Epilepsy surgery in tuberous sclerosis: a review

2012 ◽  
Vol 32 (3) ◽  
pp. E5 ◽  
Author(s):  
Linton T. Evans ◽  
Richard Morse ◽  
David W. Roberts

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.

2019 ◽  
Author(s):  
S Tumpa ◽  
R Thornton ◽  
M Tisdall ◽  
T Baldeweg ◽  
KJ Friston ◽  
...  

AbstractThe presence of interictal epileptiform discharges on electroencephalography (EEG) may indicate increased epileptic seizure risk and on invasive EEG are the signature of the irritative zone. In highly epileptogenic lesions – such as cortical tubers in tuberous sclerosis – these discharges can be recorded with intracranial stereotactic EEG as part of the evaluation for epilepsy surgery. Yet the network mechanisms that underwrite the generation and spread of these discharges remain poorly understood, limiting their current diagnostic use.Here, we investigate the dynamics of interictal epileptiform discharges using a combination of quantitative analysis of invasive EEG recordings and mesoscale neural mass modelling of cortical dynamics. We first characterise spatially organised local dynamics of discharges recorded from 36 separate tubers in 8 patients with tuberous sclerosis. We characterise these dynamics with a set of competing explanatory network models using dynamic causal modelling. Bayesian model comparison of plausible network architectures suggests that the recurrent coupling between neuronal populations within – and adjacent to – the tuber core explains the travelling wave dynamics observed in these patient recordings.Our results – based on interictal activity – unify competing theories about the pathological organisation of epileptic foci and surrounding cortex in patients with tuberous sclerosis. Coupled oscillator dynamics have previously been used to describe ictal activity, where fast travelling ictal discharges are commonly observed within the recruited seizure network. The interictal data analysed here add the insight that this functional architecture is already established in the interictal state. This links observations of interictal EEG abnormalities directly to pathological network coupling in epilepsy, with possible implications for epilepsy surgery approaches in tuberous sclerosis.Significance StatementInterictal epileptiform discharges (IEDs) are clinically important markers of an epileptic brain. Here we link local IED spread to network coupling through a combination of clinical recordings in paediatric patients with tuberous sclerosis complex, quantitative EEG analysis of interictal discharges spread, and Bayesian inference on coupled neural mass model parameters. We show that the kinds of interictal discharges seen in our patients require recurrent local network coupling extending beyond the putative seizure focus and that in fact only those recurrent coupled networks can support seizure-like and interictal dynamics when run in simulation. Our findings provide a novel integrated perspective on emergent epileptic dynamics in human patients.


2006 ◽  
Vol 37 (03) ◽  
Author(s):  
C Krahn-Peper ◽  
IEB Tuxhorn ◽  
K Ahlbory ◽  
F Behne ◽  
H Pannek

2004 ◽  
Vol 19 (9) ◽  
pp. 687-689 ◽  
Author(s):  
Howard L. Weiner ◽  
Nina Ferraris ◽  
Josiane LaJoie ◽  
Daniel Miles ◽  
Orrin Devinsky

QJM ◽  
2021 ◽  
Vol 114 (Supplement_1) ◽  
Author(s):  
Osama Salah Mohamed El Sharkawy ◽  
Zeinab Anwar El kabbany ◽  
Neveen Tawakol Younis ◽  
Khaled Aboulfotouh Ahmad ◽  
Ahmed Darwish Mahmoud ◽  
...  

Abstract Objective To select patients with drug resistant epilepsy following up in Pediatrics Neurology Outpatient Clinic of Children's Hospital, Ain Shams University who are candidates for epilepsy surgery and to detect outcome of epilepsy surgery in such children as regards seizures control. Methods This prospective study was conducted over a period of 36 months and comprises of 3 stages. Stage 1 includes selection of candidates for epilepsy surgery and preoperative evaluation. Evaluation included clinical assessment, video EEG, MRI epilepsy protocol. Stage 2 include surgery phase where decision of surgery was made by a multidisciplinary team. Stage 3 includes post-operative evaluation as regards Seizures frequency, Seizures Severity using Chalfont score, Engel Epilepsy Surgery Outcome Scale and the International League Against Epilepsy (ILAE) outcome classification. Data was tabulated and analyzed with SSPS package for windows. Results 17 patients underwent epilepsy surgery. Results revealed significant decrease in seizures frequency and severity at 6 and 12 months after surgery. As regards Engel Epilepsy Surgery Outcome Scale 11 (64.7%) patients were class I at 12 months. As regards the ILAE outcome classification 10 (58.8%) patients are class 1 at 12 months. Conclusions epilepsy surgery can be a hope for patients with drug resistant epilepsy who are well selected and evaluated preoperatively. New studies on larger number and for longer duration are recommended.


Neurosurgery ◽  
2019 ◽  
Vol 66 (Supplement_1) ◽  
Author(s):  
Hernán F J González ◽  
Srijata Chakravorti ◽  
Sarah E Goodale ◽  
Kanupriya Gupta ◽  
Daniel O Claassen ◽  
...  

Abstract INTRODUCTION The effects of temporal lobe epilepsy (TLE) on subcortical arousal structures remain incompletely understood. Here we evaluate thalamic arousal network functional connectivity in TLE and examine changes after epilepsy surgery. METHODS We examined 26 adult TLE patients and 26 matched control participants and used resting-state functional magnetic resonance imaging (fMRI) to measure functional connectivity between the thalamus (entire thalamus and 19 bilateral thalamic nuclei) and both neocortex and brainstem ascending reticular activating system (ARAS) nuclei. Postoperative imaging was completed for 19 patients > 1 yr after surgery and compared to preoperative baseline. RESULTS Before surgery, TLE patients demonstrated abnormal thalamo-occipital functional connectivity, losing the normal negative fMRI correlation between the intralaminar central lateral (CL) nucleus and medial occipital lobe seen in controls (P < .001, paired t-test). Patients also had abnormal connectivity between ARAS and CL, lower ipsilateral intrathalamic connectivity, and smaller ipsilateral thalamic volume compared to controls (P < .05 for each, paired t-tests). Abnormal brainstem-thalamic connectivity was associated with impaired visuospatial attention (? = −0.50, P = .02, Spearman's rho), while lower intrathalamic connectivity and volume were related to higher frequency of consciousness-sparing seizures (P < .02, Spearman's rho). After epilepsy surgery, patients with improved seizures showed partial recovery of thalamo-occipital and brainstem-thalamic connectivity, with values more closely resembling controls (P < .01 for each, ANOVA). CONCLUSION Overall, TLE patients demonstrate impaired connectivity in thalamic arousal networks that may be involved in visuospatial attention, but these disturbances may partially recover after successful epilepsy surgery. Thalamic arousal network dysfunction may contribute to morbidity in TLE.


2018 ◽  
Vol 160 (2) ◽  
pp. 347-354 ◽  
Author(s):  
Subodh Kumar ◽  
Awadhesh Kumar Mishra ◽  
Ashwani Sethi ◽  
Ajay Mallick ◽  
Nidhi Maggon ◽  
...  

Objective To compare the outcomes of various techniques of endoscopic dacryocystorhinostomy (DCR). Study Design Retrospective case record analysis. Settings Tertiary care referral center. Subject and Methods Retrospective analysis of case records was carried out pertaining to the period from January 1996 to September 2017 with respect to patients who had undergone endoscopic DCR with either the standard technique or one of its modifications. Case notes showing well-documented preoperative evaluation, operative details, postoperative assessment, and minimum 6-month follow-up were considered. The outcomes were measured on the basis of patients’ postoperative symptoms, clinical examination, and sac-syringing results. Results A total of 423 patients were included in the study. Of these, 169 underwent standard endoscopic DCR; 87, endoscopic DCR with stent; 19, endoscopic DCR with mitomycin C; 62, powered DCR; 29, laser-assisted DCR; and 57, balloon DCR. There was no statistically significant difference in success rates, recurrences, or complications of various techniques at 3 or 6 months. Mean operating time was lowest for balloon DCR (mean ± SD, 27.1 ± 3.1 minutes), followed by standard endoscopic DCR (38.2 ± 3.6 minutes; P = .001). Conclusion Standard endoscopic DCR and its more sophisticated modifications were equally effective and safe in managing distal nasolacrimal drainage obstruction. Balloon DCR, followed by standard endoscopic DCR, was significantly faster than other techniques.


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