Long-term outcomes of resective epilepsy surgery after invasive presurgical evaluation in children with tuberous sclerosis complex and bilateral multiple lesions

Arya, Ravindra; Tenney, Jeffrey R.; Horn, Paul S.; Greiner, Hansel M.; Holland, Katherine D.; Leach, James; Gelfand, Michael J.; Rozhkov, Leonid; Fujiwara, Hisako; Rose, Douglas F.; Franz, David Neal; Mangano, Francesco T.

doi:10.3171/2014.10.peds14107

Cited by 61 publications

(66 citation statements)

References 16 publications

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“…The recent EXIST 3 study showing higher efficacy of adjunctive everolimus therapy in patients with treatment-refractory seizures associated with TSC compared to placebo, is promising in this regard. 30,31 This might be the main limiting factor for epilepsy surgery but should be a reminder to refer patients with pharmacoresistant epilepsy early on to expert epilepsy surgery centers to define the possibility of such therapy that is showing fair results, even in patients with multiple foci. 23 There has been substantial progress in understanding of diagnosis and treatment of epilepsy in patients with TSC during the last decade.…”

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confidence: 99%

“…Epilepsy surgery for patients with TSC needs specific expertise, as there is often more than a single tuber focus and surgery may need invasive monitoring of seizures to determine the resection area. 30,31 This might be the main limiting factor for epilepsy surgery but should be a reminder to refer patients with pharmacoresistant epilepsy early on to expert epilepsy surgery centers to define the possibility of such therapy that is showing fair results, even in patients with multiple foci. 32 The correlation between intellectual ability and seizure control in TSC has been reported.…”

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confidence: 99%

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Epilepsy in tuberous sclerosis complex: Findings from the TOSCA Study

Nabbout

Belousova

Benedik³

et al. 2018

Epilepsia Open

145

167

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Summary Objective To present the baseline data of the international TuberOus SClerosis registry to increase disease Awareness (TOSCA) with emphasis on the characteristics of epilepsies associated with tuberous sclerosis complex (TSC). Methods Retrospective and prospective patients’ data on all aspects of TSC were collected from multiple countries worldwide. Epilepsy variables included seizure type, age at onset, type of treatment, and treatment outcomes and association with genotype, seizures control, and intellectual disability. As for noninterventional registries, the study protocol did not specify any particular clinical instruments, laboratory investigations, or intervention. Evaluations included those required for diagnosis and management following local best practice. Results Epilepsy was reported in 83.6% of patients (1852/2216) at baseline; 38.9% presented with infantile spasms and 67.5% with focal seizures. The mean age at diagnosis of infantile spasms was 0.4 year (median <1 year; range <1‐30 years) and at diagnosis of focal seizures was 2.7 years (median 1 year; range <1‐66 years). A total of 1469 patients (79.3%) were diagnosed with epilepsy <2 years. The rate of infantile spasms was higher in patients with a TSC2 mutation than in patients with a TSC1 mutation (47.3% vs 23%). ɣ‐aminobutyric acid (GABA)ergic drugs were the most common treatment modality for both infantile spasms (78.7%) and focal seizures (65.5%). Infantile spasms and focal seizures were controlled in 76.3% and 58.2% of patients, respectively. Control of seizures was associated with lower rates of intellectual disability in both groups. Significance This registry reports the largest international cohort of patients with TSC. Findings confirmed the typical onset pattern of infantile spasms and other focal seizures in the first 2 years of life, and the high rates of infantile spasms in patients with TSC2 mutation. Our results underscored the occurrence of focal seizures at all ages, including an onset that preceded emergence of infantile spasms. Seizure control was shown to be associated with lower rates of intellectual disability but did not preclude the presence of intellectual disability.

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confidence: 99%

mentioning

confidence: 99%

Epilepsy in tuberous sclerosis complex: Findings from the TOSCA Study

Nabbout

Belousova

Benedik³

et al. 2018

Epilepsia Open

145

167

View full text Add to dashboard Cite

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“…After epilepsy surgery, authors have reported rates of seizure freedom (Engel Class I outcome) between 48 and 84% depending on the follow-up interval (Arya et al, 2015;Fallah et al, 2015;Liang et al, 2017;Weiner, 2006;Zhang et al, 2013). An extensive presurgical evaluation is carried out and each patient is discussed at a multidisciplinary conference to determine surgical candidacy.…”

Section: Treatment Strategiesmentioning

confidence: 99%

“…An extensive presurgical evaluation is carried out and each patient is discussed at a multidisciplinary conference to determine surgical candidacy. After epilepsy surgery, authors have reported rates of seizure freedom (Engel Class I outcome) between 48 and 84% depending on the follow-up interval (Arya et al, 2015;Fallah et al, 2015;Liang et al, 2017;Weiner, 2006;Zhang et al, 2013). Positive predictors of seizure freedom after surgery include younger age at seizure onset, greater extent of resection, focal EEG abnormalities, EEG and MRI concordance, and the absence of generalized seizures or significant developmental delay (Fallah et al, 2013;Fallah et al, 2015;Zhang et al, 2013).…”

Section: Treatment Strategiesmentioning

confidence: 99%

Central nervous system manifestations of tuberous sclerosis complex

Karas

Krueger

et al. 2018

American J of Med Genetics Pt C

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Tuberous sclerosis complex (TSC) is a neurocutaneous autosomal-dominant genetic syndrome marked by development of hamartomatous lesions arising from dysfunction of the mammalian target of rapamycin (mTOR) pathway. Although TSC remains a heterogeneous clinical entity, the recent inclusion of genetic diagnostic criteria reflects advancement in our understanding of its underlying etiopathogenesis. Abnormal cellular growth, differentiation, and migration result in multisystem sequelae, with neurologic manifestations of TSC representing the primary cause of morbidity and mortality for the majority of individuals. Modern imaging techniques aid in the diagnosis of TSC and guide treatment strategies by revealing central nervous system findings. Cortical tubers are the namesake lesion of the disorder and occur in up to 90% of cases, often exerting significant epileptogenic potential. Subependymal nodules are found in 80% of patients as calcified tumors lining the ependyma of the lateral ventricles. In some cases, these nodules are thought to progress to subependymal giant cell astrocytomas and may present with obstructive hydrocephalus. Retinal astrocytic hamartomas are also common, present in 50% of patients. Surgery remains the treatment of choice for large or symptomatic lesions, though clinical trials have highlighted a potential role for mTOR pathway antagonism. A multidisciplinary approach is necessary for achieving optimal patient outcomes.

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“…Previous studies in TSC showed that an aggressive, often multilobar resection can render children with a large or multifocal epileptogenic zone seizure-free in a substantial number of cases. 23,24 In children with a larger epileptogenic zoneoften with multilesional or nonlesional MRI-interictal spike populations may represent irritative zones that are distinct from or only barely overlapping with the SOZ. This is the principle behind the decision to perform IEM even when there are frequent ECoG abnormalities.…”

Section: Clinical Implications Of Ecog Abnormalitiesmentioning

confidence: 99%

Preresection intraoperative electrocorticography (ECoG) abnormalities predict seizure‐onset zone and outcome in pediatric epilepsy surgery

et al. 2016

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SUMMARYObjective: The predictive value of intraoperative electrocorticography (ECoG) in pediatric epilepsy surgery is unknown. In a population of children undergoing ECoG followed typically by invasive extraoperative monitoring (IEM) and resection, we aimed to determine the relationship between frequent ECoG abnormalities and the seizure onset zone and outcome after resection. Methods: We retrospectively identified 103 children with preresection ECoG of sufficient technical quality. ECoG records were scored based on electrode location and frequency, blinded to the seizure-onset zone and outcome. Electrographic seizure and spike locations were identified. Locations of seizures and spike populations were then compared to the location of seizure-onset zone defined by IEM using subdural electrodes and resection margin. Results: Electrographic seizures were identified in 11 (11%) of 103 patients. A spike population of one or more was noted in 79 (77%) of 103 patients. In 50 (63%) of 79 patients, spike populations correlated with seizure-onset zone location. The overall surgical outcome was good (ILAE 1 to 3) in 53 (52%) of 101 patients. Outcome was good in seven (78%) of nine patients when electrographic seizure location was resected. The best outcomes were obtained with resection of both the seizure-onset zone and ECoG abnormalities to include seizures and spike locations (22/33 good outcome, 67%, p = 0.008). There was a significantly better outcome in children with complete resection of ECoG-identified spike populations (14/26, 62% good outcome) compared to when none were resected (4/14, 29%, p = 0.043). Significance: Electrographic seizures and frequent spikes are frequently seen on preresection ECoG in children. The brain locations corresponding to these discharges are highly concordant with the seizure-onset zone; resection of these regions is correlated with good seizure outcome. Further research is needed to design interventions that increase the reliability of ECoG prediction of the epileptogenic zone and obviate the need for IEM.

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Long-term outcomes of resective epilepsy surgery after invasive presurgical evaluation in children with tuberous sclerosis complex and bilateral multiple lesions

Cited by 61 publications

References 16 publications

Epilepsy in tuberous sclerosis complex: Findings from the TOSCA Study

Epilepsy in tuberous sclerosis complex: Findings from the TOSCA Study

Central nervous system manifestations of tuberous sclerosis complex

Preresection intraoperative electrocorticography (ECoG) abnormalities predict seizure‐onset zone and outcome in pediatric epilepsy surgery

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