Disrupting the epileptogenic network with stereoelectroencephalography-guided radiofrequency thermocoagulation.

Stereoelectroencephalography-guided radiofrequency thermocoagulation (SEEG-guided RF-TC) is a treatment option for focal drug-resistant epilepsy. In previous studies, this technique has shown seizure reduction by ≥50% in 50% of patients at 1 year. However, the relationship between the location of the ablation within the epileptogenic network and clinical outcomes remains poorly understood. Seizure outcomes were analyzed for patients who underwent SEEG-guided RF-TC and across subgroups depending on the location of the ablation within the epileptogenic network, defined as SEEG sites involved in seizure generation and spread. Eighteen patients who had SEEG-guided RF-TC were included. SEEG-guided seizure-onset zone ablation (SEEG-guided SOZA) was performed in 12 patients, and SEEG-guided partial seizure-onset zone ablation (SEEG-guided P-SOZA) in 6 patients. The early spread was ablated in three SEEG-guided SOZA patients. Five patients had ablation of a lesion. The seizure freedom rate in the cohort ranged between 22% and 50%, and the responder rate between 67% and 85%. SEEG-guided SOZA demonstrated superior results for both outcomes compared to SEEG-guided P-SOZA at 6 months (seizure freedom p = .294, responder rate p = .014). Adding the early spread ablation to SEEG-guided SOZA did not increase seizure freedom rates but exhibited comparable effectiveness regarding responder rates, indicating a potential network disruption.

Practice Guidelines for Canadian Neurophysiology Laboratories During the COVID-19 Pandemic.

The COVID-19 pandemic has had a major impact on clinical practice. Safe standards of practice are essential to protect health care workers while still allowing them to provide good care. The Canadian Society of Clinical Neurophysiologists, the Canadian Association of Electroneurophysiology Technologists, the Association of Electromyography Technologists of Canada, the Board of Registration of Electromyography Technologists of Canada, and the Canadian Board of Registration of Electroencephalograph Technologists have combined to review current published literature about safe practices for neurophysiology laboratories. Herein, we present the results of our review and provide our expert opinion regarding the safe practice of neurophysiology during the COVID-19 pandemic in Canada.

Involvement of the posterior cingulate gyrus in temporal lobe epilepsy: A study using stereo-EEG.

OBJECTIVE: To analyze the involvement of the posterior cingulate gyrus (PCG) during mesial temporal lobe seizures (MTLS). METHODS: We retrospectively reviewed the stereo-EEG (SEEG) recordings of patients with MTLS performed in our institution from February 2013 to December 2020. Only patients who had electrode implantation in the PCG were included. Patients with lesions that could potentially alter the seizure spread pathways were excluded. We assessed the propagation patterns of MTLS with respect to the different structures sampled. RESULTS: Nine of 97 patients who had at least one seizure originating in the mesial temporal region met the inclusion criteria. A total of 174 seizures were analyzed. The PCG was the first site of propagation in most of the cases (8/9 patients and 77.5% of seizures, and 7/8 patients and 65.6% of seizures after excluding an outlier patient). The fastest propagation times were towards the contralateral mesial temporal region and ipsilateral PCG. Seven patients underwent standard anterior temporal lobectomy and, of these, all but one were Engel 1 at last follow up. CONCLUSION: We found the PCG to be the first propagation site of MTLS in this group of patients. These results outline the relevance of the PCG in SEEG planning strategies. Further investigations are needed to corroborate whether fast propagation to the PCG predicts a good surgical outcome.

Defective Fas expression exacerbates neurotoxicity in a model of Parkinson's disease.

Fas (CD95), a member of the tumor necrosis factor-receptor superfamily, has been studied extensively as a death-inducing receptor in the immune system. However, Fas is also widely expressed in a number of other tissues, including in neurons. Here, we report that defects in the Fas/Fas ligand system unexpectedly render mice highly susceptible to neural degeneration in a model of Parkinson's disease. We found that Fas-deficient lymphoproliferative mice develop a dramatic phenotype resembling clinical Parkinson's disease, characterized by extensive nigrostriatal degeneration accompanied by tremor, hypokinesia, and loss of motor coordination, when treated with the neurotoxin 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) at a dose that causes no neural degeneration or behavioral impairment in WT mice. Mice with generalized lymphoproliferative disease, which express a mutated Fas ligand, display an intermediate phenotype between that of lymphoproliferative and WT mice. Moreover, Fas engagement directly protects neuronal cells from MPTP/1-methyl-4-phenylpyridinium ion toxicity in vitro. Our data show that decreased Fas expression renders dopaminergic neurons highly susceptible to degeneration in response to a Parkinson-causing neurotoxin. These findings constitute the first evidence for a neuroprotective role for Fas in vivo.

Potential Pitfalls in Pre-implantation Genetic Diagnosis in a Patient with Tuberous Sclerosis and Isolated Mosaicism for a TSC2 Variant in Renal Tissue.

Tuberous sclerosis complex (TSC) is an autosomal dominant disorder that displays a wide spectrum of clinical manifestations, often affecting multiple organs including the kidneys, brain, lungs, and skin. A pathogenic mutation in either the TSC1 or TSC2 gene can be detected in almost 85% of the cases, with mosaicism accounting for about half of the remaining cases. We report a case of TSC diagnosed clinically, requesting genetic counselling regarding reproductive risks. No mutation was identified on initial testing of peripheral blood; however, mosaicism for a likely pathogenic frameshift variant in TSC2 was detected at a level of 15% in renal angiomyolipoma tissue. Despite widespread clinical manifestations of TCS, this variant was not detected in skin fibroblasts or saliva, raising the possibility this is an isolated somatic mutation in renal tissue with the underlying germline mutation not yet identified. This case highlights the difficulties when counselling patients with mosaicism regarding their reproductive risks and prenatal diagnostic options.

Critically ill benign EEG variants: Is there such a thing?

Despite growing use of critical care electroencephalography (ccEEG) to detect seizures and status epilepticus in the intensive care unit (ICU), integrating ccEEG findings with traditionally described benign EEG variants (BEVs) is a relatively new concept. BEV-like waveforms are now increasingly encountered in the ICU, and have also been explicitly included in proposed definitions of brief potentially ictal rhythmic discharges (BIRDs) in the ICU, bringing to the fore the question of if and which EEG patterns in critically ill patients can be safely deemed "benign". Though well-characterized as benign in healthy outpatients at low pre-test risk for neurologic disease, the significance of BEVs in the ICU remains largely unknown. Simultaneously, there has been mounting evidence to suggest that certain BEVs can arise from heterogeneous intracranial sources, including some pathologic generators. We conducted an extensive literature review on all known BEVs to assess what is known of BEVs in the ICU. Here we discuss critically ill BEVs and how to interpret them.

Breakthrough spikes in rapid eye movement sleep from the epilepsy monitoring unit are associated with peak seizure frequency.

STUDY OBJECTIVES: Rapid eye movement sleep (REM) usually suppresses interictal epileptiform discharges (IED) and seizures. However, breakthrough IEDs in REM sometimes continue. We aimed to determine if the amount of IED and seizures in REM, or REM duration, is associated with clinical trajectories. METHODS: Continuous electroencephalogram (EEG) recordings from the epilepsy monitoring unit (EMU) were clipped to at least 3 h of concatenated salient findings per day including all identified REM. Concatenated EEG files were analyzed for nightly REM duration and the "REM spike burden" (RSB), defined as the proportion of REM occupied by IED or seizures. Patient charts were reviewed for clinical data, including patient-reported peak seizure frequency. Logistic and linear regressions were performed, as appropriate, to explore associations between two explanatory measures (duration of REM and RSB) and six indicators of seizure activity (clinical trajectory outcomes). RESULTS: The median duration of REM sleep was 43.3 (IQR 20.9-73.2) min per patient per night. 59/63 (93.7%) patients achieved REM during EMU admission. 39/59 (66.1%) patients had breakthrough IEDs or seizures in REM with the median RSB at 0.7% (IQR 0%-8.4%). Every 1% increase in RSB was associated with 1.69 (95% CI = 0.47-2.92) more seizures per month during the peak seizure period of one's epilepsy (p = 0.007). CONCLUSIONS: Increased epileptiform activity during REM is associated with increased peak seizure frequency, suggesting an overall poorer epilepsy trajectory. Our findings suggest that RSB in the EMU is a useful biomarker to help guide about what to expect over the course of one's epilepsy.