Figure 235

Occipital paroxysms with fixation off sensitivity of an 11-year-old boy with idiopathic childhood occipital epilepsy. Occipital paroxysms occur immediately after and as long as fixation and central vision are eliminated by any means (eyes closed, darkness, plus 10 spherical lenses, Ganzfeld stimulation). Under these conditions, even in the presence of light, eye opening does not inhibit the occipital paroxysms. Conversely, occipital paroxysms are totally inhibited by fixation and central vision. Symbols of eyes open without glasses indicate conditions in which fixation is possible. Symbols of eyes with glasses indicate conditions in which central vision and fixation are eliminated. At age 18 years, he is entirely normal and is not receiving medication.

These syndromes may mimic symptomatic occipital lobe epilepsies, and neuroimaging, preferably MRI, is indicated. No other investigations, except EEG, are routinely required.

Treatment. Given the frequency of seizures in idiopathic childhood occipital epilepsy, including the likelihood of occasional GTCS, regular AED treatment is considered necessary in most if not all subjects. There are no controlled studies comparing alternatives, although carba-mazepine appears to be most often used in subjects who are not photosensitive. It is appropriate to attempt withdrawal after two seizure-free years, although there is a significant risk of relapse.

Some subjects with idiopathic photosensitive occipital lobe epilepsy who are only mildly photosensitive and who do not have spontaneous seizures can remain seizure free by avoiding precipitants. Others will require AED treatment. Broad spectrum agents, such as sodium valproate and levetiracetam, active against focal and generalized seizures and photosensi-tivity, would appear to be reasonable choices. However, it appears that carbamazepine, not usually considered a useful drug for photosensitivity, may sometimes be effective.

Course and Prognosis. The prognosis for both idio-pathic childhood occipital epilepsy and idiopathic photosensitive occipital lobe epilepsy is variable. A majority of the former, perhaps 50% to 60%, have remission of seizures within 2-4 years of them starting. However, in a significant minority seizures will continue into adulthood. In those with idiopathic photosensitive occipital lobe epilepsy who are only mildly photosensitive and can control their exposure to relevant provoking factors, freedom from seizures may be easy. For others, particularly those who are highly photosensitive, the likelihood of seizures continuing into adult life is high.

Atypical Evolutions of the Benign Focal Epilepsies of Childhood

Less than 1% of children with rolandic epilepsy have so-called atypical evolutions (24, 32, 83). These include the development of severe linguistic, cognitive, or behavioral problems. If such problems develop in a child with rolandic epilepsy, a sleep EEG should be obtained, because continuous spike-and-wave during slow-wave sleep (CSWS) may be present. The Landau-Kleffner syndrome is sometimes said to develop from rolandic epilepsy. CSWS may also be seen in children with opercular status characterized by continuous positive or negative myoclonias around the mouth or elsewhere in the face and pseudobulbar problems. Atypical focal epilepsy of childhood in which other seizure types, including tonic and atypical absence seizures, occur may also develop in children with otherwise typical rolandic epilepsy.

There are also case reports of atypical evolutions in Panayiotopoulos syndrome, including the development of absences and drop attacks (32, 84, 85) and in idiopathic childhood occipital epilepsy with cognitive deterioration and CSWS (86).

Carbamazepine is sometimes implicated in precipitating such atypical evolutions (87, 88).

Other Described Benign Focal Epilepsies of Childhood

The syndromes discussed previously are the only benign focal epilepsies of childhood currently recognized by the ILAE. However, others have been proposed and are more or less well characterized. They include the following.

Benign Childhood Seizures with Affective Symptoms (89). This is reported to have its onset between 2 and 9 years of age and is characterized by multiple, usually short, daytime and nighttime seizures in which the predominant symptom appears to be fear or terror, accompanied by autonomic disturbances (pallor, sweating, abdominal pain, and salivation), arrest of speech, and mild impairment of consciousness with automatisms. Interictal EEG shows sharp and slow wave complexes similar to those in rolandic epilepsy but located in the frontotemporal and parietotemporal electrodes. Remission in 1 to 2 years from onset is expected. This is likely to be an intermediate phenotype between Panayiotopoulos syndrome and rolandic epilepsy.

Benign Childhood Epilepsy with Parietal Spikes and Frequent Giant Somatosensory Evoked Potentials (28, 90). This putative disorder is mainly defined by its interictal EEG features reflected in its name. These features are, however, said to often be associated with a phenotype characterized by mainly daytime versive seizures, which are infrequent and have an excellent prognosis.

Benign Childhood Focal Seizures Associated with Frontal or Midline Spikes (5). Again this putative disorder is mainly defined by its interictal EEG features. These EEG features can be seen in children with febrile seizures, rolandic epilepsy, Panayiotopoulos syndrome, and idio-pathic childhood occipital epilepsy.

Benign Focal Epilepsy in Infants with Central and Vertex Spikes and Waves During Sleep (91, 92). Benign focal epilepsy in infants with central and vertex spikes and waves during sleep has been recently described as a new benign syndrome. In terms of age of onset, it is on the borderline between benign infantile seizures and Panayio-topoulos syndrome. Age at onset is in the first 2 years of life with both sexes equally affected. Infants are normal and all tests other than EEG are normal. Seizures consist mainly of staring, motor arrest, facial cyanosis, loss of consciousness, and stiffening of the arms. Clonic convulsions and automatisms are rare. Duration is from 1 to 5 minutes. Seizures are mainly diurnal (but may also occur during sleep) and may occur in clusters, but are generally infrequent (1-3 per year). Interictal EEG abnormalities are seen only in non-REM sleep and consist of small, mostly singular, spikes and waves local/zed at the vertex and central electrodes.

There is a strong family history of epilepsy with benign epilepsies prevailing. The prognosis is excellent with remission of seizures, normal development, and normalization of the EEG before the age of 4 years.

Benign Focal Seizures of Adolescence (5, 93). This syndrome of the second decade, and predominantly occurring in males, features a single seizure or a single cluster of seizures over a period of up to 36 hours. The seizures are mainly diurnal, with consciousness initially preserved. The main manifestations are focal clonic jerking, usually without a Jacksonian march, and somatosen-sory symptoms. Secondary GTCS occur in about 50% of cases. EEG and brain neuroimaging are normal. The prognosis is excellent and treatment is not required.


Commission on Classification and Terminology of the International League Against Epilepsy. Proposal for revised classification of epilepsies and epileptic syndromes. Epilepsia

1989; 30:389-399.

Geelhoed M, Boerrigter AO, Camfield P, Geerts AT, et al. The accuracy of outcome prediction models for childhood-onset epilepsy. Epilepsia 2005; 46:1526-1532.

3. Engel J Jr. A proposed diagnostic scheme for people with epileptic seizures and with epilepsy: Report of the ILAE Task Force on Classification and Terminology. Epilepsia 2001; 42:796-803.

4. Riikonen R. Long-term outcome of patients with West syndrome. Brain Dev 2001; 23:683-687.

5. Panayiotopoulos CP. Benign childhood focal seizures and related epileptic syndromes. In: Panayiotopoulos CP, ed. The Epilepsies: Seizures, Syndromes and Management. Oxford: Bladon Medical Publishing, 2005:223-269.

6. Panayiotopoulos CP. Benign childhood partial epilepsies: benign childhood seizure susceptibility syndromes [editorial]. J Neurol Neurosurg Psychiatry 1993; 56:2-5.

7. Vadlamudi L, Harvey AS, Connellan MM, Milne RL, et al. Is benign rolandic epilepsy genetically determined? Ann.Neurol 2004; 56:129-132.

8. Bray PF,.Wiser WC. Evidence for a genetic etiology of temporal-central abnormalities in focal epilepsy. N Engl J Med 1964; 271:926-933.

9. Heijbel J, Blom S, Rasmuson M. Benign epilepsy of childhood with centrotemporal EEG foci: a genetic study. Epilepsia 1975; 16:285-293.

10. Neubauer BA, Hahn A, Stepham U, Doose H. Clinical spectrum and genetics of Rolandic epilepsy. Adv Neurol 2002; 89:475-479.

11. Neubauer BA, Fiedler B, Himmelein B, Kampfer F, et al. Centrotemporal spikes in families with rolandic epilepsy: linkage to chromosome 15q14. Neurology 1998; 51:1608-1612.

12. Scheffer IE, Berkovic SF. The genetics of human epilepsy. Trends Pharmacol Sci 2003; 24:428-433.

13. Gutierrez-Delicado E, Serratosa JM. Genetics of the epilepsies. Curr Opin Neurol 2004; 17:147-153.

14. Hirose S, Mitsudome A, Okada M, Kaneko S. Genetics of idiopathic epilepsies. Epilepsia 2005; 46 Suppl 1:38-43.

15. Coppola G, Castaldo P, Miraglia DG, Bellini G, et al. A novel KCNQ2 K+ channel mutation in benign neonatal convulsions and centrotemporal spikes. Neurology 2003; 61:131-134.

16. Berkovic SF, Heron SE, Giordano L, Marini C, et al. Benign familial neonatal-infantile seizures: characterization of a new sodium channelopathy. Ann Neurol 2004; 55:550-557.

17. Roll P, Massacrier A, Pereira S, Robaglia-Schlupp A, et al. New human sodium/glucose cotransporter gene (KST1): identification, characterization, and mutation analysis in ICCA (infantile convulsions and choreoathetosis) and BFIC (benign familial infantile convulsions) families. Gene 2002; 285:141-148.

18. Guerrini R, Bonanni P, Nardocci N, Parmeggiani L, et al. Autosomal recessive rolan-dic epilepsy with paroxysmal exercise-induced dystonia and writer's cramp: delineation of the syndrome and gene mapping to chromosome 16p12-11.2. Ann Neurol 1999; 45:344-352.

19. Panayiotopoulos CP. Benign childhood epilepsy with centrotemporal spikes or Rolandic seizures. In: Panayiotopoulos CP, ed. Benign Childhood Partial Seizures and Related Epileptic Syndromes. London: John Libbey & Company, 1999:33-100.

20. Dalla Bernardina B, Sgro M, Fejerman N. Epilepsy with centro-temporal spikes and related syndromes. In: Roger J, Bureau M, Dravet C, Genton P, et al, eds. Epileptic Syndromes in Infancy, Childhood and Adolescence. 4th ed. Montrouge, France: John Libbey Eurotext, 2005:203-225.

21. Beaussart M, Loiseau P, Roger H. The discovery of "benign rolandic epilepsy." In: Berkovic SF, Genton P, Hirsch E, Picard F, eds. Genetics of Focal Epilepsies. London: John Libbey & Company, 1999:3-6.

22. Lombroso CT. Sylvian seizures and midtemporal spike foci in children. Arch Neurol 1967; 17:52-59.

23. Bouma PA, Bovenkerk AC, Westendorp RG, Brouwer OF. The course of benign partial epilepsy of childhood with centrotemporal spikes: a meta-analysis. Neurology 1997; 48:430-437.

24. Fejerman N, Caraballo R, Tenembaum SN. Atypical evolutions of benign localization-related epilepsies in children: are they predictable? Epilepsia 2000; 41:380-390.

25. Gregory DL,.Wong PK. Clinical relevance of a dipole field in rolandic spikes. Epilepsia 1992; 33:36^4.

26. Yoshinaga H, Amano R, Oka E, Ohtahara S. Dipole tracing in childhood epilepsy with special reference to rolandic epilepsy. Brain Topogr 1992; 4:193-199.

27. De Marco P, Tassinari CA. Extreme somatosensory evoked potential (ESEP): an EEG sign forecasting the possible occurrence of seizures in children. Epilepsia 1981; 22:569-575.

28. Fonseca LC, Tedrus GM. Somatosensory evoked spikes and epileptic seizures: a study of 385 cases. Clin Electroencephalogr 2000; 31:71-75.

29. Gibbs F A, Gibbs EL. Atlas of electroencephalography. Vol 2: Epilepsy. Reading, MA: Addison-Wesley, 1952:214-290.

30. Smith JMB, Kellaway P. Central (Rolandic) foci in children: an analysis of 200 cases. Electroencephalogr Clin Neurophysiol 1964; 17:460-461.

31. Eeg-Olofsson O. The development of the electroencephalogram in normal children and adolescents from the age of 1 through 21 years. Acta Paediatr Scand Suppl 1970; 208.

32. Kanazawa O. Benign rolandic epilepsy and related epileptic syndromes: electrophysi-ological studies including magnetoencephalography in ictal and interictal phenomena. In: Benjamin SM, ed. Trends in Epilepsy Research. New York: Nova Science Publishers,Inc., 2005. pp 19-54.

33. Minami T, Gondo K, Yamamoto T, Yanai S, et al. Magnetoencephalographic analysis of rolandic discharges in benign childhood epilepsy. Ann Neurol 1996; 39:326-334.

34. Gelisse P, Corda D, Raybaud C, Dravet C, et al. Abnormal neuroimaging in patients with benign epilepsy with centrotemporal spikes. Epilepsia 2003; 44:372-378.

35. Gelisse P, Genton P, Raybaud C, Thiry A, et al. Benign childhood epilepsy with centro-temporal spikes and hippocampal atrophy. Epilepsia 1999; 40:1312-1315.

36. Lundberg S, Eeg-Olofsson O, Raininko R, Eeg-Olofsson KE. Hippocampal asymmetries and white matter abnormalities on MRI in benign childhood epilepsy with centrotemporal spikes. Epilepsia 1999; 40:1808-1815.

37. Lundberg S, Weis J, Eeg-Olofsson O, Raininko R. Hippocampal region asymmetry assessed by 1H-MRS in rolandic epilepsy. Epilepsia 2003; 44:205-210.

38. Peters JM, Camfield CS, Camfield PR. Population study of benign rolandic epilepsy: Is treatment needed? Neurology 2001; 57:537-539.

39. Loiseau P, Pestre M, Dartigues JF, Commenges D, et al. Long-term prognosis in two forms of childhood epilepsy: typical absence seizures and epilepsy with rolandic (centrotemporal) EEG foci. Ann Neurol 1983; 13:642-648.

40. Deonna T, Zesiger P, Davidoff V, Maeder M, et al. Benign partial epilepsy of childhood: a longitudinal neuropsychological and EEG study of cognitive function. Dev Med Child Neurol 2000; 42:595-603.

41. Papavasiliou A, Mattheou D, Bazigou H, Kotsalis C, et al. Written language skills in children with benign childhood epilepsy with centrotemporal spikes. Epilepsy Behav

42. Vinayan KP, Biji V, Thomas SV. Educational problems with underlying neuropsychological impairment are common in children with benign epilepsy of childhood with centrotem-poral spikes (BECTS). Seizure 2005; 14:207-212.

43. Fonseca LC, Tedrus GM, Tonelotto JM, Antunes TDA, Chiodi MG. [School performance in children with benign childhood epilepsy with centrotemporal spikes]. Arq Neurop-siquiatr 2004; 62:459^62.

44. Baglietto MG, Battaglia FM, Nobili L, Tortorelli S, 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; 43:407-412.

45. Yung AW, Park YD, Cohen MJ, Garrison TN. Cognitive and behavioral problems in children with centrotemporal spikes. Pediatr Neurol 2000; 23:391-395.

46. Croona C, Kihlgren M, Lundberg S, Eeg-Olofsson O, et al. Neuropsychological findings in children with benign childhood epilepsy with centrotemporal spikes. Dev Med Child

Neurol 1999; 41:813-818.

47. Panayiotopoulos CP. Vomiting as an ictal manifestation of epileptic seizures and syndromes. J Neurol Neurosurg Psychiatr 1988; 51:1448-1451.

48. Ferrie CD, Grunewald RA. Panayiotopoulos syndrome: a common and benign childhood epilepsy [commentary]. Lancet 2001; 357:821-823.

49. Koutroumanidis M. Panayiotopoulos syndrome: a common benign but underdiagnosed and unexplored early childhood seizure syndrome [editorial]. BMJ 2002; 324:1228-1229.

50. Panayiotopoulos CP. Panayiotopoulos syndrome: a common and benign childhood epileptic syndrome. London: John Libbey & Company, 2002.

51. Panayiotopoulos CP. Autonomic seizures and autonomic status epilepticus peculiar to childhood: diagnosis and management. Epilepsy Behav 2004; 5:286-295.

52. Covanis A, Ferrie CD, Koutroumanidis M, Oguni H, et al. Panayiotopoulos syndrome and Gastaut type idiopathic childhood occipital epilepsy. In: Roger J, Bureau M, Dravet C, Genton P, et al, eds. Epileptic Syndromes in Infancy, Childhood and Adolescence. 4th ed, with video. Montrouge, France: John Libbey Eurotext, 2005:227-253.

53. Panayiotopoulos CP. Panayiotopoulos syndrome. In: Panayiotopoulos CP, ed. The Epilepsies: Seizures, Syndromes and Management. Oxford: Bladon Medical Publishing, 2005:235-248.

54. Ferrie C, Caraballo R, Covanis A, Demirbilek V, et al. Panayiotopoulos syndrome: a consensus view. Dev Med Child Neurol 2006; 48:236-240.

55. Panayiotopoulos CP. Inhibitory effect of central vision on occipital lobe seizures.

Neurology 1981; 31:1330-1333.

56. Panayiotopoulos CP. Benign childhood epilepsy with occipital paroxysms: a 15-year prospective study. Ann Neurol 1989; 26:51-56.

57. Panayiotopoulos CP. Extraoccipital benign childhood partial seizures with ictal vomiting and excellent prognosis. J Neurol Neurosurg Psychiatry 1999; 66:82-85.

58. Ferrie CD. Nonconvulsive status epilepticus in the benign focal epilepsies of childhood with particular reference to autonomic status epilepticus in Panayiotopoulos syndrome. Epileptic Disord 2005; 7:291-293.

59. Sanders S, Rowlinson S, Manidakis I, Ferrie CD, et al. The contribution of the EEG technologists in the diagnosis of Panayiotopoulos syndrome (susceptibility to early onset benign childhood autonomic seizures). Seizure 2004; 13:565-573.

60. Ferrie CD, Beaumanoir A, Guerrini R, Kivity S, et al. Early-onset benign occipital seizure susceptibility syndrome. Epilepsia 1997; 38:285-293.

61. Oguni H, Hayashi K, Imai K, Hirano Y, et al. Study on the early-onset variant of benign childhood epilepsy with occipital paroxysms otherwise described as early-onset benign occipital seizure susceptibility syndrome. Epilepsia 1999; 40:1020-1030.

62. Caraballo R, Cersosimo R, Medina C, Fejerman N. Panayiotopoulos-type benign childhood occipital epilepsy: a prospective study. Neurology 2000; 55:1096-1100.

63. Kivity S, Ephraim T, Weitz R, Tamir A. Childhood epilepsy with occipital paroxysms: clinical variants in 134 patients. Epilepsia 2000; 41:1522-1523.

64. Lada C, Skiadas K, Theodorou V, Covanis A. A study of 43 patients with Panayiotopoulos syndrome: a common and benign childhood seizure suceptibility. Epilepsia 2003; 44:81-88.

65. Ohtsu M, Oguni H, Hayashi K, Funatsuka M, et al. EEG in children with early-onset benign occipital seizure susceptibility syndrome: Panayiotopoulos syndrome. Epilepsia

2003; 44:435-442.

66. Parisi P, Ferri R, Pagani J, Cecili M, et al. Ictal video-polysomnography and EEG spectral analysis in a child with severe Panayiotopoulos syndrome. Epileptic.Disord 2005; 7:333-339.

67. Verrotti A, Salladini C, Trotta D, di Corcia G, et al. Ictal cardiorespiratory arrest in Panayiotopoulos syndrome. Neurology 2005; 64:1816-1817.

68. Beaumanoir A. Semiology of occipital seizures in infants and children. In: Andermann F, Beaumanoir A, Mira L, Roger J, et al, eds. Occipital Seizures and Epilepsies in Children. London: John Libbey and Company, 1993:71-86.

69. Vigevano F, Lispi ML, Ricci S. Early onset benign occipital susceptibility syndrome: video-EEG documentation of an illustrative case. Clin Neurophysiol 2000; 111 Suppl 2:S81-S86.

70. Demirbilek V, Dervent A. Panayiotopoulos syndrome: video-EEG illustration of a typical seizure. Epileptic.Disord 2004; 6:121-124.

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