The role of genetic factors in determining risk for status epilepticus (SE) was examined in twins identified using the population-based Virginia Twin Registry. Concordance rates for SE were 0.38 for monozygotic (MZ) and 0.00 for dizygotic (DZ) twins, with the rate in MZs being significantly increased over DZs. The prevalence of SE in MZ co-twins of affected individuals was as high as 0.55. Clinical presentation of SE was evaluated, and no association was found between occurrence of SE and age at onset or seizure etiology. Genetic factors contribute to risk for SE.
Serial EEG During Human Status Epilepticus: Evidence for PLED as an Ictal Pattern Garzon E, Fernandes RM, Sakamoto AC Neurology 2001;57:1175–1183 Objective To analyze the relationship between periodic lateralized epileptiform discharges (PLED) and status epilepticus (SE), to evaluate the relationship between mortality and periodic patterns, and to determine whether a stereotypic sequence of EEG patterns exists during human SE. Methods The authors performed a prospective clinical and electrographic study comprising 62 episodes of SE, 55 patients, and 254 ictal/postictal EEG recordings. Serial daily EEG were obtained in all cases. Results Partial SE was the predominant clinical type. Four distinct ictal EEG patterns were identified: intermittent EEG seizures (IES), merging EEG seizures (MES), continuous ictal discharges (CID), and periodic epileptiform discharges (PED) which could be lateralized (PLED) or bilateral (PBED). IES was the most common ictal pattern. In the same record, only one combination of ictal patterns was observed corresponding to an association of PLED or PBED and MES pattern. Serial EEG demonstrated that approximately one-third of SE resolved before the second EEG, another one-third persisted and maintained the same ictal pattern throughout the entire evolution, and the final one-third showed variable ictal EEG patterns. PLED were also unequivocally associated with epileptic seizures, and in some patients were the initial ictal pattern. Conclusion PLED can be an ictal pattern; and, in contrast to previous observations, no stereotyped sequence of ictal EEG patterns was found. PLED/PBED were not a terminal ictal pattern in every case, and outcome was more related to age and etiology than to specific ictal EEG patterns.
Summary Objective Evaluate the seizure‐reduction response and safety of brain‐responsive stimulation in adults with medically intractable partial‐onset seizures of neocortical origin. Methods Patients with partial seizures of neocortical origin were identified from prospective clinical trials of a brain‐responsive neurostimulator (RNS System, NeuroPace). The seizure reduction over years 2–6 postimplantation was calculated by assessing the seizure frequency compared to a preimplantation baseline. Safety was assessed based on reported adverse events. Additional analyses considered safety and seizure reduction according to lobe and functional area (e.g., eloquent cortex) of seizure onset. Results There were 126 patients with seizures of neocortical onset. The average follow‐up was 6.1 implant years. The median percent seizure reduction was 70% in patients with frontal and parietal seizure onsets, 58% in those with temporal neocortical onsets, and 51% in those with multilobar onsets (last observation carried forward [LOCF] analysis). Twenty‐six percent of patients experienced at least one seizure‐free period of 6 months or longer and 14% experienced at least one seizure‐free period of 1 year or longer. Patients with lesions on magnetic resonance imaging (MRI; 77% reduction, LOCF) and those with normal MRI findings (45% reduction, LOCF) benefitted, although the treatment response was more robust in patients with an MRI lesion (p = 0.02, generalized estimating equation [GEE]). There were no differences in the seizure reduction in patients with and without prior epilepsy surgery or vagus nerve stimulation. Stimulation parameters used for treatment did not cause acute or chronic neurologic deficits, even in eloquent cortical areas. The rates of infection (0.017 per patient implant year) and perioperative hemorrhage (0.8%) were not greater than with other neurostimulation devices. Significance Brain‐responsive stimulation represents a safe and effective treatment option for patients with medically intractable epilepsy, including adults with seizures of neocortical onset, and those with onsets from eloquent cortex.
Thursday, April 30April 14, 2020Free AccessThe Risk of Renal Hemorrhage in Adult Patients Burdened by Kidney Manifestations of Tuberous Sclerosis. (4085)Lauren Neal, Mary Silvia, MD, Jane Boggs, MD, Theodore B. Stem, Jr, MD, Daniel B. Rukstalis, MD, Matthew C. Miles, MD, and Roy Strowd, MDAuthors Info & AffiliationsApril 14, 2020 issue94 (15_supplement)https://doi.org/10.1212/WNL.94.15_supplement.4085 Letters to the Editor
Background: Nonconvulsive status epilepticus (NCSE) is a form of status epilepticus (SE) that is an often unrecognized cause of coma. Objective: To evaluate the presence of NCSE in comatose patients with no clinical signs of seizure activity. Methods: A total of 236 patients with coma and no overt clinical seizure activity were monitored with EEG as part of their coma evaluation. This study was conducted during our prospective evaluation of SE, where it has been validated that we identify over 95% of all SE cases at the Medical College of Virginia Hospitals. Only cases that were found to have no clinical signs of SE were included in this study. Results: EEG demonstrated that 8% of these patients met the criteria for the diagnosis of NCSE. The study included an age range from 1 month to 87 years. Conclusions: This large-scale EEG evaluation of comatose patients without clinical signs of seizure activity found that NCSE is an under-recognized cause of coma, occurring in 8% of all comatose patients without signs of seizure activity. EEG should be included in the routine evaluation of comatose patients even if clinical seizure activity is not apparent.
Although generalized convulsive status epilepticus is a life-threatening emergency, the best initial drug treatment is uncertain. We conducted a five-year randomized, double-blind, multicenter trial of four intravenous regimens: diazepam (0.15 mg per kilogram of body weight) followed by phenytoin (18 mg per kilogram), lorazepam (0.1 mg per kilogram), phenobarbital (15 mg per kilogram), and phenytoin (18 mg per kilogram). Patients were classified as having either overt generalized status epilepticus (defined as easily visible generalized convulsions) or subtle status epilepticus (indicated by coma and ictal discharges on the electroencephalogram, with or without subtle convulsive movements such as rhythmic muscle twitches or tonic eye deviation). Treatment was considered successful when all motor and electroencephalographic seizure activity ceased within 20 minutes after the beginning of the drug infusion and there was no return of seizure activity during the next 40 minutes. Analyses were performed with data on only the 518 patients with verified generalized convulsive status epilepticus as well as with data on all 570 patients who were enrolled.
OBJECTIVE:
To evaluate concordance of scalp EEG with intracranial EEG, MRI, PET, SPECT, MEG in post-surgical epilepsy outcomes.
BACKGROUND:
Multiple tests are used in presurgical evaluations. Initial video EEG monitoring and MRI are supplemented by PET, SPECT, MEG, and invasive monitoring. However, not all testing is completed in all patients. Optimized presurgical evaluation based on highly concordant test results should lead to cost effectiveness, as measured in Engel class I/II surgical outcomes.
DESIGN/METHODS:
We conducted a retrospective review of adult and pediatric epilepsy surgical evaluations including scalp +/- invasive EEG, MRI, MEG, PET and SPECT. Study included 24 patients at Wake Forest Baptist Hospital between 2010 and 2013. Surgeries include resections and laser ablation, but excluded corpus callosotomies. We reviewed chart notes for subsequent seizure control. We compared localization by testing with temporal or extratemporal surgical location.
RESULTS:
Of 24 patients, 17 were Engel Class I or II outcome after follow up of 6 -36 months. By scalp EEG, 9/17 had a single focus, most of these with mesial temporal sclerosis. All 17 patients had MEG and 7 patients also had invasive EEG. 15 had PET, and 14 had ictal SPECT. Of the 7 patients with suboptimal surgical outcome all had a MEG, 5 had invasive EEG and 7 had PET, 6 had ictal SPECT. MEG and/or invasive EEG which were concordant with initial scalp EEG more frequently predicted better outcomes than SPECT and PET.
CONCLUSIONS:
While presurgical epilepsy evaluations consist of multiple tests, physician and patient preferences, convenience and even insurance authorization determine which tests can be performed for an individual. Although we found all tests contributed potentially useful information, we conclude that MEG and invasive monitoring yield information more likely to predict good outcome. Further study is needed to define MEG utility, especially in non-lesional compared to lesional epilepsy. Disclosure: Dr. Boles has nothing to disclose. Dr. Boggs has received research support from Visualase. Dr. Valerie has nothing to disclose. Dr. O9Donovan has received research support from UCB Pharma and Visualase.
