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
- 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
- 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
- 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
- 5
Bebin E M, Kelly P J, Gomez M R.
Surgical treatment for epilepsy in cerebral tuberous sclerosis.
Epilepsia.
1993;
34
651-657
- 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
- 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
- 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
- 9
Crino P B, Henske E P.
New developments in the neurobiology of the tuberous sclerosis complex.
Neurology.
1999;
53
1384-1390
- 10
Curatolo P.
Neurological manifestations of tuberous sclerosis complex.
Childs Nerv Syst.
1996;
12
515-521
- 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
- 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
- 13
Erba G, Duchowny M.
Partial epilepsy and tuberous sclerosis: Indications for surgery in disseminated disease.
J Epilepsy.
1990;
3 (Suppl)
315-319
- 14
Evans J C, Curtis J.
The radiological appearances of tuberous sclerosis.
Br J Radiol.
2000;
73
91-98
- 15
Franz D N.
Diagnosis and management of tuberous sclerosis complex.
Semin Pediatr Neurol.
1998;
5
253-268
- 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
- 17 Gomez M R.
Tuberous sclerosis. Gomez MR Tuberous Sclerosis. 2nd ed. New York; Raven 1988: 9-19
- 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
- 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
- 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
- 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
- 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
- 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
- 24
Ohmori I, Ohtsuka Y, Ohno S, Oka E.
Analysis of ictal EEGs of epilepsy associated with tuberous sclerosis.
Epilepsia.
1998;
39
1277-1283
- 25
Perot P, Weir B, Rasmussen T.
Tuberous sclerosis. Surgical therapy for seizures.
Arch Neurol.
1966;
15
498-506
- 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
- 27
Roach E S, Williams D P, Laster D W.
Magnetic resonance imaging in tuberous sclerosis.
Arch Neurol.
1987;
44
301-303
- 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
- 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
- 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
Dr. Ingrid Tuxhorn
Epilepsy-Center Bethel
Klinik Mara I
Maraweg 21
33617 Bielefeld
Germany
Email: tux@mara.de