Long-Term Outcome of Difficult-to-Treat Epilepsy in Childhood

Abstract

So far little is known about the long-term outcome of difficult-to-treat epilepsy syndromes of childhood. The aim of this study was to evaluate precise long-term data concerning the course of such epilepsies after 20 years. By means of a questionnaire we assessed the current situation of patients who had been treated as in-patients at the Department of Children and Adolescents at our Centre due to their difficult-to-treat epilepsy. Of 287 patients who met the inclusion criteria (diagnosis of difficult-to-treat epilepsy according to the final hospital record and hospitalisation for a therapy-resistant epilepsy), 176 were traced successfully and 81 completed and returned the questionnaire. A significant improvement of seizure frequency was reported by 49.7% and complete remission (with or without AEDs) by 25.9% of the patients. Social integration and work was attained by 21%. Cognitive impairment and treatment with more than 3 antiepileptic drugs proved to be significant negative outcome predictors. Among the patient population addressed in this study a high percentage had neurological impairments and/or cognitive deficiencies. Still, with 25.9% an unexpectedly high proportion of patients reached complete clinical remission after 20 years. Our data indicate that the natural course of difficult-to-treat epilepsies of childhood may be better than previously suggested.

Key words

difficult-to-treat epilepsy - epileptic syndromes in childhood - long-term outcome - social integration

References

• 1 Annegers JF, Hauser WA, Elvebback LR. Remission of seizures and relapse in patients with epilepsy.  Epilepsia. 1979;  20 729-737
• 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;  51 676-685
• 3 Berg AT, Levy SR, Novotny EJ. et al . Predictors of intractable epilepsy in childhood: a case-control study.  Epilepsia. 1996;  37 24-30
• 4 Brorson LO, Wranne L. Long-term prognosis in childhood epilepsy: survival and seizure prognosis.  Epilepsia. 1987;  28 324-330
• 5 Camfield P, Camfield C. Long-term prognosis for symptomatic (secondarily) generalized epilepsies: A population-based study.  Epilepsia. 2007;  48 1128-1132
• 6 Camfiled P, Camfield C, Gordon K. et al . Outcome of childhood epilepsy: A population-based study with a simple predictive scoring system for those treated with medication.  J Pediatr. 1993;  122 861-868
• 7 Chawla S, Aneja S, Kahsyap R. et al . Etiology and clinical predictors of intractable epilepsy.  Pediatr Neurol. 2002;  27 186-191
• 8 Conrad P. The meaning of medications: another look at compliance.  Soc Sci Med. 1985;  20 29-37
• 9 Goldsmith IL, Zupanc ML, Buchhatler JR. Long-term seizure outcome in 74 patients with Lennox-Gastaut syndrome: effects of incorporating MRI head imaging in defining the cryptogenic subgroup.  Epilepsia. 2000;  41 395-399
• 10 Gururaj A, Sztiha L, Hertecant J. et al . Clinical predictors of intractable childhood epilepsy.  J Psychosomat Res. 2006;  61 343-347
• 11 Hauser WA, Annegers JF, Rocca WA. Descriptive epidemiology of epilepsy: contributions of population-based studies from Rochester, Minnesota.  Mayo Clin Proc. 1996;  7 576-586
• 12 Hoffmann-Riem M, Diener W, Benninger C. et al . Nonconvulsive status epilepticus – a possible cause of mental retardation in patients with Lennox-Gastaut syndrome.  Neuropediatrics.. 2000;  31 169-174
• 13 Huttenlocher PR, Hapke RJ. A follow-up study of intractable seizures in childhood.  Ann Neurol. 1990;  28 699-705
• 14 Oguni H, Hayashi K, Osawa M. Long-term prognosis of Lennox-Gastaut syndrome.  Epilepsia. 1996;  37 (S 03) 44-47
• 15 Riikonen R. Long-term outcome of patients with West syndrome.  Brain Dev. 2001;  23 683-687
• 16 Shinnar S, O’Dell C, Berg AT. Distribution of epilepsy syndromes in a cohort of children prospectively monitored from the time of their first unprovoked seizure.  Epilepsia. 1999;  40 1378-1383
• 17 Sillanpää M. Remission of seizures and predictors of intractability in long-term follow-up.  Epilepsia. 1993;  34 930-936
• 18 Specht U. Lennox-Gastaut Syndrom bei Erwachsenen. In: Krämer G, Sälke-Kellermann RA, (Eds) Das Lennox-Gastaut Syndrom. Epilepsie-Berichte 5,. Blackwell; Berlin Wien; 1998: 103-114
• 19 Steinhoff BJ, Nitsche M, Naumann M. et al . Kork, Germany: Diakonie Kork Epilepsiezentrum. Epilepsy Centre, Kork.  Seizure. 2003;  12 (S 01) S27-S31
• 20 Strzelcyk A, Reese JP, Dodel R. et al . Cost of epilepsy: a systematic review.  Pharamcoeconomics. 2008;  26 463-476
• 21 Trevathan E. Infantile spasms and Lennox-Gastaut syndrome.  J Child Neurol. 2002;  17 2S9-2S22
• 22 Trostle JA. Medical compliance as an ideology.  Soc Sci Med. 1998;  27 1299-1308
• 23 Yagi K. Evolution of Lennox-Gastaut syndrome: a long-term longitudinal study.  Epilepsia. 1996;  37 (S 03) 48-51

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