On the Surgical Treatment of Refractory Epilepsy in Tuberous Sclerosis Complex

Baumgartner, James E.; Wheless, James W.; Kulkarni, Sri; Northrup, Hope; Au, Kit Sing; Smith, Andrew C.; Brookshire, Bannie

doi:10.1159/000121276

Cited by 53 publications

(38 citation statements)

References 12 publications

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“…Of nine operated cases reported by Bebin et al, six became seizure-free and two had a significant seizure reduction [5]. Other authors also report good results of surgical treatment in TSC [3,4,6,13]. Seizure outcome was not classified by Engel criteria in any of the mentioned publications but the overall percentage of seizure-free patients after surgery is 50 ± 60 %.…”

Section: Discussionmentioning

confidence: 99%

“…Ictal seizure patterns (SzP) and subclinical SzP were found in only one location in three patients (no. 4,7,8), in one location and generalized in patients 5 and 6, unilateral in patients 2 and 9, in two locations and generalized in patient 3, and bilateral in patient 1. The patterns from invasive recording with subdural grid electrodes were strongly localized to one focus or tuber.…”

Section: Eeg Datamentioning

confidence: 92%

“…Patient 8 only showed regional IED, and four patients (2,4,6,9) showed IED restricted to one hemisphere. Three patients (1,3,5) showed independent bilateral or generalized IED.…”

Section: Eeg Datamentioning

confidence: 95%

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Epilepsy Surgery Outcome in Children with Focal Epilepsy due to Tuberous Sclerosis Complex

et al. 2002

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kinsonism and dementia. Brain MRI show iron in the substantia nigra and globus pallidus, as well as a halo of T1 hyperintense signal in the substantia nigra and cerebral peduncles. Male and female patients were phenotypically similar. Conclusion: We describe the first X-lined form of NBIA, due to mutations in WDR45. The phenotypic similarity between the sexes is likely due to somatic mosaicism in surviving males. WDR45 encodes a beta-propeller protein postulated to play a role in autophagy, a novel disease mechanism in NBIA. The newly termed BPAN (beta-propellar associated neurodegenera-tion) appears to be a significant cause of NBIA. Objectives: The hypothesis of epileptic encephalopathy posits that epileptiform discharge promotes changes in the connection between neurons in early childhood due to ongoing developmental structural plasticity. The presence of remodelled cortical architecture is evaluated in infants following presentation with 'idiopathic' WS. Methods: Cross-sectional study. Quantitative magnetic resonance imaging (3D structural MRI and diffusion tensor imaging) data was compared for 22 patients with newly diagnosed WS and 12 healthy age controls using voxel based morphometry (VBM) and tract-based spatial statistics (TBSS). Results: WS patients showed decreased white matter volume of the temporal lobes compared to controls (p<0.005), but did not show difference in white matter integrity on DTI metrics (frac-tional anisotropy and mean diffusivity). WS patients showed increased temporal lobe grey matter density (p<0.005), particularly in the perirhinal cortex. The structural changes occurred bilaterally. Conclusions: Following idiopathic West Syndrome a number of persistent neurological deficits referable to the temporal lobe are observed, including impaired receptive language, and combined visual-and-auditory agnosia. These are likely to reflect an altered connection pattern within the temporal lobe that is demonstrated here. The changes were found despite successful resolution of infantile spasm seizures on treatment, highlighting a need for treatment options that prevent the alteration of connection architecture in WS. Objective: To review the clinical features determining the offer of epilepsy surgery and surgical outcome in children with Tuberous Sclerosis Complex (TSC). Methods: Clinical data of 122 patients with TSC and complex epilepsy, referred to Great Ormond Street Hospital for epilepsy surgery from 2000 to 2012, were reviewed retrospectively. Pre-surgical evaluation included ictal video electroencephalogram (EEG) monitoring and brain Magnetic Resonance Imaging (MRI) in all, with selected patients undergoing ictal/interictal Single Photon Emission Computed Tomography (SPECT) and/or intracranial EEG. Patients undergoing surgery were followed at 6 months, 1 and 2 years post-operatively; seizure outcome was measured using Modified Engel classification. Results: Of the 122 patients screened, 53 were discussed at the Epilepsy Surgery Meeting based on the presence of continuing seizures, latera...

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Section: Discussionmentioning

confidence: 99%

Section: Eeg Datamentioning

confidence: 92%

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Epilepsy Surgery Outcome in Children with Focal Epilepsy due to Tuberous Sclerosis Complex

et al. 2002

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“…Up to one third of children affected with TSC develop infantile spasms; vigabatrin is the preferred treatment in these patients [44]. Several studies have shown excellent results once epileptogenic tubers were removed from individuals with TSC complicated by drug-resistant seizures [45,46]. …”

Section: Syndromic Causesmentioning

confidence: 99%

Clinical review of genetic epileptic encephalopathies

Noh

Asher

Graham

2012

European Journal of Medical Genetics

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Seizures are a frequently encountered finding in patients seen for clinical genetics evaluations. The differential diagnosis for the cause of seizures is quite diverse and complex, and more than half of all epilepsies have been attributed to a genetic cause. Given the complexity of such evaluations, we highlight the more common causes of genetic epileptic encephalopathies and emphasize the usefulness of recent technological advances. The purpose of this review is to serve as a practical guide for clinical geneticists in the evaluation and counseling of patients with genetic epileptic encephalopathies. Common syndromes will be discussed, in addition to specific seizure phenotypes, many of which are refractory to anti-epileptic agents. Divided by etiology, we overview the more common causes of infantile epileptic encephalopathies, channelopathies, syndromic, metabolic, and chromosomal entities. For each condition, we will outline the diagnostic evaluation and discuss effective treatment strategies that should be considered.

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“…The assessment of whether one leading seizure source is responsible for all or most of the patient's seizures therefore is of critical importance during presurgical evaluation and usually requires invasive recordings. In recent studies, MEG was useful for better defining the leading epileptogenic focus in patients with tuberous sclerosis [104,105].…”

Section: Tuberous Sclerosismentioning

confidence: 99%

Magnetoencephalography in presurgical epilepsy evaluation

et al. 2002

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The introduction of whole-head magnetoencephalography (MEG) systems facilitating simultaneous recording from the entire brain surface has led to a major breakthrough of MEG in presurgical epilepsy evaluation. Localizations of the interictal spike zone with MEG showed excellent agreement with invasive electrical recordings, were useful to clarify the spatial relationship of the irritative spike zone to structural lesions, and could attribute epileptic activity to lobar subcompartments both in temporal lobe and extratemporal epilepsy. MEG was especially useful for the study of patients with non-lesional neocortical epilepsy and of patients with large lesions, where it provided unique information on the epileptogenic zone. It could reliably localize sensorimotor cortex prior to surgical procedures adjacent to central fissure. MEG language mapping yielded concordant results with the Wada test and cortical stimulation studies. MEG localizations of epileptic activity and essential brain regions were successfully integrated into frameless stereotaxy systems providing accurate functional information intraoperatively. Because MEG and EEG yield both complementary and confirmatory information, combined MEG-EEG recordings in conjunction with advanced source modeling techniques will further improve the noninvasive evaluation of epilepsy patients and constantly reduce the need for invasive procedures.

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On the Surgical Treatment of Refractory Epilepsy in Tuberous Sclerosis Complex

Cited by 53 publications

References 12 publications

Epilepsy Surgery Outcome in Children with Focal Epilepsy due to Tuberous Sclerosis Complex

Epilepsy Surgery Outcome in Children with Focal Epilepsy due to Tuberous Sclerosis Complex

Clinical review of genetic epileptic encephalopathies

Magnetoencephalography in presurgical epilepsy evaluation

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