Journal of Pediatric Epilepsy 2016; 05(03): 142-146
DOI: 10.1055/s-0036-1585064
Review Article
Georg Thieme Verlag KG Stuttgart · New York

Treatment of Classic Syndromes in Idiopathic Focal Epilepsies in Childhood

Thalia Valeta
1   Departments of Neurology and Psychiatry, University of Athens, Athens, Greece
2   Department of Clinical Neurophysiology and Epilepsies, St. Thomas' Hospital, London, United Kingdom
,
C. P. Panayiotopoulos
2   Department of Clinical Neurophysiology and Epilepsies, St. Thomas' Hospital, London, United Kingdom
› Author Affiliations
Further Information

Publication History

14 March 2016

13 April 2016

Publication Date:
01 August 2016 (online)

Abstract

Of the classic syndromes of idiopathic (benign) focal epilepsies in childhood, more than half of children with Rolandic epilepsy and Panayiotopoulos syndrome do not need antiepileptic drug (AED) treatment. Monotherapy with carbamazepine, levetiracetam, sodium valproate, or clobazam is usually recommended for those with seizures that are unusually frequent (more than three), lengthy, distressing, or otherwise significantly interfering with the child's life. Other factors in favor of treatment include a short interval between the first three seizures, a younger age of onset (less than 4 years), or a presence of recurrent tonic–clonic seizures and diurnal seizures. In contrast to Rolandic epilepsy and Panayiotopoulos syndrome that may not need treatment, idiopathic childhood occipital epilepsy of Gastaut should be treated because seizures are frequent and secondarily generalized convulsions are probably unavoidable without medication. It is likely that all AEDs licensed as monotherapy for focal seizures and mainly carbamazepine and levetiracetam are suitable.

The electroencephalogram does not predict prognosis or AED treatment needs, though there are views supporting that interictal spikes have an adverse effect on cognition.

Strategies of withdrawing medication in idiopathic focal epilepsies in childhood differ among experts, although all agree that there is no need to continue medication 1 to 3 years after the last seizure, and certainly not after the age of 14 years, when most benign childhood focal seizures remit, or age 16, when they are practically nonexistent.

In the acute stage of status epilepticus, control of the seizures is of paramount importance. Convulsive status epilepticus is a life threatening condition and should be terminated as soon as possible. Autonomic status epilepticus needs thorough evaluation for proper diagnosis and management. Benzodiazepines, intravenously, in rectal or buccal preparations are commonly used to terminate long lasting seizures and status epilepticus. Early parental treatment is more effective than late emergency treatment. Buccal midazolam is probably the first choice medication for out-of-hospital termination of status epilepticus.

Parental education and psychological support are the cornerstone of the optimal management because despite excellent prognosis, idiopathic focal epilepsies in childhood usually have a dramatic impact to parents.

 
  • References

  • 1 Panayiotopoulos CP, Michael M, Sanders S, Valeta T, Koutroumanidis M. Benign childhood focal epilepsies: assessment of established and newly recognized syndromes. Brain 2008; 131 
Pt 9
 2264-2286
  • 2 Fejerman N, Caraballo RH , eds. Benign Focal Epilepsies in Infancy, Childhood and Adolescence. Montrouge, France: John Libbey Eurotext; 2007
  • 3 Panayiotopoulos CP, Bureau M, Caraballo RH, Dalla Bernardina B, Valeta T. Idiopathic focal epilepsies in childhood. In: Bureau M, Genton P, Dravet C, et al., eds. Epileptic Syndromes in Infancy, Childhood and Adolescence. 5th ed. with video. Montrouge, France: John Libbey Eurotext; 2012: 217-254
  • 4 Berg AT, Berkovic SF, Brodie MJ , et al. Revised terminology and concepts for organization of seizures and epilepsies: report of the ILAE Commission on Classification and Terminology, 2005-2009. Epilepsia 2010; 51 (4) 676-685
  • 5 ILAE Commission on Classification and Terminology. Online diagnostic manual of the epilepsies. 2014. Available at https://www.epilepsydiagnosis.org/. Accessed March 14, 2016
  • 6 Koutroumanidis M. Panayiotopoulos syndrome: an important electroclinical example of benign childhood system epilepsy. Epilepsia 2007; 48 (6) 1044-1053
  • 7 Ferrie C, Caraballo R, Covanis A , et al. Panayiotopoulos syndrome: a consensus view. Dev Med Child Neurol 2006; 48 (3) 236-240
  • 8 Panayiotopoulos CP. Panayiotopoulos Syndrome: A Common and Benign Childhood Epileptic Syndrome. London: John Libbey; 2002
  • 9 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 (12) 1536-1542
  • 10 Mellish LC, Dunkley C, Ferrie CD, Pal DK. Antiepileptic drug treatment of rolandic epilepsy and Panayiotopoulos syndrome: clinical practice survey and clinical trial feasibility. Arch Dis Child 2015; 100 (1) 62-67
  • 11 Oguni H. Treatment of benign focal epilepsies in children: when and how should be treated?. Brain Dev 2011; 33 (3) 207-212
  • 12 Ambrosetto G, Tassinari CA. Antiepileptic drug treatment of benign childhood epilepsy with rolandic spikes: is it necessary?. Epilepsia 1990; 31 (6) 802-805
  • 13 Peters JM, Camfield CS, Camfield PR. Population study of benign rolandic epilepsy: is treatment needed?. Neurology 2001; 57 (3) 537-539
  • 14 Valeta T, ‘Metamyth’© and Dramatherapy: An Innovative Approach for People with Epilepsy. In: Schrader C. , ed. Ritual Theatre. The Power of Dramatic Ritual in Personal Development Groups and Clinical Practice. London: Jessica Kingsley Publishers; 2012: 275-290
  • 15 Valeta T. Psychosocial aspects, parental reactions and need in idiopathic focal epilepsies. Epileptic Disord 2016; 18 (3) 19-22
  • 16 Valeta T, Sogawa Y, Moshe SL. Impact of focal seizures on patients and family. In: Panayiotopoulos CP, Benbadis S, Sisodiya S, eds. Volume 5: Focal Epilepsies: Seizures, Syndromes and Management. Oxford: Medicinae; 2008: 230-238
  • 17 Valeta T. Parental attitude, reaction and education in benign childhood focal seizures. In: Panayiotopoulos CP, ed. The Epilepsies: Seizures, Syndromes and Management. Oxford: Bladon Medical Publishing; 2005: 258-261
  • 18 Fejerman N. Benign childhood epilepsy with centrotemporal spikes. In: Panayiotopoulos CP, ed. Atlas of Epilepsies. London: Springer; 2010: 957-963
  • 19 Caraballo R, Cersósimo R, Fejerman N. Panayiotopoulos syndrome: a prospective study of 192 patients. Epilepsia 2007; 48 (6) 1054-1061
  • 20 Glauser T, Ben-Menachem E, Bourgeois B , et al. ILAE treatment guidelines: evidence-based analysis of antiepileptic drug efficacy and effectiveness as initial monotherapy for epileptic seizures and syndromes. Epilepsia 2006; 47 (7) 1094-1120
  • 21 Wheless JW, Clarke DF, Carpenter D. Treatment of pediatric epilepsy: expert opinion, 2005. J Child Neurol 2005; 20 (Suppl. 01) S1-S56 , quiz S59–S60
  • 22 Wheless JW, Clarke DF, Arzimanoglou A, Carpenter D. Treatment of pediatric epilepsy: European expert opinion, 2007. Epileptic Disord 2007; 9 (4) 353-412
  • 23 Verrotti A, D'Adamo E, Parisi P, Chiarelli F, Curatolo P. Levetiracetam in childhood epilepsy. Paediatr Drugs 2010; 12 (3) 177-186
  • 24 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 (5) 281-284
  • 25 Verrotti A, Parisi P, Loiacono G , et al. Levetiracetam monotherapy for childhood occipital epilepsy of Gastaut. Acta Neurol Scand 2009; 120 (5) 342-346
  • 26 Kossoff EH, Los JG, Boatman DF. A pilot study transitioning children onto levetiracetam monotherapy to improve language dysfunction associated with benign rolandic epilepsy. Epilepsy Behav 2007; 11 (4) 514-517
  • 27 García C, Rubio G. Efficacy and safety of levetiracetam in the treatment of Panayiotopoulos syndrome. Epilepsy Res 2009; 85 (2–3) 318-320
  • 28 Bourgeois BF. Drug treatment of benign focal epilepsies of childhood. Epilepsia 2000; 41 (8) 1057-1058
  • 29 Andrade R, García-Espinosa A, Machado-Rojas A, García-González ME, Trápaga-Quincoses O, Morales-Chacón LM. A prospective, open, controlled and randomised study of clobazam versus carbamazepine in patients with frequent episodes of rolandic epilepsy [in Spanish]. Rev Neurol 2009; 49 (11) 581-586
  • 30 Milburn-McNulty P, Powell G, Sills GJ, Marson AG. Sulthiame monotherapy for epilepsy. Cochrane Database Syst Rev 2014; 3: CD010062
  • 31 Borggraefe I, Bonfert M, Bast T , et al; German HEAD Study Group. Levetiracetam vs. sulthiame in benign epilepsy with centrotemporal spikes in childhood: a double-blinded, randomized, controlled trial (German HEAD Study). Eur J Paediatr Neurol 2013; 17 (5) 507-514
  • 32 Kramer U, Shahar E, Zelnik N , et al. Carbamazepine versus sulthiame in treating benign childhood epilepsy with centrotemporal spikes. J Child Neurol 2002; 17 (12) 914-916
  • 33 Rating D, Wolf C, Bast T ; Sulthiame Study Group. Sulthiame as monotherapy in children with benign childhood epilepsy with centrotemporal spikes: a 6-month randomized, double-blind, placebo-controlled study. Epilepsia 2000; 41 (10) 1284-1288
  • 34 Wirrell E, Sherman EM, Vanmastrigt R, Hamiwka L. Deterioration in cognitive function in children with benign epilepsy of childhood with central temporal spikes treated with sulthiame. J Child Neurol 2008; 23 (1) 14-21
  • 35 Corda D, Gelisse P, Genton P, Dravet C, Baldy-Moulinier M. Incidence of drug-induced aggravation in benign epilepsy with centrotemporal spikes. Epilepsia 2001; 42 (6) 754-759
  • 36 Kikumoto K, Yoshinaga H, Oka M , et al. EEG and seizure exacerbation induced by carbamazepine in Panayiotopoulos syndrome. Epileptic Disord 2006; 8 (1) 53-56
  • 37 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 (11) 1657-1660
  • 38 Cerminara C, Montanaro ML, Curatolo P, Seri S. Lamotrigine-induced seizure aggravation and negative myoclonus in idiopathic rolandic epilepsy. Neurology 2004; 63 (2) 373-375
  • 39 Ferrie CD, Beaumanoir A, Guerrini R , et al. Early-onset benign occipital seizure susceptibility syndrome. Epilepsia 1997; 38 (3) 285-293
  • 40 Bouma PA, Peters AC, Brouwer OF. Long term course of childhood epilepsy following relapse after antiepileptic drug withdrawal. J Neurol Neurosurg Psychiatry 2002; 72 (4) 507-510
  • 41 National Institute for Clinical Excellence. The epilepsies: the diagnosis and management of the epilepsies in adults and children in primary and secondary care. 1–73. 2012. London, National Institute for Clinical Excellence. Clinical Guideline 20. Available at http://www.nice.org.uk/cg137 . Accessed March 14, 2016
  • 42 Panayiotopoulos Ced. Occipital seizures and related epileptic syndromes. In: Benign Childhood Partial Seizures and Related Epileptic Syndromes. London: John Libbey; 1999: 101 228;
  • 43 Incecik F, Herguner OM, Altunbasak S. First-drug treatment failures in 42 Turkish children with idiopathic childhood occipital epilepsies. J Neurosci Rural Pract 2015; 6 (3) 300-303
  • 44 Verrotti A, Beccaria F, Fiori F, Montagnini A, Capovilla G. Photosensitivity: epidemiology, genetics, clinical manifestations, assessment, and management. Epileptic Disord 2012; 14 (4) 349-362
  • 45 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 (6) 1165-1172
  • 46 Trinka E, Cock H, Hesdorffer D , et al. A definition and classification of status epilepticus—Report of the ILAE Task Force on Classification of Status Epilepticus. Epilepsia 2015; 56 (10) 1515-1523
  • 47 Shorvon S. Clinical trials in acute repetitive seizures and status epilepticus. Epileptic Disord 2012; 14 (2) 138-147
  • 48 Shorvon S. The treatment of status epilepticus. Curr Opin Neurol 2011; 24 (2) 165-170
  • 49 Ferrie CD. Autonomic status epilepticus. In: Panayiotopoulos CP, ed. Atlas of Epilepsies. London: Springer; 2010: 559-562
  • 50 Panayiotopoulos Ced. Status epilepticus. In: A Clinical Guide to Epileptic Syndromes and Their Treatment. Revised 2nd ed. London: Springer; 2010: 65-95
  • 51 Lacroix L, Fluss J, Gervaix A, Korff CM. Benzodiazepines in the acute management of seizures with autonomic manifestations: anticipate complications!. Epilepsia 2011; 52 (10) e156-e159
  • 52 Valeta T. Psychosocial impact of epilepsy in children and family. In: Panayiotopoulos CP, ed. Atlas of Epilepsies. London: Springer; 2010: 1371-1373
  • 53 Valeta T, de Boer HM. Stigma and discrimination in epilepsy. In: Panayiotopoulos CP, ed. Atlas of Epilepsies. London: Springer; 2010: 1363-1366
  • 54 Valeta T. Parental reactions in benign childhood focal seizures. Epilepsia 2012; 53 (5) 222-223