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PURPOSE: The 2HELPS2B score is an invaluable tool for assessing seizure risk in critically ill patients with unconsciousness. However, this can be challenging for non-epileptologists to use owing to its reliance on electroencephalogram (EEG) analysis. Thus, identifying clinical manifestations associated with high 2HELPS2B scores is crucial. METHODS: We examined patients who underwent EEG for acute impaired consciousness in the emergency department between 2020 and 2022. We evaluated the clinical manifestations immediately prior to the EEG tests and identified those associated with a 2HELPS2B score ≥ 2. Additionally, we investigated clinical outcomes in accordance with these manifestations and the 2HELPS2B score. RESULTS: A total of 78 patients were included in this study. While the median 2HELPS2B score was 1 (range: 0-6), 13 patients (16.6%) showed electrographic/electroclinical seizures or status epilepticus and 16 patients (20.5%) showed ictal-interictal continuum in their EEGs. Abnormal muscle tonus (p = 0.034) and eye deviation (p = 0.021) were Significantly associated with a 2HELPS2B score ≥ 2. The presence of these manifestations (p < 0.001) and a 2HELPS2B score ≥ 2 (p < 0.001) were both significantly associated with a favorable response to anti-seizure medication. Conversely, patients with a 2HELPS2B score ≥ 2 who exhibited these clinical manifestations were more likely to be non-dischargeable (p = 0.053), have prolonged intensive care unit stays (p = 0.002), or require extended ventilator use (p = 0.082). CONCLUSION: Abnormal muscle tonus and eye deviation were significant manifestations compatible with a 2HELPS2B score ≥ 2 and may indicate an increased risk of seizures or the severity of the epileptic condition.

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The medial side of the operculum is invisible from the lateral surface of cerebral cortex, and its functions remain largely unexplored using direct evidence. Non-invasive and invasive studies have proved functions on peri-sylvian area including the inferior frontal gyrus (IFG) and superior temporal gyrus within the language-dominant hemisphere for semantic processing during verbal communication. However, within the non-dominant hemisphere, there was less evidence of its functions except for pitch or prosody processing. Here we add direct evidence for the functions of the non-dominant hemisphere, the causal involvement of the medial IFG for subjective auditory perception, which is affected by the context of the condition, regarded as a contribution in higher order auditory perception. The phenomenon was clearly distinguished from absolute and invariant pitch perception which is regarded as lower order auditory perception. Electrical stimulation of the medial surface of pars triangularis of IFG in non-dominant hemisphere via depth electrode in an epilepsy patient rapidly and reproducibly elicited perception of pitch changes of auditory input. Pitches were perceived as either higher or lower than those given without stimulation and there was no selectivity for sound type. The patient perceived sounds as higher when she had greater control over the situation when her eyes were open and there were self-cues, and as lower when her eyes were closed and there were investigator-cues. Time-frequency analysis of electrocorticography signals during auditory naming demonstrated medial IFG activation, characterized by low-gamma band augmentation during her own vocal response. The overall evidence provides a neural substrate for altered perception of other vocal tones according to the condition context.

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OBJECTIVE: This study aimed to identify seizure outcomes in people with epilepsy (PWE) following severe acute respiratory syndrome coronavirus 2 (SARS-CoV2) messenger RNA vaccination. METHODS: We examined PWE (n = 332, age ≥ 14 years) treated in four tertiary hospitals between 2021 and 2022 to assess the incidence of seizure worsening following vaccination using closed questions. We identified the clinical factors associated with worsening and 6-month vaccination outcomes. We also conducted a nationwide survey on self-reported seizure worsening using open questions, to which 261 general practitioners from 99 institutes contributed. RESULTS: Of the 282 PWE vaccinated in the four hospitals, 16 (5.7%) exhibited seizure worsening; most of them emerged within 48 h of vaccination and were not sustained. Thus, all PWE were at baseline condition 6 months after their vaccination. PWE with seizure worsening were more significantly associated with focal impaired awareness seizures (p < 0.001), high seizure frequency (p = 0.025), and drug-resistant epilepsy (p = 0.007) at baseline compared to PWE without worsening. Multivariate logistic regression analysis revealed that focal impaired awareness seizures were independently associated with worsening (odds ratio, 7.0; 95% confidence interval, 1.50-32.77). A nationwide survey of 5156 PWE data (real-world data) confirmed an extremely low incidence rate of self-reported seizure worsening (0.43%). SIGNIFICANCE: Some PWE, particularly refractory focal epilepsy, exhibit seizure worsening. However, the worsening events were infrequent, non-sustainable, and probably under-reported by PWE, suggesting that there is little evidence that worsening seizures discourage current and future vaccinations.

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OBJECTIVE: To determine whether magnetoencephalography (MEG) can identify epileptiform discharges mimicking small sharp spikes (SSSs) on scalp electroencephalography (EEG) in patients with temporal lobe epilepsy (TLE). METHODS: We retrospectively reviewed simultaneous scalp EEG and MEG recordings of 83 consecutive patients with TLE and 49 with extra-TLE (ETLE). RESULTS: SSSs in scalp EEG were detected in 15 (18.1%) of 83 TLE patients compared to only two (4.1%) of 49 ETLE patients (p = 0.029). Five of the 15 TLE patients had MEG spikes with concurrent SSSs in EEG, but neither of the 2 ETLE patients. Three of these 5 TLE patients had additional interictal epileptiform discharges (IEDs) in EEG and MEG. Equivalent current dipoles (ECDs) of MEG spikes with concurrent SSSs and IEDs showed no difference in temporal lobe localization and horizontal orientation, whereas ECD moments were smaller in MEG spikes with concurrent SSSs than those with IEDs. CONCLUSIONS: SSSs were more common in TLE than in ETLE. At least some morphologically diagnosed SSSs are true but low-amplitude epileptiform discharges in TLE which can be identified with simultaneous MEG. SIGNIFICANCE: Simultaneous MEG is useful to identify epileptiform discharges mimicking SSSs in patients with TLE.

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Lupus myelitis (LM) is a rare but serious complication of systemic lupus erythematosus (SLE). In 2009, Birnbaum et al. suggested that LM could be classified into two subtypes, namely gray and white matter myelitis, based on neurological examination findings. Here we describe three cases of this disorder, one with signs of white matter dysfunction and two with signs of gray matter dysfunction. We discuss the potential role of autoantibodies in the development of LM.

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Aortic dissection is an infrequent but important cause of acute ischemic stroke (AIS), and must not be overlooked because of a possible worse outcome, especially with the use of an intravenous recombinant tissue plasminogen activator. We report a case of left carotid artery dissection and AIS originating from localized aortic arch dissection, pathologically caused by cystic medial necrosis in the tunica media.

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A 76-year-old woman was admitted to our hospital because of transthyretin-related familial amyloid polyneuropathy (TTR-FAP). She had developed bilateral vitreous opacity at the age of 58 and paroxysmal atrial fibrillation at the age of 62. She suffered gait disturbance and dysesthesia of the limbs at the age of 68 and was diagnosed with FAP involving a homozygous Val30Met mutation in the amyloidogenic transthyretin (ATTR) gene after a genetic test. Her parents were cousins, and her aunt's medical history included pacemaker implantation and polyneuropathy. At the age of 74, the patient developed gait disturbance and dysesthesia of her extremities. A neurological examination revealed visual loss, hearing impairment, distal muscle weakness, dysesthesia, and decreased sensation in all modalities in her extremities. She could neither walk nor remain standing without support. Brain magnetic resonance imaging (MRI) revealed a low intensity lesion on the surface of the cerebellum on T2*-weighted images and susceptibility-weighted images. A low intensity pattern that was indicative of the classical type of superficial siderosis was detected. At the age of 76, when she was admitted to our hospital because of the deterioration of her gait disturbance and dysesthesia, brain MRI showed that the patient's cerebellar atrophy and hemosiderin deposition had worsened. Some reports suggest that FAP patients that are homozygous for the ATTR Val30Met mutation are more likely to develop central nervous involvement than those that are heterozygous for the mutation. Superficial siderosis may be responsible for the central nervous involvement.

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