Download PDF
Neuropediatrics 2002; 33(1): 21-26
DOI: 10.1055/s-2002-23595
Original Article

Georg Thieme Verlag Stuttgart · New York

Epilepsy Surgery in Children with Focal Cortical Dysplasia (FCD): Results of Long-Term Seizure Outcome

S. Kloss1 , T. Pieper2 , H. Pannek1 , H. Holthausen2 , I. Tuxhorn1
  • 1 Pediatric Epilepsy Surgery Program, Epilepsiezentrum Bethel, Klinik Mara, Bielefeld, Germany
  • 2 Behandlungszentrum Vogtareuth, Vogtareuth, Germany
Further Information

Publication History

Publication Date:
03 April 2002 (online)

    Permissions and Reprints

Abstract

The purpose of this study was to assess the effect of epilepsy surgery on seizure outcome in children and adolescents under 18 years with intractable epilepsy due to focal cortical dysplasia. We analysed clinical data, such as age at seizure onset, epilepsy course, localisation of focus from presurgical evaluation, MRI, tissue pathology and seizure outcome in 68 patients 6 months to 9 years after epilepsy surgery. Seizure outcome was classified according to the Engel classification. Mean age at seizure onset was 7 months, ranging from the first days of life to 7 years. All patients had medically intractable epilepsy. Localisation of the lesion was predominantly extratemporal: posterior (uni- or multilobar) 43 %, frontal without central region 26 %, multilobar involving central area 19 % and temporal in 12 %. MRI signs typically seen in cortical dysplasia (FCD) such as localised blurring of gray-white matter junction was found in 68 %, dysgyria in 62 %, thickening of the cortical ribbon in 46 % and T2 signal elongation of the subcortical white matter in 40 % of the patients' MRI. Age at surgery ranged from 5 months to 16 years; 14 patients were under 2 years when operated on. In 34 patients (6 patients under 3 years) subdural grid electrode evaluation was performed prior to surgery. Pathology revealed focal cortical dysplasia without balloon cells (type I) in 60 %, FCD of the balloon cell subtype (type II) in 40 % of the specimens. Postoperative complications were subdural hygroma in 5 and an increased motor deficit in 2 patients. Up to two years after epilepsy surgery 50 % of the children were seizure free (Engel class I), 10 % Engel class II, 33 % Engel class III and 7 % unchanged (Engel class IV). Long-term seizure outcome (> 3 years post surgery) in 32 patients showed similar results (class I 50 %, class II 19 %, class III 28 %, class IV 3 %). Complete resection of the dysplastic lesion was significantly correlated with favorable seizure outcome, whereas seizure outcome was not significantly different in patients with mild (type I) or balloon cell (type II) FCD. Children operated after 6 years of age had no better outcome than children operated in infancy or at preschool age. Epilepsy surgery resulted in good (class I and II) seizure control in 60 % of children with intractable epilepsy due to focal cortical dysplasia.

Key words

Focal Cortical Dysplasia - Epilepsy Surgery - Seizure Outcome

References

  • 1 Asarnow R F, LoPresti C, Guthrie D. et al . Developmental outcomes in children receiving resection surgery for medically intractable spasms.  Dev Med Child Neurol. 1997;  39 430-440
    PubMedGoogle Scholar
  • 2 Brännström T, Nordborg C, Olivecrona M, Silvewnius H, Sourander P. Disorders of neuronal migration in surgically resected epilepsy patients.  Epilepsia. 1993;  34 71
    PubMedGoogle Scholar
  • 3 Chugani H T, Shewmon D A, Shields W D. et al . Surgery for intractable infantile spasms: neuroimaging perspectives.  Epilepsia. 1993;  34 764-771
    CrossrefPubMedGoogle Scholar
  • 4 Duchowny M, Harvey A S, Goldstein R, Bruce J, Jayakar P, Resnick T, Alvarez L, Altman N. Cortical dysplasia in children with intractable partial epilepsy.  Epilepsia. 1994;  35 (Suppl 8) 14
    PubMedGoogle Scholar
  • 5 Duchowny M, Jayakar P, Resnick T, Harvey A S, Alvarez L, Dean P, Gilman J, Yaylali I, Morrison G, Prats A, Altman N, Birchansky S, Bruce J. Epilepsy surgery in the first three years of life.  Epilepsia. 1998;  39 737-743
    PubMedGoogle Scholar
  • 6 Edwards J C, Wyllie E, Ruggeri P M, Bingaman W, Lüders H, Kotagal P, Dinner D S, Morris H H, Prayson R A, Comair Y G. Seizure outcome after surgery for epilepsy due to malformation of cortical development.  Neurology. 2000;  55 1110-1114
    PubMedGoogle Scholar
  • 7 Engel J, van Ness J, Rasmussen P C, Ojemann T B, Ojemann L M. Outcome with respect to epileptic seizures. Engel J Surgical Treatment of the Epilepsies. 1993: 609-622
    Google Scholar
  • 8 Ho S S, Kuzniecky R I, Gilliam F, Faught E, Morawetz R. Temporal lobe developmental malformations and epilepsy.  Neurology. 1998;  748-754
    PubMedGoogle Scholar
  • 9 Holmes G L. Epilepsy in the developing brain: lessons from the laboratory and clinic.  Epilepsia. 1997;  38 12-30
    PubMedGoogle Scholar
  • 10 Holthausen H, Teixeira V A, Tuxhorn I, Pieper T, Ebner A, Kerdar M, Schulz R, Pannek H, Lahl R. Epilepsy surgery in children and adolescents with focal cortical dysplasia. Tuxhorn I, Holthausen H, Boenigk H Pediatric Epilepsy Syndromes and their Surgical Treatment. London; Libbey Company 1997: 199-215
    Google Scholar
  • 11 Hong S C, Kang K S, Seo D W, Hong S B, Lee M, Nam D H, Lee J I, Kim J S, Shin H J, Park K, Eoh W, Suh Y L, Kim J H. Surgical treatment of intractable epilepsy accompanying cortical dysplasia.  J Neurosurg. 2000;  93 766-773
    PubMedGoogle Scholar
  • 12 Keene D L, Jimenez C C, Ventureyra E. Cortical microdysplasia and surgical outcome in refractory epilepsy of childhood.  Pediatr Neurosurg. 1998;  29 69-72
    CrossrefPubMedGoogle Scholar
  • 13 Kuzniecky R I, Barkovich A J. Pathogenesis and pathology of focal malformations of cortical development and epilepsy.  J Clin Neurophysiol. 1996;  13 468-480
    PubMedGoogle Scholar
  • 14 Kuzniecky R, Garcia J H, Faught E, Morewetz R B. Cortical dysplasias in temporal lobe epilepsy: magnetic imaging correlation.  Ann Neurol. 1991;  29 293-298
    CrossrefPubMedGoogle Scholar
  • 15 Lee B CP, Schmidt R E, Hatfield G A, Bourgeois B, Park T S. MRI of focal cortical dysplasia.  Neuroradiology. 1998;  40 675-683
    CrossrefPubMedGoogle Scholar
  • 16 Lüders H, Murphy D, Awad I. et al . Quantitative analysis of seizure frequency 1 week and 6, 12 and 24 months after surgery for epilepsy.  Epilepsia. 1994;  35 1174-1178
    PubMedGoogle Scholar
  • 17 Mahira T, Matsuda K, Tottori T. et al . Focal cortical dysplasia and epilepsy surgery.  No To Hattatsu. 1997;  29 134-144
    PubMedGoogle Scholar
  • 18 Mischel P S, Nguyen L P, Vinters H V. Cerebral cortical dysplasia associated with pediatric epilepsy. Review of neuropathological features and proposal for a grading system.  J Neuropathol Exp Neurol. 1995;  54 137-153
    CrossrefPubMedGoogle Scholar
  • 19 Mukahira K, Oguni H, Awaya Y, Tanaka T, Saito K, Shimizu H, Oda M, Arai N, Suzuki I, Osawa M. Study on surgical treatment in intractable childhood epilepsy.  Brain Dev. 1998;  20 154-164
    CrossrefPubMedGoogle Scholar
  • 20 Palmini A, Andermann F, Olivier A, Tampieri D, Robitaille Y. Focal neuronal migration disorders and intractable partial epilepsy: results of surgical treatment.  Ann Neurol. 1991;  30 750-757
    CrossrefPubMedGoogle Scholar
  • 21 Palmini A, Costa da Costa J, Andermann F, Dubeau F, Gambardella A, Kim H I, Paglioli-Neto E, Paglioli E, Olivier A, Andermann E, Quesney F, Gloor P. Surgical results in epilepsy patients with localized cortical dysplastic lesions. Tuxhorn I, Holthausen H, Boenigk H Pediatric Epilepsy Syndromes and their Surgical Treatment. London; Libbey Company 1997: 216-224
    Google Scholar
  • 22 Palmini A, Gambardella A, Andermann F. et al . Operative strategies for patients with cortical dysplastic lesions and intractable epilepsy.  Epilepsia. 1994;  35 S57-71
    PubMedGoogle Scholar
  • 23 Pieper T, Tuxhorn I, Holthausen H, Ebner A, Schulz R. Surgical outcome after temporal lobectomy in children with temporal lobe epilepsy.  Epilepsia. 1996;  37 4 , (abstract)
    PubMedGoogle Scholar
  • 24 Raymond A A, Fish D R, Sisidiya S M, Alsanjari N, Stevens J M, Shorvon S D. Abnormalities of gyration, heterotopias, tuberous sclerosis, focal cortical dysplasias, microdysgenesis, dysembryoplastic neuroepithelial tumour and dysgeneis of the archicortex in epilepsy. Clinical, EEG and neuroimaging features in 100 adult patients.  Brain. 1995;  118 629-660
    CrossrefPubMedGoogle Scholar
  • 25 Sisodiya A M, Free S L, Stevens J M, Fish D R, Shorvon S D. Widespread structural changes in patients with cortical dysgenesis and epilepsy.  Brain. 1995;  118 1039-1050
    PubMedGoogle Scholar
  • 26 Sisodiya S M. Surgery for malformations of cortical development causing epilepsy.  Brain. 2000;  123 1075-1091
    CrossrefPubMedGoogle Scholar
  • 27 Taylor D C, Falconer M A, Bruton C J, Corsellis J AN. Focal dysplasia of the cerebral cortex in epilepsy.  J Neurol Neurosurg Psychiatr. 1971;  34 369-387
    PubMedGoogle Scholar
  • 28 Villemure J G. Hemispherectomy: techniques and complications. Wyllie E The Treatment of Epilepsy: Principles and Practice. Philadelphia; Lea and Fibinger 1993: 1116-1119
    Google Scholar
  • 29 Villemure J G, Rasmussen T. Functional hemispherectomy: Methodology.  J Epilepsy. 1990;  3 S177-182
    CrossrefPubMedGoogle Scholar
  • 30 Vining E PG, Freeman J M, Pillas D J. et al . Why would you remove half a brain? The outcome of 58 children after hemispherectomy - The John Hopkins Experience: 1968 - 1996.  Pediatrics. 1997;  100 163-171
    CrossrefPubMedGoogle Scholar
  • 31 Wyllie E. Surgery for catastrophic localization-related epilepsy in infants.  Epilepsia. 1996;  37 S22-25
    PubMedGoogle Scholar
  • 32 Wyllie E, Comair Y, Kotagal P, Bulacio J, Bingaman W, Ruggieri P. Seizure outcome after epilepsy surgery in children and adoloscents.  Ann Neurol. 1998;  44 740-748
    CrossrefPubMedGoogle Scholar

M. B., Ch. B. Ingrid Tuxhorn

Head, Section of Pediatric Epilepsy
Epilepsy Center Bethel
Klinik Mara

Maraweg 21

33617 Bielefeld

Germany

Email: tux@mara.de

      >