Clinical manifestations and outcomes associated with a high 2HELPS2B score in patients with acute impaired consciousness.

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.

Vagus nerve size determined via ultrasonography is associated with white matter lesions in patients with vascular risk factors.

PURPOSE: The cross-sectional area (CSA) of the cervical vagus nerve (VN), as assessed through ultrasonography, might be linked to autonomic nervous system dysfunction. Hypertension is the primary factor associated with cerebral white matter lesions (WMLs), but there is also evidence of a connection with autonomic nervous system dysfunction. However, the associations between WMLs and VN size are unclear. Our objective was to investigate the associations between WMLs and VN size in patients with vascular risk factors. METHODS: The CSA of the VN was evaluated using carotid ultrasonography in patients with a history of stroke (acute or chronic) and comorbidities (n = 196, 70.2 ± 12.7 years). Common carotid artery (CCA) intima-media thickness and interadventitial diameter (IAD) were also measured. The severity of the WMLs was assessed by the Fazekas classification and Scheltens' scale. RESULTS: The CSA of the right VN (2.08 ± 0.65 mm2) was significantly greater than that of the CSA of the left VN (1.56 ± 0.44 mm2) (P < 0.001). Multiple linear regression analyses revealed that older age, hypertension, increased right CCA IAD, and decreased CSA of the right VN (standardized partial regression coefficient [ß] - 0.226; P < 0.001) were independently associated with the severity of WMLs (Scheltens' scale). A decreased CSA of the left VN was also associated with the severity of WMLs (ß = - 0.239; P < 0.001). CONCLUSION: VN size determined via ultrasonography was associated with the severity of WMLs. While these findings do not establish a causal relationship, they suggest that autonomic nervous system dysfunction is involved in the progression of WMLs.

Redefined giant somatosensory evoked potentials: Evoked epileptic complexes of excitatory and inhibitory components.

OBJECTIVE: Giant somatosensory evoked potentials (SEPs) are observed in patients with cortical myoclonus. Short-latency components (SLC), are regarded as evoked epileptic activities or paroxysmal depolarization shifts (PDSs). This study aimed to reveal the electrophysiological significance of the middle-latency component (MLC) P50 of the SEPs. METHODS: Twenty-two patients with cortical myoclonus having giant SEPs (patient group) and 15 healthy controls were included in this study. Waveform changes in SEPs before and after perampanel (PER) treatment were evaluated in the patient group. The wide range, time-frequency properties underlying the waveforms were compared between the groups. RESULTS: After PER treatment, SLC was prolonged and positively correlated with PER concentration, whereas MLC showed no correlation with PER concentration. Time-frequency analysis showed a power increase (156 Hz in all patients, 624 Hz in benign adult familial myoclonus epilepsy patients) underlying SLC and a power decrease (156 Hz, 624 Hz) underlying MLC in the patient group. CONCLUSIONS: The high-frequency power increase in SLCs and decrease in MLCs clearly reflected PDS and subsequent hyperpolarization, respectively. This relationship was similar to that of interictal epileptiform discharges, suggesting that giant SEPs evoke epileptic complexes of excitatory and inhibitory components. SIGNIFICANCE: MLCs of giant SEPs reflected inhibitory components.

An Early Neurological Indicator of Immune Effector Cell-Associated Neurotoxicity Syndrome.

We herein report a 58-year-old female patient undergoing chimeric antigen receptor T-cell (CAR-T) therapy for refractory diffuse large B-cell lymphoma (DLBCL). Following the CAR-T infusion, the patient experienced Cytokine Release Syndrome (CRS), which was subsequently remitted. However, aphasia was observed five days post-infusion, and a loss of consciousness occurred on the sixth day. Brain MRI revealed a possibly high signal intensity in the mesial temporal region. The patient was diagnosed with immune effector cell-associated neurotoxicity syndrome (ICANS) secondary to CRS and received treatment with dexamethasone, which promptly improved her consciousness. As the diagnosis of ICANS was confirmed following the emergence of aphasia, vigilant cognitive monitoring of cognitive function is crucial in patients following CAR-T therapy.

Irregular Tremulous Movements and Infrequent Seizures: A Clinical-Electrophysiological Diagnosis of Benign Adult Familial Myoclonus Epilepsy.

We report a case involving a 31-year-old male without any known precipitating injuries presenting with involuntary finger movements and rare seizures. There was a noted family history of tremulous movements. Yet the characteristics of his finger movements were irregular and not typical of essential tremor (ET). Electrophysiological examinations, including video EEG, showed no epileptic discharges, and brain MRI results were normal. However, somatosensory evoked potentials (SEP) revealed the presence of giant SEP, and a positive cortical (C)-reflex was observed, leading to a clinical diagnosis of benign adult familial myoclonus epilepsy (BAFME). Management with valproic acid and perampanel resulted in a significant reduction of symptoms. This case highlights the necessity of considering BAFME in the differential diagnosis for atypical tremorous finger movements, especially with a relevant family history, and the critical role of electrophysiological findings indicative of cortical hyperexcitability.

Chronic Visual Abnormality in an Elderly Patient With Temporal Lobe Epilepsy.

A 79-year-old woman visited our department for chronic visual field abnormalities with a floating sensation for two months. Neurological and ophthalmologic examinations yielded normal results, except for brain MRI indicating left hippocampal atrophy. Cognitive function tests were normal. EEG revealed frequent spikes and slow waves in the left frontotemporal region, corroborated by reduced accumulation in 123I-iomazenil single photon emission computed tomography. A diagnosis of temporal lobe epilepsy was established, and treatment with lacosamide resulted in a remarkable improvement in symptoms and EEG findings. Mild focal seizures from the temporal region might cause mild impaired awareness, resulting in the patient's report as a sensation of uncertainty between the self and the outside world, mimicking ophthalmologic abnormalities. The repeated nature of the seizures contributed to the absence of the term "transient" in symptom description. Diagnosing epilepsy in the elderly proves challenging due to nonspecific complaints.

Mucuna beans administered through hydrogen-infused superheated steam in advanced Parkinson's disease.

This retrospective review on patients with Parkinson's disease, focusing on using mucuna beans (MB), its dosing, and administration methods. Two hundred patients taking 1-3 g of MP dissolved in hot water daily orally. Besides, MB administration via enema may be viable, especially when oral L-dopa efficacy is insufficient.

Emergency medical service response for cases of stroke-suspected seizure: A population-based study.

OBJECTIVES: We evaluated the on-scene time of emergency medical services (EMS) for cases where discrimination between acute stroke and epileptic seizures at the initial examination was difficult and identified factors linked to delays in such scenarios. MATERIALS AND METHODS: A retrospective review of cases with suspected seizure using the EMS database of fire departments across six Japanese cities between 2016 and 2021 was conducted. Patient classification was based on transport codes. We defined cases with stroke-suspected seizure as those in whom epileptic seizure was difficult to differentiate from stroke and evaluated their EMS on-scene time compared to those with epileptic seizures. RESULTS: Among 30,439 cases with any seizures, 292 cases of stroke-suspected seizure and 8,737 cases of epileptic seizure were included. EMS on-scene time in cases of stroke-suspected seizure was shorter than in those with epileptic seizure after propensity score matching (15.1±7.2 min vs. 17.0±9.0 min; p = 0.007). Factors associated with delays included transport during nighttime (odds ratio [OR], 1.73, 95 % confidence interval [CI] 1.02-2.93, p = 0.041) and transport during the 2020-2021 pandemic (OR, 1.77, 95 % CI 1.08-2.90, p = 0.022). CONCLUSION: This study highlighted the difference between the characteristics in EMS for stroke and epileptic seizure by evaluating the response to cases with stroke-suspected seizure. Facilitating prompt and smooth transfers of such cases to an appropriate medical facility after admission could optimize the operation of specialized medical resources.

Specific consistency score for rational selection of epilepsy resection surgery candidates.

OBJECTIVE: Degree of indication for epilepsy surgery is determined by taking multiple factors into account. This study aimed to investigate the usefulness of the Specific Consistency Score (SCS), a proposed score for focal epilepsy to rate the indication for epilepsy focal resection. METHODS: This retrospective cohort study included patients considered for resective epilepsy surgery in Kyoto University Hospital from 2011 to 2022. Plausible epileptic focus was tentatively defined. Cardinal findings were scored based on specificity and consistency with the estimated laterality and lobe. The total points represented SCS. The association between SCS and the following clinical parameters was assessed by univariate and multivariate analysis: (1) probability of undergoing resective epilepsy surgery, (2) good postoperative seizure outcome (Engel I and II or Engel I only), and (3) lobar concordance between the noninvasively estimated focus and intracranial electroencephalographic (EEG) recordings. RESULTS: A total of 131 patients were evaluated. Univariate analysis revealed higher SCS in the (1) epilepsy surgery group (8.4 [95% confidence interval (CI) = 7.8-8.9] vs. 4.9 [95% CI = 4.3-5.5] points; p < .001), (2) good postoperative seizure outcome group (Engel I and II; 8.7 [95% CI = 8.2-9.3] vs. 6.4 [95% CI = 4.5-8.3] points; p = .008), and (3) patients whose focus defined by intracranial EEG matched the noninvasively estimated focus (8.3 [95% CI = 7.3-9.2] vs. 5.4 [95% CI = 3.5-7.3] points; p = .004). Multivariate analysis revealed areas under the curve of .843, .825, and .881 for Parameters 1, 2, and 3, respectively. SIGNIFICANCE: SCS provides a reliable index of good indication for resective epilepsy surgery and can be easily available in many institutions not necessarily specializing in epilepsy.

Therapeutic Time Window With DWI-ADC (Diffusion-Weighted Imaging-Apparent Diffusion Coefficient) Match and Periodic Discharges for Status Epilepticus.

A man in his 70s with alcoholic dementia was admitted for acute, prolonged impaired consciousness. Blood and cerebrospinal fluid findings were unremarkable. Brain MRI revealed multiple high-signal cortical regions. Following diazepam and levetiracetam administration, electroencephalography (EEG) revealed <1 Hz lateralized periodic discharges, indicating that the seizures were ceasing. The periodic discharges had disappeared during the gradual recovery process by day 10; however, cortical arterial spin labeling findings persisted only in regions exhibiting cytotoxic edema. Without additional anti-seizure medication, no seizure recurred, but cognitive dysfunction remained. He was transferred to a rehabilitation hospital with the continued oral administration of levetiracetam at 1,000 mg/day. DWI-ADC (diffusion-weighted imaging-apparent diffusion coefficient) match may suggest an indication of a missed suitable treatment window for seizures.

On-scene time delays for epileptic seizures in developed community-based integrated care system regions.

BACKGROUND: Delayed on-scene time by emergency medical services (EMS) can have detrimental effects on critical cases for people with epilepsy (PWE). In preparation for a super-aged society, a Community-based Integrated Care System is crucial to manage healthcare costs. However, sufficient coordination irrespective of sociomedical changes among medical providers is challenging. AIM: This study aimed to evaluate on-scene time delays in the treatment of PWE, identify factors associated with such delays, and clarify regional differences. The focus was on the volume of acute care beds in regions with a developed Community-based Integrated Care System. METHODS: This population-based observational study evaluated on-scene time delays in the treatment of PWE across six major cities in western Japan between 2017 and 2021. In addition, we also evaluated the association between regional differences focusing on volume of acute care beds ("Reduced region" and "Preserved region", as cities with numbers of acute care beds per 1,000 people below and above the national average, respectively) along with sociomedical factors associated with on-scene time delays. RESULTS: This study included 8,737 PWE transported by EMS, with a mean on-scene time for EMS ranging from 12.9 ± 6.8 min to 21.7 ± 10.6 min. On-scene time delays were evident in Reduced regions, with an increase of 1.45 min (95 % confidence interval 0.86-2.03 min, p < 0.001). A high total EMS call volume independently influenced on-scene time delays during the middle period of the pandemic in Reduced regions. CONCLUSION: Optimal coordination must be facilitated to ensure the effective functioning of the Community-based Integrated Care System, particularly during unusual circumstances.

Attacks With Urinary Incontinence Without Convulsions: Complete Atrioventricular Block Mimicking an Epileptic Seizure.

Differentiating between syncope and epileptic seizures can be challenging when a specific medical history is not available. We herein report a 70s man who exhibited recurrent, brief unresponsiveness while at rest on five occasions over a year. While there were no convulsions, the patient consistently reported urinary incontinence. These events were preceded by an epigastric rising sensation without chest symptoms, suggesting a possible diagnosis of temporal lobe epilepsy, and subsequent EEG revealed temporal semi-rhythmic delta activity. In contrast, the ECG revealed a left bundle branch block, while the initial Holter ECG showed no abnormalities. However, subsequent follow-up examinations revealed a complete atrioventricular block necessitating permanent pacemaker implantation. It is important to exercise caution in the interpretation of EEG findings. Moreover, instances of 'urinary incontinence without convulsion' may indicate non-epileptic events.

Exacerbation of Repetitive Falls Due to Atonic Seizures Following Perampanel Administration.

A 47-year-old man presented with tonic-clonic seizures characterized by convulsions. He repeatedly exhibited seizures despite treatment with four anti-seizure medications. During the titration process of perampanel (PER), the seizures paradoxically increased in intensity and frequency, resulting in trauma. Video electroencephalogram monitoring revealed interictal rapid rhythms and generalized spikes and documented atonic seizures. Thus, the patient was diagnosed with Lennox-Gastaut syndrome. Upon discontinuation of PER, the patient's atonic seizures with falls improved, probably suggesting a paradoxical effect of PER. A non-competitive antagonist selective for AMPA (α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid) receptors may have caused the weakness and delayed recovery from prolonged atonia that caused injuries.

Paradoxical effects of levetiracetam in people with epilepsy with rhythmic epileptiform discharges.

OBJECTIVE: To elucidate the incidence and risk factors for paradoxical effects (i.e., increased seizure frequency, increased seizure severity, or onset of new seizure types) of levetiracetam (LEV) in people with epilepsy (PWE) and identify the usefulness of electroencephalography (EEG) in predicting these effects. METHODS: We examined data for consecutive PWE treated with LEV. All PWE underwent EEG and magnetic resonance imaging (MRI) before LEV administration. We also evaluated the incidence of paradoxical LEV effects and conducted multivariate logistic regression analyses to identify the associated factors. RESULTS: In total, 210 (66.2%) of 317 PWEs treated in our department had a history of LEV use. The incidence of paradoxical LEV effects was 5.2% (n = 11) and was significantly associated with a high LEV dose (p = 0.029), high seizure frequency (p = 0.005), temporal lobe epilepsy (p = 0.004), focal awareness seizure (p = 0.004), focal impaired awareness seizure (p = 0.007), spike (p = 0.015), rhythmic epileptiform discharges (REDs; p = 0.003), and MRI-identified focal cortical dysplasia (FCD; p < 0.0001). Multivariate analyses revealed that REDs (odds ratio [OR] = 5.35, p = 0.048, 95% confidence interval [CI]: 1.01-28.21) were independently associated with paradoxical LEV effects. CONCLUSIONS: Paradoxical LEV effects occurred in PWE, particularly in those with drug-resistant focal epilepsy. Furthermore, the occurrence of REDs in EEG was an independent factor associated with the paradoxical effects of LEV in PWE.

On-scene time delays for epileptic seizures in emergencies during a social pandemic: A population-based study.

OBJECTIVES: The on-scene time of Emergency Medical Services (EMS), including time for hospital selection, is critical for people in an emergency. However, the outbreak of the novel coronavirus disease 2019 (COVID-19) led to longer delays in providing immediate care for individuals with non-COVID-19-related emergencies, such as epileptic seizures. This study aimed to examine factors associated with on-scene time delays for people with epilepsy (PWE) with seizures needing immediate amelioration. MATERIALS & METHODS: We conducted a population-based retrospective cohort study for PWE transported by EMS between 2016 and 2021. We used data from the Hiroshima City Fire Service Bureau database, divided into three study periods: "Pre period", the period before the COVID pandemic (2016-2019); "Early period", the early period of the COVID pandemic (2020); and "Middle period", the middle period of the COVID pandemic (2021). We performed linear regression modeling to identify factors associated with changes in EMS on-scene time for PWE during each period. In addition, we estimated the rate of total EMS call volume required to maintain the same on-scene time for PWE transported by EMS during the pandemic expansion. RESULTS: Among 2,205 PWE transported by EMS, significant differences in mean age and prevalence of impaired consciousness were found between pandemic periods. Total EMS call volume per month for all causes during the same month <5,000 (-0.55 min, 95% confidence interval [CI] -1.02 - -0.08, p = 0.022) and transport during the Early period (-1.88 min, 95%CI -2.75 - -1.00, p < 0.001) decreased on-scene time, whereas transport during the Middle period (1.58 min, 95%CI 0.70 - 2.46, p < 0.001) increased on-scene time for PWE transported by EMS. The rate of total EMS call volume was estimated as 0.81 (95%CI -0.04 - 1.07) during the expansion phase of the pandemic to maintain the same degree of on-scene time for PWE transported by EMS before the pandemic. CONCLUSIONS: On-scene time delays on PWE in critical care settings were observed during the Middle period. When the pandemic expanded, the EMS system required resource allocation to maintain EMS for time-sensitive illnesses such as epileptic seizures. Timely system changes are critical to meet dramatic social changes.

Immediate and 6-month seizure outcomes following first and second SARS-CoV2 mRNA vaccinations: A multicenter study with a nationwide survey.

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.

Reversible Brain Atrophy in Cryptogenic New-onset Refractory Status Epilepticus.

Cryptogenic new-onset refractory status epilepticus (C-NORSE) is a neurologic emergency condition characterized by refractory status epilepticus (RSE) of unknown cause. Brain atrophy in a setting of C-NORSE is usually irreversible. A 33-year-old woman who was highly suspected of C-NORSE once showed mild frontotemporal atrophy on brain magnetic resonance imaging (MRI), but follow-up MRI revealed recovery of the brain atrophy. Her cognitive function also gradually improved, with a reduction in seizure frequency. Early initiation of intensive immunotherapy with anti-seizure medications may have minimized irreversible brain damage associated with RSE, resulting in a relatively good outcome.

Are patients with Parkinson's disease at a lower risk of catching the common cold? Propensity score matching.

INTRODUCTION: Accumulating evidence indicating that inflammatory responses play crucial roles in Parkinson's disease (PD) development provided a hypothesis that physiological alpha-synuclein may contribute to inflammatory responses against infections during non-advanced stages of PD. Thus, we examined the risk of catching a common cold in patients with PD as compared to other common brain diseases. METHODS: We extracted PD (non-advanced; without dementia) and control (AD: Alzheimer's disease, migraine, epilepsy, and ischemic stroke) patient data from insurance claim data available between 2010 and 2021. After confirming the clinical PD diagnosis, we investigated factors associated with cold diagnoses and used propensity score matching to identify differences in the incidence of colds between PD and control patients. RESULTS: Diagnosis of colds in PD patients (n = 726) and controls (AD = 377, migraine = 1019, epilepsy = 3414, ischemic stroke = 6943) was found in 1186 (9.5%) patients, which was independently associated with being female (odds ratio: OR 1.59; 95%CI 1.41-1.79; P < 0.0001), follow-up by neurologists (OR 1.30; 95%CI 1.15-1.48; P < 0.0001), diagnosis of PD (OR 0.30; 95%CI 0.20-0.45; P < 0.0001) and COVID-19 pandemic period (OR 0.58; 95%CI 0.47-0.72; P < 0.0001). After propensity score matching, the incidence of colds was significantly lower in PD (3.4%) versus in controls; AD (9.8%; P < 0.0001), migraine (13.3%; P < 0.0001), epilepsy (11.0%; P < 0.0001), ischemic stroke (8.8%; P < 0.0001). CONCLUSIONS: Patients with PD were less likely to be diagnosed with colds. However, several confounding factors will need to be examined. Moreover, alpha-synuclein may provide protective resistance to viral infections by activating the immune system due to chronic inflammation in non-advanced PD patients.