Focal Status Epilepticus Research Articles

Pharmacotherapy for Refractory and Super-Refractory Status Epilepticus in Adults.

Patients with prolonged seizures that do not respond to intravenous benzodiazepines and a second-line anticonvulsant suffer from refractory status epilepticus and those with seizures that do not respond to continuous intravenous anesthetic anticonvulsants suffer from super-refractory status epilepticus. Both conditions are associated with significant morbidity and mortality. A strict pharmacological treatment regimen is urgently required, but the level of evidence for the available drugs is very low. Refractory complex focal status epilepticus generally does not require anesthetics, but all intravenous non-anesthetizing anticonvulsants may be used. Most descriptive data are available for levetiracetam, phenytoin and valproate. Refractory generalized convulsive status epilepticus is a life-threatening emergency, and long-term clinical consequences are eminent. Administration of intravenous anesthetics is mandatory, and drugs acting at the inhibitory gamma-aminobutyric acid (GABA)A receptor such as midazolam, propofol and thiopental/pentobarbital are recommended without preference for one of those. One in five patients with anesthetic treatment does not respond and has super-refractory status epilepticus. With sustained seizure activity, excitatory N-methyl-d-aspartate (NMDA) receptors are increasingly expressed post-synaptically. Ketamine is an antagonist at this receptor and may prove efficient in some patients at later stages. Neurosteroids such as allopregnanolone increase sensitivity at GABAA receptors; a Phase 1/2 trial demonstrated safety and tolerability, but randomized controlled data failed to demonstrate efficacy. Adjunct ketogenic diet may contribute to termination of difficult-to-treat status epilepticus. Randomized controlled trials are needed to increase evidence for treatment of refractory and super-refractory status epilepticus, but there are multiple obstacles for realization. Hitherto, prospective multicenter registries for pharmacological treatment may help to improve our knowledge.

Prognosis of status epilepticus in elderly patients.

To evaluate the clinical features and prognosis of status epilepticus (SE) in patients above 70years old. Retrospective analysis of all patients ≥70 years old with SE registered prospectively during 4years. Follow-up after discharge was performed. Ninety patients were evaluated. Acute symptomatic etiology was the most prevalent. The mean number of antiepileptic drugs (AEDs) used was 2.7±1.2, and 21% of the patients required sedation. A poor outcome was considered when death (31.1%) or developing of new neurological impairment at discharge (32.2%) occurred. After multivariate analysis, four variables predicted a poor outcome: acute symptomatic etiology (OR: 6.320; 95% CI: 1.976-20.217; P=.002), focal motor SE type (OR: 9.089; 95% CI: 2.482-33.283; P=.001), level of consciousness (OR: 4.596; 95% CI: 1.903-11.098; P=.001), and SE duration >12hours (OR: 3.763; 95% CI: 1.130-12.530; P=.031). Independent predictive factors of mortality were SE duration >12hours (OR: 4.306; 95% CI: 1.044-17.757; P=.043), modified Status Epilepticus Severity Score (mSTESS) (OR: 2.216; 95% CI: 1.313-3.740; P=.003), and development of complications (OR: 3.334; 95% CI: 1.004-11.070, P=.049). Considering long-term mortality, age (HR 1.036; 95% CI 1.001-1.071; P=.044), a potentially fatal underlying cause (HR 2.609; 95% CI 1.497- 4.548; P=.001), and mSTESS score >4 (HR 1.485; 95% CI 1.158-1.903; P=.002) remained as predictive factors. There was no association between sedation and the number of AEDs used with outcome at discharge or long-term mortality (P>.05). SE above 70years old has a high morbimortality. Prognosis is not related to treatment aggressiveness.

Postoperative tension pneumocephalus resulting in encephalopathy and seizure activity: Answer

A.CSF leakage Tension pneumocephalus—symptomatic intracranial air, confirmed by head CT—is a rare phenomenon that has been reported in multiple settings (e.g., trauma, infection) [[1]Schirmer C.M. Heilman C.B. Bhardwaj A. Pneumocephalus: case illustrations and review.Neurocrit Care. 2010; 13: 152-158Crossref PubMed Scopus (123) Google Scholar]. Additionally, air accumulation has been linked to post-operative CSF leakage [[2]Banu M.A. Szentirmai O. Mascarenhas L. et al.Pneumocephalus patterns following endonasal endoscopic skull base surgery as predictors of postoperative CSF leaks.J Neurosurg. 2014; 121: 961-975Crossref PubMed Scopus (42) Google Scholar]. A few cases have attributed seizures to pneumocephalus, but corresponding EEG findings have not been reported [[3]Lam K.K. Prasad A. Fehlings M.G. Venkatraghavan L. Pneumocephalus: an unusual case of postoperative seizure after intradural spine surgery.Can J Anaesth. 2014; 61: 969-970Crossref PubMed Scopus (9) Google Scholar]. CSF leakage after supratentorial tumor resection is often due to hydrocephalus. As this patient's preoperative and postoperative imaging did not show gross hydrocephalus, increased intracranial pressure secondary to edema combined with intraoperative involvement of the temporal horn of the lateral ventricle are most likely responsible for CSF leakage, which then permitted one-way entry and accumulation of air (“ball-valve” mechanism), eventually causing symptoms. The most likely explanation for new onset persistent right-sided weakness three days following a clinical seizure is recurrent left hemisphere-onset focal subclinical electrographic seizures. The weakness cannot be explained by Todd’s paresis as this does not correlate with ictal activity on EEG. This patient’s EEG was consistent with focal non-convulsive status epilepticus (NCSE), with electrographic seizures emerging from cerebral regions of complex structural pathology secondary to tumor resection. The patient continued to be in NCSE despite receiving adequate anticonvulsive therapy. Following shunt placement, the patient improved cognitively, and right-sided weakness/sensation and NCSE resolved, suggesting that mass effect of pneumocephalus contributed to patient’s clinical course. This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors. The authors report no disclosures. Postoperative tension pneumocephalus resulting in encephalopathy and seizure activity: QuestionJournal of Clinical NeuroscienceVol. 47PreviewA 46-year-old man underwent subtotal resection of a 2.8 × 2.7-cm left temporoparietal glioblastoma (Fig. 1A-B). Levetiracetam 500 mg BID was started prophylactically for seizures. He was discharged home in good condition. Five days later and following staple removal, he presented with headache, gait ataxia, and confusion. On exam, he was disoriented but showed no motor or sensory deficits. Head CT revealed extensive intracranial air with a 4-mm midline shift (Fig. 1C-D). Two subgaleal angiocatheters were placed to relieve air pressure. Full-Text PDF

Ictal pain in focal non-convulsive status epilepticus

We report an adult with acute unilateral pain as isolated manifestation of acute symptomatic focal non-convulsive status epilepticus. Pain is rarely a manifestation of epileptic seizures. Traditionally, painful seizures have...

Author response: Therapeutic coma for status epilepticus: Differing practices in a prospective multicenter study.

We thank Dr. Sethi for the interest in our article.1 We agree the management of status epilepticus (SE) should be progressive using successive lines of antiseizure drugs (ASD), as recommended by the European and American guidelines.2,3 Therapeutic coma (TC) is sometimes needed; however, since the benefits of TC are not yet clear,4 we strongly believe that a “shotgun” approach should be avoided because it might harm the patient.5 We demonstrated that a more conservative approach was not associated with increased mortality, but avoiding TC correlated with a shorter length of hospital stay and a trend towards a better functional outcome.1 The best approach, as Dr. Sethi suggests, is probably to strictly adhere to the proposed treatment algorithms,3 using correctly dosed benzodiazepines followed by one nonsedating ASD. Eventually, after a careful case-by-case risk–benefit assessment, a decision to start TC may be taken. TC is often designated for patients with refractory generalized convulsive SE (awaiting further studies); nevertheless, in patients with focal SE and partly preserved consciousness, subsequent trials of nonsedating ASD before considering TC appears more appropriate.4

Mirror Image Video Artifact: An Under-Reported Digital Video-EEG Artifact

Synchronous video recording can be helpful in EEG recordings, especially in recognition of seizures and in rejection of artifacts. However, video recordings themselves are also subject to the risk of contamination by artifacts. We report a unique case in which a digital video artifact was identified, occurring during synchronous video-EEG recording, albeit independently of the EEG tracing itself. A synchronous digital video-EEG recording was performed on a 67-year-old male who presented in focal motor status epilepticus. During the initial review of the data, right-sided abnormalities on EEG apparently corresponded with (ipsilateral) right arm motor activity on video, suggesting a nonsensical anatomical localization. However, review of the patient’s chart and discussion with the EEG technologist led to the recognition that the video data recorded a mirror image of the true findings of left arm motor activity. Review of the software settings led to the discovery that the video recording was inverted along the vertical axis, leading to mirror image video artifact. Recognition of this video artifact allowed for accurate interpretation of the study—that right hemispheric EEG abnormalities correlated appropriately with (contralateral) left arm twitching. Effective communication between the EEG reading physician, the treating team, and the EEG technologist is critical for recognition of such artifacts, for proper EEG interpretation, and for appropriate patient management. Mirror image video artifact affirms that bedside evaluation, astute technologists, and attentive EEG reading physicians remain important, even in the presence of video recording.

Lacosamide in status epilepticus: Systematic review of current evidence.

The intravenous formulation of lacosamide (LCM) and its good overall tolerability and safety favor the use in status epilepticus (SE). The aim of this systematic review was to identify and evaluate studies reporting on the use of LCM in SE. We performed a systematic literature search of electronic databases using a combined search strategy from 2008 until October 2016. Using a standardized assessment form, information on the study design, methodologic framework, data sources, efficacy, and adverse events attributed to LCM were extracted from each publication and systematically reported. In total, 522 SE episodes (51.7% female) in 486 adults and 36 children and adolescents were evaluated with an overall LCM efficacy of 57%. Efficacy was comparable between use in nonconvulsive (57%; 82/145) and generalized-convulsive (61%; 30/49; p = 0.68) SE, whereas overall success rate was better in focal motor SE (92%; 34/39, p = 0.013; p < 0.001). The efficacy with later positioning of LCM decreased from 100% to 20%. The main adverse events during treatment of SE are dizziness, abnormal vision, diplopia, and ataxia. Overall, lacosamide is well tolerated and has no clinically relevant drug-drug interactions. The available data regarding the use of LCM in SE are promising, with a success rate of 57%. The strength of LCM is the lack of interaction potential and the option for intravenous use in emergency situations requiring rapid uptitration.

Should Posterior Reversible Encephalopathy Syndrome Be Mainly Considered an Epileptic Disorder? Results of a Sequential Neurophysiological Study in a Pediatric Cohort.

Despite a wide number of studies trying to define clinical, physiopathological, and neuroradiological features of posterior reversible encephalopathy syndrome (PRES), the true nature of symptoms is still not fully understood. We studied a standard cohort of 24 pediatric patients, affected by hemato-oncological diseases, with a neuroradiological diagnosis consistent with PRES identified from 2006 to 2013. Ten of them developed PRES after hematopoietic stem cell transplantation. We analyzed the sequence of clinical, radiological, and electrophysiological data. In all the patients who were recorded at the onset of the first symptoms, electroencephalograms showed focal nonconvulsive seizures or status epilepticus (SE). We found a sensitivity of 100% for electroencephalogram (EEG) with a good correlation between clinical signs and the localization of seizures, whereas computed tomography scans showed a sensitivity of 50% only. Following prompt treatment, intensive care unit admission rate was only 8%. PRES is a multifactorial neurologic event with focal nonconvulsive seizures or SE as the main feature in pediatric patients. Clinical manifestations are epileptic in nature, and prompt EEG recording is useful for diagnosis and supports an earlier treatment, potentially preventing the appearance of complications such as generalized seizures or refractory SE.

Electro-clinical-etiological associations of epilepsia partialis continua in 57 Chinese children

ObjectiveEpilepsia partialis continua (EPC) was one type of focal status epilepticus. The aim of this study was to analyze the clinical and electroencephalography (EEG) characteristics, and outcome of 57 child-onset patients with EPC according to different etiologies, and further explore the electro-clinical-etiological associations. MethodsWe retrospectively reviewed 57 children diagnosed with EPC in our department over last ten years. Etiology, clinical and EEG data, and outcome were categorized and analyzed. ResultsFor the 57 child-onset patients, EPC was caused by different etiologies, including immune-related disease (43.9%), focal lesions (17.5%), inborn errors of metabolism (24.6%), and unknown (14.0%). EEG background abnormalities showed generalized slowing in 45 patients (78.9%) and focal slowing in two patients (3.5%). Nineteen patients (33.3%) presented clear correlation of ictal EEG/EMG and the remaining 38 patients (66.7%) showed no clear correlation of ictal EEG/EMG. Both EEG background activity and ictal EEG/EMG correspondence among different etiologies had statistical significance (P<0.05). The ictal patterns without clear EEG/EMG correspondence in immune-related disease and the ictal patterns with clear EEG/EMG correspondence in focal lesions were more prominent (P<0.05). ConclusionThis is the first study of child-onset EPC with a large series in a pediatric epilepsy center in China. The most common cause for EPC was immune-related disease. The EEG background activity and the EEG/EMG correspondence might be influenced by the etiologies of EPC to some degree. These findings might guide the direction of EPC diagnosis in conjunction with other examinations.

Cerebrospinal Fluid Pleocytosis in Critical Care Patients With Seizures.

To assess the etiology of cerebrospinal fluid (CSF) pleocytosis in critical care patients with seizure(s) or status epilepticus (SE). Many previous studies, some performed decades ago, concluded that CSF pleocytosis may be entirely attributable to seizure activity. We undertook a retrospective chart review of adult patients with an admitting or acquired diagnosis of seizure(s) or SE in critical care units at the Winnipeg Health Sciences Centre between 2009 and 2012. Patients were identified through a critical care information database at a tertiary care center. We limited our study to patients who had lumbar punctures at our center within 5 days of seizure(s) or SE. Of 426 patients with seizures in critical care units, 51 met the inclusion criteria. Seizure subtypes included focal seizures (5 or 10%), generalized seizures (14 or 27%), and SE (32 or 63%). Twelve (seven with SE) of the 51 (24%) were found to have CSF pleocytosis. A probable etiological cause for the CSF pleocytosis was identified in all 12 cases. We conclude that seizures do not directly induce a CSF pleocytosis. Instead, the CSF pleocytosis more likely reflects the underlying acute or chronic brain process responsible for the seizure(s). This was not readily apparent in early studies without magnetic resonance imaging (MRI) of the brain and currently available laboratory investigations. An etiological cause of CSF pleocytosis must always be sought when patients present with seizures and it should never be assumed that seizures are the cause.

Intravenous lacosamide in seizure emergencies: Observations from a hospitalized in-patient adult population

Purposeto evaluate the efficacy and safety of intravenous (IV) lacosamide (LCM) in the treatment of seizure clusters (SC) and status epilepticus (SE) in hospitalized adult patients. Methodswe prospectively analyzed treatment response, seizure outcome, and adverse effects of IV LCM in 38 patients with seizure emergencies (15 with SC, 23 with SE) during a hospital stay. The loading dose of IV LCM was 200–400mg and the maintenance dose was 200–400mg daily. Response to IV LCM was evaluated within 20min, 4h and 24h of LCM infusion. Resultsan acute anti-seizure effect after IV LCM was especially evident when it was first used – (SC) or second line (established SE) treatment. In particular, 87% of SC patients (13/15) and 80% of established SE (8/10) demonstrated response to LCM treatment, while no patients with super-refractory SE (0/8) responded to IV LCM according to our criteria. The loading of IV LCM was well tolerated, with mild adverse effects (2/38 temporary dizziness). In most patients, during and after administration of the loading dose of IV LCM a temporary (30min–1h) sedation was observed. No ECG and laboratory values-changes were documented in any of the patients. ConclusionsLCM is an effective and well-tolerated treatment when used to treat SC in hospitalized adult patients. As add-on therapy, it may be useful to stop seizure activity in patients with focal SE not responding to first/second-line intravenous AEDs.

Epileptic Phenotype of Two Siblings with Asparagine Synthesis Deficiency Mimics Neonatal Pyridoxine-Dependent Epilepsy.

We report the cases of a brother and a sister of nonconsanguineous parents who developed progressive microcephaly and had tremor, irritability, spasticity, startle reflexes, and permanent erratic myoclonus since birth. Focal clonic seizures, status epilepticus, and infantile spasms appeared later, during the first months of life, while erratic myoclonic jerks persisted. Electroencephalogram initially showed multifocal spikes that evolved into modified hypsarrhythmia and then discontinuous activity, evoking the progressive nature of the condition. Magnetic resonance imaging showed brain atrophy and poor myelination. Plasma and cerebrospinal fluid asparagine levels were normal or moderately reduced on repeat testing. Both infants died at the age of 8 months in status epilepticus. Neuropathology of the brother revealed diffuse neuronal loss and astrocytic gliosis predominating in superficial layers of temporal and frontal lobes and in thalamus with almost absent myelin, as a consequence of the neuronal death. Whole exome sequencing of the siblings and parents revealed compound heterozygous c.1439C > T (p.Ser480Phe) and c.1648C > T (p.Arg550Cys) mutations in the ASNS gene, indicating asparagine synthetase (ASNS) deficiency. Electroclinical epileptic phenotype and neuropathological findings of ASNS deficiency are reminiscent of neonatal pyridoxine-dependent epilepsy, thus suggesting common pathophysiological mechanism possibly related to cytotoxic glutamate accumulation.

Focal status epilepticus as a manifestation of idiopathic hypertrophic cranial pachymeningitis

BackgroundIdiopathic hypertrophic cranial pachymeningitis (IHCP) is an uncommon disease of unknown etiology characterized by thickening of the cerebral dura mater with possible associated inflammation. The most frequently described clinical symptoms include headache, cranial nerve palsy, and cerebellar dysfunction. Epilepsy and/or status epilepticus as main presentation is very uncommon. Case presentationTwo consecutive cases are presented of patients manifesting focal status epilepticus secondary to IHCP, with clinical, laboratory [blood test and cerebrospinal fluid (CSF) analysis], neuroradiologic [magnetic resonance imaging (MRI) at 3 Tesla and digital subtraction angiography (DSA)], and therapeutic data. One patient underwent meningeal biopsy; pathology findings are also included. Corticosteroid therapy resulted in clinical improvement in both cases, and neuroimaging showed decreased abnormal morphology, compared to initial findings. ConclusionIn the diagnostic approach to focal status epilepticus or epilepsy, IHCP must be considered a potential, although extremely infrequent, cause. Anti-inflammatory treatment is an effective addition to antiepileptic drug therapy in patients with IHCP.

New ILAE versus previous clinical status epilepticus semiologic classification: Analysis of a hospital-based cohort.

In 2015, the International League Against Epilepsy (ILAE) issued a new status epilepticus (SE) classification, including a detailed semiologic axis. This study assesses frequencies of SE forms in a cohort of adult patients, and explores differences and practical implications as compared to a seizure-type-bound classification. The prospective adult SE registry of the Lausanne University Hospital (CHUV) was considered over 5 years (2011-2015); each SE episode was retrospectively reclassified for its semiology according to the new ILAE scheme. Mortality rates were retrieved for each subgroup of SE. Among 488 SE episodes, according to the seizure-type-bound classification, 230 (47%) had a generalized convulsive, and 29 (6%) had a nonconvulsive SE in coma; both categories overlapped almost perfectly between the two classifications. However, the 84 episodes with focal SE without consciousness impairment and the 141 episodes with consciousness impairment were each translated into two major (and five sub-) categories of the new ILAE classification, having markedly different mortality rates. In addition, of 140 episodes labeled as focal motor SE according to the new classification, 54% had concomitant consciousness impairment, whereas 46% did not; again, mortality rates were heterogeneous. Although generalized convulsive and nonconvulsive SE in coma show an almost perfect correspondence across SE semiologic classifications, focal SE is markedly heterogeneous and appears to be better reflected in the new classification, offering more clinically relevant subdivisions, also differing in mortality rates. This refined knowledge may allow the development of clinical prognostic scores that are more precise than existing tools, and should be taken into account for epidemiologic studies.

Pearls & Oy-sters: Hemicrania epileptica: Unfolding the mystery of an unremitting migraine.

Sheikh et al.1 described a typical migraine persisting for 3 days and resolving after antiepileptic treatment as hemicrania epileptica (HE). EEG recording showed focal status epilepticus and transient brain MRI abnormalities attributed to ongoing seizure. This condition is rare and the proposed criteria for HE bring about the temporal relationship between onset and cessation of migraine with ictal EEG discharges. While migraine and occipital seizures are sometimes difficult to differentiate solely on …

A novel de novo dominant negative mutation in DNM1L impairs mitochondrial fission and presents as childhood epileptic encephalopathy

DNM1L encodes dynamin-related protein 1 (DRP1/DLP1), a key component of the mitochondrial fission machinery that is essential for proper functioning of the mammalian brain. Previously reported probands with de novo missense mutations in DNM1L presented in the first year of life with severe encephalopathy and refractory epilepsy, with several dying within the first several weeks after birth. In contrast, we report identical novel missense mutations in DNM1L in two unrelated probands who experienced normal development for several years before presenting with refractory focal status epilepticus and subsequent rapid neurological decline. We expand the phenotype of DNM1L-related mitochondrial fission defects, reveal common unique clinical characteristics and imaging findings, and compare the cellular impact of this novel mutation to the previously reported A395D lethal variant. We demonstrate that our R403C mutation, which resides in the assembly region of DRP1, acts by a dominant-negative mechanism and reduces oligomerization, mitochondrial fission activity, and mitochondrial recruitment of DRP1, but to a lesser extent compared to the A395D mutation. In contrast to the initial report of neonatal lethality resulting from DNM1L mutation and DRP1 dysfunction, our results show that milder DRP1 impairment is compatible with normal early development and subsequently results in a distinct set of neurological findings. In addition, we identify a common pathogenic mechanism whereby DNM1L mutations impair mitochondrial fission. © 2016 Wiley Periodicals, Inc.

Current Trends in Treatment of Status Epilepticus and Refractory Status Epilepticus.

Status epilepticus is a heterogeneous disorder with varied definitions and presentations. Taken together, all forms of status epilepticus carry significant morbidity and mortality, much of which is dictated by the underlying etiology. Generalized convulsive status epilepticus, which represents a common form, is a true neurologic emergency that requires emergent management. Treatment focuses on stabilizing the patient and aggressive medical management to achieve the timely termination of seizures. For other forms of status epilepticus including nonconvulsive and focal status epilepticus, the goal remains early seizure termination, but the use of intravenous medications should be weighed against the risks associated with these therapies. The diagnostic evaluation of status epilepticus is guided by the patient's history and should be broad, including a screen for toxins, electrolytes, structural abnormalities, and central nervous system infectious and autoimmune/inflammatory etiologies. Considerable research is still needed to improve our understanding of the mechanisms, consequences, and therapy of status epilepticus.

Treatment of Focal Status Epilepticus plus Epileptic Spasms with Leukotomy in an Infant with TSC 1 Mutation: A Case Study

ABSTRACTBackground: Focal status epilepticus and catastrophic epilepsy are not rare in infants. Epilepsy surgery can be safely done in selected infants to cure epilepsy.Patient description: We report on an infant who began having drug-resistant status epilepticus at 2 weeks of age and developed epileptic spasms. We discuss in detail how the clinical and electroencephalographic data were used to reach a consensus for epilepsy surgery and why a particular surgical approach was preferred over other alternatives.Method: Presurgical evaluation consisted of 32-channel scalp video EEG using the standard 10–20 system of electrodes, 3-Tesla brain magnetic resonance imaging, and 18 F-fluorodeoxyglucose positron emission tomography. The surgery consisted of resection of the extensive epileptogenic lesion, in addition to disconnection of the left frontal lobe anterior to the motor strip.Result: The infant underwent epilepsy surgery at three months of age. At two-year follow up, she remained seizure free, with no focal motor deficit and the epileptic encephalopathy resolved. The disconnected left frontal lobe shows epileptiform discharges restricted to the disconnected tissue.Conclusion: We highlight the importance of epilepsy surgery in selected infants to achieve seizure freedom and to reverse epileptic encephalopathy. In the process, we demonstrate how epileptic spasms, although clinically and electrographically generalized, resolved after disconnecting the epileptogenic zone.

Are There Any Specific EEG Findings in Autoimmune Epilepsies?

This study evaluated the EEG findings of patients whose seizures were associated with a possible autoimmune etiology. Our aim was to find clues to distinguish patients with antineuronal antibodies (Ab) through EEG studies. We reviewed our database and identified antineuronal Ab positive epilepsy patients with or without autoimmune encephalitis. These patients had Abs to N-methyl-d-aspartate receptor (NMDAR) (n = 5), glycine receptor (GLY-R) (n = 5), contactin-associated protein-like 2 (CASPR-2) (n = 4), uncharacterized voltage-gated potassium channel complex (VGKC) antigens (n = 2), glutamic acid decarboxylase (GAD) (n = 2), Hu (n = 1), and amphiphysin (n = 1). The control group consisted of 21 seronegative epilepsy or encephalopathy patients with similar clinical features. EEG findings were compared between the groups in a blindfolded design. We did not find any significant difference in EEG findings between antineuronal Ab positive epilepsy patients and seronegative control group. It was remarkable that four seropositive but none of the seronegative patients presented with nonconvulsive status epilepticus (NCSE) or focal motor status epilepticus. Continuous theta and delta rhythms were observed in 5 (71%) seropositive patients with autoimmune encephalitis and 2 (25%) seronegative patients. Eight (40 %) seropositive patients showed a frontal intermittent rhythmic delta activity (FIRDA) pattern as opposed to 5 (24%) seronegative patients. Two patients with NMDAR Ab positivity showed rhythmic delta waves superimposed with beta frequency activity resembling "delta brush" pattern. EEG seems as a limited diagnostic tool in differentiating epilepsy and/or encephalopathy patients with a possible autoimmune etiology from those without. However, antineuronal Abs associated with encephalitis should be considered in the etiology of status epilepticus forms. A possible autoimmune etiology for seizures may be considered in the presence of continuous slow waves, FIRDA, and delta brush pattern in the EEG.

Short-term mortality and prognostic factors related to status epilepticus.

Status epilepticus (SE) is associated with significant morbidity and mortality, and there is some controversy concerning predictive indicators of outcome. Our main goal was to determine mortality and to identify factors associated with SE prognosis. This prospective study in a tertiary-care university hospital, included 105 patients with epileptic seizures lasting more than 30 minutes. Mortality was defined as death during hospital admission. The case-fatality rate was 36.2%, which was higher than in previous studies. In univariate analysis, mortality was associated with age, previous epilepsy, complex focal seizures; etiology, recurrence, and refractoriness of SE; clinical complications, and focal SE. In multivariate analysis, mortality was associated only with presence of clinical complications. Mortality associated with SE was higher than reported in previous studies, and was not related to age, specific etiology, or SE duration. In multivariate analysis, mortality was independently related to occurrence of medical complications.