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Sleep-related epilepsy syndromes

Erik K St Louis, MD, MS
Section Editors
Alon Y Avidan, MD, MPH
Paul Garcia, MD
Deputy Editor
Janet L Wilterdink, MD


Epileptic seizures frequently manifest during the sleep state and are often difficult to distinguish from parasomnias and other nonepileptic events, particularly when seizures occur exclusively during sleep. While most epilepsies have a diurnal component (ie, seizures may occur during both sleep and wake), several epilepsy syndromes have an especially intimate and robust relationship with sleep.

This topic reviews the pathophysiology of seizures and epilepsy related to sleep and the clinical features, diagnosis, and treatment of common sleep-related epilepsy syndromes. The clinical features, diagnosis, and treatment of seizures and epilepsy more generally are reviewed elsewhere. (See "Evaluation and management of the first seizure in adults" and "Overview of the management of epilepsy in adults" and "Clinical and laboratory diagnosis of seizures in infants and children" and "Seizures and epilepsy in children: Classification, etiology, and clinical features" and "Seizures and epilepsy in children: Initial treatment and monitoring".)


Although the International League Against Epilepsy does not formally classify sleep-related epilepsies, in practice they can be divided into three major categories:

Pure sleep epilepsies, in which seizures occur exclusively or predominantly during sleep (eg, benign epilepsy of childhood with centrotemporal spikes, nocturnal frontal lobe epilepsy) (see 'Benign focal epilepsies of childhood' below and 'Nocturnal (sleep-related) focal epilepsies' below)

Sleep-accentuated epilepsies, in which seizures occur during both wakefulness and sleep but epileptiform activity is potentiated during sleep (eg, certain epileptic encephalopathies, including Lennox-Gastaut syndrome and Landau-Kleffner syndrome) (see 'Sleep-accentuated epilepsies' below)

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Literature review current through: Nov 2017. | This topic last updated: Mar 24, 2017.
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  1. Thomas RH, King WH, Johnston JA, Smith PE. Awake seizures after pure sleep-related epilepsy: a systematic review and implications for driving law. J Neurol Neurosurg Psychiatry 2010; 81:130.
  2. Ekizoglu E, Baykan B, Bebek N, et al. Sleep characteristics of patients with epilepsy with pure sleep-related seizures. Epilepsy Behav 2011; 21:71.
  3. Yaqub BA, Waheed G, Kabiraj MM. Nocturnal epilepsies in adults. Seizure 1997; 6:145.
  4. D'Alessandro R, Guarino M, Greco G, et al. Risk of seizures while awake in pure sleep epilepsies: a prospective study. Neurology 2004; 62:254.
  5. Fernández LB, Salas-Puig J. Pure sleep seizures: risk of seizures while awake. Epileptic Disord 2007; 9:65.
  6. Malow BA, Lin X, Kushwaha R, Aldrich MS. Interictal spiking increases with sleep depth in temporal lobe epilepsy. Epilepsia 1998; 39:1309.
  7. Sammaritano M, Gigli GL, Gotman J. Interictal spiking during wakefulness and sleep and the localization of foci in temporal lobe epilepsy. Neurology 1991; 41:290.
  8. Kumar P, Raju TR. Seizure susceptibility decreases with enhancement of rapid eye movement sleep. Brain Res 2001; 922:299.
  9. Rocamora R, Andrzejak RG, Jiménez-Conde J, Elger CE. Sleep modulation of epileptic activity in mesial and neocortical temporal lobe epilepsy: a study with depth and subdural electrodes. Epilepsy Behav 2013; 28:185.
  10. Usami K, Matsumoto R, Kobayashi K, et al. Sleep modulates cortical connectivity and excitability in humans: Direct evidence from neural activity induced by single-pulse electrical stimulation. Hum Brain Mapp 2015; 36:4714.
  11. Parrino L, Smerieri A, Spaggiari MC, Terzano MG. Cyclic alternating pattern (CAP) and epilepsy during sleep: how a physiological rhythm modulates a pathological event. Clin Neurophysiol 2000; 111 Suppl 2:S39.
  12. Parrino L, Halasz P, Tassinari CA, Terzano MG. CAP, epilepsy and motor events during sleep: the unifying role of arousal. Sleep Med Rev 2006; 10:267.
  13. Parrino L, Smerieri A, Terzano MG. Combined influence of cyclic arousability and EEG synchrony on generalized interictal discharges within the sleep cycle. Epilepsy Res 2001; 44:7.
  14. Bower MR, Stead M, Bower RS, et al. Evidence for consolidation of neuronal assemblies after seizures in humans. J Neurosci 2015; 35:999.
  15. Sarkis RA, Alam J, Pavlova MK, et al. Sleep-dependent memory consolidation in the epilepsy monitoring unit: A pilot study. Clin Neurophysiol 2016; 127:2785.
  16. Ng M, Pavlova M. Why are seizures rare in rapid eye movement sleep? Review of the frequency of seizures in different sleep stages. Epilepsy Res Treat 2013; 2013:932790.
  17. Herman ST, Walczak TS, Bazil CW. Distribution of partial seizures during the sleep--wake cycle: differences by seizure onset site. Neurology 2001; 56:1453.
  18. St Louis EK, Genilo P, Granner MA, Zimmerman B. Sleep-onset mesial temporal seizures arise from light NREM sleep. Epilepsia 2004; 45(Suppl 7):86.
  19. Bazil CW, Castro LH, Walczak TS. Reduction of rapid eye movement sleep by diurnal and nocturnal seizures in temporal lobe epilepsy. Arch Neurol 2000; 57:363.
  20. Crespel A, Baldy-Moulinier M, Coubes P. The relationship between sleep and epilepsy in frontal and temporal lobe epilepsies: practical and physiopathologic considerations. Epilepsia 1998; 39:150.
  21. Malow BA, Passaro E, Milling C, et al. Sleep deprivation does not affect seizure frequency during inpatient video-EEG monitoring. Neurology 2002; 59:1371.
  22. Quigg M, Gharai S, Ruland J, et al. Insomnia in epilepsy is associated with continuing seizures and worse quality of life. Epilepsy Res 2016; 122:91.
  23. Tinuper P, Bisulli F, Cross JH, et al. Definition and diagnostic criteria of sleep-related hypermotor epilepsy. Neurology 2016; 86:1834.
  24. Steinlein OK, Mulley JC, Propping P, et al. A missense mutation in the neuronal nicotinic acetylcholine receptor alpha 4 subunit is associated with autosomal dominant nocturnal frontal lobe epilepsy. Nat Genet 1995; 11:201.
  25. Steinlein OK, Magnusson A, Stoodt J, et al. An insertion mutation of the CHRNA4 gene in a family with autosomal dominant nocturnal frontal lobe epilepsy. Hum Mol Genet 1997; 6:943.
  26. Nobili L, Proserpio P, Combi R, et al. Nocturnal frontal lobe epilepsy. Curr Neurol Neurosci Rep 2014; 14:424.
  27. Nobili L, Cardinale F, Magliola U, et al. Taylor's focal cortical dysplasia increases the risk of sleep-related epilepsy. Epilepsia 2009; 50:2599.
  28. Tassi L, Garbelli R, Colombo N, et al. Electroclinical, MRI and surgical outcomes in 100 epileptic patients with type II FCD. Epileptic Disord 2012; 14:257.
  29. Proserpio P, Cossu M, Francione S, et al. Epileptic motor behaviors during sleep: anatomo-electro-clinical features. Sleep Med 2011; 12 Suppl 2:S33.
  30. Provini F, Plazzi G, Tinuper P, et al. Nocturnal frontal lobe epilepsy. A clinical and polygraphic overview of 100 consecutive cases. Brain 1999; 122 ( Pt 6):1017.
  31. Terzaghi M, Sartori I, Mai R, et al. Coupling of minor motor events and epileptiform discharges with arousal fluctuations in NFLE. Epilepsia 2008; 49:670.
  32. Terzaghi M, Sartori I, Mai R, et al. Sleep-related minor motor events in nocturnal frontal lobe epilepsy. Epilepsia 2007; 48:335.
  33. Provini F, Plazzi G, Lugaresi E. From nocturnal paroxysmal dystonia to nocturnal frontal lobe epilepsy. Clin Neurophysiol 2000; 111 Suppl 2:S2.
  34. Oldani A, Zucconi M, Ferini-Strambi L, et al. Autosomal dominant nocturnal frontal lobe epilepsy: electroclinical picture. Epilepsia 1996; 37:964.
  35. Oldani A, Zucconi M, Asselta R, et al. Autosomal dominant nocturnal frontal lobe epilepsy. A video-polysomnographic and genetic appraisal of 40 patients and delineation of the epileptic syndrome. Brain 1998; 121 ( Pt 2):205.
  36. Tinuper P, Cerullo A, Cirignotta F, et al. Nocturnal paroxysmal dystonia with short-lasting attacks: three cases with evidence for an epileptic frontal lobe origin of seizures. Epilepsia 1990; 31:549.
  37. Meierkord H, Fish DR, Smith SJ, et al. Is nocturnal paroxysmal dystonia a form of frontal lobe epilepsy? Mov Disord 1992; 7:38.
  38. Bernasconi A, Andermann F, Cendes F, et al. Nocturnal temporal lobe epilepsy. Neurology 1998; 50:1772.
  39. Ferri L, Bisulli F, Nobili L, et al. Auditory aura in nocturnal frontal lobe epilepsy: a red flag to suspect an extra-frontal epileptogenic zone. Sleep Med 2014; 15:1417.
  40. Nobili L, Cossu M, Mai R, et al. Sleep-related hyperkinetic seizures of temporal lobe origin. Neurology 2004; 62:482.
  41. Ryvlin P, Minotti L, Demarquay G, et al. Nocturnal hypermotor seizures, suggesting frontal lobe epilepsy, can originate in the insula. Epilepsia 2006; 47:755.
  42. Gibbs SA, Figorilli M, Casaceli G, et al. Sleep Related Hypermotor Seizures with a Right Parietal Onset. J Clin Sleep Med 2015; 11:953.
  43. Derry CP, Davey M, Johns M, et al. Distinguishing sleep disorders from seizures: diagnosing bumps in the night. Arch Neurol 2006; 63:705.
  44. Manni R, Terzaghi M, Repetto A. The FLEP scale in diagnosing nocturnal frontal lobe epilepsy, NREM and REM parasomnias: data from a tertiary sleep and epilepsy unit. Epilepsia 2008; 49:1581.
  45. Lugaresi E, Cirignotta F. Hypnogenic paroxysmal dystonia: epileptic seizure or a new syndrome? Sleep 1981; 4:129.
  46. Lugaresi E, Cirignotta F, Montagna P. Nocturnal paroxysmal dystonia. J Neurol Neurosurg Psychiatry 1986; 49:375.
  47. Park SA, Lee BI, Park SC, et al. Clinical courses of pure sleep epilepsies. Seizure 1998; 7:369.
  48. Nobili L, Francione S, Mai R, et al. Surgical treatment of drug-resistant nocturnal frontal lobe epilepsy. Brain 2007; 130:561.
  49. Hirshkowitz M, Whiton K, Albert SM, et al. National Sleep Foundation's updated sleep duration recommendations: final report. Sleep Health 2015; 1:233.
  50. St Louis EK. Diagnosing And Treating Co-Morbid Sleep Apnea In Neurological Disorders. Pract Neurol (Fort Wash Pa) 2010; 9:26.
  51. St Louis EK. Diagnosing and Treating Co-morbid Sleep Apnea in Neurological Disorders, Part II. Pract Neurol (Fort Wash Pa) 2010; 9:26.
  52. Pornsriniyom D, Kim Hw, Bena J, et al. Effect of positive airway pressure therapy on seizure control in patients with epilepsy and obstructive sleep apnea. Epilepsy Behav 2014; 37:270.
  53. Thurman DJ, Logroscino G, Beghi E, et al. The burden of premature mortality of epilepsy in high-income countries: A systematic review from the Mortality Task Force of the International League Against Epilepsy. Epilepsia 2017; 58:17.
  54. Levira F, Thurman DJ, Sander JW, et al. Premature mortality of epilepsy in low- and middle-income countries: A systematic review from the Mortality Task Force of the International League Against Epilepsy. Epilepsia 2017; 58:6.
  55. Mostacci B, Bisulli F, Vignatelli L, et al. Incidence of sudden unexpected death in nocturnal frontal lobe epilepsy: a cohort study. Sleep Med 2015; 16:232.
  56. Krauss GL, Ampaw L, Krumholz A. Individual state driving restrictions for people with epilepsy in the US. Neurology 2001; 57:1780.
  57. Arzimanoglou A, French J, Blume WT, et al. Lennox-Gastaut syndrome: a consensus approach on diagnosis, assessment, management, and trial methodology. Lancet Neurol 2009; 8:82.
  58. Fernández IS, Chapman KE, Peters JM, et al. The tower of Babel: survey on concepts and terminology in electrical status epilepticus in sleep and continuous spikes and waves during sleep in North America. Epilepsia 2013; 54:741.
  59. JANZ D. The grand mal epilepsies and the sleeping-waking cycle. Epilepsia 1962; 3:69.