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Neuropediatrics 2002; 33(5): 255-261
DOI: 10.1055/s-2002-36740
Original Article

Georg Thieme Verlag Stuttgart · New York

Epilepsy Surgery Outcome in Children with Focal Epilepsy due to Tuberous Sclerosis Complex

M. Karenfort 1 , 2 , B. Kruse 1 , H. Freitag 1 , H. Pannek 1 , I. Tuxhorn 1
  • 1Epilepsy-Center Bethel, Klinik Mara I, Bielefeld, Germany
  • 2Department of Neuropediatrics, University of Göttingen, Göttingen, Germany
Further Information

Publication History

Received: 18 February 2002

Accepted after Revision: 2 August 2002

Publication Date:
21 January 2003 (online)

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Abstract

Tuberous sclerosis complex (TSC) is frequently associated with focal epilepsy due to cerebral tubers. Seizures are the first symptoms in most patients with brain involvement. These epilepsies are frequently severe, drug-resistant and may have a negative impact on the child's global development. Although most epilepsies are multicentric, these patients may be candidates for epilepsy surgery, if it is possible to determine a leading epileptogenic tuber. Nine patients with TSC were examined with long-term video-EEG monitoring, different neuroimaging techniques and neuropsychological tests. A main epileptogenic tuber could be identified in all of our patients. We found good correlations between neuroimaging and EEG. Surgery was performed in eight patients. Seizure outcome was good in all. Two patients became seizure-free, one patient had a single prolonged seizure five days postoperatively, four patients had a significant seizure reduction of more than 75 % and one patient had a seizure reduction of more than 50 % after surgery without additional neurologic deficits. In conclusion, patients with TSC and drug-resistant epilepsy may benefit from epilepsy surgery with reduction in frequency and severity of seizures as well as improved mental and behavioural development leading to a better quality of life. In view of recent developments in functional and metabolic imaging, primary epileptogenic lesions will be more easily detectable in patients with diffuse brain involvement in TSC and surgical treatment may be more specifically applied at an earlier age to a selected subgroup of patients with this disorder.

Key words

Tuberous Sclerosis Complex - Epilepsy Surgery - Developmental Improvement - Main Epileptogenic Tuber - Drug-Resistant Epilepsy

References

  • 1 American Electroencephalographic Society . Guideline thirteen. Guidelines for standard electrode position nomenclature.  J Clin Neurophysiol. 1994;  11 111-113
    PubMedSearch in Google Scholar
  • 2 Asano E, Chugani D C, Muzik O, Shen C, Juhasz C, Janisse J. et al . Multimodality imaging for improved detection of epileptogenic foci in tuberous sclerosis complex.  Neurology. 2000;  54 1976-1984
    PubMedSearch in Google Scholar
  • 3 Avellino A M, Berger M S, Rostomily R C, Shaw C M, Ojemann G A. Surgical management and seizure outcome in patients with tuberous sclerosis.  J Neurosurg. 1997;  87 391-396
    PubMedSearch in Google Scholar
  • 4 Baumgartner J E, Wheless J W, Kulkarni S, Northrup H, Au K S, Smith A. et al . On the surgical treatment of refractory epilepsy in tuberous sclerosis complex.  Pediatr Neurosurg. 1997;  27 311-318
    PubMedSearch in Google Scholar
  • 5 Bebin E M, Kelly P J, Gomez M R. Surgical treatment for epilepsy in cerebral tuberous sclerosis.  Epilepsia. 1993;  34 651-657
    PubMedSearch in Google Scholar
  • 6 Bye A M, Matheson J M, Tobias V H, Mackenzie R A. Selective epilepsy surgery in tuberous sclerosis.  Aust Paediatr J. 1989;  25 243-245
    PubMedSearch in Google Scholar
  • 7 Christophe C, Sekhara T, Rypens F, Ziereisen F, Christiaens F, Dan B. MRI spectrum of cortical malformations in tuberous sclerosis complex.  Brain Dev. 2000;  22 487-493
    CrossrefPubMedSearch in Google Scholar
  • 8 Chugani D C, Chugani H T, Muzik O, Shah J R, Shah A K, Canady A. et al . Imaging epileptogenic tubers in children with tuberous sclerosis complex using alpha-[11 C]methyl-L-tryptophan positron emission tomography.  Ann Neurol. 1998;  44 858-866
    CrossrefPubMedSearch in Google Scholar
  • 9 Crino P B, Henske E P. New developments in the neurobiology of the tuberous sclerosis complex.  Neurology. 1999;  53 1384-1390
    PubMedSearch in Google Scholar
  • 10 Curatolo P. Neurological manifestations of tuberous sclerosis complex.  Childs Nerv Syst. 1996;  12 515-521
    PubMedSearch in Google Scholar
  • 11 Cusmai R, Chiron C, Curatolo P, Dulac O, Tran-Dinh S. Topographic comparative study of magnetic resonance imaging and electroencephalography in 34 children with tuberous sclerosis.  Epilepsia. 1990;  31 747-755
    CrossrefPubMedSearch in Google Scholar
  • 12 Dulac O, Lemaitre A, Plouin P. Maladie de Bourneville: aspects cliniques et électroencéphalographiques de l'épilepsie dans la première année.  Bol Lega Ital Epil. 1984;  45/46 39-42
    PubMedSearch in Google Scholar
  • 13 Erba G, Duchowny M. Partial epilepsy and tuberous sclerosis: Indications for surgery in disseminated disease.  J Epilepsy. 1990;  3 (Suppl) 315-319
    PubMedSearch in Google Scholar
  • 14 Evans J C, Curtis J. The radiological appearances of tuberous sclerosis.  Br J Radiol. 2000;  73 91-98
    PubMedSearch in Google Scholar
  • 15 Franz D N. Diagnosis and management of tuberous sclerosis complex.  Semin Pediatr Neurol. 1998;  5 253-268
    PubMedSearch in Google Scholar
  • 16 Franz D N, Tudor C, Leonard J, Egelhoff J C, Byars A, Valerius K. et al . Lamotrigine therapy of epilepsy in tuberous sclerosis.  Epilepsia. 2001;  42 935-940
    CrossrefPubMedSearch in Google Scholar
  • 17 Gomez M R. Tuberous sclerosis. Gomez MR Tuberous Sclerosis. 2nd ed. New York; Raven 1988: 9-19
    Search in Google Scholar
  • 18 Guerreiro M M, Andermann F, Andermann E, Palmini A, Hwang P, Hoffman H J. et al . Surgical treatment of epilepsy in tuberous sclerosis: strategies and results in 18 patients.  Neurology. 1998;  51 1263-1269
    CrossrefPubMedSearch in Google Scholar
  • 19 Husain A M, Foley C M, Legido A, Chandler D A, Miles D K, Grover W D. Tuberous sclerosis complex and epilepsy: prognostic significance of electroencephalography and magnetic resonance imaging.  J Child Neurol. 2000;  15 81-83
    CrossrefPubMedSearch in Google Scholar
  • 20 Husain A M, Foley C M, Legido A, Chandler D A, Miles D K, Grover W D. West syndrome in tuberous sclerosis complex.  Pediatr Neurol. 2000;  23 233-235
    CrossrefPubMedSearch in Google Scholar
  • 21 Jambaque I, Cusmai R, Curatolo P, Cortesi F, Perrot C, Dulac O. Neuropsychological aspects of tuberous sclerosis in relation to epilepsy and MRI findings.  Dev Med Child Neurol. 1991;  33 698-705
    PubMedSearch in Google Scholar
  • 22 Jozwiak S, Schwartz R A, Janniger C K, Bielicka-Cymerman J. Usefulness of diagnostic criteria of tuberous sclerosis complex in pediatric patients.  J Child Neurol. 2000;  15 652-659
    PubMedSearch in Google Scholar
  • 23 Koh S, Jayakar P, Dunoyer C, Whiting S E, Resnick T J, Alvarez L A. et al . Epilepsy surgery in children with tuberous sclerosis complex: presurgical evaluation and outcome.  Epilepsia. 2000;  41 1206-1213
    CrossrefPubMedSearch in Google Scholar
  • 24 Ohmori I, Ohtsuka Y, Ohno S, Oka E. Analysis of ictal EEGs of epilepsy associated with tuberous sclerosis.  Epilepsia. 1998;  39 1277-1283
    PubMedSearch in Google Scholar
  • 25 Perot P, Weir B, Rasmussen T. Tuberous sclerosis. Surgical therapy for seizures.  Arch Neurol. 1966;  15 498-506
    PubMedSearch in Google Scholar
  • 26 Roach E S, Gomez M R, Northrup H. Tuberous sclerosis complex consensus conference: revised clinical diagnostic criteria.  J Child Neurol. 1998;  13 624-628
    CrossrefPubMedSearch in Google Scholar
  • 27 Roach E S, Williams D P, Laster D W. Magnetic resonance imaging in tuberous sclerosis.  Arch Neurol. 1987;  44 301-303
    PubMedSearch in Google Scholar
  • 28 Romanelli P, Weiner H L, Najjar S, Devinsky O. Bilateral resective epilepsy surgery in a child with tuberous sclerosis: case report. (discussion 735).  Neurosurgery. 2001;  49 732-734
    PubMedSearch in Google Scholar
  • 29 Shepherd C W, Houser O W, Gomez M R. MR findings in tuberous sclerosis complex and correlation with seizure development and mental impairment.  AJNR Am J Neuroradiol. 1995;  16 149-155
    PubMedSearch in Google Scholar
  • 30 Takanashi J, Sugita K, Fujii K, Niimi H. MR evaluation of tuberous sclerosis: increased sensitivity with fluid-attenuated inversion recovery and relation to severity of seizures and mental retardation.  AJNR Am J Neuroradiol. 1995;  16 1923-1928
    PubMedSearch in Google Scholar

Dr. Ingrid Tuxhorn

Epilepsy-Center Bethel
Klinik Mara I

Maraweg 21

33617 Bielefeld

Germany

Email: tux@mara.de