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Benign focal epilepsies of childhood

Selim R Benbadis, MD
Section Editor
Douglas R Nordli, Jr, MD
Deputy Editor
Janet L Wilterdink, MD


Benign focal (partial) epilepsies of childhood are electroclinical syndromes of unknown or genetic cause that occur in developmentally and neurologically normal children and have a benign course, remitting prior to adulthood. These epilepsy syndromes are distinguished from symptomatic focal epilepsy, which refers to epilepsy that results from brain injury or other structural brain disease. Thus, the benign focal epilepsies of childhood can be viewed as a spectrum of conditions with "functional" or "nonlesional" focal epileptogenicity, each characterized by location and seizure type(s). This is sometimes referred to as the benign childhood susceptibility syndrome [1,2].

The best-described syndromes are:

Benign (childhood) epilepsy with centrotemporal spikes (BCECTS or BECTS) or benign rolandic epilepsy

Benign occipital epilepsy of childhood (Gastaut syndrome)

Panayiotopoulos syndrome

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Literature review current through: Nov 2017. | This topic last updated: Oct 06, 2017.
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  1. Panayiotopoulos CP. Benign childhood partial epilepsies: benign childhood seizure susceptibility syndromes. J Neurol Neurosurg Psychiatry 1993; 56:2.
  2. Panayiotopoulos CP. Benign childhood focal seizures and related epileptic syndromes. In: A Clinical Guide to Epileptic Syndromes and Their Treatment, 2nd ed, Panayiotopoulos CP (Ed), Springer, London 2007. p.285.
  3. Lüders H, Lesser RP, Dinner DS, et al. Benign focal epilepsy of childhood. In: Epilepsy: Electroclinical Syndromes, Lüders H, Lesser RP (Eds), Springer-Verlag, London 1987. p.303.
  4. Kramer U, Zelnik N, Lerman-Sagie T, Shahar E. Benign childhood epilepsy with centrotemporal spikes: clinical characteristics and identification of patients at risk for multiple seizures. J Child Neurol 2002; 17:17.
  5. Kramer U, Nevo Y, Neufeld MY, et al. Epidemiology of epilepsy in childhood: a cohort of 440 consecutive patients. Pediatr Neurol 1998; 18:46.
  6. Cavazzuti GB. Epidemiology of different types of epilepsy in school age children of Modena, Italy. Epilepsia 1980; 21:57.
  7. Vears DF, Tsai MH, Sadleir LG, et al. Clinical genetic studies in benign childhood epilepsy with centrotemporal spikes. Epilepsia 2012; 53:319.
  8. Bouma PA, Bovenkerk AC, Westendorp RG, Brouwer OF. The course of benign partial epilepsy of childhood with centrotemporal spikes: a meta-analysis. Neurology 1997; 48:430.
  9. Vadlamudi L, Harvey AS, Connellan MM, et al. Is benign rolandic epilepsy genetically determined? Ann Neurol 2004; 56:129.
  10. Vadlamudi L, Kjeldsen MJ, Corey LA, et al. Analyzing the etiology of benign rolandic epilepsy: a multicenter twin collaboration. Epilepsia 2006; 47:550.
  11. Neubauer BA. The genetics of rolandic epilepsy. Epileptic Disord 2000; 2 Suppl 1:S67.
  12. Bali B, Kull LL, Strug LJ, et al. Autosomal dominant inheritance of centrotemporal sharp waves in rolandic epilepsy families. Epilepsia 2007; 48:2266.
  13. Pruna D, Persico I, Serra D. Lack of association with the 15q14 candidate region for benign epilepsy of childhood with centro-temporal spikes in a Sardinian population. Epilepsia 2000; 41:164.
  14. Neubauer BA, Waldegger S, Heinzinger J, et al. KCNQ2 and KCNQ3 mutations contribute to different idiopathic epilepsy syndromes. Neurology 2008; 71:177.
  15. Lemke JR, Lal D, Reinthaler EM, et al. Mutations in GRIN2A cause idiopathic focal epilepsy with rolandic spikes. Nat Genet 2013; 45:1067.
  16. Lesca G, Rudolf G, Bruneau N, et al. GRIN2A mutations in acquired epileptic aphasia and related childhood focal epilepsies and encephalopathies with speech and language dysfunction. Nat Genet 2013; 45:1061.
  17. Gregory DL, Wong PK. Topographical analysis of the centrotemporal discharges in benign rolandic epilepsy of childhood. Epilepsia 1984; 25:705.
  18. Loiseau P, Beaussart M. The seizures of benign childhood epilepsy with Rolandic paroxysmal discharges. Epilepsia 1973; 14:381.
  19. Uematsu S, Lesser R, Fisher RS, et al. Motor and sensory cortex in humans: topography studied with chronic subdural stimulation. Neurosurgery 1992; 31:59.
  20. Penfield W, Jasper H. Epilepsy and the Functional Anatomy of the Brain, Little, Brown and Co, Boston 1954.
  21. Lombroso CT. Sylvian seizures and midtemporal spike foci in children. Arch Neurol 1967; 17:52.
  22. Ma CK, Chan KY. Benign childhood epilepsy with centrotemporal spikes: a study of 50 Chinese children. Brain Dev 2003; 25:390.
  23. Gregory DL, Farrell K, Wong PK. Partial status epilepticus in benign childhood epilepsy with centrotemporal spikes: are independent right and left seizures a risk factor? Epilepsia 2002; 43:936.
  24. Dai AI, Weinstock A. Postictal paresis in children with benign rolandic epilepsy. J Child Neurol 2005; 20:834.
  25. Dai AI, Demiryürek S. The Clinical Implications of Todd Paralysis in Children With Benign Rolandic Epilepsy. J Child Neurol 2016; 31:289.
  26. Cherian A, Baheti NN, Menon RN, et al. Atonic variant of benign childhood epilepsy with centrotemporal spikes (atonic-BECTS): a distinct electro-clinical syndrome. Brain Dev 2012; 34:511.
  27. Baglietto MG, Battaglia FM, Nobili L, et al. Neuropsychological disorders related to interictal epileptic discharges during sleep in benign epilepsy of childhood with centrotemporal or Rolandic spikes. Dev Med Child Neurol 2001; 43:407.
  28. Lindgren S, Kihlgren M, Melin L, et al. Development of cognitive functions in children with rolandic epilepsy. Epilepsy Behav 2004; 5:903.
  29. Northcott E, Connolly AM, Berroya A, et al. The neuropsychological and language profile of children with benign rolandic epilepsy. Epilepsia 2005; 46:924.
  30. Clarke T, Strug LJ, Murphy PL, et al. High risk of reading disability and speech sound disorder in rolandic epilepsy families: case-control study. Epilepsia 2007; 48:2258.
  31. Perkins FF Jr, Breier J, McManis MH, et al. Benign rolandic epilepsy -- perhaps not so benign: use of magnetic source imaging as a predictor of outcome. J Child Neurol 2008; 23:389.
  32. Boatman DF, Trescher WH, Smith C, et al. Cortical auditory dysfunction in benign rolandic epilepsy. Epilepsia 2008; 49:1018.
  33. Kavros PM, Clarke T, Strug LJ, et al. Attention impairment in rolandic epilepsy: systematic review. Epilepsia 2008; 49:1570.
  34. Vago C, Bulgheroni S, Franceschetti S, et al. Memory performance on the California Verbal Learning Test of children with benign childhood epilepsy with centrotemporal spikes. Epilepsy Behav 2008; 13:600.
  35. Lillywhite LM, Saling MM, Harvey AS, et al. Neuropsychological and functional MRI studies provide converging evidence of anterior language dysfunction in BECTS. Epilepsia 2009; 50:2276.
  36. Danielsson J, Petermann F. Cognitive deficits in children with benign rolandic epilepsy of childhood or rolandic discharges: a study of children between 4 and 7 years of age with and without seizures compared with healthy controls. Epilepsy Behav 2009; 16:646.
  37. Cerminara C, D'Agati E, Lange KW, et al. Benign childhood epilepsy with centrotemporal spikes and the multicomponent model of attention: a matched control study. Epilepsy Behav 2010; 19:69.
  38. Tovia E, Goldberg-Stern H, Ben Zeev B, et al. The prevalence of atypical presentations and comorbidities of benign childhood epilepsy with centrotemporal spikes. Epilepsia 2011; 52:1483.
  39. Smith AB, Kavros PM, Clarke T, et al. A neurocognitive endophenotype associated with rolandic epilepsy. Epilepsia 2012; 53:705.
  40. Vannest J, Tenney JR, Gelineau-Morel R, et al. Cognitive and behavioral outcomes in benign childhood epilepsy with centrotemporal spikes. Epilepsy Behav 2015; 45:85.
  41. Tang SS, Clarke T, Owens J, Pal DK. Sleep behavior disturbances in rolandic epilepsy. J Child Neurol 2011; 26:239.
  42. Smith AB, Bajomo O, Pal DK. A meta-analysis of literacy and language in children with rolandic epilepsy. Dev Med Child Neurol 2015; 57:1019.
  43. Wickens S, Bowden SC, D'Souza W. Cognitive functioning in children with self-limited epilepsy with centrotemporal spikes: A systematic review and meta-analysis. Epilepsia 2017; 58:1673.
  44. Berroya AM, Bleasel AF, Stevermuer TL, et al. Spike morphology, location, and frequency in benign epilepsy with centrotemporal spikes. J Child Neurol 2005; 20:188.
  45. Blom S, Heijbel J. Benign epilepsy of children with centro-temporal EEG foci. Discharge rate during sleep. Epilepsia 1975; 16:133.
  46. Frost JD Jr, Hrachovy RA, Glaze DG. Spike morphology in childhood focal epilepsy: relationship to syndromic classification. Epilepsia 1992; 33:531.
  47. Camfield P, Camfield C. Epileptic syndromes in childhood: clinical features, outcomes, and treatment. Epilepsia 2002; 43 Suppl 3:27.
  48. Shevell MI, Rosenblatt B, Watters GV, et al. "Pseudo-BECRS": intracranial focal lesions suggestive of a primary partial epilepsy syndrome. Pediatr Neurol 1996; 14:31.
  49. Wirrell EC, Camfield PR, Gordon KE, et al. Benign rolandic epilepsy: atypical features are very common. J Child Neurol 1995; 10:455.
  50. Datta A, Sinclair DB. Benign epilepsy of childhood with rolandic spikes: typical and atypical variants. Pediatr Neurol 2007; 36:141.
  51. Gelisse P, Corda D, Raybaud C, et al. Abnormal neuroimaging in patients with benign epilepsy with centrotemporal spikes. Epilepsia 2003; 44:372.
  52. Peters JM, Camfield CS, Camfield PR. Population study of benign rolandic epilepsy: is treatment needed? Neurology 2001; 57:537.
  53. Tzitiridou M, Panou T, Ramantani G, et al. Oxcarbazepine monotherapy in benign childhood epilepsy with centrotemporal spikes: a clinical and cognitive evaluation. Epilepsy Behav 2005; 7:458.
  54. Bello-Espinosa LE, Roberts SL. Levetiracetam for benign epilepsy of childhood with centrotemporal spikes-three cases. Seizure 2003; 12:157.
  55. Gastaut H. A new type of epilepsy: benign partial epilepsy of childhood with occipital spike-waves. Clin Electroencephalogr 1982; 13:13.
  56. Coppola G, Franzoni E, Verrotti A, et al. Levetiracetam or oxcarbazepine as monotherapy in newly diagnosed benign epilepsy of childhood with centrotemporal spikes (BECTS): an open-label, parallel group trial. Brain Dev 2007; 29:281.
  57. Hughes JR. Benign epilepsy of childhood with centrotemporal spikes (BECTS): to treat or not to treat, that is the question. Epilepsy Behav 2010; 19:197.
  58. Rating D, Wolf C, Bast T. Sulthiame as monotherapy in children with benign childhood epilepsy with centrotemporal spikes: a 6-month randomized, double-blind, placebo-controlled study. Sulthiame Study Group. Epilepsia 2000; 41:1284.
  59. Bourgeois BF. Antiepileptic drugs, learning, and behavior in childhood epilepsy. Epilepsia 1998; 39:913.
  60. Corda D, Gelisse P, Genton P, et al. Incidence of drug-induced aggravation in benign epilepsy with centrotemporal spikes. Epilepsia 2001; 42:754.
  61. Catania S, Cross H, de Sousa C, Boyd S. Paradoxic reaction to lamotrigine in a child with benign focal epilepsy of childhood with centrotemporal spikes. Epilepsia 1999; 40:1657.
  62. Braathen G, Andersson T, Gylje H, et al. Comparison between one and three years of treatment in uncomplicated childhood epilepsy: a prospective study. I. Outcome in different seizure types. Epilepsia 1996; 37:822.
  63. Loiseau P, Duché B, Cordova S, et al. Prognosis of benign childhood epilepsy with centrotemporal spikes: a follow-up study of 168 patients. Epilepsia 1988; 29:229.
  64. Callenbach PM, Bouma PA, Geerts AT, et al. Long term outcome of benign childhood epilepsy with centrotemporal spikes: Dutch Study of Epilepsy in Childhood. Seizure 2010; 19:501.
  65. Camfield CS, Camfield PR. Rolandic epilepsy has little effect on adult life 30 years later: a population-based study. Neurology 2014; 82:1162.
  66. Berg AT, Nickels K, Wirrell EC, et al. Mortality risks in new-onset childhood epilepsy. Pediatrics 2013; 132:124.
  67. Doumlele K, Friedman D, Buchhalter J, et al. Sudden Unexpected Death in Epilepsy Among Patients With Benign Childhood Epilepsy With Centrotemporal Spikes. JAMA Neurol 2017; 74:645.
  68. Fejerman N, Caraballo R, Tenembaum SN. Atypical evolutions of benign localization-related epilepsies in children: are they predictable? Epilepsia 2000; 41:380.
  69. Monjauze C, Broadbent H, Boyd SG, et al. Language deficits and altered hemispheric lateralization in young people in remission from BECTS. Epilepsia 2011; 52:e79.
  70. Datta AN, Oser N, Bauder F, et al. Cognitive impairment and cortical reorganization in children with benign epilepsy with centrotemporal spikes. Epilepsia 2013; 54:487.
  71. Bulgheroni S, Franceschetti S, Vago C, et al. Verbal dichotic listening performance and its relationship with EEG features in benign childhood epilepsy with centrotemporal spikes. Epilepsy Res 2008; 79:31.
  72. Lin JJ, Riley JD, Hsu DA, et al. Striatal hypertrophy and its cognitive effects in new-onset benign epilepsy with centrotemporal spikes. Epilepsia 2012; 53:677.
  73. Taylor I, Berkovic SF, Kivity S, Scheffer IE. Benign occipital epilepsies of childhood: clinical features and genetics. Brain 2008; 131:2287.
  74. Covanis A. Panayiotopoulos syndrome: a benign childhood autonomic epilepsy frequently imitating encephalitis, syncope, migraine, sleep disorder, or gastroenteritis. Pediatrics 2006; 118:e1237.
  75. Lada C, Skiadas K, Theodorou V, et al. A study of 43 patients with panayiotopoulos syndrome, a common and benign childhood seizure susceptibility. Epilepsia 2003; 44:81.
  76. Koutroumanidis M. Panayiotopoulos syndrome: an important electroclinical example of benign childhood system epilepsy. Epilepsia 2007; 48:1044.
  77. Caraballo R, Cersósimo R, Fejerman N. Panayiotopoulos syndrome: a prospective study of 192 patients. Epilepsia 2007; 48:1054.
  78. Panayiotopoulos CP. Benign childhood epilepsy with occipital paroxysms: a 15-year prospective study. Ann Neurol 1989; 26:51.
  79. Du JC, Chien YH, Weng WC, et al. Clinical analysis of childhood occipital lobe epilepsy in 43 Taiwanese patients. Pediatr Neurol 2007; 36:387.
  80. Oguni H, Hayashi K, Funatsuka M, Osawa M. Study on early-onset benign occipital seizure susceptibility syndrome. Pediatr Neurol 2001; 25:312.
  81. Ferrie C, Caraballo R, Covanis A, et al. Panayiotopoulos syndrome: a consensus view. Dev Med Child Neurol 2006; 48:236.
  82. Panayiotopoulos CP. Benign childhood epileptic syndromes with occipital spikes: new classification proposed by the International League Against Epilepsy. J Child Neurol 2000; 15:548.
  83. Michael M, Tsatsou K, Ferrie CD. Panayiotopoulos syndrome: an important childhood autonomic epilepsy to be differentiated from occipital epilepsy and acute non-epileptic disorders. Brain Dev 2010; 32:4.
  84. Specchio N, Trivisano M, Di Ciommo V, et al. Panayiotopoulos syndrome: a clinical, EEG, and neuropsychological study of 93 consecutive patients. Epilepsia 2010; 51:2098.
  85. Ferrie CD, Caraballo R, Covanis A, et al. Autonomic status epilepticus in Panayiotopoulos syndrome and other childhood and adult epilepsies: a consensus view. Epilepsia 2007; 48:1165.
  86. Koutroumanidis M, Ferrie CD, Valeta T, et al. Syncope-like epileptic seizures in Panayiotopoulos syndrome. Neurology 2012; 79:463.
  87. De Rose P, Perrino F, Lettori D, et al. Visual and visuoperceptual function in children with Panayiotopoulos syndrome. Epilepsia 2010; 51:1205.
  88. Specchio N, Trivisano M, Claps D, et al. Documentation of autonomic seizures and autonomic status epilepticus with ictal EEG in Panayiotopoulos syndrome. Epilepsy Behav 2010; 19:383.
  89. Panayiotopoulos CP, Michael M, Sanders S, et al. Benign childhood focal epilepsies: assessment of established and newly recognized syndromes. Brain 2008; 131:2264.
  90. Ozkara C, Benbir G, Celik AF. Misdiagnosis due to gastrointestinal symptoms in an adolescent with probable autonomic status epilepticus and Panayiotopoulos syndrome. Epilepsy Behav 2009; 14:703.
  91. Hirano Y, Oguni H, Funatsuka M, et al. Neurobehavioral abnormalities may correlate with increased seizure burden in children with Panayiotopoulos syndrome. Pediatr Neurol 2009; 40:443.
  92. Caraballo RH, Cersósimo RO, Fejerman N. Childhood occipital epilepsy of Gastaut: a study of 33 patients. Epilepsia 2008; 49:288.
  93. Caraballo RH, Cersósimo RO, Fejerman N. Late-onset, "Gastaut type", childhood occipital epilepsy: an unusual evolution. Epileptic Disord 2005; 7:341.
  94. Kivity S, Ephraim T, Weitz R, Tamir A. Childhood epilepsy with occipital paroxysms: clinical variants in 134 patients. Epilepsia 2000; 41:1522.
  95. Tsai ML, Lo HY, Chaou WT. Clinical and electroencephalographic findings in early and late onset benign childhood epilepsy with occipital paroxysms. Brain Dev 2001; 23:401.
  96. Shahar E, Barak S. Favorable outcome of epileptic blindness in children. J Child Neurol 2003; 18:12.
  97. Caraballo R, Koutroumanidis M, Panayiotopoulos CP, Fejerman N. Idiopathic childhood occipital epilepsy of Gastaut: a review and differentiation from migraine and other epilepsies. J Child Neurol 2009; 24:1536.
  98. Martinović Z. Clinical correlations of electroencephalographic occipital epileptiform paroxysms in children. Seizure 2001; 10:379.
  99. Verrotti A, Parisi P, Loiacono G, et al. Levetiracetam monotherapy for childhood occipital epilepsy of gastaut. Acta Neurol Scand 2009; 120:342.
  100. Panayiotopoulos CP. Elementary visual hallucinations, blindness, and headache in idiopathic occipital epilepsy: differentiation from migraine. J Neurol Neurosurg Psychiatry 1999; 66:536.