Journal of Pediatric Epilepsy 2015; 04(03): 096-101
DOI: 10.1055/s-0035-1556738
Review Article
Georg Thieme Verlag KG Stuttgart · New York

Generalized Epilepsy Syndromes of Adolescence

Debopam Samanta
1   Department of Pediatrics, University of Arkansas for Medical Sciences, Little Rock, Arkansas, United States
,
Erin Willis
1   Department of Pediatrics, University of Arkansas for Medical Sciences, Little Rock, Arkansas, United States
› Author Affiliations
Further Information

Publication History

12 June 2014

01 December 2014

Publication Date:
17 August 2015 (online)

Abstract

“Idiopathic” generalized epilepsies (juvenile absence epilepsy, juvenile myoclonic epilepsy, and epilepsy with generalized tonic–clonic seizures alone) are the most frequent group of epilepsies with onset during adolescence and constitute 15 to 20% of all epilepsies. These syndromes have overlapping clinical features and genetic origins as well as similar electroencephalographic findings. There are primarily three seizure types that occur with these epilepsy syndromes: generalized tonic–clonic, absence, and myoclonic seizures. Standard neurologic examination and neuroimaging studies do not generally reveal any abnormality. Potential precipitating factors are many, including sleep deprivation, fatigue, alcohol use, photic stimulation, and menstruation. The characteristic electroencephalographic abnormality consists of diffuse, bilateral, symmetric, and synchronous 4 to 6 Hz polyspike and wave complexes. Idiopathic generalized epilepsies are generally associated with low mortality and typically respond well to treatment. The most effective antiepileptic drug for these disorders is sodium valproate, but it should be used with caution in adolescent females who are of childbearing age. Levetiracetam, lamotrigine, topiramate, and zonisamide can also be used as effective monotherapy. Carbamazepine, oxcarbamazepine, phenytoin, gabapentin, and vigabatrin may exacerbate myoclonic and absence seizures in juvenile myoclonic epilepsy.

 
  • References

  • 1 Commission on Classification and Terminology of the International League Against Epilepsy. Proposal for revised classification of epilepsies and epileptic syndromes. Epilepsia 1989; 30 (4) 389-399
  • 2 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
  • 3 Jallon P, Latour P. Epidemiology of idiopathic generalized epilepsies. Epilepsia 2005; 46 (Suppl. 09) 10-14
  • 4 Genton P, Gelisse P. The history of juvenile myoclonic epilepsy. Epilepsy Behav 2013; 28 (Suppl. 01) S2-S7
  • 5 Camfield CS, Striano P, Camfield PR. Epidemiology of juvenile myoclonic epilepsy. Epilepsy Behav 2013; 28 (Suppl. 01) S15-S17
  • 6 Delgado-Escueta AV. Advances in genetics of juvenile myoclonic epilepsies. Epilepsy Curr 2007; 7 (3) 61-67
  • 7 Manganotti P, Bongiovanni LG, Fuggetta G, Zanette G, Fiaschi A. Effects of sleep deprivation on cortical excitability in patients affected by juvenile myoclonic epilepsy: a combined transcranial magnetic stimulation and EEG study. J Neurol Neurosurg Psychiatry 2006; 77 (1) 56-60
  • 8 Badawy RA, Macdonell RA, Jackson GD, Berkovic SF. Why do seizures in generalized epilepsy often occur in the morning?. Neurology 2009; 73 (3) 218-222
  • 9 Murthy JM, Rao CM, Meena AK. Clinical observations of juvenile myoclonic epilepsy in 131 patients: a study in South India. Seizure 1998; 7 (1) 43-47
  • 10 Vijai J, Cherian PJ, Stlaja PN, Anand A, Radhakrishnan K. Clinical characteristics of a South Indian cohort of juvenile myoclonic epilepsy probands. Seizure 2003; 12 (7) 490-496
  • 11 Canevini MP, Mai R, Di Marco C , et al. Juvenile myoclonic epilepsy of Janz: clinical observations in 60 patients. Seizure 1992; 1 (4) 291-298
  • 12 Jayalakshmi SS, Srinivasa Rao B, Sailaja S. Focal clinical and electroencephalographic features in patients with juvenile myoclonic epilepsy. Acta Neurol Scand 2010; 122 (2) 115-123
  • 13 Vollmar C, O'Muircheartaigh J, Barker GJ , et al. Motor system hyperconnectivity in juvenile myoclonic epilepsy: a cognitive functional magnetic resonance imaging study. Brain 2011; 134 (Pt 6) 1710-1719
  • 14 Chifari R, Piazzini A, Turner K, Canger R, Canevini MP, Wolf P. Reflex writing seizures in two siblings with juvenile myoclonic epilepsy. Acta Neurol Scand 2004; 109 (3) 232-235
  • 15 da Silva Sousa P, Lin K, Garzon E, Ceiki Sakamoto A, Yacubian EM. Language- and praxis-induced jerks in patients with juvenile myoclonic epilepsy. Epileptic Disord 2005; 7 (2) 115-121
  • 16 Pulsipher DT, Seidenberg M, Guidotti L , et al. Thalamofrontal circuitry and executive dysfunction in recent-onset juvenile myoclonic epilepsy. Epilepsia 2009; 50 (5) 1210-1219
  • 17 Woermann FG, Free SL, Koepp MJ, Sisodiya SM, Duncan JS. Abnormal cerebral structure in juvenile myoclonic epilepsy demonstrated with voxel-based analysis of MRI. Brain 1999; 122 (Pt 11) 2101-2108
  • 18 Baise-Zung C, Guilhoto LM, Grossmann RM. Juvenile myoclonic epilepsy: non-classic electroencephalographical presentation in adult patients. Eur J Neurol 2006; 13 (2) 171-175
  • 19 Panayiotopoulos CP, Obeid T, Tahan AR. Juvenile myoclonic epilepsy: a 5-year prospective study. Epilepsia 1994; 35 (2) 285-296
  • 20 Appleton R, Beirne M, Acomb B. Photosensitivity in juvenile myoclonic epilepsy. Seizure 2000; 9 (2) 108-111
  • 21 Hrachovy RA, Frost Jr JD. The EEG in selected generalized seizures. J Clin Neurophysiol 2006; 23 (4) 312-332
  • 22 Panayiotopoulos CP, Obeid T, Waheed G. Absences in juvenile myoclonic epilepsy: a clinical and video-electroencephalographic study. Ann Neurol 1989; 25 (4) 391-397
  • 23 Holmes MD, Quiring J, Tucker DM. Evidence that juvenile myoclonic epilepsy is a disorder of frontotemporal corticothalamic networks. Neuroimage 2010; 49 (1) 80-93
  • 24 Hantas S. Idiopathic generalized epilepsy syndromes of childhood and adolescence. In: Wyllie E, Cascino GD, Gidal BE, Goodkin HP, , eds. Wyllie's Treatment of Epilepsy: Principles and Practice. 5th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2011: 258-268
  • 25 Marson AG, Al-Kharusi AM, Alwaidh M , et al; SANAD Study group. The SANAD study of effectiveness of valproate, lamotrigine, or topiramate for generalised and unclassifiable epilepsy: an unblinded randomised controlled trial. Lancet 2007; 369 (9566) 1016-1026
  • 26 Morris GL, Hammer AE, Kustra RP, Messenheimer JA. Lamotrigine for patients with juvenile myoclonic epilepsy following prior treatment with valproate: results of an open-label study. Epilepsy Behav 2004; 5 (4) 509-512
  • 27 Beran RG, Berkovic SF, Dunagan FM , et al. Double-blind, placebo-controlled, crossover study of lamotrigine in treatment-resistant generalised epilepsy. Epilepsia 1998; 39 (12) 1329-1333
  • 28 Biton V, Bourgeois BF ; YTC/YTCE Study Investigators. Topiramate in patients with juvenile myoclonic epilepsy. Arch Neurol 2005; 62 (11) 1705-1708
  • 29 Labate A, Colosimo E, Gambardella A, Leggio U, Ambrosio R, Quattrone A. Levetiracetam in patients with generalised epilepsy and myoclonic seizures: an open label study. Seizure 2006; 15 (3) 214-218
  • 30 Berkovic SF, Knowlton RC, Leroy RF, Schiemann J, Falter U ; Levetiracetam N01057 Study Group. Placebo-controlled study of levetiracetam in idiopathic generalized epilepsy. Neurology 2007; 69 (18) 1751-1760
  • 31 Kothare SV, Valencia I, Khurana DS, Hardison H, Melvin JJ, Legido A. Efficacy and tolerability of zonisamide in juvenile myoclonic epilepsy. Epileptic Disord 2004; 6 (4) 267-270
  • 32 Penry JK, Dean JC, Riela AR. Juvenile myoclonic epilepsy: long-term response to therapy. Epilepsia 1989; 30 (Suppl. 04) S19-S23 , discussion S24–S27
  • 33 Nicolson A, Marson AG. When the first antiepileptic drug fails in a patient with juvenile myoclonic epilepsy. Pract Neurol 2010; 10 (4) 208-218
  • 34 Noachtar S, Andermann E, Meyvisch P, Andermann F, Gough WB, Schiemann-Delgado J ; N166 Levetiracetam Study Group. Levetiracetam for the treatment of idiopathic generalized epilepsy with myoclonic seizures. Neurology 2008; 70 (8) 607-616
  • 35 Mantoan L, Walker M. Treatment options in juvenile myoclonic epilepsy. Curr Treat Options Neurol 2011; 13 (4) 355-370
  • 36 Knott C, Panayiotopoulos CP. Carbamazepine in the treatment of generalised tonic clonic seizures in juvenile myoclonic epilepsy. J Neurol Neurosurg Psychiatry 1994; 57 (4) 503
  • 37 Genton P, Gelisse P, Thomas P, Dravet C. Do carbamazepine and phenytoin aggravate juvenile myoclonic epilepsy?. Neurology 2000; 55 (8) 1106-1109
  • 38 Perucca E. Birth defects after prenatal exposure to antiepileptic drugs. Lancet Neurol 2005; 4 (11) 781-786
  • 39 Meador KJ, Baker GA, Browning N , et al; NEAD Study Group. Fetal antiepileptic drug exposure and cognitive outcomes at age 6 years (NEAD study): a prospective observational study. Lancet Neurol 2013; 12 (3) 244-252
  • 40 Cunnington M, Tennis P ; International Lamotrigine Pregnancy Registry Scientific Advisory Committee. Lamotrigine and the risk of malformations in pregnancy. Neurology 2005; 64 (6) 955-960
  • 41 Nicolson A, Appleton RE, Chadwick DW, Smith DF. The relationship between treatment with valproate, lamotrigine, and topiramate and the prognosis of the idiopathic generalised epilepsies. J Neurol Neurosurg Psychiatry 2004; 75 (1) 75-79
  • 42 Geithner J, Schneider F, Wang Z , et al. Predictors for long-term seizure outcome in juvenile myoclonic epilepsy: 25-63 years of follow-up. Epilepsia 2012; 53 (8) 1379-1386
  • 43 Senf P, Schmitz B, Holtkamp M, Janz D. Prognosis of juvenile myoclonic epilepsy 45 years after onset: seizure outcome and predictors. Neurology 2013; 81 (24) 2128-2133
  • 44 Unterberger I, Trinka E, Luef G, Bauer G. Idiopathic generalized epilepsies with pure grand mal: clinical data and genetics. Epilepsy Res 2001; 44 (1) 19-25
  • 45 Elwes RD, Johnson AL, Shorvon SD, Reynolds EH. The prognosis for seizure control in newly diagnosed epilepsy. N Engl J Med 1984; 311 (15) 944-947
  • 46 Annegers JF, Hauser WA, Elveback LR. Remission of seizures and relapse in patients with epilepsy. Epilepsia 1979; 20 (6) 729-737
  • 47 Tovia E, Goldberg-Stern H, Shahar E, Kramer U. Outcome of children with juvenile absence epilepsy. J Child Neurol 2006; 21 (9) 766-768
  • 48 Obeid T. Clinical and genetic aspects of juvenile absence epilepsy. J Neurol 1994; 241 (8) 487-491
  • 49 Kostov H, Larsson PG, Røste GK. Is vagus nerve stimulation a treatment option for patients with drug-resistant idiopathic generalized epilepsy?. Acta Neurol Scand Suppl 2007; 187: 55-58