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Journal of Neurology

, Volume 251, Issue 10, pp 1235–1241 | Cite as

Long-term prognosis for childhood and juvenile absence epilepsy

  • Eugen TrinkaEmail author
  • Sarah Baumgartner
  • Iris Unterberger
  • Josef Unterrainer
  • Gerhard Luef
  • Edda Haberlandt
  • Gerhard Bauer
ORIGINAL COMMUNICATION

Abstract.

Purpose:

To analyse prognostic factors for long term seizure remission in patients with childhood (CAE) and juvenile absence epilepsy (JAE).

Study design:

A retrospective analysis of a hospital based prevalence cohort.

Methods:

The cohort consisted of 163 patients (104 females, 59 males) treated at the Universitätsklinik für Neurologie, Innsbruck between 1970 and 1997. All had absences according to the ILAE classification. Follow up was in 1999 to 2000. We assessed multiple clinical and EEG factors as predictors of outcome and compared a classification according to the predominant pattern of seizure recurrence (pyknoleptic, PA or non pyknoleptic absence, NPA) with the ILAE classification with respect to prognosis.

Results:

The mean age at seizure onset was 10.9 years (range, 3 to 27); age at follow up was 36.7 years (range, 13 to 81); duration of follow up was 25.8 years (range, 3 to 69). Sixty four patients (39 %) had CAE and 64 (39 %) JAE, while 35 (22%) had typical absences but could not be clearly defined as either CAE or JAE, and were therefore called “the overlap group”. Patients with JAE or patients in the overlap group developed more often generalized tonic clonic seizures (GTCS) (p<0.001) and myoclonic attacks (p<0.05) during the course of the disease. At follow up 36 (56 %) of patients with CAE, 40 (62%) with JAE and 19 (54 %) of the overlap group were seizure free for at least two years (p=ns). When classified according to the predominant absence pattern at seizure onset 42 (51%) patients with PA and 53 (65%) with NPA were in remission (p=ns). In a stepwise binary logistic regression analysis the pattern of absence (PA or NPA) together with the later development of additional seizure types (myoclonias or GTCS), but not the CAE/JAE classification was predictive for long term lack of remission with a correct prediction of 66% of all patients.

Conclusion:

Only 58% of patients with absences were in remission after a long term follow up. CAE and JAE are closely related syndromes with large overlap of the age of onset. A classification according to the predominant seizure pattern at onset, together with later development of myoclonic attacks or GTCS is useful in predicting seizure remission in absence epilepsies.

Key words

childhood absence epilepsy juvenile absence epilepsy absence seizure epilepsy prognosis 

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References

  1. 1.
    Commission on Classification and Terminology of the International League Against Epilepsy (1981) Proposal for revised clinical and electroencephalographic classification of epileptic seizures. Epilepsia 22:489–501PubMedGoogle Scholar
  2. 2.
    Commission on Classification and Terminology of the International League Against Epilepsy (1989) Proposal for revised classification of epilepsies and epileptic syndromes. Epilepsia 30:389–399PubMedGoogle Scholar
  3. 3.
    Andermann F, Berkovic SF (2001) Idiopathic generalized epilepsy with generalized and other seizures in adolescence. Epilepsia 42:317–320CrossRefPubMedGoogle Scholar
  4. 4.
    Bartolomei F, Roger J, Bureau M, Genton P, Dravet C, Viallat D, Gastaut JL (1997) Prognostic factors for childhood and juvenile absence epilepsies. Eur Neurol 37:169–175PubMedGoogle Scholar
  5. 5.
    Bauer G (1972) Catamnestic studies in 3-sec spike and wave carriers. Fortschr Neurol Psychiatr Grenzgeb 41:177–224PubMedGoogle Scholar
  6. 6.
    Berkovic SF, Andermann F, Andermann E, Gloor P (1987) Concepts of absence epilepsies: discrete syndromes or biological continuum? Neurology 37:993–1000PubMedGoogle Scholar
  7. 7.
    Bouma PA, Westendorp RG, van Dijk JG, Peters AC, Brouwer OF (1996) The outcome of absence epilepsy: a metaanalysis. Neurology 47:802–808PubMedGoogle Scholar
  8. 8.
    Delgado-Escueta AV, Medina MT, Serratosa JM, Castroviejo IP, Gee MN, Weissbecker K, Westling BW, Fong CY, Alonso ME, Cordova S, Shah P, Khan S, Sainz J, Rubio-Donnadieu F, Sparkes RS (1999) Mapping and positional cloning of common idiopathic generalized epilepsies: juvenile myoclonus epilepsy and childhood absence epilepsy. Adv Neurol 79:351–374PubMedGoogle Scholar
  9. 9.
    Dieterich E, Baier WK, Doose H, Tuxhorn I, Fichsel H (1985) Longterm follow-up of childhood epilepsy with absences. I. Epilepsy with absences at onset. Neuropediatrics 16:149–154PubMedGoogle Scholar
  10. 10.
    Dieterich E, Doose H, Baier WK, Fichsel H (1985) Longterm follow-up of childhood epilepsy with absences. II. Absence-epilepsy with initial grand mal. Neuropediatrics 16:155–158PubMedGoogle Scholar
  11. 11.
    Doose H, Volzke E, Scheffner D (1965) Course forms of infantile epilepsies with spike waves absences. Arch Psychiatr Nervenkr 207:394–415PubMedGoogle Scholar
  12. 12.
    Feucht M, Fuchs K, Pichlbauer E, Hornik K, Scharfetter J,Goessler R, Fureder T, Cvetkovic N, Sieghart W, Kasper S, Aschauer H (1999) Possible association between childhood absence epilepsy and the gene encoding GABRB3. Biol Psychiatry 46:997–1002CrossRefPubMedGoogle Scholar
  13. 13.
    Fong GC, Shah PU, Gee MN, Serratosa JM, Castroviejo IP, Khan S, Ravat SH, Mani J, Huang Y, Zhao HZ, Medina MT, Treiman LJ, Pineda G, Delgado-Escueta AV (1998) Childhood absence epilepsy with tonic-clonic seizures and electroencephalogram 3–4-Hz spike and multispike-slow wave complexes: linkage to chromosome 8q24. Am J Hum Genet 63:1117–1129CrossRefPubMedGoogle Scholar
  14. 14.
    Haug K, Warnstedt M, Alekov AK, Sander T, Ramirez A, Poser B, Maljevic S, Hebeisen S, Kubisch C, Rebstock J, Horvath S, Hallmann K, Dullinger JS, Rau B, Haverkamp F, Beyenburg S, Schulz H, Janz D, Giese B, Muller-Newen G, Propping P, Elger CE, Fahlke C, Lerche H, Heils A (2003) Mutations in CLCN2 encoding a voltage-gated chloride channel are associated with idiopathic generalized epilepsies. Nat Genet 33:527–532CrossRefPubMedGoogle Scholar
  15. 15.
    Hirsch E, Blanc-Platier A, arescaux C (1994) What are the relevant criteria for a better classification of epileptic syndromes with typical absences? In: Malafosse A, Genton P, Marescaux C, Hirsch E, Broglin D, Bernasconi R (eds) Idiopathic generalized epilepsies: clinical, experimental and genetic aspects. Libbey, London, pp 87–93Google Scholar
  16. 16.
    Hughes JR, Kaydanova Y (1997) Long-term studies on patients with absence and bilateral spike-wave complexes: 430 patients, up to 52 years follow-up. Clin Electroencephalogr 28:193–206PubMedGoogle Scholar
  17. 17.
    Janz D (1969) Die Epilepsien: Spezielle Pathologie und Therapie. Thieme, StuttgartGoogle Scholar
  18. 18.
    Janz D, Beck-Mannagetta G, Spröder B, Spröder J, Waltz S (1994) Childhood absence epilepsy (pyknolepsy) and juvenile absence epilepsy: one or two syndromes? In:Wolf P (ed) Epileptic seizures and syndromes. Libbey, London, pp 115–126Google Scholar
  19. 19.
    Jouvenceau A, Eunson LH, Spauschus A, Ramesh V, Zuberi SM, Kullmann DM, Hanna MG (2001) Human epilepsy associated with dysfunction of the brain P/Q-type calcium channel. Lancet 358:801–807CrossRefPubMedGoogle Scholar
  20. 20.
    Kananura C, Haug K, Sander T, Runge U, Gu W, Hallmann K, Rebstock J, Heils A, Steinlein OK (2002) A splice-site mutation in GABRG2 associated with childhood absence epilepsy and febrile convulsions. Arch Neurol 59:1137–1141CrossRefPubMedGoogle Scholar
  21. 21.
    Loiseau P (1992) Childhood absence epilepsy. In: Roger J, Bureau M, Dravet C, Dreifuss FE, Perret A, Wolf P (eds) Epileptic syndromes in infancy, childhood and adolescence. Libbey, London, pp 135–151Google Scholar
  22. 22.
    Loiseau P, Duche B, Pedespan JM (1995) Absence epilepsies. Epilepsia 36:1182–1186PubMedGoogle Scholar
  23. 23.
    Panayiotopoulos C (1994) The clinical spectrum of typical absence seizures and absence epilepsies. In: Malafosse A, Genton P, Hirsch E, Marescaux C, Broglin D, Bernasconi R (eds) Idiopathic generalized epilepsies: clinical, experimental and genetic aspects. Libbey, London, pp 75–85Google Scholar
  24. 24.
    Robinson R, Taske N, Sander T,Heils A, Whitehouse W, Goutieres F, Aicardi J, Lehesjoki AE, Siren A, Laue FM, Kjeldsen MJ, Panayiotopoulos C, Kennedy C, Ferrie C, Rees M, Gardiner RM (2002) Linkage analysis between childhood absence epilepsy and genes encoding GABAA and GABAB receptors, voltage-dependent calcium channels, and the ECA1 region on chromosome 8q. Epilepsy Res 48:169–179CrossRefPubMedGoogle Scholar
  25. 25.
    Sato S, Dreifuss FE, Penry JK, Kirby DD, Palesch Y (1983) Long-term follow-up of absence seizures. Neurology 33:1590–1595PubMedGoogle Scholar
  26. 26.
    Sugimoto Y, Morita R, Amano K, Fong CY, Shah PU, Castroviejo IP, Khan S, Delgado-Escueta AV, Yamakawa K (2000) Childhood absence epilepsy in 8q24: refinement of candidate region and construction of physical map. Genomics 68:264–272CrossRefPubMedGoogle Scholar
  27. 27.
    Wallace RH, Marini C, Petrou S, Harkin LA, Bowser DN, Panchal RG, Williams DA, Sutherland GR, Mulley JC, Scheffer IE, Berkovic SF (2001) Mutant GABA(A) receptor gamma2-subunit in childhood absence epilepsy and febrile seizures. Nat Genet 28:49–52CrossRefPubMedGoogle Scholar
  28. 28.
    Wirrell E, Camfield C, Camfield P, Dooley J (2001) Prognostic significance of failure of the initial antiepileptic drug in children with absence epilepsy. Epilepsia 42:760–763CrossRefPubMedGoogle Scholar
  29. 29.
    Wirrell EC, Camfield CS, Camfield PR, Gordon KE, Dooley JM (1996) Longterm prognosis of typical childhood absence epilepsy: remission or progression to juvenile myoclonic epilepsy. Neurology 47:912–918PubMedGoogle Scholar

Copyright information

© Steinkopff Verlag 2004

Authors and Affiliations

  • Eugen Trinka
    • 3
    Email author
  • Sarah Baumgartner
    • 1
    • 3
  • Iris Unterberger
    • 3
  • Josef Unterrainer
    • 2
  • Gerhard Luef
    • 3
  • Edda Haberlandt
    • 1
  • Gerhard Bauer
    • 3
  1. 1.Universitätsklinik für Kinder und JugendheilkundeInnsbruckAustria
  2. 2.Institut für PsychologieUniversität FreiburgFreiburgGermany
  3. 3.Universitätsklinik für NeurologieMedizinische Universität InnsbruckInnsbruckAustria

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