Bilateral Resective Epilepsy Surgery in a Child with Tuberous Sclerosis: Case Report (original) (raw)
Related papers
Selective epilepsy surgery in tuberous sclerosis
Journal of Paediatrics and Child Health, 1989
A patient with tuberous sclerosis diagnosed at the age of 5 years developed an intractable seizure disorder characterized by complex partial seizures numbering 10-20lday. lnterictal electroencephalograms (EEG) showed a right frontal epileptogenic focus. A computerized tomography scan demonstrated calcification in the right frontal region at two sites, periventricular calcification and multiple low density lesions. Neuropsychological assessment showed a verbal intelligence quotient (IQ) of 69 and a performance IQ of 88. Telemetry and video monitoring recorded 10 seizures during which the EEG showed flattening of ongoing sharp wave activity in the right frontal region. Electrocorticography further identified and localized epileptogenic tissue in the right frontal cortex and surgical removal of involved tissue and the adjacent two tubers was carried out. Twelve months after surgery the patient has had only two brief seizures.
Successful surgery in late onset epilepsy with tuberous sclerosis complex
Epileptic disorders : international epilepsy journal with videotape, 2009
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).
Epilepsy surgery in tuberous sclerosis: The Dutch experience
Seizure, 2007
We report the pre-surgical identifi cation of epileptogenic tubers and post-surgical outcome of 25 patients with tuberous sclerosis in The Netherlands. The pre-surgical evaluation of the Dutch Comprehensive Epilepsy Surgery Programme comprised a detailed seizure history, interictal and ictal video EEG registrations, 3D FLAIR MRI scans, neuropsychological testing and if possible magneto-encephalography.
Epilepsy surgery in tuberous sclerosis complex: early predictive elements and outcome
Childs Nervous System, 2008
Aim The aim of the study was to evaluate the surgical treatment of epilepsy and detection of possible early surgery predictive elements in patients with tuberous sclerosis complex (TSC). Materials and methods Forty-two TSC patients with epilepsy were selected and divided into two main groups: definite and fruste forms. Definite forms were divided into different groups: patients with pharmacologically controlled epilepsy, patients with pharmacoresistant epilepsy excluded from surgery after an extensive presurgical assessment, and patients with a pharmacoresistant epilepsy who underwent surgery. We compared the definite TSC groups to identify elements that predict surgical candidacy. Second, we compared all operated patients to assess surgical outcome. Conclusion We found several factors that could predict a surgical intervention even if identification of patients with refractory epilepsy who can benefit from surgery is an evolving process. Also, several positive factors for good surgical outcome were identified. Patients with the fruste form had excellent surgical outcome.
Predictors of seizure-free outcome after epilepsy surgery for pediatric tuberous sclerosis complex
Epilepsia, 2013
Purpose: Variable predictors of postsurgical seizure outcome have been reported in children with tuberous sclerosis complex (TSC). We analyzed a large surgical series of pediatric TSC patients in order to identify prognostic factors crucial for selection of subjects for epilepsy surgery. Methods: Thirty-three children with TSC who underwent excisional epilepsy surgery at Miami Children's Hospital were retrospectively reviewed. A total of 29 clinical, neuropsychological, electroencephalography (EEG), magnetic resonance imaging (MRI), and surgical variables were analyzed and related to seizure outcomes. Univariate Barnard's exact test, Wilcoxon's rank-sum test, and multivariate statistical Cox's model were used to examine the significance of associations between the variables and seizure outcome. Key Findings: Eighteen patients (55%) have been seizure-free 2 years after (final) surgery; postoperative complications occurred in five subjects (15%). Complete removal of epileptogenic tissue detected by both MRI and intracranial EEG, regional scalp interictal EEG patterns, and agreement of interictal and ictal EEG localization were the most powerful predictors of seizure-free outcome. Other significant predictors included occurrence of regional scalp ictal EEG patterns, fewer brain regions affected by tubers, presence of preoperative hemiparesis, and one-stage surgery. Remaining factors such as age at seizure onset, incidence of infantile spasms or other seizure types, duration of epilepsy, seizure frequency, mental retardation, as well as types and extent of resections did not influence outcome. Significance: Perioperative features rather than preoperative variables are the most important determinants of postsurgical seizure outcome in patients with TSC. Our findings may assist in the surgical management of these patients.
Noninvasive testing, early surgery, and seizure freedom in tuberous sclerosis complex
Neurology, 2010
The unambiguous identification of the epileptogenic tubers in individuals with tuberous sclerosis complex (TSC) can be challenging. We assessed whether magnetic source imaging (MSI) and coregistration of 18 fluorodeoxyglucose PET (FDG-PET) with MRI could improve the identification of the epileptogenic regions noninvasively in children with TSC.
Resective surgery in tuberous Sclerosis complex, from Penfield to 2018: A critical review
Revue Neurologique, 2019
Medically treated patients suffering from tuberous sclerosis complex (TSC) have less than 30 % chance of achieving a sustained remission. Both the international TSC consensus conference in 2012, and the panel of European experts in 2012 and 2018 have concluded that surgery should be considered for medically refractory TSC patients. However, surgery remains currently underutilized in TSC. Case series, metaanalyses and guidelines all agree that a 50 to 60 % chance of long term seizure freedom can be achieved after surgery in TSC patients and a presurgical workup should be done as early as possible after failure of two appropriate AEDs. The presence of infantile spasms, the second most common seizure type in TSC, had initially been a barrier to surgical planning but is now no longer considered a contraindication for surgery in TSC patients. TSC patients undergoing presurgical evaluation range from those with few tubers and good anatomo-electro-clinical correlations to patients with a significant "tuber burden" in whom the limits of the epileptogenic zone is much more difficult to define. Direct surgery is often possible in patients with a good electro-clinical and MRI correlation. For more complex cases, invasive monitoring is often mandatory and bilateral investigations can be necessary. Multiple non-invasive tools have been shown to be helpful in determining the placement of these invasive electrodes and in planning the resection scheme. Additionally, at an individual level, multimodality imaging can assist in identifying the epileptogenic zone. Increased availability of investigations that can be performed without sedation in young and/or cognitively impaired children such as MEG and HR EEG would most probably be of great benefit in the TSC population. Of those selected for invasive EEG, rates of seizure freedom following surgery are close to cases where invasive monitoring is not required, strengthening the important and efficient role of intracranial investigations in drug-resistant TSC associated epilepsy.
Frontiers in Neurology, 2018
Tuberous sclerosis complex is a genetic systematic disorder characterized by hamartomas in multiple organs. Cortical tubers, the hamartomas in the cerebrum, cause multifocal refractory seizures. In certain cases, epileptic foci potentially involve language areas, and hence, extra-and intraoperative cortical mapping can help identify anterior and posterior areas, thus avoiding postsurgical language impairment. We report on a 21-year-old female with tuberous sclerosis complex experiencing refractory partial seizures due to two epileptic foci in the left hemisphere overlapping anterior and posterior language areas. To completely evaluate both language areas, we performed stepwise resections beginning from the anterior to the posterior epileptic focus. Although the patient presented with expressive aphasia following anterior resection, it was possible to conduct language tests during every resection. Postoperatively, she presented with expressive aphasia, comprehension deficits, left-right disorientations, and arithmetic deficits. The language dysfunctions almost disappeared at 5 weeks after the surgery and were completely resolved at 6 months after surgery. At postoperative 9 months, she was free from seizures.