Noninvasive testing, early surgery, and seizure freedom in tuberous sclerosis complex

Wu, Joyce; Salamon, Noriko; Kirsch, Heidi E.; Mantle, Mary; Nagarajan, S.; Kurelowech, Lacey; Aung, Moe H.; Sankar, Raman; Shields, W. Donald; Mathern, Gary W.

doi:10.1212/wnl.0b013e3181ce5d9e

Cited by 113 publications

(82 citation statements)

References 15 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…We observed in the present study that preoperative epilepsy duration of 7 years or less was a significant predictor of postoperative seizure freedom. Other investigators have described a similar relationship between epilepsy duration and seizure outcomes, 41,58 sug gesting that earlier operative therapy may improve the likelihood of Engel Class I outcome. Furthermore, sev eral studies have shown that pediatric patients who at tain seizure control postoperatively may also benefit from improved intellectual and cognitive ability, 42,53 better memory function, 40 decreased medication use and side effects, 32,46 fewer behavioral problems, 45 and overall im proved quality of life 38,40 compared with patients with continued seizures.…”

Section: Discussionmentioning

confidence: 76%

Seizure outcomes after resective surgery for extra–temporal lobe epilepsy in pediatric patients

Englot

Breshears

Sun

et al. 2013

PED

140

View full text Add to dashboard Cite

While temporal lobe epilepsy (TLE) is the most common epilepsy syndrome in adults, seizures in children are more often extratemporal in origin. Extra–temporal lobe epilepsy (ETLE) in pediatric patients is often medically refractory, leading to significantly diminished quality of life. Seizure outcomes after resective surgery for pediatric ETLE vary tremendously in the literature, given diverse patient and epilepsy characteristics and small sample sizes. The authors performed a systematic review and meta-analysis of studies including 10 or more pediatric patients (age ≤ 19 years) published over the last 20 years examining seizure outcomes after resective surgery for ETLE, excluding hemispherectomy. Thirty-six studies were examined. These 36 studies included 1259 pediatric patients who underwent resective surgery for ETLE. Seizure freedom (Engel Class I outcome) was achieved in 704 (56%) of these 1259 patients postoperatively, and 555 patients (44%) continued to have seizures (Engel Class II–IV outcome). Shorter epilepsy duration (≤ 7 years, the median value in this study) was more predictive of seizure freedom than longer (> 7 years) seizure history (odds ratio [OR] 1.52, 95% confidence interval [CI] 1.07–2.14), suggesting that earlier intervention may be beneficial. Also, lesional epilepsy was associated with better seizure outcomes than nonlesional epilepsy (OR 1.34, 95% CI 1.19–1.49). Other predictors of seizure freedom included an absence of generalized seizures (OR 1.61, 95% CI 1.18–2.35) and localizing ictal electroencephalographic findings (OR 1.55, 95% CI 1.24–1.93). In conclusion, seizure outcomes after resective surgery for pediatric ETLE are less favorable than those associated with temporal lobectomy, but seizure freedom may be more common with earlier intervention and lesional epilepsy etiology. Children with continued debilitating seizures despite failure of multiple medication trials should be referred to a comprehensive pediatric epilepsy center for further medical and surgical evaluation.

show abstract

Section: Discussionmentioning

confidence: 76%

Seizure outcomes after resective surgery for extra–temporal lobe epilepsy in pediatric patients

Englot

Breshears

Sun

et al. 2013

PED

140

View full text Add to dashboard Cite

show abstract

“…However, the ability to identify epileptogenic tubers was increased with the FDG-PET/MRI-coregistered images correlating with the epileptogenic tuber removed at the time of surgery in 88% of patients. Similarly in a report by Wu and colleagues, 37 a subset of patients who were not initially considered surgical candidates were able to undergo focal resection following localization with FDG-PET/MRI coregistration.…”

Section: Novel Methods For Preoperative Evaluation In Tscmentioning

confidence: 77%

“…In 10 patients with hemispheric or generalized ictal EEG onset, magnetic source imaging was localizing in 8 patients. 37 As in other areas of epilepsy research and surgery, MEG is developing an increased role in seizure localization.…”

Section: Novel Methods For Preoperative Evaluation In Tscmentioning

confidence: 99%

“…1 Tonic seizures, moderate to severe mental retardation, and older age at the time of resection have been associated with seizure recurrence following epilepsy surgery. 17,37 There does not appear to be a difference in outcome following resection of temporal compared with extratemporal tubers. 4 Aside from the success of epilepsy surgery in seizure control, there are long-term benefits predicted for developmental and cognitive outcomes.…”

mentioning

confidence: 98%

See 1 more Smart Citation

Epilepsy surgery in tuberous sclerosis: a review

Evans¹,

Morse

Roberts³

2012

FOC

View full text Add to dashboard Cite

Seizures are the initial manifestation of tuberous sclerosis complex (TSC) in 90% of individuals. The prevalence of epilepsy in TSC is 80%–90% with a large proportion refractory to antiepileptic drugs. A review of the literature of epilepsy surgery in TSC demonstrates impressive success rates for seizure-free outcomes. These studies describe a number of novel noninvasive methods for seizure localization including PET, SPECT, and magnetoencephalography. Additionally, there is a subset of patients with TSC with bilateral, multifocal, or generalized epileptiform discharges that would have previously been excluded from resection. New developments in neuroimaging and invasive monitoring with intracranial electrodes are useful methods in identifying an epileptogenic tuber in these individuals with refractory epilepsy. The authors offer a survey of the literature and description of these methods. Additionally they present an illustrative case of ictal SPECT and intracranial electroencephalography used in the preoperative evaluation of a 10-year-old girl with intractable seizures and TSC. This patient ultimately underwent resection of an epileptogenic region within the occipital lobe.

show abstract

“…5 Therefore, aggressive treatment, including epilepsy surgery when applicable, is warranted. [6][7][8][9] However, identifying epileptogenic tubers is challenging because most patients have multiple lesions and multifocal epileptiform abnormalities on scalp EEG. MRI and PET with 2-deoxy-2-( 18 F) fluoro-D-glucose (FDG) can locate the tubers, but cannot identify the epileptogenic tuber(s).…”

mentioning

confidence: 99%

α-[ ¹¹ C]-Methyl- l -tryptophan–PET in 191 patients with tuberous sclerosis complex

Chugani

Luat²,

Kumar³

et al. 2013

Neurology

View full text Add to dashboard Cite

a-[ 11 C]-Methyl-L-tryptophan-PET in 191 patients with tuberous sclerosis complex ABSTRACTObjectives: This was an observational study done on a large cohort of patients with tuberous sclerosis complex (TSC) to determine whether i) the presence of a-[ 11 C]-methyl-L-tryptophan (AMT) hotspots is related to the duration of seizure intractability, ii) the presence of AMT hotspots is related to specific TSC gene mutations, and iii) there is concordance between areas with an AMT hotspot and seizure lateralization/localization on scalp EEG.Methods: One hundred ninety-one patients (mean age: 6.7 years; median: 5 years; range: 3 months to 37 years) with TSC and intractable epilepsy were included. All patients underwent AMT-PET scan. AMT uptake in each tuber and normal-appearing cortex was measured and correlated with clinical, scalp EEG, and, if available, electrocorticographic data. Results:The longer the duration of seizure intractability, the greater the number of AMT hotspots (r 5 0.2; p 5 0.03). AMT hotspots were seen in both TSC1 and TSC2. There was excellent agreement in seizure focus lateralization between ictal scalp EEG and AMT-PET (Cohen k 0.94) in 68 of 95 patients in whom both ictal video-EEG and AMT-PET showed lateralizing findings; in 28 of 68 patients (41%), AMT was more localizing. Furthermore, AMT-PET was localizing in 10 of 17 patients (58%) with nonlateralized ictal EEG. Tuberous sclerosis complex (TSC) is a neurocutaneous disorder characterized by multiple cortical tubers, caused by TSC1 gene (hamartin; chromosome 9q34) or TSC2 gene (tuberin; chromosome 16p13.3) mutations. Neurobehavioral problems and tuber numbers are more frequent among patients with the TSC2 than the TSC1 mutation. [1][2][3] Epilepsy is present in most cases, and when medically refractory (60%), 4 cognitive outcome is poor. 5 Therefore, aggressive treatment, including epilepsy surgery when applicable, is warranted. 6-9 However, identifying epileptogenic tubers is challenging because most patients have multiple lesions and multifocal epileptiform abnormalities on scalp EEG. MRI and PET with 2-deoxy-2-( 18 F) fluoro-D-glucose (FDG) can locate the tubers, but cannot identify the epileptogenic tuber(s). Occasionally, ictal SPECT may show the seizure focus, 10 but seizures in children with TSC are often brief and not ideal for ictal SPECT studies. Interictal magnetoencephalography has also been used. 11 We previously reported, in a relatively small number of patients with TSC, that a-[ 11 C]-methyl-Ltryptophan (AMT)-PET scanning may differentiate between epileptogenic tubers (high AMT uptake) and nonepileptogenic tubers (low AMT uptake) and that the location of high AMT uptake corresponds to the seizure focus on EEG. 6,12,13

show abstract

Noninvasive testing, early surgery, and seizure freedom in tuberous sclerosis complex

Abstract: Background:The unambiguous identification of the epileptogenic tubers in individuals with tuber-

Cited by 113 publications

References 15 publications

Seizure outcomes after resective surgery for extra–temporal lobe epilepsy in pediatric patients

Seizure outcomes after resective surgery for extra–temporal lobe epilepsy in pediatric patients

Epilepsy surgery in tuberous sclerosis: a review

α-[ ¹¹ C]-Methyl- l -tryptophan–PET in 191 patients with tuberous sclerosis complex

Contact Info

Product

Resources

About

Noninvasive testing, early surgery, and seizure freedom in tuberous sclerosis complex

Abstract: Background:The unambiguous identification of the epileptogenic tubers in individuals with tuber-

Cited by 113 publications

References 15 publications

Seizure outcomes after resective surgery for extra–temporal lobe epilepsy in pediatric patients

Seizure outcomes after resective surgery for extra–temporal lobe epilepsy in pediatric patients

Epilepsy surgery in tuberous sclerosis: a review

α-[ 11 C]-Methyl- l -tryptophan–PET in 191 patients with tuberous sclerosis complex

Contact Info

Product

Resources

About

α-[ ¹¹ C]-Methyl- l -tryptophan–PET in 191 patients with tuberous sclerosis complex