Absence status epilepticus in adults

Nonconvulsive status epilepticus in adults: Electroclinical differences between proper and comatose forms

Nonconvulsive status epilepticus (NCSE) is a type of status epilepticus (SE) lacking a predominant motor manifestation. The annual prevalence of NCSE is estimated to reach 10 to 20 cases in every 100,000 people. While almost half of all SE cases are nonconvulsive, there are several different types of NCSE: 1) epileptic absence SE, 2) epileptic focal seizure SE with consciousness disturbance (complex partial SE), 3) de novo NCSE of late onset, 4) NCSE due to acute brain injury or prolonged consciousness disturbance after convulsive SE. An electroencephalography (EEG) evaluation is necessary to diagnose NCSE. However, continuous EEG (cEEG) monitoring over at least 24 hours is preferable to detect NCSE, as cognitive disturbances due to this condition may fluctuate over time. In addition, neuroimaging techniques, such as MRI with arterial spin labeled sequences or single photon emission computed tomography (SPECT) can demonstrate hyperperfused areas in cases of focal onset. Thus, patients presenting with alternative cognitive disturbance with or without mild confusion should be evaluated using cEEG monitoring or blood flow imaging so as not to overlook treatable NCSE.

Determining the prognosis in nonconvulsive status epilepticus (NCSE) is complicated by several factors: under‐recognition of NCSE with its spontaneous resolution (thus decreasing the “denominator” of total cases that will have a poor outcome); incorrect diagnosis of NCSE based on misinterpretation of EEG “epileptiform” activity; mis‐classification of certain EEG patterns as NCSE ( e.g. PLEDs; triphasic waves); and grouping of different populations that have markedly different co‐morbidities (ambulatory patients with NCSE together with comatose patients with electrographic seizure activity on EEG). There are almost no prospective studies with premorbid neuropsychometric studies, and retrospective studies typically include isolated cases, or case series that include conditions in which the cause of NCSE itself causes cognitive morbidity. To summarize available data, absence status (ambulatory generalized non‐convulsive status epilepticus) would appear to carry no lasting morbidity. Complex partial status epilepticus in ambulatory patients rarely results in measurable permanent neurologic deficit, although rarely, short or longstanding deficits may clearly occur. Because intensive treatment with intravenous anticonvulsants ( e.g. benzodiazepines or phenytoin) can confer morbidity, the equation has not yet been made as to whether the morbidity of such intensive treatment for all cases of NCSE exceeds the morbidity of the disease itself. Larger, prospective studies will be needed to truly determine the prognosis in the different types of NCSE, stratified according to associated degrees of impairment (minimally impaired, moderately obtunded, comatose).

Nearly 10years after its introduction into the market, the significance of lacosamide in genetic generalized epilepsies is still unclear. Its new mode of action may qualify lacosamide as a therapeutic agent in this entity, but only a limited number of cases have been published so far. To describe the efficacy of lacosamide as treatment in a patient with the absence status epilepticus. We report on a 28-year-old woman with genetic generalized epilepsy who suffered recurrent absence status epilepticus during video-EEG-monitoring. After treatment failure of first- and second-line medication, lacosamide was administered. The outcome in this patient was evaluated, and a systematic literature review was performed for the use of lacosamide in the absence status epilepticus. After application of 400mg lacosamide intravenously, the absence status epilepticus terminated within 30minutes. No further seizures or epileptiform discharges reoccurred until the end of video-EEG-Monitoring 3days later. The role of lacosamide as a therapeutic option in patients with the absence status epilepticus is unclear. Only two cases have been reported so far with conflicting results. Further randomized controlled studies are required to validate the relevance of lacosamide as treatment for status epilepticus in genetic generalized and the absence epilepsy.

Background: In the elderly, nonconvulsive status epilepticus (NCSE) is frequent, however there are still gaps in knowledge about the types of presentation, ictal EEG patterns and prognosis. It is questioned whether the prognosis is related to the etiological factor. Objective: To evaluate the prognosis and clinical-EEG variables in older adults with NCSE according to remote or primary acute etiology. Methods: Clinical-EEG data and the occurrence of death at 30 days in 90 elderly patients diagnosed with NCSE of structural etiology (primary acute or remote) treated at the clinical neurology service of the PUC-Campinas Hospital were evaluated and compared. Results: The mean age was 72.6±8.3 years and 57(63.3%) cases were female, 53(58.9%) cases had acute etiology and 37(41.1%) had remote etiology. The NCSE type was - NCSE-coma in 41(45.6%) cases and NCSE-without coma in 49(54.4%) cases. No significant difference was observed in gender and age according to the type of etiology. There was no difference in the ictal EEG pattern (Salzburg Criterion - A1, A2, A3, A4) according to acute or remote etiology. Death within 30 days occurred in 32(35.6%) cases, with no difference according to etiology. Previous history of seizures/epilepsy occurred more and significantly in older adults with NCSE of remote etiology (Fisher’s exact test, 6(11.3%) vs 17(45.9%), p< 0.000). There was a difference in the type of NCSE according to the etiology, with a higher occurrence of NCSE-coma in cases with acute etiology (chi-square test; 30 vs 11, p=0.012). Conclusion: In the elderly with NCSE, there were differences in the clinical variables according to the acute/remote etiology. Previous history of seizures/epilepsy occurred more frequently in the NCSE of remote etiology, and the clinical presentation of coma was more frequent in the elderly with NCSE of acute etiology. Mortality in this sample was high, with no difference according to acute/remote etiology in elderly patients with NCSE. FAPESP 2023/01226-4.

ObjectiveIn Western settings, non‐convulsive status epilepticus (NCSE) and non‐convulsive seizures (NCSz) are associated with high mortality. In comatose patients, interictal epileptiform discharges (IEDs) identified on routine electroencephalogram (EEG) are predictive of NCSE/NCS. Little is known regarding the prevalence, causes, or outcomes of NCSE/NCSz in sub‐Saharan Africa (SSA). We sought to investigate the prevalence of IEDs and NCSE/NCSz at a single teaching institution in SSA.MethodsFrom October 3, 2017, to May 21, 2018, adult inpatients on the internal medicine service at Zambia's University Teaching Hospital (UTH) with a Glasgow Coma Score (GCS) of ≤10 were identified, excluding patients with mechanical ventilation or open head wounds. Signed consent by a proxy was required for enrollment and 30‐minute EEG. Chart abstractions provided coma duration, presence/absence of clinical seizures during/prior to admission, history of epilepsy, and presumed coma etiology. A structured neurological examination was completed. Patients were followed to discharge or death. Risk factors for IEDs were evaluated.ResultsOf 392 eligible patients, 250 had EEGs. EEGs were not completed on eligible patients due to death (74), improved GCS (37), transfer within UTH (25), or lack of proxy (6). NCSE occurred in 22 of 250 (8.8%), NCSz in 3 of 250 (1.2%), and IEDs in 46 of 250 (18.4%) patients. Of the 250, 197 (78.8%) died. No specific risk factors for IEDs were identified.SignificanceIf the association between IEDs and NCSE among monitored populations in developed settings holds true for SSA, a projected 17%‐21% of comatose African adults have NCSE. No clinical characteristics identified those at risk.

The diagnosis of nonconvulsive status epilepticus (NCSE) relies largely on electroencephalography (EEG) findings. The lack of a unified EEG terminology, and of evidence-based EEG criteria, leads to varying criteria for and ability to diagnose NCSE. We propose a unified terminology and classification system for NCSE, using, as a template, the Standardised Computer-based Organised Reporting of EEG (SCORE). This approach integrates the terminology recently proposed for the rhythmic and periodic patterns in critically ill patients, the electroclinical classification of NCSE (type of NCSE) and the context for the pathologic conditions and age-related epilepsy syndromes. We propose flexible EEG criteria that employ the SCORE system to assemble a database for determining evidence-based EEG criteria for NCSE.

Introduction: Status epilepticus is one of the most common neurological emergency, with high morbidity and mortality and an often poor functional prognosis in survivors. We carried out the present study, to describe the intra-hospital clinical course and to identify the risk factors for intra-hospital death of patients hospitalized for status epilepticus in Ouagadougou, Burkina Faso. Patients and methods: it was a prospective, cross-sectional, multi-center hospital, descriptive and analytical study, which took place from January 1, 2015 to December 31, 2019, involving patients consecutively hospitalized in the university hospitals of Ouagadougou, for status epilepticus. Sociodemographic characteristics, clinical data on admission, biological assessment on admission, EEG characteristics, brain scan and / or brain MRI results ; etiological diagnosis of status epilepticus and intra hospital evolutionary data of the patients, have been analyzed. Univariate analysis between the general characteristics of patients and the intra-hospital mortality, was performed in order to identify the risk factors for the intrahospital mortality. Results: The mean age of the patients was 36.6 years with an M / F sex ratio of 1.4 ; 66 patients (72.5%) had no clinical history of epilepsy, while 25 patients (27.5%) were already known to have epilepsy. The generalized tonic-clonic SE immediately (50.5%) and the focal secondarily generalized convulsive status epilepticus (29.7%) were the most common seizure types. The average duration of an SE episode was 18 hours. status epilepticus were subdivided into status epilepticus symptomatic of acute brain conditions with 61 cases (67%), dominated by central nervous system infections and acute strokes, and status epilepticus of non-acute or sequelae etiologies with 30 cases (33%), dominated by the sequelae of head trauma. Intra-hospital complications were dominated by prolonged post-critical mental confusion, 45 cases (49.4%) and aspiration pneumonia, 30 cases (33%). In-hospital mortality was 12.1% (11 patients). At the end of hospitalization, 49 patients (53.9%) still had moderate to severe disabilities. In univariate analysis, comatose non convulsive status epilepticus (p = 0.003), and altered consciousness on admission (p = 0.026), were the risk factors for in-hospital mortality. Conclusion: Prognosis of status epilepticus in Burkina Faso remains unfavorable with early mortality in more than one in 10 patients and poor functional outcome in more than half of the patients. Altered consciousness on admission and non-convulsive comatose status epilepticus were risk factors for early mortality. The fight against infectious diseases, the prevention of vascular risk factors, the promotion of helmet wearing in cyclists and motorcyclists, early admission of patients sufferinf of status epilepticus, and adequate and standardized management of status epilepticus, will help reduce the frequency and severity of this disease in our African regions.

DOOR syndrome concomitant with non-convulsive status epilepticus and hyperintense cerebellar cortex on T2-weighted imaging

100 patients suffering from status epilepticus (SE) were investigated. 63 patients suffered from SE with tonic-clonic seizures, 15 patients from absence status, 19 patients from SE with simple partial seizures and 3 patients from SE with complex partial seizures. SE with tonic-clonic seizures seems to be common, SE with complex-partial seizures a rare type of SE. The latter might, however, often be misdiagnosed because of its nonconvulsive symptomatology. In patients with absence status and SE with complex-partial seizures SE used to occur during the course of a chronic epileptic disorder, whereas in SE with simple partial seizures and SE with tonic-clonic seizures, acute cerebral diseases lead in most cases to SE in patients who had never before suffered from epileptic seizures. Thus, the interval between SE and the onset of epilepsy was in 46 out of 82 patients (56%) with the latter seizure types less than 2 years. Besides etiology, precipitating factors are of great importance in triggering SE, especially alcohol abuse and withdrawal of antiepileptic drug therapy. For the diagnosis of SE the EEG is highly decisive, although epileptiform discharges (generalized spike wave paroxysm) regularly are detected only in absence status. In the other types of SE, EEG signs are often only a slowing of background activity while epileptiform discharges were found in only 29% of the patients. None of the patients died during SE or in the following 4 weeks. Therapy was with benzodiazepines in 45%, phenytoin in 20%, benzodiazepines and Phenytoin in 26%, benzodiazepines, phenytoin and phenobarbetal in 7% and clomethiazol in 2%.

Absence status epilepticus (ASE) is a rare but treatable condition, and when present in older adults, it can be misinterpreted as encephalopathy or behavioral changes. Our case discusses a 63-year-old patient with myelofibrosis and allogeneic stem cell transplant with late-onset de novo status epilepticus. This case report adds to the rare body of literature discussing de novo ASE whose clinical presentation can be indistinguishable from other encephalopathic or behavioral conditions. Moreover, its occurrence during oncologic treatment warrants clinicians to be on the lookout for similar presentations and encourages future reports of this condition in association with similar therapies. This case report provides value to providers treating patients with similar oncologic therapies and highlights the need for ASE to be further studied as it is a possible rare complication of allogeneic transplantation of stem cells.

Electrolytes Disturbances and Seizures

Ring chromosome 20 (r[20]) syndrome is a rare epilepsy-associated chromosomal disorder often accompanied by intellectual disability and psychiatric symptoms. It lacks distinctive physical features and typically exhibits normal brain imaging; therefore, its diagnosis is frequently delayed, especially when clinicians are unfamiliar with it. Nonconvulsive status epilepticus (NCSE), a hallmark of this syndrome, can be overlooked even by epilepsy specialists, who interpret it as a psychiatric symptom, particularly in patients with limited expressive capacity. A 42-year-old woman with a long-standing history of drug-resistant epilepsy and behavioral symptoms was ultimately diagnosed with r(20) syndrome. She developed tonic seizures and fear episodes at age 6 and was diagnosed with epilepsy. After age 11, drug-resistant NCSE emerged and was not clearly identified as status epilepticus; it was considered to involve impaired awareness seizures and psychiatric symptoms. At age 41, perampanel (PER) was initiated, triggering aggressive behavior and new backward-falling episodes, further complicating her clinical presentation. Video electroencephalogram (EEG) confirmed NCSE, and chromosomal analysis identified r(20). After PER discontinuation, aggression and backward-falling episodes resolved. Lacosamide was introduced, reducing the frequency of NCSE from several daily episodes to 2-3 per week. This case underscores the diagnostic challenges of r(20) syndrome and the risk of misdiagnosing NCSE as a psychiatric illness. Additionally, PER may exacerbate psychiatric symptoms in r(20) cases with intellectual disability. Precise seizure classification and confirmation by ictal EEG, along with integrated neuropsychiatric care, are essential for accurate diagnosis and effective management.

De novo status epilepticus is associated with adverse outcome: An 11-year retrospective study in Hong Kong

Non-convulsive status epilepticus in adults: clinical forms and treatment