Skip to main content
PMC home page
Journal of Neurology, Neurosurgery, and Psychiatry logoLink to Journal of Neurology, Neurosurgery, and Psychiatry
. 2003 Feb;74(2):192–196. doi: 10.1136/jnnp.74.2.192

Idiopathic generalised epilepsy of adult onset: clinical syndromes and genetics

C Marini 1, M King 1, J Archer 1, M Newton 1, S Berkovic 1
PMCID: PMC1738270  PMID: 12531947

Abstract

Objective: To study the clinical features and genetics of idiopathic generalised epilepsy (IGE) beginning in adult life.

Methods: Consecutive patients with IGE, defined as generalised seizures with spike or polyspike and wave on EEG, were studied in the setting of a first seizure clinic where an early postictal EEG record is part of the protocol. Patients were divided into two groups: "classical IGE" with onset before 20 years and inclusive of all the IGE subsyndromes recognised by the international classification; and "adult onset IGE", when seizure onset was at age 20 years or later. Seizure patterns, clinical features, and genetics of the adult onset group were examined.

Results: Of 121 patients with an electro-clinical diagnosis of IGE, 34 (28%) were diagnosed as adult onset IGE. The seizure patterns in these 34 cases were tonic–clonic seizures + absences (3), tonic–clonic seizures + myoclonus (6), and tonic–clonic seizures alone (25). Tonic–clonic seizures were often precipitated by alcohol or sleep deprivation. The proportion of affected first and second degree relatives did not differ between the classical and adult onset IGE groups. Twenty adult onset cases were treated with sodium valproate, four with other antiepileptic drugs, and 10 were untreated. Follow up of 32 of the 34 cases (for 31 (22) months (mean (SD)) showed that tonic–clonic seizures recurred in eight patients: five with identified provocative factors and three without.

Conclusions: Adult onset IGE is a relatively frequent and benign disorder. Seizures are usually provoked and are easy to control. Patients in this age group may often be misdiagnosed as having non-lesional partial epilepsy. Early postictal EEG and sleep deprivation studies may improve the detection of these patients. Pedigree analysis suggests that adult onset IGE, like classical IGE, has a genetic aetiology.

Full Text

The Full Text of this article is available as a PDF (220.5 KB).

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Andermann F., Berkovic S. F. Idiopathic generalized epilepsy with generalized and other seizures in adolescence. Epilepsia. 2001 Mar;42(3):317–320. doi: 10.1046/j.1528-1157.2001.36400.x. [DOI] [PubMed] [Google Scholar]
  2. Appleton R. E., Panayiotopoulos C. P., Acomb B. A., Beirne M. Eyelid myoclonia with typical absences: an epilepsy syndrome. J Neurol Neurosurg Psychiatry. 1993 Dec;56(12):1312–1316. doi: 10.1136/jnnp.56.12.1312. [DOI] [PMC free article] [PubMed] [Google Scholar]
  3. Berkovic S. F., Bladin P. F. Absence status in adults. Clin Exp Neurol. 1983;19:198–207. [PubMed] [Google Scholar]
  4. Cutting S., Lauchheimer A., Barr W., Devinsky O. Adult-onset idiopathic generalized epilepsy: clinical and behavioral features. Epilepsia. 2001 Nov;42(11):1395–1398. doi: 10.1046/j.1528-1157.2001.14901.x. [DOI] [PubMed] [Google Scholar]
  5. Gastaut H., Gastaut J. L., Gonçalves e Silva G. E., Fernandez Sanchez G. R. Relative frequency of different types of epilepsy: a study employing the classification of the International League Against Epilepsy. Epilepsia. 1975 Sep;16(3):457–461. doi: 10.1111/j.1528-1157.1975.tb06073.x. [DOI] [PubMed] [Google Scholar]
  6. Gastaut H. Individualisation des epilepsies dites "benignes" ou "fonctionnelles" aux differents ages de la vie. Appreciation des variations correspondantes de la predisposition epileptique a ces ages. Rev Electroencephalogr Neurophysiol Clin. 1981 Dec;11(3-4):346–366. doi: 10.1016/s0370-4475(81)80072-x. [DOI] [PubMed] [Google Scholar]
  7. Gilliam F., Steinhoff B. J., Bittermann H. J., Kuzniecky R., Faught E., Abou-Khalil B. Adult myoclonic epilepsy: a distinct syndrome of idiopathic generalized epilepsy. Neurology. 2000 Oct 10;55(7):1030–1033. doi: 10.1212/wnl.55.7.1030. [DOI] [PubMed] [Google Scholar]
  8. Gram L., Alving J., Sagild J. C., Dam M. Juvenile myoclonic epilepsy in unexpected age groups. Epilepsy Res. 1988 Mar-Apr;2(2):137–140. doi: 10.1016/0920-1211(88)90032-0. [DOI] [PubMed] [Google Scholar]
  9. Janz D., Beck-Mannagetta G., Sander T. Do idiopathic generalized epilepsies share a common susceptibility gene? Neurology. 1992 Apr;42(4 Suppl 5):48–55. [PubMed] [Google Scholar]
  10. Janz D. The idiopathic generalized epilepsies of adolescence with childhood and juvenile age of onset. Epilepsia. 1997 Jan;38(1):4–11. doi: 10.1111/j.1528-1157.1997.tb01073.x. [DOI] [PubMed] [Google Scholar]
  11. Jeavons P. M. Nosological problems of myoclonic epilepsies in childhood and adolescence. Dev Med Child Neurol. 1977 Feb;19(1):3–8. doi: 10.1111/j.1469-8749.1977.tb08014.x. [DOI] [PubMed] [Google Scholar]
  12. King M. A., Newton M. R., Jackson G. D., Fitt G. J., Mitchell L. A., Silvapulle M. J., Berkovic S. F. Epileptology of the first-seizure presentation: a clinical, electroencephalographic, and magnetic resonance imaging study of 300 consecutive patients. Lancet. 1998 Sep 26;352(9133):1007–1011. doi: 10.1016/S0140-6736(98)03543-0. [DOI] [PubMed] [Google Scholar]
  13. Loiseau J., Crespel A., Picot M. C., Duché B., Ayrivié N., Jallon P., Loiseau P. Idiopathic generalized epilepsy of late onset. Seizure. 1998 Dec;7(6):485–487. doi: 10.1016/s1059-1311(98)80007-1. [DOI] [PubMed] [Google Scholar]
  14. Mikami M., Yasuda T., Terao A., Nakamura M., Ueno S., Tanabe H., Tanaka T., Onuma T., Goto Y., Kaneko S. Localization of a gene for benign adult familial myoclonic epilepsy to chromosome 8q23.3-q24.1. Am J Hum Genet. 1999 Sep;65(3):745–751. doi: 10.1086/302535. [DOI] [PMC free article] [PubMed] [Google Scholar]
  15. Oller-Daurella L., Sorel L. L'épilepsie grand mal bénigne de l'adulte. Acta Neurol Belg. 1989 Jan-Feb;89(1):38–45. [PubMed] [Google Scholar]
  16. Panayiotopoulos C. P., Koutroumanidis M., Giannakodimos S., Agathonikou A. Idiopathic generalised epilepsy in adults manifested by phantom absences, generalised tonic-clonic seizures, and frequent absence status. J Neurol Neurosurg Psychiatry. 1997 Nov;63(5):622–627. doi: 10.1136/jnnp.63.5.622. [DOI] [PMC free article] [PubMed] [Google Scholar]
  17. Plaster N. M., Uyama E., Uchino M., Ikeda T., Flanigan K. M., Kondo I., Ptácek L. J. Genetic localization of the familial adult myoclonic epilepsy (FAME) gene to chromosome 8q24. Neurology. 1999 Oct 12;53(6):1180–1183. doi: 10.1212/wnl.53.6.1180. [DOI] [PubMed] [Google Scholar]
  18. Schwartz M. S., Scott D. F. Isolated petit-mal status presenting de novo in middle age. Lancet. 1971 Dec 25;2(7739):1399–1401. doi: 10.1016/s0140-6736(71)90672-6. [DOI] [PubMed] [Google Scholar]
  19. Unterberger I., Trinka E., Luef G., Bauer G. Idiopathic generalized epilepsies with pure grand mal: clinical data and genetics. Epilepsy Res. 2001 Apr;44(1):19–25. doi: 10.1016/s0920-1211(00)00210-2. [DOI] [PubMed] [Google Scholar]

Articles from Journal of Neurology, Neurosurgery, and Psychiatry are provided here courtesy of BMJ Publishing Group

RESOURCES