Use of ketamine in Super Refractory Status Epilepticus: a systematic review.

OBJECTIVE: This review specifically investigates ketamine's role in SRSE management. METHODS: PubMed, EMBASE, and Google Scholar databases were searched from inception to May 1st, 2023, for English-language literature. Inclusion criteria encompassed studies on SRSE in humans of all ages and genders treated with ketamine. RESULTS: In this systematic review encompassing 19 studies with 336 participants, age ranged from 9 months to 86 years. Infections, anoxia, and metabolic issues emerged as the common causes of SRSE, while some cases had unknown origins, termed as NORSE (New Onset RSE) or FIRESs (Febrile Infection-Related Epilepsy Syndrome). Most studies categorized SRSE cases into convulsive (N = 105) and non-convulsive (N = 197). Ketamine was used after failed antiepileptics and anesthetics in 17 studies, while in others, it was a first or second line of treatment. Dosages varied from 0.5 mg/kg (bolus) and 0.2-15 mg/kg/hour (maintenance) in adults and 1-3 mg/kg (bolus) and 0.5-3 mg/kg/hour (maintenance) in pediatrics, lasting one to 30 days. Ketamine was concurrently used with other drugs in 40-100% of cases, most frequently propofol and midazolam. Seizure resolution rate varied from 53.3 to 91% and 40-100% in larger (N = 42-68) and smaller case series (N = 5-20) respectively. Seizure resolution occurred in every case of case report except in one in which the patient died. Burst suppression in EEG was reported in 12 patients from two case series and two case reports. Recurrence was reported in 11 patients from five studies. The reported all-cause mortality varied from 38.8 to 59.5% and 0-36.4% in larger and smaller case series., unrelated directly to ketamine dosage or duration. SIGNIFICANCE: Ketamine demonstrates safety and effectiveness in SRSE, offering advantages over GABAergic drugs by acting on NMDA receptors, providing neuroprotection, and reducing vasopressor requirement.

Prognostic value of Salzburg nonconvulsive status epilepticus criteria: The SACE score.

OBJECTIVE: This study was undertaken to investigate the association between the Salzburg nonconvulsive status epilepticus (NCSE) criteria and in-hospital outcome, to determine the predictive accuracy of the Status Epilepticus Severity Score (STESS), modified STESS (mSTESS), Epidemiology-Based Mortality Score in Status Epilepticus (EMSE), and END-IT (encephalitis, NCSE, diazepam resistance, imaging features, and tracheal intubation) in NCSE patients, and to develop a new prognostic score specifically designed for NCSE patients. METHODS: Clinical and electroencephalographic (EEG) data of adult patients treated for NCSE from 2020 to 2023 were retrospectively assessed. Age, sex, modified Rankin Scale at admission, comorbidities, history of seizures, etiology, status epilepticus type, and outcome were collected from the patients' digital charts. EEG data were assessed and categorized applying the Salzburg NCSE criteria. In-hospital death was defined as the primary outcome. RESULTS: A total of 116 NCSE patients were included. Multivariable logistic regression revealed that Salzburg NCSE criterion A2 (ictal morphological, spatial, and temporal evolution) was associated with in-hospital survival. The best STESS cutoff was ≥4 (sensitivity = .62, specificity = .69, accuracy = 67%). mSTESS ≥ 5 reached a sensitivity of .68, a specificity of .57, and an overall accuracy of 60%, EMSE ≥ 64 a sensitivity of .82, a specificity of .39, and an overall accuracy of 52%, and END-IT ≥ 3 a sensitivity of .65, a specificity of .44, and an overall accuracy of 50%. Through a hypothesis-generating approach, we developed the SACE score, which integrates EEG features (criterion A2) with patient age (with a 75-year cutoff), history of seizures, and level of consciousness. With a cutoff of ≥3, it had a sensitivity of .77, a specificity of .74, and an overall accuracy of 76%, performing better than other prognostic scores. SIGNIFICANCE: We developed a new user-friendly scoring system, the SACE score, which integrates EEG features with other established outcome-related variables assessable in early stages, to assist neurologists and neurointensivists in making more tailored prognostic decisions for NCSE patients.

Presumed first episode of nonconvulsive status epilepticus as the cause of postoperative disorder of consciousness following the completion of general anesthesia: A case report.

Key Clinical Message: Disorder of consciousness can lead to irreversible sequelae without proper intervention. Consequently, early diagnosis and treatment are of paramount importance in patients with disorder of consciousness. Abstract: Disorder of consciousness (DOC) has various etiologies. Here, we report a case in which DOC following general anesthesia was suspected as being due to the first episode of nonconvulsive status epilepticus (NCSE). An elderly man in his 80s underwent uneventful tumor resection surgery under general anesthesia for extramammary Paget's disease. After the procedure, he regained consciousness following anesthesia discontinuation and was extubated. Soon after extubation, however, although his respiratory status remained stable, his level of consciousness deteriorated to a Glasgow Coma Scale (GCS) score of E1V1M1. Head computed tomography and magnetic resonance imaging scans indicated no abnormal findings. Subsequently, involuntary movements were noted in his left upper limb. Suspecting an epilepsy episode, diazepam was administered, leading to an improvement in the level of consciousness (GCS: E4V5M6). Based on the improvement in consciousness after diazepam administration, we strongly suspected NCSE.

Ketamine to Prevent Endotracheal Intubation in Adults with Refractory Non-convulsive Status Epilepticus: A Case Series.

BACKGROUND: Non-convulsive status epilepticus (NCSE) is defined as status epilepticus (SE) with no obvious motor phenomenon and is diagnosed based on electroencephalogram (EEG). Refractory SE (RSE) is the persistence of seizures despite treatment with an adequately dosed first-line and second-line agents. Although guidelines for convulsive RSE include third-line agents such as intravenous anesthetic drugs (midazolam, propofol, or barbiturates), the therapeutic approach to NCSE is not well outlined. Treatment with traditional anesthetics invariably includes endotracheal intubation, which is associated with significant adverse events. Comparatively, ketamine, a non-competitive N-methyl-D-aspartate receptor antagonist is not associated with significant cardiorespiratory depression and may help in avoiding intubation. OBJECTIVE: In this case series, we describe our experience with the early use of intravenous ketamine as the first anesthetic agent in patients with refractory NCSE to avoid endotracheal intubation. METHODS: We present a case series of nine patients managed in the Neurointensive Care Unit at a university-affiliated tertiary care hospital. The study was approved by the hospital and university institutional review boards and the requirement for informed consent was waived for retrospective analysis of existing data, per institutional policy. All cases of SE were identified from a prospective database, and a subsequent retrospective chart review identified all patients with a diagnosis of refractory NCSE in whom ketamine was used as the first anesthetic agent. The primary endpoint was the avoidance of endotracheal intubation while on ketamine infusion. The secondary endpoint was defined as cessation of both clinical and electrographic seizures recorded on continuous EEG within 24 h of ketamine administration. RESULTS: A total of nine patients experiencing refractory NCSE were included in this case series, with a median age of 61 (range 26-72) years and seven patients were male. The primary endpoint, avoiding intubation, was achieved in five out of nine (55%) cases. Six patients experienced resolution of refractory NCSE with ketamine administration as the sole anesthetic agent. Four patients required endotracheal intubation and three patients had a failure of seizure cessation with ketamine. Hypersalivation and pneumonia were the most common ketamine associated adverse events. In non-intubated patients, no deaths occurred. One patient was discharged home, four to subacute rehabilitation, one to a long term acute care hospital, and one patient to hospice. CONCLUSION: The use of ketamine as the primary anesthetic agent may be a reasonable option to avoid endotracheal intubation in a subset of patients with refractory NCSE. This study is limited by its small sample size, retrospective design, and reliance on information obtained from chart review.

Nonconvulsive Status Epilepticus: A Review for Emergency Clinicians.

BACKGROUND: Status epilepticus is associated with significant morbidity and mortality and is divided into convulsive status epilepticus and nonconvulsive status epilepticus (NCSE). OBJECTIVE: This review provides a focused evaluation of NCSE for emergency clinicians. DISCUSSION: NCSE is a form of status epilepticus presenting with prolonged seizure activity. This disease is underdiagnosed, as it presents with nonspecific signs and symptoms, most commonly change in mental status without overt convulsive motor activity. Causes include epilepsy, cerebral pathology or injury, any systemic insult such as infection, and drugs or toxins. Mortality is primarily related to the underlying condition. Patients most commonly present with altered mental status, but other signs and symptoms include abnormal ocular movements and automatisms such as lip smacking or subtle motor twitches in the face or extremities. The diagnosis is divided into electrographic and electroclinical, and although electroencephalogram (EEG) is recommended for definitive diagnosis, emergency clinicians should consider this disease in patients with prolonged postictal state after a seizure with no improvement in mental status, altered mental status with acute cerebral pathology (e.g., stroke, hypoxic brain injury), and unexplained altered mental status. Assessment includes laboratory evaluation and neuroimaging with EEG. Management includes treating life-threatening conditions, including compromise of the airway, hypoglycemia, hyponatremia, and hypo- or hyperthermia, followed by rapid cessation of the seizure activity with benzodiazepines and other antiseizure medications. CONCLUSIONS: An understanding of the presentation and management of NCSE can assist emergency clinicians in the care of these patients.

Cefepime-Induced Neurotoxicity or Nonconvulsive Status Epilepticus (NCSE): A Controversy Revisited.

Neurotoxicity is a well-known side effect of cefepime among patients commonly present with altered mental status and typical electroencephalogram (EEG) findings of generalized periodic discharges (GPDs). Some practitioners consider this pattern as encephalopathy and often treat it with the withdrawal of cefepime only, while others are at times concerned with non-convulsive status epilepticus (NCSE) and treat it with antiseizure medications (ASMs) in addition to the withdrawal of cefepime to accelerate the recovery. We present a case series of two patients who developed cefepime-induced altered mental status and EEG findings of GPDs at a rate of 2-2.5 Hz concerning for the ictal-interictal continuum (IIC). Both cases were treated as possible NCSE with ASMs in addition to the withdrawal of cefepime, resulting in different clinical outcomes. The first case showed clinical and EEG improvement shortly after the administration of parenteral benzodiazepines and ASMs. The other case showed electrographic improvement but did not show significant improvement in mentation, and the patient died eventually.

Sponge EEG is equivalent regarding signal quality, but faster than routine EEG.

Objective: Emergency diagnostics, such as acquisition of an electroencephalogram (EEG), are of great diagnostic importance, but there is often a lack of experienced personnel. Wet active electrode sponge-based electroencephalogram (sp-EEG) systems can be applied rapidly and by inexperienced personnel. This makes them an attractive alternative to routine EEG (r-EEG) systems in these settings. Here, we examined the feasibility and signal quality of sp-EEG compared to r-EEG. Methods: In this case-control, single-blind, non-randomized study, EEG recordings using a sp- and a r-EEG system were performed in 18 individuals with a variety of epileptiform discharges and 11 healthy control subjects. The time was stopped until all electrodes in both systems displayed adequate skin-electrode impedances. The resulting 58 EEGs were visually inspected by 7 experienced, blinded neurologists. Raters were asked to score physiological and pathological graphoelements, and to distinguish between the different systems by visual inspection of the EEGs. Results: Time to signal acquisition for sp-EEG was significantly faster (4.8 min (SD 2.01) vs. r-EEG 13.3 min (SD 2.72), p < 0.001). All physiological and pathological graphoelements of all 58 EEGs could be identified. Raters were unable to distinguish between sp-EEG or r-EEG based on visual inspection of the EEGs alone. Conclusions: Sp-EEG represents a feasible alternative to r-EEG in emergency diagnostics or resource-limited settings. Significance: Given shortage of trained personnel or resources, the easy implementation and comparable quality of a novel sp-EEG system may increase general availability of EEG and thus improve patient care.

Nonconvulsive status epilepticus characteristics in glioma patients: a retrospective study.

PURPOSE: Epilepsy is a common complication of gliomas. The diagnosis of nonconvulsive status epilepticus (NCSE) is challenging because it causes impaired consciousness and mimics glioma progression. NCSE complication rate in the general brain tumor patient population is approximately 2%. However, there are no reports focusing on NCSE in glioma patient population. This study aimed to reveal the epidemiology and features of NCSE in glioma patients to enable appropriate diagnosis. METHODS: We enrolled 108 consecutive glioma patients (45 female, 63 male) who underwent their first surgery between April 2013 and May 2019 at our institution. We retrospectively investigated glioma patients diagnosed with tumor-related epilepsy (TRE) or NCSE to explore disease frequency of TRE/NCSE and patient background. NCSE treatment approaches and Karnofsky Performance Status Scale (KPS) changes following NCSE were surveyed. NCSE diagnosis was confirmed using the modified Salzburg Consensus Criteria (mSCC). RESULTS: Sixty-one out of 108 glioma patients experienced TRE (56%), and five (4.6%) were diagnosed with NCSE (2 female, 3 male; mean age, 57 years old; WHO grade II 1, grade III 2, grade IV 2). All NCSE cases were controlled by stage 2 status epilepticus treatment as recommended in the Clinical Practice Guidelines for Epilepsy by the Japan Epilepsy Society. The KPS score significantly decreased after NCSE. CONCLUSION: Higher prevalence of NCSE in glioma patients was observed. The KPS score significantly decreased after NCSE. Actively taking electroencephalograms analyzed by mSCC may facilitate accurate NCSE diagnosis and improve the activities of daily living in glioma patients.

Syntheses and crystal structures of bis-(4-methyl-pyridine-κN)bis-(seleno-cyanato-κN)zinc(II) and catena-poly[[bis-(4-methyl-pyridine-κN)cadmium(II)]-di-µ-seleno-cyanato-κ2 N:Se;κ2 Se:N].

The reactions of Zn(NO3)2 .6H2O and Cd(NO3)2 .4H2O with KSeCN and 4-meth-yl-pyridine (C6H7N; 4-picoline) lead to the formation of crystals of bis-(4-methyl-pyridine-κN)bis-(seleno-cyanato-κN)zinc(II), [Cd(NCSe)2(C6H7N)2] (1), and catena-poly[[bis-(4-methyl-pyridine-κN)cadmium(II)]-di-µ-seleno-cyanato-κ2 N:Se;κ2 Se:N], [Cd(NCSe)2(C6H7N)2] n (2), suitable for single-crystal X-ray diffraction. The asymmetric unit of compound 1 consists of one Zn cation that is located on a twofold rotation axis as well as one seleno-cyanate anion and one 4-methyl-pyridine ligand in general positions. The Zn cations are tetra-hedrally coordinated by two terminal N-bonding thio-cyanate anions and two 4-methyl-pyridine ligands, forming discrete complexes. The asymmetric unit of compound 2 consists of two crystallographically independent Cd cations, of which one is located on a twofold rotation axis and the second on a center of inversion, as well as two crystallographically independent seleno-cyanate anions and two crystallographically independent 4-methyl-pyridine ligands in general positions. The Cd cations are octa-hedrally coordinated by two N- and two S-bonding seleno-cyanate anions and two 4-methyl-pyridine ligands and are linked into chains by pairs of seleno-cyanate anions. Within the chains, the Cd cations show an alternating cis-cis-trans and all-trans coordination and therefore, the chains are corrugated. PXRD investigations prove that the Zn compound was obtained as a pure phase and that the Cd compound contains a very small amount of an additional and unknown phase. In the IR spectrum of 1, the CN stretching vibration of the seleno-cyanate anion is observed at 2072 cm-1, whereas in the 2 it is shifted to 2094 cm-1, in agreement with the crystal structures.

Analysis of the Spatiotemporal Evolution of the Net Carbon Sink Efficiency and Its Influencing Factors at the City Level in Three Major Urban Agglomerations in China.

The implementation of carbon peaking and carbon neutrality is an essential measure to reduce greenhouse gas emissions and actively respond to climate change. The net carbon sink efficiency (NCSE), as an effective tool to measure the carbon budget capacity, is important in guiding the carbon emission reduction among cities and the maintenance of sustainable economic development. In this paper, NCSE values are used as a measure of the carbon budget capacity to measure the spatiotemporal evolution of the carbon neutral capacity of three major urban agglomerations (UAs) in China during 2007-2019. The clustering characteristics of the NCSE of these three major UAs, and various influencing factors such as carbon emissions, are analyzed using a spatiotemporal cube model and spatial and temporal series clustering. The results reveal the following. (1) From the overall perspective, the carbon emissions of the three major UAs mostly exhibited a fluctuating increasing trend and a general deficit during the study period. Moreover, the carbon sequestration showed a slightly decreasing trend, but not much fluctuation in general. (2) From the perspective of UAs, the cities in the Beijing-Tianjin-Hebei UA are dominated by low-low clustering in space and time; this clustering pattern is mainly concentrated in Beijing, Xingtai, Handan, and Langfang. The NCSE values in the Yangtze River Delta UA centered on Shanghai, Nanjing, and the surrounding cities exhibited high-high clustering in 2019, while Changzhou, Ningbo, and the surrounding cities exhibited low-high clustering. The NCSE values of the remaining cities in the Pearl River Delta UA, namely Guangzhou, Shenzhen, and Zhuhai, exhibited multi-cluster patterns that were not spatially and temporally significant, and the spatiotemporal clusters were found to be scattered. (3) In terms of the influencing factors, the NCSE of the Beijing-Tianjin-Hebei UA was found to be significantly influenced by the industrial structure and GDP per capita, that of the Yangtze River Delta UA was found to be significantly influenced by the industrial structure, and that of the Pearl River Delta UA was found to be significantly influenced by the population density and technology level. These findings can provide a reference and suggestions for the governments of different UAs to formulate differentiated carbon-neutral policies.

Pitfalls in EEG Analysis in Patients With Nonconvulsive Status Epilepticus: A Preliminary Study.

Objective: Electroencephalography (EEG) interpretations through visual (by human raters) and automated (by computer technology) analysis were still not reliable for the diagnosis of nonconvulsive status epilepticus (NCSE). This study aimed to identify typical pitfalls in the EEG analysis and make suggestions as to how those pitfalls might be avoided. Methods: We analyzed the EEG recordings of individuals who had clinically confirmed or suspected NCSE. Epileptiform EEG activity during seizures (ictal discharges) was visually analyzed by 2 independent raters. We investigated whether unreliable EEG visual interpretations quantified by low interrater agreement can be predicted by the characteristics of ictal discharges and individuals' clinical data. In addition, the EEG recordings were automatically analyzed by in-house algorithms. To further explore the causes of unreliable EEG interpretations, 2 epileptologists analyzed EEG patterns most likely misinterpreted as ictal discharges based on the differences between the EEG interpretations through the visual and automated analysis. Results: Short ictal discharges with a gradual onset (developing over 3 s in length) were liable to be misinterpreted. An extra 2 min of ictal discharges contributed to an increase in the kappa statistics of >0.1. Other problems were the misinterpretation of abnormal background activity (slow-wave activities, other abnormal brain activity, and the ictal-like movement artifacts), continuous interictal discharges, and continuous short ictal discharges. Conclusion: A longer duration criterion for NCSE-EEGs than 10 s that is commonly used in NCSE working criteria is recommended. Using knowledge of historical EEGs, individualized algorithms, and context-dependent alarm thresholds may also avoid the pitfalls.

Prognostic indicators of non-convulsive status epilepticus in intensive care unit.

BACKGROUND: To determine the rate of non-convulsive status epilepticus with/without prominent motor phenomena (SE-PM/ NCSE) and predictive value of electroclinical findings of continious electroencephalography (cEEG) monitoring of these patients and its association with prognosis in intensive care units (ICU). METHODS: We retrospectively collected data of 218 patients whose cEEG was performed in ICU between 2016 and 2018. The cEEG for NCSE diagnosis was evaluated according to Salzburg Consensus Criteria (SCC). RESULTS: The mean age of patients was 57.09 ± 18.9 (16-95) years and 49.1% (107) were female. Of 218 patients, 32 (14.7%) had SE-PM/NCSE. According to SCC the rate of NCSE (NCSE + possible NCSE) was 9.6% (n = 21). Prior to cEEG recording, 38.9% (n = 85) of overall patients had a history of seizure/convulsion, and 22.7% (n = 21) of these patients diagnosed with NCSE based on cEEG. The mortality rates in critically ill patients were 41.3% (30.8%, 42.8%; for SE-PM and NCSE respectively). Prognosis was associated with age, epilepsy diagnosis, having convulsion/seizure history on follow-up, GCS, need for ventilation, kind of drugs, sepsis diagnosis, and minimum frequency of background activity of the cEEG (p = 0.001, 0.002, 0.001, 0.020, 0.001, 0.001, 0.001, 0.0001 respectively). CONCLUSIONS: NCSE findings are mostly found in patients who were comatose and had seizure/convulsion history on follow-up. Mortality is higher in patients diagnosed with NCSE followed in the ICU compared to SE-PM.

Nonconvulsive status epilepticus following cardiac arrest: overlooked, untreated and misjudged.

AIMS: Seizures and status epilepticus (SE) are detected in almost a third of the comatose cardiac arrest survivors. As the literature is quite exhaustive regarding SE with motor symptoms in those patients, little is known about nonconvulsive SE (NCSE). Our aim was to compile the evidence from the literature of the frequency and outcome of NCSE in adult patients remaining in coma after resuscitation. METHODS: The medical search PubMed was screened for most relevant articles reporting the emergence and outcome of NCSE in comatose post-resuscitated adult patients. RESULTS: We identified 11 cohort studies (four prospective observational, seven retrospective) including 1092 patients with SE in 29-96% and NCSE reported in 1-20%. EEG evaluation started at a median of 9.5 h (range 7.5-14.8) after cardiac arrest, during sedation and targeted temperature management (TTM). Favorable outcome after NCSE occurred in 24.5%. We found no study reporting EEG to detect or exclude NCSE in patients remaining in coma prior to the initiation of TTM and without sedation withing the first hours after ROSC. DISCUSSION: Studies on NCSE after ROSC are scarce and unsystematic, reporting favorable outcome in every fourth patient experiencing NCSE after ROSC. This suggests that NCSE is often overlooked and outcome after NCSE is not always poor. The low data quality does not allow firm conclusions regarding the effects of NCSE on outcome calling for further investigation. In the meantime, clinicians should avoid equating NCSE after ROSC with poor prognosis.

Rapid Response EEG: Current State and Future Directions.

PURPOSE OF REVIEW: To critically appraise the literature on the application, methods, and advances in emergency electroencephalography (EEG). RECENT FINDINGS: The development of rapid EEG (rEEG) technologies and other reduced montage approaches, along with advances in machine learning over the past decade, has increased the rate and access to EEG acquisition. These achievements have made EEG in the emergency setting a practical diagnostic technique for detecting seizures, suspected nonconvulsive status epilepticus (NCSE), altered mental status, stroke, and in the setting of sedation. Growing evidence supports using EEG to expedite medical decision-making in the setting of suspected acute neurological injury. This review covers approaches to acquiring EEG in the emergency setting in the adult and pediatric populations. We also cover the clinical impact of this data, the time associated with emergency EEG, and the costs of acquiring EEG in these settings. Finally, we discuss the advances in artificial intelligence for rapid electrophysiological interpretation.

Comparing Seizures Captured by Rapid Response EEG and Conventional EEG Recordings in a Multicenter Clinical Study.

Objective: A recent multicenter prospective study (DECIDE trial) examined the use of Ceribell Rapid Response EEG (Rapid-EEG) in the emergent evaluation and management of critically ill patients suspected to have non-convulsive seizures. We present a detailed, patient-level examination of seizures detected either on initial Rapid-EEG or subsequent conventional EEG within 24 h to investigate whether seizures were missed on Rapid-EEG due to the exclusion of midline/parasagittal coverage. Methods: We identified from 164 patients studied in the DECIDE trial those who had seizures detected on Rapid-EEG but not conventional EEG (n = 6), conventional EEG but not Rapid-EEG (n = 4), or both Rapid-EEG and conventional EEG (n = 9). We examined the electrographic characteristics of ictal and interictal findings on both devices, especially their detection in lateral or midline/parasagittal chains, and patient clinical histories to identify contributors toward discordant seizure detection. Results: Seizures detected on both EEG systems had similar electrographic appearance and laterality. Seizures detected only on conventional EEG (within 24 h following Rapid-EEG) were visible in the temporal chains, and external clinical factors (e.g., treatment with anti-seizure medications, sedation, and duration of recordings) explained the delayed presentation of seizures. Patients with seizures detected only by Rapid-EEG were treated with anti-seizure medications, and subsequent conventional EEG detected interictal highly epileptiform patterns with similar laterality. Conclusions: Our case series demonstrates that electrographic data obtained from initial Rapid-EEG and subsequent conventional EEG monitoring are largely concordant relative to morphology and laterality. These findings are valuable to inform future investigation of abbreviated EEG systems to optimize management of suspected non-convulsive seizures and status epilepticus. Future, larger studies could further investigate the value of Rapid-EEG findings for forecasting and predicting seizures in long-term EEG recordings.

Nonconvulsive status epilepticus due to pneumocephalus after suprasellar arachnoid cyst fenestration with transsphenoidal surgery: illustrative case.

BACKGROUND: Nonconvulsive status epilepticus (NCSE) requires prompt diagnosis and treatment, particularly after neurosurgical procedures for cerebral damage. Here, the authors reported an extremely rare case of suprasellar arachnoid cyst presenting with NCSE after cyst fenestration with transsphenoidal surgery, which was associated with pneumocephalus. OBSERVATIONS: A 61-year-old man presented with visual impairment and was diagnosed with a suprasellar arachnoid cyst on magnetic resonance imaging (MRI). The patient received cyst fenestration with endonasal transsphenoidal surgery. His visual symptoms improved immediately after the operation; however, on postoperative day 3, semicoma appeared and was prolonged. The patient was diagnosed with NCSE due to pneumocephalus based on MRI and electroencephalography (EEG) findings. The administration of antiepileptic drugs (AEDs) improved his clinical symptoms and the abnormal findings on MRI and EEG. LESSONS: This is the first case of NCSE with pneumocephalus after transsphenoidal surgery for a suprasellar arachnoid cyst. Pneumocephalus due to cerebrospinal fluid leakage can cause NCSE. Arterial spin labeling perfusion imaging and diffusion-weighted imaging are as useful for differentially diagnosing NCSE as EEG and AED tests.

Non-convulsive Status Epilepticus in SEMA6B-Related Progressive Myoclonic Epilepsy: A Case Report With Literature Review.

Progressive myoclonic epilepsy (PME) is a group of rare diseases characterized by progressive myoclonus, cognitive impairment, ataxia, and other neurologic deficits. PME has high genetic heterogeneity, and more than 40 genes are reportedly associated with this disorder. SEMA6B encodes a member of the semaphorin family and was first reported to cause PME in 2020. Herein, we present a rare case of PME due to a novel SEMA6B gene mutation in a 6-year-old boy born to healthy non-consanguineous Chinese parents. His developmental milestones were delayed, and he developed recurrent atonic seizures and myoclonic seizures without fever at 3 years and 11 months of age. He experienced recurrent myoclonic seizures, non-convulsive status epilepticus (NCSE), atonic seizures, and atypical absence seizures during the last 2 years. At different time points since onset, valproic acid, levetiracetam, piracetam, and clobazam were used to control the intractable seizures. Notably, NCSE was controlled by a combination of piracetam with clobazam and valproic acid instead of intravenous infusion of midazolam and phenobarbital. Due to the limited number of cases reported to date, the clinical description of our case provides a better understanding of the genotype-phenotype correlations associated with PME and indicate that piracetam may be effective against NCSE in patients with SEMA6B-related PME.

[A case of myopathy, myocarditis, and encephalitis with nonconvulsive status epileptics after immune checkpoint inhibitor therapy for ureter cancer].

A 72-year-old man, who had received pembrolizumab of immune checkpoint inhibitor (ICI) over 6 months for ureter cancer, developed progressive skeletal muscle weakness, dysarthria, dyspnea, and consciousness disturbance over the past two weeks. The systemic work-up tests documented an encephalitis, myopathy, and myocarditis. Multiple autoimmune antibodies of anti-Tr, anti-titin, anti-kv1.4, anti-GM1 and anti-GD1a were positive in the serum. Although myopathy and myocarditis responded to high-dose steroid pulse therapy, encephalopathy deteriorated. Electroencephalogram showed a fluctuated pattern of rhythmic delta activity with fast waves, and a rapid response to intravenous diazepam revealed a condition of nonconvulsive status epileptics (NCSE). The patient had an uneventful course after anti-epileptic medication. The ICIs therapy may trigger a broader activation of multiple autoimmune mechanisms. When an encephalitis by immune-related adverse events does not respond to standard immunotherapy, NCSE may be a main pathophysiological mechanism, thereby anti-epileptics being an alternative treatment option.

De novo aphasic status epilepticus: Finally making the diagnosis by long-term EEG.

Aphasic status epilepticus (SE) is a rare manifestation of non-convulsive SE (NCSE) and may occasionally be under-recognized. We report a 69-year-old male patient with a pre-existing left parietal oligodendroglioma WHO III after two resections and radio-chemotherapy. The patient was left with some word finding difficulties but had no history of overt seizures. He developed aphasic NCSE, which was only detected by long-term electroencephalography (EEG) monitoring. The 24-hour EEG revealed paroxysmal rhythmic theta-delta activity in left posterior regions that propagated to left temporo-parietal areas. Rhythmic activity appeared every 15-30 min and lasted for 10-110 s. Aphasia was continuously present with superimposed short-lasting clinical deteriorations during the day. Magnetic resonance imaging showed peri-ictal edema on diffusion-weighted images in the insula and fronto-parietal cortex, which supported the diagnosis of SE. NCSE persisted for seven months. The patient recovered upon addition of intravenous phenytoin. One should not only consider aphasic SE when language impairment is episodic, but also when there are prolonged manifestations, especially when the typical differential diagnoses have been excluded. Intravenous therapy may be required to terminate NCSE. With this report, we would like to draw attention to aphasic SE as a rare phenomenon that may be difficult to diagnose and delay management in clinical practice.