Successful surgery in late onset epilepsy with tuberous sclerosis complex.
[Case records of Epileptic Disorders. Anatomo-electro clinical correlations. Case 01-2009]. Tuberous sclerosis complex (TSC) is a multisystem genetic disorder with variable phenotypic expression, caused by mutations in one of the two tumor suppressor genes, TSC1 or TSC2. Epilepsy is the most common neurological presentation and seizures are often medically intractable. Definition of the epileptogenic zone during presurgical evaluation is challenging given the multiple potentially epileptogenic lesions visible on MRI. However, TSC patients may nevertheless achieve seizure freedom, when preoperative evaluation yields concordant results. The strategies used in these patients vary substantially among different epilepsy surgery centres. We present a 21-year-old right-handed, intellectually not impaired woman with TSC and medically intractable seizures since the age of 15 years. Careful multi-stage presurgical evaluation, including prolonged video-EEG-monitoring, cerebral high resolution MRI, ictal and interictal [99m Tc]HMPAO-SPECT, [18 F]FDG-PET and further invasive recordings with subdural and depth electrodes led to the identification of an epileptogenic tuber with concordant seizure onset zone in the right neocortical temporal lobe. A tailored resection was performed leading to excellent surgical outcome (follow-up 12 months, Engel class I).
Department of Neurology, Innsbruck Medical University, Innsbruck, Austria.
Iris
I
Unterberger
Giorgi
G
Kuchukhidze
Gerald
G
Walser
Judith
J
Dobesberger
Florian
F
Koppelstaetter
Martin
M
Ortler
Richard
R
Bauer
Eugen
E
Trinka
D017668:Age of Onset; D001921:Brain / Q000503:physiopathology / Q000601:surgery; D004569:Electroencephalography; D004827:Epilepsy / Q000209:etiology / Q000503:physiopathology / Q000601:surgery*; D005260:Female; D019788:Fluorodeoxyglucose F18; D006801:Humans; D008279:Magnetic Resonance Imaging; D019635:Neurosurgical Procedures / Q000379:methods; D049268:Positron-Emission Tomography / Q000379:methods; D019275:Radiopharmaceuticals; D019690:Technetium Tc 99m Exametazime; D013702:Temporal Lobe / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology* / Q000601:surgery*; D015899:Tomography, Emission-Computed, Single-Photon / Q000379:methods; D016896:Treatment Outcome; D014402:Tuberous Sclerosis / Q000150:complications* / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000601:surgery*; D055815:Young Adult
D019275:Radiopharmaceuticals; D019788:Fluorodeoxyglucose F18; D019690:Technetium Tc 99m Exametazime