Anti-myelin oligodendrocyte glycoprotein antibody-associated cerebral cortical encephalitis (MOG-CCE) occasionally exhibits cerebrospinal fluid (CSF) anti-N-methyl-D-aspartate receptor (NMDAR) antibody co-positivity; however, its clinical relevance remains unclear. This study examined the clinical characteristics of NMDAR antibody-positive and NMDAR antibody-negative MOG-CCE to clarify its clinical implications. Consecutive patients with MOG-CCE admitted to our hospital between January 2015 and July 2025 were included. Clinical, laboratory, and imaging findings, as well as treatments and outcomes, were descriptively analyzed according to the CSF anti-NMDAR antibody status. Ten cases with MOG-CCE were included, with five being NMDAR antibody-positive MOG-CCE. Compared to patients with NMDAR antibody-negative MOG-CCE, patients with NMDAR antibody-positive MOG-CCE appeared to have a higher frequency of psychiatric symptoms (80% vs. 20%), abnormal behavior (80% vs. 0%), and movement disorder (60% vs. 0%). Beyond fluid attenuated inversion recovery hyperintensity and gadolinium enhancement, cerebral perfusion imaging demonstrated regional hyperperfusion in most cases, irrespective of CSF anti-NMDAR antibody status. Functional outcomes were similar (median modified Rankin Scale at final follow-up 0 vs. 0). This study suggests that cases with NMDAR antibody-positive MOG-CCE may represent a synergistic overlap between MOG-CCE and NMDARE, characterized by NMDARE-like symptoms. Cerebral perfusion evaluation may serve as a supportive tool for the assessment of MOG-CCE.

OBJECTIVE:Infraslow activity (ISA) <.1 Hz is supposed to represent epileptogenicity; however, its activity is rarely recorded in clinical practice. Therefore, we have focused on the previously reported short infraslow activity (SISA) with a time constant (TC) of 2 s, always accompanied by burst suppression in acute anoxic encephalopathy (AnE). As the association between SISA and seizures has not been evaluated, this study aimed to elucidate the ictogenicity of SISA. METHODS:Electroencephalographies (EEGs) were evaluated in 16 consecutive comatose patients who showed burst suppression in 1155 EEGs at two tertiary emergency care facilities. Eight of these patients had acute AnE (acute AnE group), whereas the remaining patients had metabolic encephalopathy or were sedated for status epilepticus (nonacute AnE group). SISA was defined as a waveform with a duration of >3 s and an amplitude >10 μV with a 2 s TC. The SISA amplitude was compared between patients with and without posthypoxic myoclonus or acute symptomatic seizures. RESULTS:SISA was observed in all patients. Acute symptomatic seizure or posthypoxic myoclonus occurred in seven of eight patients in the acute AnE group but none in the nonacute AnE group. The SISA amplitude was significantly higher in seven patients with acute symptomatic seizure or posthypoxic myoclonus than in nine without them. Furthermore, high-amplitude SISA (>120 μV) was specific for acute symptomatic seizure or posthypoxic myoclonus occurrence. SIGNIFICANCE:High-amplitude SISA accompanied by burst suppression is associated with acute symptomatic seizure or posthypoxic myoclonus and can be an ictogenicity marker in critical care patients.

ABSTRACT Convulsive status epilepticus (CSE) is a life‐threatening neurological emergency that requires prompt intervention. While several benzodiazepines are available as first‐line treatments, the optimal choice among them remains unclear. Therefore, we conducted a systematic review and meta‐analysis to evaluate the comparative effectiveness of these benzodiazepines for CSE. We conducted a comprehensive literature search of MEDLINE, the Cochrane Central Register of Controlled Trials, and Igaku Chuo Zasshi. We included randomized controlled trials involving adult patients with CSE who received benzodiazepines as first‐line treatment. Prehospital studies were also considered eligible. Two reviewers independently screened the studies and extracted relevant data. The outcomes were seizure termination, mortality, intubation, favorable neurological outcomes, seizure recurrence, and duration of hospitalization. Risk ratios (RRs) with 95% confidence intervals were calculated using a random‐effects model with inverse variance weighting. Among 3478 studies screened, three randomized controlled trials met the inclusion criteria: two comparing intravenous lorazepam with intravenous diazepam, and one comparing intramuscular midazolam with intravenous lorazepam. Intravenous lorazepam was associated with a higher seizure termination rate (RR 1.24, 95% CI: 1.03–1.49) to intravenous diazepam without an increase in adverse outcomes. Similarly, intramuscular midazolam was associated with higher seizure termination rates than intravenous lorazepam in prehospital settings (RR 1.16, 95% CI: 1.06–1.27). The present study demonstrated that intravenous lorazepam is more effective than intravenous diazepam. Furthermore, intramuscular midazolam may be more associated with higher seizure termination rates than intravenous lorazepam. Further evidence is needed to clarify the efficacy of intravenous lorazepam and both intravenous and intramuscular midazolam in adult patients.

A previously healthy 23-year-old man developed acute-onset fever, headache, and a rapidly deteriorating altered mental status, occurring 11 days after the resolution of self-limiting respiratory symptoms. The cerebrospinal fluid and brain MRI findings were consistent with meningoencephalitis. A recent M. pneumoniae infection was confirmed using nasopharyngeal PCR and serological testing. Despite appropriate antimicrobial therapy, his neurological deterioration progressed. The addition of steroids and intravenous immunoglobulin resulted in a rapid and complete neurological recovery. This case underscores the immune-mediated pathogenesis of M. pneumoniae-associated meningoencephalitis, particularly in cases with a delayed neurological onset following respiratory prodromes. Clinicians should therefore consider early immunotherapy in conjunction with antibiotics, even in adult populations.

A 23-year-old woman with ulcerative colitis (UC) presented with fever, headache, an altered mental status, and bloody stool. A neurological examination, MRI, and electroencephalography revealed right-side dominant encephalopathy. Although a serological analysis revealed a primary cytomegalovirus (CMV) infection, the cerebrospinal fluid (CSF) showed no evidence of a viral load, although the CSF IL-6 levels were markedly elevated (1,670 pg/mL). Encephalopathy rapidly improved with steroid therapy; however, the presence of bloody stool persisted. A colon biopsy revealed concurrent CMV enteritis, which improved with ganciclovir. This case shows that steroid-responsive encephalopathy can occur during a primary CMV infection. Additionally, CMV enteritis complicated by a primary CMV infection should be considered in patients with UC.

EEG is useful for evaluation of pathophysiology and prognostication of neurocritically ill patients, as it provides non-invasive, real-time monitoring of cerebral function. There have been recently a lot of advances in research on critical care EEG according to the American Clinical Neurophysiology Society's Standardized Critical Care EEG Terminology. Based on the latest knowledge, this review discusses clinical utilization of EEG in neurocritically ill patients, including critical care continuous EEG monitoring, and key points of interpretation of critical care EEG, classifying main purposes into three points: detection of electrographic and electroclinical seizures, consideration of special encephalopathies, and evaluation and prognostication of cerebral function. Neurologists should have fundamental ability to read and interpret critical care EEG and support treating physicians in terms of therapeutic strategy.

Anti-leucine-rich glioma-inactivated 1 (LGI1) encephalitis is a treatable form of limbic encephalitis, marked by frequent focal seizures and cognitive decline (particularly memory disturbance); however, it can be difficult to diagnose in patients with subtle cognitive decline. Ictal pouting, a rare seizure feature, has not yet been reported in anti-LGI1 encephalitis. A 73-year-old man with anti-LGI1 encephalitis presented with subacute onset of frequent ictal pouting without apparent cognitive decline. Steroid treatment alone resolved seizures and improved subtle visual memory. Middle-aged and older patients experiencing subacute-onset frequent focal seizures should be thoroughly evaluated for memory disturbances to determine the need for antiLGI1 antibody measurement.

Antibodies to aquaporin-4 (AQP4-Ab) are sensitive and highly specific serum markers of neuromyelitis optica spectrum disorder (NMOSD). A 32-year-old man presenting with left optic neuritis was diagnosed with NMOSD on the basis of AQP4-Ab positivity by enzyme-linked immunosorbent assay (ELISA). Three years later, ELISA AQP4-Ab was also positive when he presented with left leg involuntary movement with right frontal medial cortex hyperintensity on magnetic resonance imaging (MRI). Three years thereafter, he had left facial involuntary movement with hyperintense lesions from the right temporal lobe to the insular cortex on MRI. His serum was negative for AQP4-Ab by ELISA and cell-based assay (CBA), but positive for myelin oligodendrocyte glycoprotein (MOG) antibodies by CBA. We retested his preserved serum; the CBA test was negative for AQP4 but positive for MOG antibodies throughout the entire period. The ELISA AQP4-Ab test result was a false positive, and the patient was diagnosed with MOG antibody-associated disease. The results of ELISA AQP4-Ab should be interpreted with caution, especially at low titers.

Preventing relapse of myelin oligodendrocyte glycoprotein-immunoglobulin G-associated disease (MOGAD) with steroids and immunosuppressants is sometimes difficult. There is no standard treatment for refractory cases. We present the case of a 17-year-old female patient with longitudinally extensive myelitis, asymptomatic bilateral optic neuritis, and positive serum MOG-IgG. While taking steroids and several immunosuppressants during the following 14 months, she suffered from two symptomatic relapses in the cerebrum and spinal cord, and multiple asymptomatic relapses in the cerebrum. The patient was negative for MOG-IgG at the second relapse of myelitis. Subcutaneous ofatumumab has suppressed relapse for 13 months. Ofatumumab can be considered a therapeutic option for refractory MOGAD.

Microbial tests are essential for appropriate management for acute meningitis and encephalitis, but it often takes several days to identify the results of culture tests or PCR. BioFire FilmArray® meningitis/encephalitis panel (ME panel) is a rapid multiplex PCR assay that targets 14 bacteria, viruses, and yeast in 1 hour. In this single-center retrospective study, we reviewed adult patients who underwent ME panel test in parallel with conventional microbial tests from January to August 2021. Eighteen of 70 patients (26%) tested positive by ME panel, of which 8 patients (11%) were helpful in altering treatment strategy. Fifty-two patients (74%) could stop empirical treatment such as acyclovir or antibiotics due to negative results on ME panel. All results of ME panel were same as traditional assays. Use of ME panel can contribute to early diagnosis and treatment.

BACKGROUND AND OBJECTIVES:The functional outcome and mortality of patients with poststroke epilepsy (PSE) have not been assessed in a prospective study. Previous reports have suggested that patients with PSE may suffer from prolonged functional deterioration after a seizure. In this study, we prospectively investigated the functional outcome and mortality of patients with PSE and analyzed the effect of seizure recurrence on the outcomes. METHODS:This is part of the Prognosis of Post-Stroke Epilepsy study, a multicenter, prospective observational cohort study, where 392 patients with PSE (at least 1 unprovoked seizure more than 7 days after the onset of the last symptomatic stroke) were followed for at least 1 year at 8 hospitals in Japan. This study included only PSE patients with a first-ever seizure and assessed their functional decline and mortality at 1 year. Functional decline was defined as an increase in modified Rankin Scale (mRS) score at 1 year compared with baseline, excluding death. The associations between the seizure recurrence and the outcomes were analyzed statistically. RESULTS:A total of 211 patients (median age of 75 years; median mRS score of 3) were identified. At 1 year, 50 patients (23.7%) experienced seizure recurrence. Regarding outcomes, 25 patients (11.8%) demonstrated functional decline and 20 (9.5%) had died. Most patients died of pneumonia or cardiac disease (7 patients each), and no known causes of death were directly related to recurrent seizures. Seizure recurrence was significantly associated with functional decline (odds ratio [OR] 2.96, 95% CI 1.25-7.03, p = 0.01), even after adjusting for potential confounders (adjusted OR 3.26, 95% CI 1.27-8.36, p = 0.01), but not with mortality (OR 0.79, 95% CI 0.25-2.48, p = 0.68). Moreover, there was a significant trend where patients with more recurrent seizures were more likely to have functional decline (8.7%, 20.6%, and 28.6% in none, 1, and 2 or more recurrent seizures, respectively; p = 0.006). DISCUSSION:One-year functional outcome and mortality of patients with PSE were poor. Seizure recurrence was significantly associated with functional outcome, but not with mortality. Further studies are needed to ascertain whether early and adequate antiseizure treatment can prevent the functional deterioration of patients with PSE.

Abstract Poststroke epilepsy is a major ischaemic/haemorrhagic stroke complication. Seizure recurrence risk estimation and early therapeutic intervention are critical, given the association of poststroke epilepsy with worse functional outcomes, quality of life and greater mortality. Several studies have reported risk factors for seizure recurrence; however, in poststroke epilepsy, the role of EEG in predicting the risk of seizures remains unclear. This multicentre observational study aimed to clarify whether EEG findings constitute a risk factor for seizure recurrence in patients with poststroke epilepsy. Patients with poststroke epilepsy were recruited from the PROgnosis of POst-Stroke Epilepsy study, an observational multicentre cohort study. The enrolled patients with poststroke epilepsy were those admitted at selected hospitals between November 2014 and June 2017. All patients underwent EEG during the interictal period during admission to each hospital and were monitored for seizure recurrence over 1 year. Board-certified neurologists or epileptologists evaluated all EEG findings. We investigated the relationship between EEG findings and seizure recurrence. Among 187 patients with poststroke epilepsy (65 were women with a median age of 75 years) admitted to the lead hospital, 48 (25.7%) had interictal epileptiform discharges on EEG. During the follow-up period (median, 397 days; interquartile range, 337–450 days), interictal epileptiform discharges were positively correlated with seizure recurrence (hazard ratio, 3.82; 95% confidence interval, 2.09–6.97; P < 0.01). The correlation remained significant even after adjusting for age, sex, severity of stroke, type of stroke and generation of antiseizure medications. We detected periodic discharges in 39 patients (20.9%), and spiky/sharp periodic discharges were marginally associated with seizure recurrence (hazard ratio, 1.85; 95% confidence interval, 0.93–3.69; P = 0.08). Analysis of a validation cohort comprising 187 patients with poststroke epilepsy from seven other hospitals corroborated the association between interictal epileptiform discharges and seizure recurrence. We verified that interictal epileptiform discharges are a risk factor for seizure recurrence in patients with poststroke epilepsy. Routine EEG may facilitate the estimation of seizure recurrence risk and the development of therapeutic regimens for poststroke epilepsy.

Autoimmune epilepsy (AE) is an inflammatory disease of the central nervous system with symptoms that have seizures that are refractory to antiepileptic drugs. Since the diagnosis of AE tends to rely on a limited number of anti-neuronal antibody tests, a more comprehensive analysis of the immune background could achieve better diagnostic accuracy. This study aimed to compare the characteristics of anti-neuronal antibody-positive autoimmune epilepsy (AE/Ab(+)) and antibody-negative suspected autoimmune epilepsy (AE/Ab(-)) groups. A total of 23 patients who met the diagnostic criteria for autoimmune encephalitis with seizures and 11 healthy controls (HC) were enrolled. All patients were comprehensively analyzed for anti-neuronal antibodies; 13 patients were identified in the AE/Ab(+) group and 10 in the AE/Ab(-) group. Differences in clinical characteristics, including laboratory and imaging findings, were evaluated between the groups. In addition, the immunophenotype of peripheral blood mononuclear cells (PBMCs) and CSF mononuclear cells, particularly B cells and circulating Tfh (cTfh) subsets, and multiplex assays of serum and CSF were analyzed using flow cytometry. Patients with AE/Ab(+) did not show any differences in clinical parameters compared to patients with AE/Ab(-). However, the frequency of plasmablasts within PBMCs and CSF in patients with AE/Ab(+) was higher than that in patients with AE/Ab(-) and HC, and the frequency of cTfh17 cells and inducible T-cell co-stimulator (ICOS) expressing cTfh17 cells within cTfh subsets was higher than that in patients with AE/Ab(-). Furthermore, the frequency of ICOShighcTfh17 cells was positively correlated with that of the unswitched memory B cells. We also found that IL-12, IL-23, IL-6, IL-17A, and IFN-γ levels were elevated in the serum and IL-17A and IL-6 levels were elevated in the CSF of patients with AE/Ab(+). Our findings indicate that patients with AE/Ab(+) showed increased differentiation of B cells and cTfh subsets associated with antibody production. The elevated frequency of plasmablasts and ICOS expressing cTfh17 shift in PBMCs may be indicative of the presence of antibodies in patients with AE.

A 48-year-old woman developed painless bilateral acute visual loss with a progressive visual field defect over 2 weeks. At the initial visit, her best-corrected visual acuity was 6/9 (right) and 6/15 (left). There was a left relative afferent pupillary defect. There was no ptosis, and ocular motility was full. Her retina appeared normal without papilloedema. Her critical flicker fusion frequency was 17 Hz in the right eye and 13 Hz in the left eye (normal range >37 Hz).1 On Humphrey visual field analysis, she had a complete bitemporal hemianopia (figure 1A). MR scan of orbit showed enhanced optic chiasm enlargement with slight extension to the right posterior optic nerve, suggesting a neoplasm or inflammatory process such as sarcoidosis (figure 2A–C).2–4 Her cerebrospinal fluid (CSF) was clear with normal pressure, containing 6 cells/µL (≤5), protein 1.2 g/L (0.15–0.45) and glucose of …

ObjectivesBickerstaff’s brainstem encephalitis (BBE) is a rare post-infectious inflammatory disease, which causes impaired consciousness by the dysfunction of the ascending reticular activating system (ARAS). We aimed to clarify EEG changes possibly caused by the dysfunction of the ARAS in BBE.MethodsWe retrospectively investigated 15 EEGs from 5 patients with definite BBE (i.e., the positivity for serum IgG anti-GQ1b antibodies was mandatory for the diagnosis) admitted to our hospital from January 2014 through December 2019, particularly focusing on whether N1 and N2 sleep patterns were maintained.ResultsAll of the 10 EEGs recorded when patients had consciousness disturbance were abnormal. Stereotypical EEG changes correlating with their level of consciousness were identified: poorly organized posterior dominant rhythms with maintenance of sleep patterns in patients with mild consciousness disturbance (n = 5); predominant N1 and/or N2 sleep patterns even with external stimuli, including spindle coma pattern, in patients with moderate consciousness disturbance (“unarousable sleep-like” EEG) (n = 4); and generalized slow waves without N1 and N2 sleep patterns in patients with severe consciousness disturbance (n = 1). Among 5 patients, 3 (60%) had “unarousable sleep-like” EEG in their clinical course.ConclusionsPatients with BBE showed stereotypical EEG changes correlating with their level of consciousness, mostly with maintenance of N1 and N2 sleep patterns, and often exhibited characteristic “unarousable sleep-like” EEG.SignificanceThis study revealed characteristic EEG changes possibly caused by the dysfunction of the ARAS, which can be a diagnostic clue for BBE.

Objective Various neurological manifestations have been increasingly reported in coronavirus disease 2019 (COVID-19). We determined the neurological features and long-term sequelae in hospitalized COVID-19 patients. Methods We retrospectively studied 95 consecutive hospitalized patients with COVID-19 between March 1 and May 13, 2020. Acute neurological presentations (within two weeks of the symptom onset of COVID-19) were compared between 60 non-severe and 35 severely infected patients who required high-flow oxygen. In the 12 ventilated patients (the most severe group), we evaluated neurological complications during admission, subacute neurological presentations, and neurological sequelae (51 and 137 days from the onset [median], respectively). Results Of the 95 patients (mean age 53 years old; 40% women), 63% had acute neurological presentations, with an increased prevalence in cases of severe infections (83% vs. 52%, p<0.001). Impaired consciousness and limb weakness were more frequent in severe patients than in non-severe ones (0% vs. 49%; p<0.001, and 0% vs. 54%; p<0.001, respectively). In the most severe group (mean age 72 years old; 42% women), 83% of patients had neurological complications [cerebrovascular disease (17%), encephalopathy (82%), and neuropathy (55%)], and 92% had subacute neurological presentations [impaired consciousness (17%), higher brain dysfunction (82%), limb weakness (75%), and tremor (58%)]. Neurological sequelae were found in 83% of cases, including higher brain dysfunction (73%), limb weakness (50%), and tremor (58%). Conclusions Neurological manifestations are common in COVID-19, with the possibility of long-lasting sequelae.

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