Resective surgery in tuberous Sclerosis complex, from Penfield to 2018: A critical review

Ostrowsky‐Coste, Karine; Neal, Andrew; Guénot, Marc; Ryvlin, Philippe; Bouvard, Sandrine; Bourdillon, Pierre; Jung, Julien; Catenoix, Hélène; Montavont, Alexandra; Isnard, Jean; Arzimanoglou, Alexis; Rheims, Sylvain

doi:10.1016/j.neurol.2018.11.002

Cited by 22 publications

(18 citation statements)

References 94 publications

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“…Epilepsy is the most common neurological manifestation of TSC, occurring in 75%‐90% of patients, and almost two‐thirds develop drug‐resistant seizures . Epilepsy surgery can lead to seizure freedom in 50%‐60% of patients, with a subsequent improvement in quality of life and neurocognitive function . However, this management strategy is currently underutilized in TSC …”

Section: Introductionmentioning

confidence: 99%

“…In the common setting of multiple tubers, several seizure types, and discordant noninvasive investigations, intracranial electroencephalography (EEG) is required to precisely identify the epileptogenic zone (EZ) and guide surgical resection. In patients requiring invasive EEG, postresection seizure freedom rates approach 50%‐70% . However, there is a paucity of literature examining invasive EEG in TSC and even less that has analyzed neurophysiological data .…”

Section: Introductionmentioning

confidence: 99%

“…As a result, the optimal invasive EEG approach is still not defined. This leads to a wide variation in intracranial EEG evaluation and operative approach, from focal tuberectomies sparing perituberal cortex after targeted intraoperative electrocorticography (ECoG) to lobar disconnections …”

Section: Introductionmentioning

confidence: 99%

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Epileptogenicity in tuberous sclerosis complex: A stereoelectroencephalographic study

et al. 2019

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Objective:In tuberous sclerosis complex (TSC)-associated drug-resistant epilepsy, the optimal invasive electroencephalographic (EEG) and operative approach remains unclear. We examined the role of stereo-EEG in TSC and used stereo-EEG data to investigate tuber and surrounding cortex epileptogenicity. Methods: We analyzed 18 patients with TSC who underwent stereo-EEG (seven adults). One hundred ten seizures were analyzed with the epileptogenicity index (EI).In 13 patients with adequate tuber sampling, five anatomical regions of interest (ROIs) were defined: dominant tuber (tuber with highest median EI), perituber cortex, secondary tuber (tuber with second highest median EI), nearby cortex (normal-appearing cortex in the same lobe as dominant tuber), and distant cortex (in other lobes). At the seizure level, epileptogenicity of ROIs was examined by comparing the highest EI recorded within each anatomical region. At the patient level, epileptogenic zone (EZ) organization was separated into focal tuber (EZ confined to dominant tuber) and complex (all other patterns). Results: The most epileptogenic ROI was the dominant tuber, with higher EI than perituber cortex, secondary tuber, nearby cortex, and distant cortex (P < .001). A focal tuber EZ organization was identified in seven patients. This group had 80% Engel IA postsurgical outcome and distinct dominant tuber characteristics: continuous interictal discharges (IEDs; 100%), fluid-attenuated inversion recovery (FLAIR) hypointense center (86%), center-to-rim EI gradient, and stimulation-induced seizures (71%). In contrast, six patients had a complex EZ organization, characterized by nearby cortex as the most epileptogenic region and 40% Engel IA outcome.At the intratuber level, the combination of FLAIR hypointense center, continuous IEDs, and stimulation-induced seizures offered 98% specificity for a focal tuber EZ organization. Significance: Tubers with focal EZ organization have a striking similarity to type II focal cortical dysplasia. The presence of distinct EZ organizations has significant implications for EZ hypothesis generation, invasive EEG approach, and resection strategy. |

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Epileptogenicity in tuberous sclerosis complex: A stereoelectroencephalographic study

et al. 2019

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“…8 Six Japanese children, who underwent surgery for symptomatic West syndrome on follow-up, were found to have better control of seizure with modest cognitive function improvement and development of autistic features postoperatively. 43 Ostrowsky-Coste et al 44 published that seizures remission was 30% with medical therapy and higher (50-60%) with surgery. Even in epileptic spasm patients with no structural lesions in magnetic resonance imaging (MRI), surgery might have role in controlling the spams.…”

Section: Epilepsy Surgerymentioning

confidence: 98%

Outcome of West Syndrome: A Critical Review

Adhikari

Gautam

2020

J Pediatr Epilepsy

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West syndrome is a genetically heterogeneous electro-clinical syndrome starting in early infancy. Short-term goal of therapy is spasm control and with standard hormonal or vigabatrin treatment, spasms can be controlled in 60 to 80% of patients in 2 weeks to 3 months period. Hormonal treatment with oral steroid is an alternative therapy to injectable adrenocorticotropin hormone, especially in low resource areas. Vigabatrin is preferred in tuberous sclerosis patients. Long-term aim of treatment is sustained remission of seizures and better neurodevelopmental outcome. About 50 to 70% of children are spasm free for prolonged duration, but epilepsy with multiple seizure types including Lennox–Gastaut syndrome is evident in 20 to 40% of children in long-term follow-up. Though hypsarrhythmia is helpful for the diagnosis, prognostic role of the resolution of electroencephalographic abnormalities is still uncertain. Seizures can be controlled in 40 to 60% of the patients, but only 12 to 40% children have normal neurodevelopmental outcome and a third of children are left with severe disabilities. Children with unknown etiology and normal development at spasm onset have better clinical outcome. Young age at onset, nonstandard therapy have less favorable outcome. Surgery is helpful for spasm control in patients with structural lesions and refractory spasms even in the absence of structural lesions.

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“…In comparison, mTOR inhibitors were prescribed in 7.7% of patients with focal seizures and 5.5% of patients with infantile spasms. Epilepsy surgery planning in TSC faces major challenges, mainly related to the presence of multiple lesions (tubers) [17]. Considering the target of pre-surgical evaluation, it was debated whether, in the epileptogenic network, the "epileptogenic tuber'' is associated with the surrounding altered cortex or not.…”

mentioning

confidence: 99%

Surgery for drug‐resistant tuberous sclerosis complex‐associated epilepsy: who, when, and what

Specchio¹,

Pepi²,

Palma³

et al. 2021

Epileptic Disorders

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Objective. Tuberous sclerosis complex (TSC) is a multisystem genetic disorder associated with refractory early-onset epilepsy. Current evidence supports surgery as the intervention most likely to achieve long-term seizure freedom, but no specific guidelines are available on TSC pre-surgical workup. This critical review assesses which TSC patients are suitable for surgical treatment, when pre-surgical evaluation should start, and what degree of surgical resection is optimal for postsurgical outcome. Methods. We searched for publications from 2000 to 2020 in Pubmed and Embase using the terms "tuberous sclerosis,'' "epilepsy,'' and "epilepsy surgery''. To evaluate postsurgical seizure outcome, we selected only studies with at least one year of follow-up. Results. Overall, we collected data on 1,026 patients from 34 studies. Age at surgery ranged from one month to 54 years. Mean age at surgery was 8.41 years. Of the diagnostic non-invasive pre-surgical tools, MRI and video-EEG were considered most appropriate. Promising data for epileptogenic tuber detection is provided from invasive SEEG studies. Data on surgery and related outcome were available for 769 patients. Seizure freedom was seen in 64.4% of patients who underwent tuberectomy, 68.9% treated with lobectomy and 65.1% with multilobar resection. The most effective surgical approach was lobectomy, even though more recently tuberectomy associated with the resection of the perituberal area seems to be the best approach to reach seizure freedom. Published postsurgical seizure freedom rates in patients with TSC were between 65% and 75%, but reduced to 48%-57% over longer follow-up periods. Early surgery might positively affect neurodevelopmental trajectory in some patients, even though data on cognitive outcome are still to be confirmed with longitudinal studies. Significance. Considering the strong correlation between epilepsy duration and neurocognitive outcome, all patients with TSC ought to be referred early to a dedicated epilepsy centre for individually tailored pre-surgical evaluation by a multidisciplinary epilepsy surgery team.

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Resective surgery in tuberous Sclerosis complex, from Penfield to 2018: A critical review

Cited by 22 publications

References 94 publications

Epileptogenicity in tuberous sclerosis complex: A stereoelectroencephalographic study

Epileptogenicity in tuberous sclerosis complex: A stereoelectroencephalographic study

Outcome of West Syndrome: A Critical Review

Surgery for drug‐resistant tuberous sclerosis complex‐associated epilepsy: who, when, and what

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