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Objectives Status epilepticus (SE) can be associated with neuronal surface antibodies (NS-Abs) but NS-Ab detection rate remains unknown in patients with SE of unclear etiology at symptom presentation but suspected of having an autoimmune etiology (SE suspected autoimmune). We aimed to determine the NS-Ab detection rate and the clinical features that predict the presence of NS-Abs in patients with SE suspected autoimmune. Methods We retrospectively reviewed the clinical information of 137 patients with SE suspected autoimmune who underwent testing for NS-Abs between January 2007 and September 2020. NS-Abs were examined in both serum and cerebrospinal fluid (CSF) obtained at symptom onset with established assays. We classified brain magnetic resonance imaging (MRI) findings into unremarkable, autoimmune limbic encephalitis (ALE) (bilateral abnormalities highly restricted to the medial temporal lobes), ALE-Plus (ALE pattern and additional extramedial temporal lobe abnormalities), multifocal cortico-subcortical (MCS), or other pattern. We compared the clinical features between patients with and without NS-Abs. Results Forty-four patients (32.1%) had NS-Abs, including 35 N-methyl-d-aspartate receptor (NMDAR) (one with concurrent γ-aminobutyric acid B receptor [GABAbR] and α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor [AMPAR]), 5 γ-aminobutyric acid A receptor (GABAaR), 2 leucine-rich glioma-inactivated 1(LGI1), 1 GABAbR, and 1 unknown antigens. Compared with NS-Ab-negative patients, NS-Ab-positive patients were more likely to have a preceding headache (56.8% vs 26.7%), preceding psychobehavioral or memory alterations (65.9% vs 20.4%), involuntary movements (79.5% vs 16.1%), CSF pleocytosis (81.8% vs 62.0%), elevated immunoglobulin G (IgG) index (45.2% vs 15.6%), oligoclonal bands (51.5% vs 9.5%), tumor (47.7% vs 8.6%), and higher APE2 score (median of 9 vs 7), and they were less likely to have an ALE-Plus pattern (2.3% vs 23.7%). However, preceding fever and ALE or MCS pattern were not different between the two groups of patients. Significance When an autoimmune etiology was suspected, there was a relatively high likelihood (one of three patients) of identifying NS-Abs. Some clinical features (preceding symptoms, inflammatory CSF) predict a higher likelihood of finding NS-Ab positivity, but the ALE-Plus MRI pattern is more likely suggestive of NS-Ab negativity.

A 23-year-old previously healthy man (Patient 1) and a 33-year-old woman with a past history of depression (Patient 2) developed neurological symptoms approximately 1 week after receipt of the first COVID-19 mRNA vaccination and deteriorated over the next week. Patient 1 reported nausea, headache, a high fever, and retrograde amnesia. Patient 2 reported visual disturbance, headache, dysarthria, a left forearm tremor, dysesthesia of the mouth and distal limbs, and visual agnosia. PCR test results for SARS-CoV-2 were negative. Complete blood cell count, biochemistry, and antibody test and cerebrospinal fluid test findings were unremarkable. Diffusion-weighted and fluid-attenuated inversion recovery MRI of the brain showed a high signal intensity lesion at the midline of the splenium of the corpus callosum compatible with cytotoxic lesions of the corpus callosum (CLOCCs). High-dose intravenous methylprednisolone improved their symptoms and imaging findings. CLOCCs should be considered in patients with neurological manifestation after COVID-19 vaccination.

Background Eosinophilic granulomatosis with polyangiitis (EGPA) is a vasculitis characterized by abnormally high eosinophils and frequent peripheral neuropathy. Mepolizumab is an approved therapy for EGPA, but its efficacy against peripheral neuropathy remains unknown. Case presentation A 41-year-old woman was admitted in the hospital with dyspnea and neuropathy. Ground glass opacity and infiltrative shadow in the bilateral lungs were evident on chest computed tomography images. Eosinophils were increased in serum, in bronchoalveolar lavage fluid (BALF), and in transbronchial lung biopsy, and bacteria were not detected in BALF. EGPA resulting in severe eosinophilic asthma, sinusitis, pulmonary infiltrates, and peripheral neuropathy was diagnosed. Prednisolone (50 mg/day) caused remission of eosinophilic pneumonia and sinusitis, but not peripheral neuropathy. During prednisolone tapering (7 mg/day, 10 months after treatment), eosinophils were increased, and peripheral neuropathy relapsed. The humanized anti-IL-5 antibody mepolizumab (300 mg) was initially administered, followed by prednisolone. Mepolizumab caused sustained peripheral neuropathy remission and effective prednisolone tapering. Conclusions Introduction of mepolizumab combined with prednisolone may improve peripheral neuropathy.

Various types of cerebrovascular diseases can result in epilepsy in any age, especially in the elderly. Besides well-known cause of epilepsy as large cerebral infarction involving cerebral cortex and intracerebral hemorrhage, there are growing evidences of roles of subcortical infarction, chronic subdural hematoma, and superficial siderosis of the central nervous system in the pathogenesis of epilepsy. We review here the epidemiology and possible predictors of epilepsy in each type of cerebrovascular lesions and summarize the characteristics of semiology and electroencephalography findings in order to take early treatment strategy. Additionally, relevance of acute-symptomatic seizures and status epilepticus to epilepsy is discussed.

We describe a case of Trousseau's syndrome in a patient with lung carcinoma. A 69-year-old man presented with pleural effusion. Further evaluation revealed EGFR mutation-positive non-small cell carcinoma in the upper lobe with extensive lymph node, bone, and brain metastases. Administration of osimertinib, an EGFR tyrosine kinase inhibitor, resulted in partial tumor response, but caused osimertinib-induced pneumonitis 10 weeks later. Prednisolone restrained lung injury progression and was gradually tapered. However, he presented with impaired consciousness and right hemiplegia. Magnetic resonance imaging revealed a left middle cerebral artery M1 segment occlusion. D-dimer level was elevated to 19.5 μg/mL. In the absence of atherosclerotic or cardiogenic thrombi, these findings led to the diagnosis of Trousseau syndrome. Endovascular therapy, but not tissue plasminogen activator, improved his condition with no recurrences. These treatment strategies are crucial to restore function in patients with potentially disabling cerebral infarction due to Trousseau syndrome., Highlights • Systemic anticoagulation for Trousseau syndrome generally has a poor prognosis. • There is no established treatment for cancer-associated cerebral infarction. • We performed endovascular therapy for cerebral infraction due to Trousseau syndrome. • Endovascular therapy was effective for recanalization of the occluded artery. • An NIHSS score of 0 was achieved with no recurrence.

Progressive multifocal leukoencephalopathy (PML) is a fatal demyelinating disease caused by the JC polyomavirus (JCV). Most patients with PML after renal transplantation have had poor outcomes. We describe a patient with PML after renal transplantation who had a good response to the withdrawal of immunosuppressant therapy. We performed quantitative real-time PCR testing for JCV DNA in cerebrospinal fluid (CSF), and assessed mutation of the JC virus genome detected in the CSF. At the same time, we checked cranial magnetic resonance imaging (MRI). Immunosuppressant therapy was discontinued immediately. The MRI scan that followed showed markedly decreased numbers of high intensity signals, and the results of real-time PCR for JCV DNA in CSF became negative. The patient had no other neurological deficits. Withdrawal of immunosuppressant treatment has a beneficial effect on the course of PML after renal transplantation, and quantitative PCR may facilitate the immediate withdrawal of immunosuppressant agents.

Introduction Cardiac autonomic activity is susceptible to various stimuli including light. Power spectrum analysis of heart rate variability (HRV) is a method to quantify cardiovascular control. High frequency component (HF) represents cardiac parasympathetic modulation, and the ratio of low frequency component (LF)/HF is associated with cardiac sympathetic modulation. Here we investigated whether intermittent repetitive photic stimulations in different flash frequencies during conventional electroencephalography (EEG) recordings affect heart rate variability (HRV) in normal adults. Methods Data were obtained from 16 normal adult subjects (8 males), age 36.1 ± 12.1 (mean ± SD) years. EEG was recorded with scalp electrodes set in the International 10–20 system. Electrocardiography (ECG) was simultaneously recorded with electrodes set at both hands. Photic stimulation was performed by placing the stroboscope in front of the subjects’ closed eyes. The frequencies of flashes were 3, 6, 8, 10, 12, 14, 16, 18, and 20 Hz, each delivered for 10 s with the pause of 10 s. HRV indices were analyzed by calculating frequency power of 10 s bins shifted by 1.0 s (maximum entropy method, MemCalc, GMS). Results The maximum value of LF, HF, and LF/HF significantly increased as compared to those before the whole train of photic stimulation (LF before 2.5 ± 0.8 log msec2, maximum 3.4 ± 0.3 log msec2, p Conclusion The current study suggests that photic stimulations during conventional EEG recordings can significantly increase the cardiac autonomic activity in normal adults. Flash frequency can have differential effects on cardiac parasympathetic and sympathetic modulation.

Sporadic Inclusion Body Myositis (sIBM) is a refractory myositis developing in the elderly. Recently, anti-cytosolic 5'-nucleotidase 1A (cN1A) antibodies were reported to be present specifically in sIBM. To assess the clinicopathological features and usefulness of anti-cN1A positivity in muscle specimens, we evaluated a group of Japanese patients with sIBM. Among Japanese patients with myositis who underwent a muscle biopsy from 1991 through 2014 in our hospital, we identified cases of sIBM that met the European Neuromuscular Center (ENMC) criteria. We then analyzed clinical course, pathological findings, and treatment response. Of 282 patients with myositis, 35 (12%) were given a diagnosis of sIBM (24 men and 11 women). The median age at onset was 67 ± 7 (55-81) years, and that at diagnosis was 70 ± 7 years. Remarkably, 5 (14%) patients concurrently had hepatitis C virus (HCV) infection and 4 (11%) had heart disease. Pathologically, we found rimmed vacuoles in 32 (91%) patients and anti-cN1A positivity in perinuclear regions and rimmed vacuoles in 31 (89%) patients. Remarkably, anti-cN1A antibody positivity was significantly higher in sIBM than in polymyositis (30%), dermatomyositis (10%), and morphologically normal cases (0%), suggesting its high specificity for sIBM. Twelve (34%) patients received immunosuppressants, 8 (23%) received prednisone, and 5 (14%) received intravenous immunoglobulins (IVIg). Clinical symptoms transiently improved in 5 patients given prednisone and in 4 patients given IVIg, but these treatments failed to be radical therapies. In our study of Japanese patients with sIBM, anti-cN1A antibody positivity in muscle specimens was highly sensitive and specific pathologically. Therefore, the expression of anticN1A antibody may be greatly useful for the diagnosis of sIBM. Although the concurrent presence of HCV infection and heart disease was notable, other epidemiological characteristics were consistent with the results of previous studies.

Many of the lifespan-related genes have been identified in eukaryotes ranging from the yeast to human. However, there is limited information available on the longevity genes that are essential for cell proliferation. Here, we investigated whether the essential genes encoding DNA-binding transcription factors modulated the replicative lifespan of Saccharomyces cerevisiae. Heterozygous diploid knockout strains for FHL1, RAP1, REB1, and MCM1 genes showed significantly short lifespan. 1H-nuclear magnetic resonance analysis indicated a characteristic metabolic profile in the Δfhl1/FHL1 mutant. These results strongly suggest that FHL1 regulates the transcription of lifespan related metabolic genes. Thus, heterozygous knockout strains could be the potential materials for discovering further novel lifespan genes.

Triorganosilanes, which possess two aryl groups on the silicon atom, undergo the palladium-catalyzed silylation of aryl iodides. Aryl(2-furyl)silanes thus obtained are potentially useful starting materials for carbon-carbon bond-formation reactions in the presence of transition metal catalysts and tetrabutylammonium fluoride., application/pdf