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Background: Heart failure (HF) requires effective management and self-care education to improve outcomes. However, daily self-care routines necessary for managing HF can lead to psychological issues, including stress, potentially exacerbating the condition. Patient stress-coping behaviors may significantly impact prognosis., Objective: We aimed to identify stress coping styles in patients with HF and examine their impact on self-care behavior and prognosis. s., Methods: This study utilized a longitudinal prospective observational design. Patients were categorized into task-oriented (task), emotion-oriented (emotion), and avoidance (avoidance) coping groups using the Coping Inventory for Stress Situations. Stress levels and self-care behaviors were evaluated during hospitalization and 1 and 3 months after discharge. The primary outcome was a composite of HF readmission and all-cause mortality during the 180-day post-discharge period. Longitudinal changes in self-care behaviors were assessed according to stress intensity., Results: We included 151 patients (age, 74 [60-80] years; female, 37.1 %) hospitalized for HF exacerbation between August 2021 and August 2023. The task group comprised 45 patients (29.8 %); emotion group, 47 (31.1 %); and avoidance group, 59 (39.1 %). The avoidance group had a higher risk of adverse events during follow-up (task, emotion, and avoidance: 8.9 % vs. 14.9 % vs. 32.2 %, p = 0.004). The avoidance-coping style was the only independent predictor of the primary outcome (hazard ratio: 3.84, 95 % confidence interval: 1.40-10.53, p = 0.009). Self-care behaviors were notably poorer in the avoidance group, particularly under conditions of high stress., Conclusions: Stress-coping strategies impact both prognosis and self-care outcomes in patients with HF, and education programs should consider incorporating these strategies., Competing Interests: Declaration of competing interest The authors have no conflicts of interest to declare., (Copyright © 2024. Published by Elsevier B.V.)

Objective: To evaluate the function of the radial nerve in the upper arm using Magnetoneurography (MNG)., Methods: Eight asymptomatic male volunteers (age 25-63 years) and one 67-year-old female patient with radial nerve palsy were included. The radial nerve was electrically stimulated in the right wrist, and the evoked magnetic field was recorded using a 132-channel bio-magnetometer system with a superconducting quantum interference device positioned below the upper arm. The measurements were divided into distal and proximal sessions because of the extensive range of the nerve., Results: MNG recorded the radial nerve's evoked magnetic field in all cases, and the neural activity of the radial nerve at the upper arm was visualized using reconstructed currents. The mean conduction velocity calculated from the peak latency of the inward currents was 43.9 m/s for distal measurements and 57.9 m/s for proximal measurements. A 67-year-old female patient with radial nerve palsy had a disappearance of the reconstructed inward current and conduction disturbance of the axonal current, facilitating the identification of the lesion site., Conclusions: MNG allowed visualization of the radial nerve activity in the upper arm and facilitated the identification of the lesion site in a patient with radial nerve palsy., Significance: This method could be a useful diagnostic tool for patients with radial nerve palsy., Competing Interests: The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (© 2024 International Federation of Clinical Neurophysiology. Published by Elsevier B.V.)

Background: The role of activities of daily living (ADL) as a predictor of adverse outcomes in patients with pneumonia is unclear. This study aimed to assess the association between ADL, including physical and cognitive function, and death or readmission in older inpatients with pneumonia., Methods: This retrospective, single-center, observational study included consecutive older inpatients with pneumonia between October 2018 and December 2019. ADL was assessed using the Functional Independence Measure (FIM). Functional decline during hospitalization was defined as a decrease of at least 1 point in FIM at discharge from admission. The primary outcome was the time to composite 180-day mortality and readmission from any cause after discharge., Results: In total, 363 patients (median [interquartile range] age: 80 [73-86] years, male: 68 %) were divided according to the median FIM scores (≥100, n = 183 and < 100, n = 180). Among the patients, 25 experienced functional decline during hospitalization, 69 were readmitted, and 17 died. In the Kaplan-Meier analysis, both the lower FIM group and the functional decline group had significantly lower event-free rates than the higher FIM groups and the non-functional decline groups (log-rank test, p < 0.001), respectively. After multivariate analysis, both the lower FIM (adjusted HR, 2.11; 95 % CI, 1.24-3.58; p = 0.006) and functional decline (adjusted HR, 3.18; 95 % CI, 1.44-7.05; p = 0.005) were significantly associated with the primary outcome., Conclusions: In older patients hospitalized with pneumonia, ADL limitations at discharge and a decline in ADL were associated with poor outcomes., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 Elsevier Ltd. All rights reserved.)

We herein report a 79-year-old woman with subacute progressive ataxic sensory neuropathy. The patient's symptoms began with numbness in the lower extremities, which rapidly deteriorated, resulting in gait disturbance and abnormal sensations in the extremities, reaching a peak over a period of approximately two months. Nerve conduction studies revealed pure axonal-type sensory polyneuropathy. The expeditious progression of the disease initially prompted suspicion of Guillain-Barré syndrome or paraneoplastic syndrome. Nevertheless, after comprehensive evaluations, the conclusive diagnosis was confirmed as ataxic sensory neuropathy with Sjögren's syndrome. Intensive immunotherapy was administered; however, it was ineffective in halting disease progression. Consequently, this case underscores the significance of an early comprehensive diagnosis and prompt immunotherapy for ataxic sensory neuropathy associated with Sjögren's syndrome.

Electrophysiologic testing plays an important role in evaluating peripheral nerve, muscle, and neuromuscular junction diseases, aiding in diagnosis and treatment strategies by offering real-time assessment. Demyelination of peripheral nerves results in increased conduction delay, temporal dispersion, conduction block, and stimulation threshold. The localization or diffusion of these changes is crucial in understanding disease pathogenesis, necessitating stimulation at multiple points along nerve pathways. When axonal degeneration occurs, the amplitude is reduced, with mild conduction delay. Acute axonal degeneration may require 1 week to develop into Wallerian degeneration. During this time, conductivity was preserved in the nerve peripheral to the lesion. When MG or LEMS is suspected, repetitive nerve stimulation tests and single-fiber EMG are valuable for the diagnosis and pathophysiological evaluation. Notably, the latter is highly sensitive but not specific. Needle electromyography (EMG) assists in differentiating between myopathies and neurogenic diseases, and in determining whether the patient is in an acute or chronic stage. Integration of these tests contribute to an accurate diagnosis when considering the presenting symptoms.

This study investigated the oxygenation in the prefrontal cortex (PFC) during walking in a 75-year-old male patient in the acute phase of a left frontal lobe cerebral infarction complicated by severe left internal carotid artery stenosis. The patient regained independent ambulation on the fifth day after the onset of symptoms, and the study was conducted on the eighth day after the onset of symptoms. The patient rested for 10 s before walking for 70 s. Then he rested for 60 s. The levels of oxyhaemoglobin (O 2 Hb), deoxyhaemoglobin (HHb), and total haemoglobin (THb) in the PFC were quantified during the rest and walking phases using a wearable near-infrared spectroscopy device. Comparative analysis with baseline values during rest revealed decreases in the O 2 Hb, HHb, and THb in both hemispheres of the PFC during walking (O 2 Hb: -19.76/ -14.88 μmol/L, HHb: -1.18/ -2.00 μmol/L, THb: -20.96/ -16.88 μmol/L, right/left, respectively). The recovery of the O 2 Hb was delayed by 15.8 seconds in the affected hemisphere. These findings support the effectiveness of wearable NIRS for the evaluation of cerebral oxygenation during rehabilitation in patients with acute stroke to facilitate evaluations and individualise patient care., (© 2024. Oxygen Transport to Tissue International.)

Oxygenated haemoglobin (O 2 Hb) and total haemoglobin (THb) concentrations rise with increasing exercise load during the cardiopulmonary exercise test (CPX); however, this elevated response is impaired in patients with chronic heart failure. Furthermore, the changes occurring in patients during the acute phase of cardiac surgery are unknown. This study aimed to measure cerebral oxygenation in the prefrontal cortex (PFC) during CPX in patients during the acute post-operative phase following cardiovascular surgery. Fourteen patients in the acute phase of post-cardiovascular surgery period were enrolled. CPX was administered between the post-operative period and discharge. The protocol employed the ramp method (10 W/min) after 3-min rest and 3-min warm-up periods. Levels of O 2 Hb, deoxygenated haemoglobin (HHb), THb, and the regional cerebral oxygen saturation (rSO 2 ) in the PFC were measured from the resting state through the end of CPX using near-infrared spectroscopy. The mean values of O 2 Hb, HHb, and THb levels and rSO 2 were compared at rest, warm-up, anaerobic threshold, and peak points. At the peak, O 2 Hb and rSO 2 declined significantly, and HHb rose significantly compared to the respective values at rest; no significant changes were observed in THb. These findings suggest that the oxygen supply to the PFC is reduced in patients with reduced cardiac function following cardiovascular surgery., (© 2024. Oxygen Transport to Tissue International.)

This study clarifies the effects of head elevation on cerebral oxygenation in patients with acute cerebral infarction. We included 76 patients with anterior circulation cerebral infarction admitted to the stroke care unit within 7 days of symptom onset. Using near-infrared spectroscopy (NIRS), we measured regional saturation oxygen (rSO 2 ), oxygenated haemoglobin (O 2 Hb), deoxygenated haemoglobin (HHb), and total haemoglobin (THb) in both the affected hemisphere (AH) and unaffected hemisphere (UH) after raising the head to 0°, 30°, and 60° for 1 minute each. The mean arterial pressure (MAP) was measured non-invasively at each position. Our result demonstrated a significant decrease in O 2 Hb as the head elevation angle (HEA) increased, with no observable interaction between the AH and UH. A significant decrease in rSO 2 interaction was observed. In contrast, the HHb and THb levels changed significantly as the HEA increased, although no interactions were identified. No significant correlations existed between the observed changes in O 2 Hb, HHb, THb, or rSO 2 and changes in MAP at each HEA., (© 2024. Oxygen Transport to Tissue International.)

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.

Objective: Reconstruct compound median nerve action currents using magnetoneurography to clarify the physiological characteristics of axonal and volume currents and their relationship to potentials., Methods: The median nerves of both upper arms of five healthy individuals were investigated. The propagating magnetic field of the action potential was recorded using magnetoneurography, reconstructed into a current, and analyzed. The currents were compared with the potentials recorded from multipolar surface electrodes., Results: Reconstructed currents could be clearly visualized. Axonal currents flowed forward or backward in the axon, arcing away from the depolarization zone, turning about the subcutaneous volume conductor, and returning to the depolarization zone. The zero-crossing latency of the axonal current was approximately the same as the peak of its volume current and the negative peak of the surface electrode potential. Volume current waveforms were proportional to the derivative of axonal ones., Conclusions: Magnetoneurography allows the visualization and quantitative evaluation of action currents. The currents in axons and in volume conductors could be clearly discriminated with good quality. Their properties were consistent with previous neurophysiological findings., Significance: Magnetoneurography could be a novel tool for elucidating nerve physiology and pathophysiology., (Copyright © 2023 International Federation of Clinical Neurophysiology. Published by Elsevier B.V. All rights reserved.)

Background: Patients with critical COVID-19 often require invasive mechanical ventilation (IMV) and admission to the intensive care unit (ICU), resulting in a higher incidence of ICU-acquired weakness (ICU-AW) and functional decline., Objective: This study aimed to examine the causes of ICU-AW and functional outcomes in critically ill patients with COVID-19 who required IMV., Methods: This prospective, single-center, observational study included COVID-19 patients who required IMV for ≥48 h in the ICU between July 2020 and July 2021. ICU-AW was defined as a Medical Research Council sum score <48 points. The primary outcome was functional independence during hospitalization, defined as an ICU mobility score ≥9 points., Results: A total of 157 patients (age: 68 [59-73] years, men: 72.6%) were divided into two groups (ICU-AW group; n = 80 versus non-ICU-AW; n = 77). Older age (adjusted odds ratio [95% confidence interval]: 1.05 [1.01-1.11], p = 0.036), administration of neuromuscular blocking agents (7.79 [2.87-23.3], p < 0.001), pulse steroid therapy (3.78 [1.49-10.1], p = 0.006), and sepsis (7.79 [2.87-24.0], p < 0.001) were significantly associated with ICU-AW development. In addition, patients with ICU-AW had significantly longer time to functional independence than those without ICU-AW (41 [30-54] vs 19 [17-23] days, p < 0.001). The development of ICU-AW was associated with delayed time to functional independence (adjusted hazard ratio: 6.08; 95% CI: 3.05-12.1; p < 0.001)., Conclusions: Approximately half of the patients with COVID-19 requiring IMV developed ICU-AW, which was associated with delayed functional independence during hospitalization., Competing Interests: Declaration of Competing Interest All authors have nothing to disclose., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Objective: The mechanism underlying the generation of P9 far-field somatosensory evoked potentials (SEPs) is unresolved. Accordingly, we used magnetoneurography to visualize the current distribution in the body at the P9 peak latency and elucidate the origin of P9 generation., Methods: We studied five healthy male volunteers without neurological abnormalities. We recorded far-field SEPs after median nerve stimulation at the wrist to identify the P9 peak latency. Using magnetoneurography, we recorded the evoked magnetic fields in the whole body under the same stimulus conditions as the SEP recording. We analyzed the reconstructed current distribution at the P9 peak latency., Results: At the P9 peak latency, we observed the reconstructed current distribution dividing the thorax into two parts, upper and lower. Anatomically, the depolarization site at the P9 peak latency was distal to the interclavicular space and at the level of the second intercostal space., Conclusions: By visualizing the current distribution, we proved that P9 peak latency originates in the change in volume conductor size between the upper and lower thorax., Significance: We clarified that magnetoneurography analysis is affected by the current distribution due to the junction potential., (Copyright © 2023 International Federation of Clinical Neurophysiology. Published by Elsevier B.V. All rights reserved.)

Background: In older people, frailty has been recognized as an important prognostic factor. However, only a few studies have focused on multidimensional frailty as a predictor of mortality and readmission among inpatients with pneumonia., Objective: The present study aimed to assess the association between preadmission frailty and clinical outcomes after the hospitalization of older patients with pneumonia., Design: Single-center, retrospective case-control study., Setting: Acute phase hospital at Kobe, Japan., Participants: The present study included 654 consecutive older inpatients with pneumonia., Measurements: Frailty status before admission was assessed using total Kihon Checklist (KCL) score, which has been used as a self-administered questionnaire to assess comprehensive frailty, including physical, social, and cognitive status. The primary outcome was a composited 6-month mortality and readmission after discharge., Results: In total, 330 patients were analyzed (median age: 79 years, male: 70.4%, median total KCL score: 10 points), of which 68 were readmitted and 10 died within 6 months. After multivariate analysis, total KCL score was associated with a composited 6-month mortality and readmission (adjusted hazard ratio, 1.07; 95% confidence interval, 1.02-1.12; p = 0.006). The cutoff value for total KCL score determined by receiver operating characteristic curve analysis was 15 points (area under the curve = 0.610). The group with a total KCL score ≥ 15 points had significantly higher readmission or mortality rates than the groups with a total KCL score < 15 points (p < 0.001)., Conclusions: Preadmission frailty status in older patients with pneumonia was an independent risk factor for readmission and survival after hospitalization., Competing Interests: The authors declare no conflict of interest

The pathological hallmarks of Parkinson's disease (PD) are α-synuclein (αSYN)-positive inclusions referred to as Lewy bodies and Lewy neurites, collectively referred to as Lewy-related pathology (LRP). LRP is thought to propagate in an ascending manner throughout the brain as the disease progresses. LRP is visible with histologic methods and is thought to represent a later stage of the disease process, while αSYN oligomers, which are not visible with routine histologic methods, are considered earlier. There is increasing evidence to suggest that αSYN oligomers may be more toxic than visible LRP. Detecting αSYN oligomers requires special techniques, and their distribution and association with clinical features are important research objectives. In this report, we describe the distribution of αSYN oligomers in multiple cortical and subcortical regions of PD using a proximity ligation assay (PLA). We observe widespread distribution of αSYN oligomers with PLA and more restricted distribution of LRP with αSYN immunohistochemistry. The distribution of αSYN oligomers differed from LRP in that αSYN oligomer burden was significantly greater in the neocortex, while LRP was greater in vulnerable subcortical regions, including the brainstem. We also found that cognitive impairment was associated with αSYN oligomers in the hippocampus. These results suggest that αSYN oligomers may be widely distributed in PD early in the disease process and that they may contribute to cognitive impairment in PD., (© 2022. The Author(s).)

Study Design: A prospective analysis., Objective: To test if threshold-based monitoring of compound muscle action potentials (CMAPs) by stimulating the screw loaded to uninsulated extender sleeve provides a valid safety warning for percutaneous pedicle screw (PPS) placements in the lumbosacral spine., Summary of Background Data: Utility of the CMAP monitoring to PPS procedures remains controversial., Methods: A series of 202 patients underwent a total of 1664 lumbosacral PPS placements under CMAP monitoring without fluoroscopic guidance. The monitoring consisted of stimulating the PPS assembled to uninsulated extender sleeve and recording CMAPs from the vastus medialis, biceps femoris, tibialis anterior, and medial gastrocnemius. Automated steps of a threshold hunting algorithm using 0.2-ms duration pulses of increasing intensities delivered at 2/s allowed quick determination of a minimum stimulation current to evoke >100-μV amplitude CMAPs., Results: At L2 through S1 spines, postoperative CT scans identified 51 medial or inferior pedicle wall breaches of 1536 screws (3.3%) without neurologic complications. The receiver operating characteristic curve analysis determined the critical cutoff threshold value of 27 mA (74% sensitivity and 95% specificity) for predicting 35 breaches of 627 screws (5.6%) at L2 and L3, and of 17 mA (100% sensitivity and 98% specificity) for 16 of 909 (1.8%) at L4 through S1. While advancing the screw, three breaches (5.9%) showed a particularly low threshold of ≤6-mA, allowing the surgeon to immediately redirect the screw and retest the new trajectory as safe., Conclusion: Screw stimulation with threshold hunting algorithm has a distinct advantage over the time-consuming insulated pilot hole stimulation, allowing an uninterrupted flow of the surgery. The present findings have documented practical usefulness and reliability of CMAP monitoring using direct stimulation of the PPS assembled to uninsulated extender sleeve., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

The impact of prehospital notification by emergency medical services (EMS) on outcomes of endovascular therapy (EVT) for large vessel occlusion (LVO) remains unclear. We therefore explored the association between prehospital notification and clinical outcomes after EVT. In this single-center retrospective study from 2016 through 2020, we identified all LVO patients who received EVT. Based on the EMS's usage of a prehospital stroke notification system, we categorized patients into two groups, Hotline and Non-hotline. The primary outcome was good neurological outcome at 90 days; other time metrics were also evaluated. Of all 312 LVO patients, the proportion of good neurological outcomes was 94/218 (43.1%) in the Hotline group and 8/34 (23.5%) in the Non-hotline group (adjusted odds ratio 2.86; 95% confidence interval 1.12 to 7.33). Time from hospital arrival to both tissue plasminogen activator and to groin puncture were shorter in the Hotline group (30 (24 to 38) min vs 48(37 to 65) min, p < 0.001; 40 (32 to 54) min vs 76 (50 to 97) min, p < 0.001), respectively. In conclusion, prehospital notification was associated with a reduction in time from hospital arrival to intervention and improved clinical outcomes in LVO patients treated with EVT., (© 2022. The Author(s).)

Background: There is little evidence regarding relevant clinical findings for the early diagnosis of basilar artery occlusion (BAO) in the prehospital setting. We focused on "convulsive-like symptoms", including convulsive seizures and other convulsive-like movements, and examined the frequency and clinical characteristics of patients with BAO having these symptoms as an initial symptom., Methods: In this single-center case series from 2015 to 2020, we identified patients who underwent endovascular therapy (EVT) for BAO and presented with convulsive-like symptoms between the stroke onset and initiation of emergency medical care. The clinical course and neurological findings were evaluated by reviewing the run sheets of emergency medical services and medical records., Results: Among a total of 32 patients with BAO, 7 (21.9%) developed convulsive-like symptoms before EVT, of whom 6 were men and whose median age was 72 (interquartile range, 69-78) years. These 7 patients had no history of epilepsy or stroke, and the semiology of convulsive-like symptoms was generalized in 6 of them. In only 3 of the 7 cases, emergency medical services could consider the possibility of stroke on scene, and time from hospital arrival to groin puncture was longer in those who were transported without suspicion of stroke., Conclusions: 21.9% of our patients who underwent EVT for BAO experienced convulsive-like symptoms initially. We should be vigilant in the possibility of BAO when managing the first-time generalized convulsive-like symptoms occurring in older patients, which may enable to adequate triage and better management for patients with acute BAO., Competing Interests: Hirotoshi Imamura received lecture fees from Stryker Japan K.K. and Medtronic Japan Co., Ltd.; Nobuyuki Sakai received lecture fees from Stryker Japan K.K., Medtronic Japan Co., Ltd., Daiichi Sankyo Company, Limited, Asahi Intecc Co., Ltd., and Biomedical Solutions, Inc., (© 2022 The Authors.)

Importance: The effectiveness of currently approved drugs for amyotrophic lateral sclerosis (ALS) is restricted; there is a need to develop further treatments. Initial studies have shown ultrahigh-dose methylcobalamin to be a promising agent., Objective: To validate the efficacy and safety of ultrahigh-dose methylcobalamin for patients with ALS enrolled within 1 year of onset., Design, Setting, and Participants: This was a multicenter, placebo-controlled, double-blind, randomized phase 3 clinical trial with a 12-week observation and 16-week randomized period, conducted from October 17, 2017, to September 30, 2019. Patients were recruited from 25 neurology centers in Japan; those with ALS diagnosed within 1 year of onset by the updated Awaji criteria were initially enrolled. Of those, patients fulfilling the following criteria after 12-week observation were eligible for randomization: 1- or 2-point decrease in the Revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R) total score, a percent forced vital capacity greater than 60%, no history of noninvasive respiratory support and tracheostomy, and being ambulatory. The target participant number was 64 in both the methylcobalamin and placebo groups. Patients were randomly assigned through an electronic web-response system to methylcobalamin or placebo., Interventions: Intramuscular injection of methylcobalamin (50-mg dose) or placebo twice weekly for 16 weeks., Main Outcomes and Measures: The primary end point was change in ALSFRS-R total score from baseline to week 16 in the full analysis set., Results: A total of 130 patients (mean [SD] age, 61.0 [11.7] years; 74 men [56.9%]) were randomly assigned to methylcobalamin or placebo (65 each). A total of 129 patients were eligible for the full analysis set, and 126 completed the double-blind stage. Of these, 124 patients proceeded to the open-label extended period. The least square means difference in ALSFRS-R total score at week 16 of the randomized period was 1.97 points greater with methylcobalamin than placebo (-2.66 vs -4.63; 95% CI, 0.44-3.50; P = .01). The incidence of adverse events was similar between the 2 groups., Conclusions and Relevance: Results of this randomized clinical trial showed that ultrahigh-dose methylcobalamin was efficacious in slowing functional decline in patients with early-stage ALS and with moderate progression rate and was safe to use during the 16-week treatment period., Trial Registration: ClinicalTrials.gov Identifier: NCT03548311.

Immunoglobulin G therapy for chronic inflammatory demyelinating polyneuropathy (CIDP) often requires individual dose adjustments because of the heterogeneity of pathogenesis and varying catabolic rates. However, currently available pharmacokinetic studies of immunoglobulin G therapy do not consider individual differences. We conducted a pharmacokinetic study of both intravenous immunoglobulin and subcutaneous immunoglobulin in a single patient with CIDP who was dependent on high-dose immunoglobulin treatment. This patient-a 77-year-old man with symmetrical limb weakness, diffuse demyelination determined by a nerve conduction study, and lacking autoantibodies-was treated with intravenous immunoglobulin and experienced severe fluctuations in symptoms. We transitioned him to subcutaneous immunoglobulin: his serum immunoglobulin G levels stabilised and he experienced symptomatic relief. Monitoring of serum immunoglobulin G concentrations revealed volatile changes following intravenous immunoglobulin administration which stabilised following subcutaneous immunoglobulin treatment. This suggests that subcutaneous immunoglobulin is a preferable long-term treatment option, especially for high-dose immunoglobulin-dependent patients with CIDP., Competing Interests: The authors report no conflict of interest., (© 2022 The Authors.)

We herein report two P/Q-type voltage-gated calcium channel (VGCC) antibody-positive Lambert-Eaton myasthenic syndrome (LEMS) patients who responded dramatically to cholinesterase inhibitors. Patient 1, a 76-year-old man, had small-cell lung cancer and developed LEMS during chemotherapy. When symptomatic treatment was started with pyridostigmine, gait disturbance was ameliorated, and his modified Rankin scale decreased from 4 points to 3 points. Patient 2, a 68-year-old man, had cancer-free LEMS. Distigmine bromide was very effective and ameliorated not only his gait disturbance but also autonomic symptoms, and his modified Rankin scale decreased from 2 points to 1 point. Cholinesterase inhibitors alone may be effective in a small portion of LEMS patients.

Coronavirus disease 2019 (COVID-19) patients have been increasingly reported to develop various neurological manifestations. We herein present a rare case of bilateral facial nerve palsy in a patient that occurred 5 weeks after the onset of COVID-19. The patient had no motor or sensory deficits in his extremities, and there were no other diseases that may have resulted in bilateral facial palsy. Based on these findings, we concluded that the facial palsy in this case may have been triggered by COVID-19.

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.

Activities of daily living (ADL) are important prognostic factors for heart failure. The functional independent measure (FIM) has emerged as a comprehensive valid measure of ADL from both physical and cognitive perspectives. This study aimed to investigate the prognostic impact of the FIM score on clinical outcomes in hospitalized patients with acute decompensated heart failure (ADHF). We retrospectively analyzed 473 ADHF patients, with available pre-discharge FIM scores, admitted to our institution between May 2018 and May 2020. Primary outcome measures, defined as a composite of 180-day all-cause deaths and readmissions, were compared among three tertiles. The median FIM score was 102 (interquartile range: 85-115). Tertile 1 corresponded to an FIM score > 111 (n = 154), Tertile 2 to that of 90-111 (n = 167), and Tertile 3 to that of < 90 (n = 152). During follow-up, 28 deaths and 114 readmissions occurred. Patients with lower FIM scores were associated with a graded increase in the risk of primary outcome measure (p = 0.001). Even after multivariable adjustment, the results remained significant [Tertile 1 vs 3; adjusted hazard ratio: 3.28 (95% confidence interval: 1.72-6.56), p < 0.001; Tertile 2 vs 3; 2.32 (1.27-4.47), p = 0.006]. FIM scores were significantly associated with readmission or death within 180 days of discharge in hospitalized ADHF patients., (© 2021. Springer Japan KK, part of Springer Nature.)

Aim: This study aimed to assess the association between physical frailty and clinical outcomes among older patients hospitalized for pneumonia., Methods: This study examined 852 consecutive patients hospitalized for pneumonia between October 2018 and September 2020. Patients who were <65 years old, scheduled for admission, did not receive inpatient rehabilitation, or died during admission were excluded. A short physical performance battery (SPPB) test was performed by physical therapists upon discharge. The primary outcome measure was a composite endpoint of readmission or mortality due to any cause within 6 months of discharge., Results: In total, 521 patients (median age, 80 years; interquartile range, 74-86 years) were included in the analyses, and were divided into the following two groups: robust group with SPPB scores >9 (n = 150), and physical frailty group with SPPB scores ≤9 (n = 371). Of these, 346 (66.4%) patients were men; and the median SPPB score was 6 (interquartile range, 1-10). During the median follow-up period of 53 days (interquartile range, 4-180 days), 92 (17.6%) patients were readmitted and 25 (4.8%) patients died. Patients with physical frailty were at an increased risk for the primary endpoint (hazard ratio, 2.21; 95% confidence interval, 1.44-3.41; P < 0.001); the risk remained significant after adjusting for multiple variables (adjusted hazard ratio, 1.70; 95% confidence interval, 1.05-2.74; P = 0.028)., Conclusions: Among older patients with pneumonia, physical frailty status at discharge was an independent risk factor for readmission and mortality within 6 months after initial discharge. Geriatr Gerontol Int 2021; 21: 926-931., (© 2021 Japan Geriatrics Society.)

Miliary brain metastasis is a rare form of metastasis commonly associated with advanced stages of cancer. In this article, we report a case of a 38-year-old male with solitary progression of miliary brain metastasis originating from stage 4 EML-ALK-positive lung adenocarcinoma. Multiple brain lesions were first detected upon the patient's inclusion in a brigatinib clinical trial. The brain lesions dissipated, and the lung and bone lesions decreased in size after starting the brigatinib therapy; however, the brain lesions later increased in number. A biopsy revealed miliary brain metastasis of lung adenocarcinoma. The patient was successfully treated with whole brain therapy, in addition to brigatinib. This case suggests that it is possible for an isolated miliary brain metastasis to occur during the effective suppression of systemic cancer progression. Whole brain radiotherapy while continuing effective systemic therapy is a good strategy for such patients., Competing Interests: None., (© 2021 The Authors. Published by Elsevier B.V.)

We herein report a 48-year-old woman receiving eribulin mesylate for breast cancer who presented with gait disorder, distal limb paresthesia, and weakness progressing monthly. A nerve conduction study indicated demyelination with multifocal conduction block. Considering the immune-mediated pathology of her condition, she was administered intravenous immunoglobulin. Her neurological symptoms improved promptly after intravenous immunoglobulin therapy and eribulin withdrawal. Furthermore, the limb myokymia seen at the time of admission disappeared. Her symptoms continued to improve without additional treatment. We conclude that eribulin was a rare cause of demyelinating neuropathy with multifocal conduction block derived from immune-mediated pathology.

Aim: Intensive rehabilitation effectively improves physical functions in patients with acute stroke, but the frequency of intervention and its cost-effectiveness are poorly studied. This study aimed to examine the effect of early high-frequency rehabilitation intervention on inpatient outcomes and medical expenses of patients with stroke., Methods: The study retrospectively included 1759 patients with acute stroke admitted to the Kobe City Medical Center General Hospital between 2013 and 2016. Patients with a transient ischemic attack, subarachnoid hemorrhage, and those who underwent urgent surgery were excluded. Patients were divided into two groups according to the frequency of rehabilitation intervention: the high-frequency intervention group (>2 times/day, n = 1105) and normal-frequency intervention group (<2 times/day, n = 654). A modified Rankin scale score ≤2 at discharge, immobility-related complications and medical expenses were compared between the groups., Results: The high-frequency intervention group had a significantly shorter time to first rehabilitation (median [interquartile range], 19.0 h [13.1-38.4] vs. 24.7 h [16.1-49.4], P < 0.001) and time to first mobilization (23.3 h [8.7-47.2] vs. 22.8 h [5.7-62.3], P = 0.65) than the normal-frequency intervention group. Despite higher disease severity, the high-frequency intervention group exhibited favorable outcomes at discharge (modified Rankin scale, ≤2; adjusted odds ratio, 1.89; 95% confidence interval, 1.25-2.85; P = 0.002). No significant differences were observed between the two groups concerning the rate of immobility-related complications and total medical expenses during hospitalization., Conclusions: High-frequency intervention was associated with improved outcomes and decreased medical expenses in patients with stroke. Our results may contribute to reducing medical expenses by increasing the efficiency of care delivery. Geriatr Gerontol Int 2021; 21: 623-628., (© 2021 Japan Geriatrics Society.)

A 73-year-old woman with untreated diabetes mellitus visited our emergency department with a 4-day history of progressive headache, fever, and chills. She received trigger point injections (TPI) into the right sternocleidomastoid for exercise-induced ipsilateral shoulder pain, 13 days before admission and into the right trapezius, 6 days before admission. Cerebrospinal fluid (CSF) evaluation revealed pleocytosis with a predominance of neutrophils, as well as elevated protein and reduced glucose levels. Magnetic resonance imaging of the cervical spine revealed inflammatory changes of the right-sided posterior cervical muscles and the right vertebral arch of the C5-C6 vertebrae without contrast enhancement of the right posterior cervical veins. She was diagnosed with bacterial meningitis and suppurative thrombophlebitis, and empiric broad-spectrum antibiotic therapy was administered intravenously. The initial blood culture yielded Streptococcus intermedius; however, CSF culture showed no growth. She recovered completely after a 4-week course of intravenously administered ampicillin and was discharged with oral clindamycin to complete a total 6-week antibiotic course. TPI are widely used as a safe therapeutic strategy associated with few complications, and serious infections are rare. However, clinicians must remain mindful of the possibility of these complications in immunocompromised patients, such as those with diabetes mellitus who undergo TPI. (Received September 18, 2020; Accepted December 21, 2020; Published June 1, 2021).

Introduction: Idiopathic systemic capillary leak syndrome (ISCLS) is a rare cryptogenic disorder characterized by recurrent hemoconcentration, hypoalbuminemia, edema, and hypotension due to extravascular fluid leakage. This is the first report that details uncommon extensive leukoencephalopathy caused by ISCLS upon a neuropathological investigation., Case Report: A 68-year-old female had recurrent episodes of hemoconcentration, hypoalbuminemia, and generalized edema and was diagnosed with ISCLS. After 9 years, brain magnetic resonance imaging (MRI) incidentally revealed extensive leukoencephalopathy without neurological deficits. Thorough examinations ruled out other disorders, and the cerebral involvement due to ISCLS was finally diagnosed. Three years later, she developed an acute-onset coma and status epilepticus together with hypotension and hemoconcentration, which were compatible with ISCLS recurrence. Electroencephalogram and MRI were correlated with a seizure arising from the left hemisphere. Extensive leukoencephalopathy did not show notable changes for 3 years. Although treatment for ISCLS recurrence temporally improved hemoconcentration and consciousness, consciousness worsened again by marked edema of the left hemisphere, and she died of cerebral herniation. A brain autopsy revealed straggly perivascular plasma leakage around the small vessels of the deep white matter, which supported that the leukoencephalopathy was caused by ISCLS. Widespread myelin pallor and decreased axonal density with sparse astrogliosis and microgliosis were observed in the cerebral white matter and corresponded with a chronic change in the MRI., Conclusion: Current radiological and pathological observations revealed that frequent perivascular leakages could cause chronic leukoencephalopathy, were linked with the development of systemic capillary leakage in ISCLS, and provided insights into the mysterious pathophysiology.

We retrospectively examined the differences between paramedic triage and final diagnosis in the cases that were transported to our hospital between May 2016 and March 2019. About 30% of the patients with suspected stroke were diagnosed other than stroke. Some of the patients without suspected stroke were diagnosed with large vessel occlusion and were treated with mechanical thrombectomy. The time from arrival at the hospital to treatment was significantly longer in the patients without suspected stroke than with suspected stroke. To achieve a better prehospital care, we need to accept a wide range of stroke mimics, and to continuously feedback the paramedics about the importance of paralysis, cortical symptoms in stroke.

Background: Diffusion-weighted imaging (DWI) on magnetic resonance imaging (MRI) shows limited sensitivity in the acute-phase brainstem infarctions, including lateral medullary infarction (LMI), and the detailed characteristics of acute LMI patients with initially negative DWI-MRI findings have not been reported previously. Therefore, we aimed to investigate the differences in the backgrounds or symptoms of acute LMI patients with initially negative findings in standard axial DWI-MRI and those with positive findings., Methods: In this retrospective cohort study, we collected the data for 35 consecutive acute LMI patients who were hospitalized in our hospital from January 2011 to December 2018. Initial standard axial DWI-MRI was assessed, and the patients were divided into positive and negative groups. The characteristics of the two groups were compared, and the usefulness of additional thin-slice coronal DWI-MRI was also investigated., Results: Nine (26%) acute LMI patients were initially negative on standard axial DWI-MRI. The patients were independently associated with smoking history (78% vs. 23%, p = 0.021) and headache (78% vs. 31%, p = 0.046). Thin-slice coronal DWI-MRI showed positive findings in 50% of the patients with negative findings in standard axial DWI-MRI. All four patients with negative findings in both standard axial and thin-slice coronal DWI-MRI had smoking history and headache., Conclusion: Smoking history and headache were associated with initial negative results in standard axial DWI-MRI in acute LMI. Additional thin-slice coronal DWI-MRI was sometimes useful in detecting acute LMI. Follow-up MRI is important for patients showing negative findings in initial DWI-MRI.

Objective: Acute ischemic stroke due to large vessel occlusion (LVO) in hospitalized patients is relatively rare but important condition. However, unlike community-onset cases, there are only few time-saving protocols for in-hospital LVO. This study aimed to evaluate the time-saving effects of rapid response system (RRS) for the management of in-hospital LVO., Methods: We retrospectively evaluated consecutive in-hospital LVO patients who underwent mechanical thrombectomy (MT) between April 2015 and January 2020. In November 2017, we added "acute hemiparesis, eye deviation, and convulsive seizures" to the activation criteria for RRS. In this protocol, the patient is immediately transported from the ward to the emergency room (ER) by Medical Emergency Team (MET). The stroke team can then start assessment in the same manner as for community-onset cases. The time metrics between those with and without RRS intervention were compared. The primary outcome was time from detection to the first assessment by stroke team and to initial CT. To investigate the validity of the revised criteria, we also analyzed all RRS-activated cases., Results: In total, 26 patients (RRS group, 11 patients; non-RRS group, 15 patients) were included. The median time from detection to stroke team assessment (10.0 [interquartile range: IQR, 8-15] minutes vs 65.5 [18-89] minutes) and to CT (22.0 [16-31] minutes vs. 46.5 [35-93] minutes) were significantly shorter in the RRS group. RRS was activated in 34 patients (mean, 1.3/month) according to the added criteria, of whom 20 (58.8%) had cerebral infarction and 9 underwent MT. About two-thirds of the other patients developed neurological emergencies (e.g., epileptic seizure, syncope, or hypoglycemia) that required acute care., Conclusion: RRS has the potential to shorten response time efficiently in the management of in-hospital LVO. Prompt transportation of the patient to the ER by MET enables faster intervention by the stroke team., Competing Interests: Hirotoshi Imamura received lecture fees from Stryker Japan K.K. and Medtronic Japan Co., Ltd.; Nobuyuki Sakai received lecture fees from Stryker Japan K.K., Medtronic Japan Co., Ltd., Daiichi Sankyo Company, Limited, Asahi Intecc Co., Ltd., and Biomedical Solutions, Inc.; Nobuyuki Sakai received consigned research fund from DAIICHI Sankyo Company, Limited, TERUMO Corporation, JIMRO Co., Ltd., and Medtronic Japan Co., Ltd. The other authors declare no conflicts of interest., (©2021 The Japanese Society for Neuroendovascular Therapy.)

Objectives: Bickerstaff'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., Methods: We 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., Results: All 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., Conclusions: Patients 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., Significance: This study revealed characteristic EEG changes possibly caused by the dysfunction of the ARAS, which can be a diagnostic clue for BBE., Competing Interests: The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (© 2020 International Federation of Clinical Neurophysiology. Published by Elsevier B.V.)

Objective: We report a case of sustained atypical myokymia associated with short bursts of neuromyotonic discharges involving the abductor pollicis brevis (APB) muscle and describe a useful way of detecting a focal slowing involving a small number of median nerve motor fibers with a concentric needle using the filter setting for single fiber electromyography (EMG)., Methods and Results: A 62-year-old woman developed right thumb twitches at regular interval of 1.7-3.3 s (0.6-0.3 Hz), which continued for more than four months. Muscle twitches remained the same during altered hand position, psychological stress, or sleep. A concentric needle inserted in the active zone of the APB muscle revealed myokymic bursts with a characteristic of neuromyotonic discharges. Inching study, stimulating at 5 mm increment along the median nerve and recording with a concentric needle using a filter setting for single fiber EMG, revealed a focal slowing of the motor fibers at a point 5-10 mm distal from the distal crease of the wrist, an entrapment site occasionally seen in the carpal tunnel syndrome. One injection of botulinum toxin type A eliminated the myokymia, which then recurred two and a half years later, showing less prominent muscle twitches., Conclusions: Sustained atypical myokymia seen in our case represented bursts of neuromyotonic discharges originated from a focal demyelinating lesion involving a few median nerve motor fibers., (© 2021 International Federation of Clinical Neurophysiology. Published by Elsevier B.V.)

We report the case of a 27-year-old woman with acute internal carotid artery occlusion long after carotid artery revascularization by vein graft. She presented with sudden unconsciousness and left hemiparesis. Her right carotid artery was revascularized with an ipsilateral internal jugular vein graft during a carotid body tumor resection 10 years ago. Computed tomography angiography revealed a right internal carotid artery terminus occlusion. Intravenous rt-PA and mechanical thrombectomy were performed, resulting in successful recanalization. Her neurological symptoms gradually recovered. When examining the embolic source, carotid ultrasonography for the vein graft showed intimal thickening, some high-echoic plaques, and lumen dilation, but no thrombus was observed. Color Doppler imaging showed laminar flow at the graft. Angiography after thrombectomy also showed pooling of contrast at the vein graft. We suspected that the blood flow stagnation at the vein graft induced thrombus formation; therefore, anticoagulation therapy was initiated. One year later, she was independent without recurrence of stroke, and anticoagulation therapy was replaced with aspirin because she went abroad. However, a carotid ultrasonography exam the following year revealed a huge thrombus at the graft. Anticoagulation therapy was resumed; subsequently, the thrombus decreased. In conclusion, we could monitor the long-term change in the vein graft by ultrasonography. Moreover, anticoagulation therapy was more effective., (Copyright © 2020 Elsevier Inc. All rights reserved.)

We present the case of an 81-year-old woman who underwent aortic valve replacement and coronary artery bypass surgery by median sternotomy. Following the operation, she experienced distal muscle weakness in her left upper limb and numbness in the medial part of her left forearm and palm. Nerve conduction study revealed low amplitudes of her left ulnar compound muscle action potential (CMAP) and sensory nerve action potential (SNAP), radial CMAP, and medial antebrachial cutaneous SNAP. Needle electromyography showed denervation potentials in the extensor digitorum communis and abductor pollicis brevis. CT and MRI showed a left first rib fracture and a hematoma nearby. Short-T1 inversion recovery image (STIR) showed a high-intensity area in the left root of C8. Based on these findings, we diagnosed the patient with lower trunk brachial plexopathy due to hematoma.

Background: Muscle mass might be a possible predictor for walking function in patients with stroke; however, evidence is limited., Objective: To investigate whether skeletal muscle mass is associated with walking function at discharge during the acute phase., Methods: In this observational cohort study, we assessed skeletal muscle mass in patients with acute ischemic stroke using the noninvasive and portable multifrequency bio-impedance device. This device can easily be used in bedridden patients. Appendicular skeletal muscle mass was converted to skeletal muscle index (SMI) standardizing by height squared (kg/m 2 ). The primary outcome was walking function assessed by the modified Rankin Scale score at acute phase hospital discharge. Logistic regression analysis was used to determine the association between skeletal muscle mass and walking function., Results: Of the 107 patients enrolled, low SMI (SMI: male <7.0 kg/m 2 , female <5.7 kg/m 2 ) was identified in 29.9% (19.7% in men, 48.6% in women). Logistic regression analysis showed that low SMI [OR: 4.02, 95% confidence interval (CI): 1.38-11.7, p = 0.001] independently associated with walking function at discharge. Further, patients with mild and moderate severity had significant difficulty in walking when they had low SMI (p = 0.039)., Conclusions: Low skeletal muscle mass at the onset of ischemic stroke is an independent predictor of walking function at discharge during the acute phase. Our findings highlight the importance of detecting skeletal muscle mass in patients with acute ischemic stroke., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Background and Purpose: The detection rate of diffusion-weighted (DWI) hyperintense lesions varies widely in patients with transient global amnesia (TGA). The aim was to examine the association of hyperintense lesions on DWI magnetic resonance imaging (MRI) with patient characteristics, precipitating factors, clinical presentation and MRI settings in patients with TGA., Methods: In this multicenter retrospective observational study, using the standardized diagnosis entry system of electronic health records of four tertiary medical centers in the Kansai district of Japan, TGA patients (n = 261) who underwent brain MRI within 28 days of onset were examined. When the onset time was unavailable, the discovery time was used., Results: Diffusion-weighted hyperintense lesions were observed in 79 patients (30%). There were no significant differences in age, sex, vascular risk factors, precipitating factors or clinical presentation between patients with and without DWI lesions. The detection rate increased linearly 24 h after onset and then reached a plateau of 60%-80% by 84 h. After 84 h, the detection rate decreased rapidly. In a multivariate logistic regression model, MRI examination 24-84 h after onset (odds ratio 7.00, 95% confidence interval 3.50-13.99) and a thin-slice (≤3 mm) DWI sequence (odds ratio 7.59, 95% confidence interval 3.05-18.88) were independent predictors of DWI lesions., Conclusions: This study suggests that DWI hyperintense lesions in TGA are not associated with patient characteristics and clinical presentation. Brain MRI examination 24-84 h after onset and thin-slice DWI sequences enhance the detection of DWI lesions in TGA patients., (© 2020 European Academy of Neurology.)

Ninety-six patients were admitted to our hospital during the first wave of the 2020 COVID pandemic. Our hospital, a core hospital in Kobe, was in confusion at the beginning of the pandemic. The following three factors were considered important for preventing the collapse of hospitals during the pandemic based on our experiences: avoidance of contact, prompt and accurate communication, and role-sharing among community medical institutions. Of the 96 patients, 36 had severe cases with several neurological problems: 18 had consciousness disorders, 19 had generalized weakness, 7 had polyneuropathy, and 2 had severe limb weakness. There are several unsolved pathological problems, and neurologists should play important roles in the treatment of patients with COVID.

This is an extremely rare reported case of intravascular large B-cell lymphoma (IVLBCL) presenting with acute hemorrhages and numerous microbleeds. An 80-year-old man presented with consciousness disturbances after convulsion. Computed tomography revealed multiple hemorrhages, and susceptibility-weighted imaging (SWI) demonstrated numerous microbleeds. Brain biopsy showed CD20-positive cells in small vessels; accordingly, IVLBCL was diagnosed. IVLBCL should be considered as a differential diagnosis in multiple cerebral hemorrhages and microbleeds., (Copyright © 2020 Elsevier Inc. All rights reserved.)

We report a case of a 29-year-old woman with spinal muscular atrophy (SMA) type II who developed severe ketoacidosis after short-term starvation. She was hospitalized with lower respiratory tract infection. Although her symptoms improved after administration of intravenous antibiotic agents, her food intake gradually decreased. On the 7th day of hospitalization, she experienced abdominal pain followed by vomiting, after which she was unable to eat. Approximately 12 h later, she suffered from shock, accompanied with disturbance of consciousness, and she was admitted to the intensive care unit. She was diagnosed with ketoacidosis based on arterial blood gas analyses and urine test results. On receiving continuous infusion of glucose and insulin, her ketoacidosis was rapidly resolved and her symptoms completely recovered by the next day. To prevent the recurrence of ketoacidosis, we provided a diet plan based on indirect calorimetry results. However, ketoacidosis recurred twice, at 12 months and 16 months after discharge, both within 24 h of the onset of the fasting state. In addition to insufficient glycogen storage because of chronic malnutrition, poor gluconeogenesis or poor ketone body consumption due to skeletal muscle atrophy was believed to increase the risk of acute-onset, severe ketoacidosis after short-term starvation. Clinicians must note that patients with SMA are prone to ketoacidosis and that they must be promptly treated.

Introduction: Immune-related adverse events (ir-AEs) are increasingly becoming a concern, as immune checkpoint inhibitors (ICIs) are used more frequently. Herein, we present a case of fulminant cytokine release syndrome (CRS) complicated by dermatomyositis after the combination therapy with ICIs., Patient Concerns: A 70-year-old male developed dermatomyositis during the course of treatment with two ICIs, nivolumab and ipilimumab. He was treated by steroid pulse therapy, but the effect was limited. Afterwards, he had acute-onset high fever, hypotension, respiratory failure, impaired consciousness, renal failure, and coagulation abnormality at the same time. C reactive protein (CRP), creatinine kinase (CK), D-dimer, and ferritin levels were considerably elevated: CRP, 24 mg/dL; CK, 40,500 U/L; D-dimer, 290 μg/mL; ferritin, 329,000 ng/mL., Diagnosis: CRS induced by ICI combination therapy., Interventions: Given that high fever and elevated CRP level indicated potential sepsis, an antibiotic was used until the confirmation of negative blood cultures. All the simultaneous acute symptoms were supposed to be CRS. He was admitted to the intensive care unit (ICU), and temporary intubation and hemodialysis were needed. Immunosuppressive therapy was reinforced by mycophenolate mofetil together with steroid, and plasma exchange was performed for the elimination of abnormal proteins., Outcomes: The patient's clinical symptoms and laboratory parameters gradually improved and he was discharged from the ICU in a month., Conclusion: Fulminant CRS can be induced by ICI combination therapy. As the initial symptoms of CRS resemble sepsis, it is important to consider CRS as a differential diagnosis and to initiate immunosuppressive therapy early when needed. In steroid-resistant cases, early introduction of other immunosuppressive therapy and plasma exchange can be effective.

To investigate environmental factors that contribute to ultraviolet A (UVA)-induced oxidative stress, which accelerates the senescence and toxicity of skin cells, we irradiated human fibroblasts cultured in commonly used essential media with UVA and evaluated their viability and production of reactive oxygen species. The viability of fibroblasts exposed to a single dose of 3.6 J/cm 2 UVA was not reduced when cultured in Hanks balanced salt solution, but it was significantly decreased when cultured in Dulbecco's modified Eagle's medium (DMEM), which contains various amino acids and vitamins. Furthermore, cell viability was not reduced when fibroblasts were cultured in DMEM and treated with a hydrogen peroxide (H₂O₂) scavenger such as glutathione or catalase added after UVA irradiation. In addition, we confirmed that the production of H 2 O 2 was dramatically increased by UVA photosensitization when riboflavin (R) coexisted with amino acids such as tryptophan (T), and found that R with folic acid (F) produced high levels of H 2 O 2 after UVA irradiation. Furthermore, we noticed that R and F or R and T have different photosensitization mechanisms since NaN 3 , which is a singlet oxygen quencher, suppressed only R and T photosensitization. Lastly, we examined the effects of antioxidants (L-ascorbic acid, trolox, L-cysteine, and L-histidine), which are singlet oxygen or superoxide or H₂O₂ scavengers, on R and F or on R and T photosensitization, and found that 1 mM ascorbic acid, Trolox, and L-histidine were strongly photosensitized with R, and produced significant levels of H₂O₂ during UVA exposure. However, 1 mM L-cysteine dramatically suppressed H₂O₂ production by UVA photosensitization. These data suggest that a low concentration of R-derived photosensitization is elicited by different mechanisms depending on the coexisting vitamins and amino acids, and regulates cellular oxidative stress by producing H₂O₂ during UVA exposure., Competing Interests: The authors declare no conflict of interest.

Leuconostoc lactis, often found in fermented dairy products, although considered to have a low pathogenic potential, can cause life-threatening infections in immunocompromised hosts. We herein report a 62-year-old man with a history of alcoholic liver cirrhosis, hepatocellular carcinoma, and diabetes mellitus who developed a very rare case of bacterial meningitis caused by this organism. After we administered antibiotics including ampicillin, he recovered completely within two weeks. This gram-positive coccus (GPC) is sensitive to ampicillin but naturally resistant to vancomycin, while its susceptibility to ceftriaxone has not yet been established. In acute GPC meningitis in immunocompromised hosts, Leuconostoc lactis should therefore be considered as a possible pathogen.

Objective: We report a case of reversible cerebral vasoconstriction syndrome (RCVS) that occurred during early pregnancy and improved by endovascular therapy (EVT)., Case Presentation: A 30-year-old Japanese woman at 8 weeks' gestation presented with sudden occipital headache followed by left hemiparesis and convulsion. MRI on admission revealed acute infarction in the distribution of the right posterior cerebral artery (PCA), and MRA demonstrated multi-segmental vasoconstrictions in the posterior circulation. Since the episode and image findings were suggestive of RCVS and the patient was in the organogenesis period, intravenous magnesium sulfate was administered as a vasodilator. Her level of consciousness improved temporarily; however, she suddenly fell into a stupor on day 4 of the illness. Emergency MRI demonstrated a fresh infarction in the left side of pons, and the poorer visualization of the posterior circulation. We proposed selective intra-arterial infusion of nicardipine 1 mg over 1 minute through an indwelling microcatheter in the middle of the basilar artery. The left superior cerebellar artery (SCA) and P1 blood flow improved after the procedure. Her symptoms improved gradually, and follow-up MRA performed on day 15 was almost normal. Hence, we established a definite diagnosis of RCVS. She was discharged to recovery phase rehabilitation hospital with modified Rankin Scale 4 and National Institute of Health Stroke Scale (NIHSS) 5., Conclusion: RCVS can occur in early pregnancy period, and EVT is a potential therapeutic option for RCVS in this condition., Competing Interests: Nobuyuki Sakai received lecture fees from Stryker Japan K.K. and Biomedical Solutions, Inc.; Nobuyuki Sakai received a research grant from TERUMO Corporation; Nobuyuki Sakai received consigned research fund from DAIICHI SANKYO COMPANY, LIMITED, TERUMO Corporation, and JIMRO Co., Ltd. The remaining authors have no conflict of interest related to this work., (©2020 The Japanese Society for Neuroendovascular Therapy.)

Patients with paroxysmal nocturnal hemoglobinuria (PNH) have a clonal population of blood cells deficient in glycosylphosphatidylinositol-anchored (GPI-anchored) proteins, resulting from a mutation in the X-linked gene PIGA. Here we report on a set of patients in whom PNH results instead from biallelic mutation of PIGT on chromosome 20. These PIGT-PNH patients have clinically typical PNH, but they have in addition prominent autoinflammatory features, including recurrent attacks of aseptic meningitis. In all these patients we find a germ-line point mutation in one PIGT allele, whereas the other PIGT allele is removed by somatic deletion of a 20q region comprising maternally imprinted genes implicated in myeloproliferative syndromes. Unlike in PIGA-PNH cells, GPI is synthesized in PIGT-PNH cells and, since its attachment to proteins is blocked, free GPI is expressed on the cell surface. From studies of patients' leukocytes and of PIGT-KO THP-1 cells we show that, through increased IL-1β secretion, activation of the lectin pathway of complement and generation of C5b-9 complexes, free GPI is the agent of autoinflammation. Eculizumab treatment abrogates not only intravascular hemolysis, but also autoinflammation. Thus, PIGT-PNH differs from PIGA-PNH both in the mechanism of clonal expansion and in clinical manifestations.

Immune checkpoint inhibitors (ICIs) are promising drugs for various cancers. However, immune activation by ICIs can lead to immune-related adverse events (irAEs). Autoimmune encephalitis is a rare irAE, and its clinical features remain unknown. We herein report two patients with ICI-associated autoimmune encephalitis who, saliently, showed elevated adenosine deaminase (ADA) levels in the cerebrospinal fluid (CSF). This is the first report of increased ADA levels in the CSF of patients with ICI-induced autoimmune encephalitis. Although the mechanism of the ADA increase is poorly understood, elevated ADA in the CSF may be informative in the diagnosis of this rare disorder.

Neuronal intranuclear inclusion disease (NIID) is a progressive neurodegenerative disease that is characterized by eosinophilic hyaline intranuclear inclusions in neuronal and somatic cells. The wide range of clinical manifestations in NIID makes ante-mortem diagnosis difficult 1-8 , but skin biopsy enables its ante-mortem diagnosis 9-12 . The average onset age is 59.7 years among approximately 140 NIID cases consisting of mostly sporadic and several familial cases. By linkage mapping of a large NIID family with several affected members (Family 1), we identified a 58.1 Mb linked region at 1p22.1-q21.3 with a maximum logarithm of the odds score of 4.21. By long-read sequencing, we identified a GGC repeat expansion in the 5' region of NOTCH2NLC (Notch 2 N-terminal like C) in all affected family members. Furthermore, we found similar expansions in 8 unrelated families with NIID and 40 sporadic NIID cases. We observed abnormal anti-sense transcripts in fibroblasts specifically from patients but not unaffected individuals. This work shows that repeat expansion in human-specific NOTCH2NLC, a gene that evolved by segmental duplication, causes a human disease.