures recorded in patients undergoing neurosurgical treatment for drug-resistant epilepsy, together with the demonstration of clear-cut epileptiform discharges in some patients, permitted the Bologna group to demonstrate that short-lasting, stereotyped attacks of NPD were in fact epileptic seizures originating in the mesial surface of the frontal lobes (Tinuper et al. 1990, 2002; Provini et al. 1999). The term “nocturnal frontal lobe epilepsy” (NFLE) was therefore adopted. Meanwhile, in 1994 Scheffer et al. first described a large Australian family with nocturnal frontal lobe seizures (NFLS) inherited in an autosomal dominant manner and named this condition “autosomal dominant nocturnal frontal lobe epilepsy” (ADNFLE). Following the initial recognition, more than a hundred families have been described worldwide (Picard et al. 2006).

Original languageEnglish
Title of host publicationThe Causes of Epilepsy: Common and Uncommon Causes in Adults and Children
PublisherCambridge University Press
Number of pages4
ISBN (Print)9780511921001, 9780521114479
Publication statusPublished - Jan 1 2011

ASJC Scopus subject areas

  • Medicine(all)


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