Serial Nerve Conduction Studies in Guillain-Barré Syndrome: Its Usefulness and Precise Timing.

PURPOSE: Nerve conduction study (NCS) is essential for subclassifying Guillain-Barré syndrome (GBS). It is well known that the GBS subclassification can change through serial NCSs. However, the usefulness of serial NCSs is debatable, especially in patients with early stage GBS. METHODS: Follow-up NCS data within 3 weeks (early followed NCS, EFN) and within 3 to 10 weeks (late-followed NCS, LFN) were collected from 60 patients with GBS who underwent their first NCS (FN) within 10 days after symptom onset. Each NCS was classified into five subtypes (normal, demyelinating, axonal, inexcitable, and equivocal), according to Hadden's and Rajabally's criteria. We analyzed the frequency of significant changes in classification (SCCs) comprising electrodiagnostic aggravation and subtype shifts between demyelinating and axonal types according to follow-up timing. RESULTS: Between FN and EFN, 33.3% of patients with Hadden's criteria and 18.3% with Rajabally's criteria showed SCCs. Between FN and LFN, 23.3% of patients with Hadden's criteria and 21.7% with Rajabally's criteria showed SCCs, of which 71.4% (Hadden's criteria) and 46.2% (Rajabally's criteria) already showed SCCs from the EFN. The conditions of delayed SCCs between EFN and LFN were very early FN, mild symptoms at the FN, or persistent electrophysiological deterioration 3 weeks after symptom onset. CONCLUSIONS: A substantial proportion of patients with GBS showed significant changes in neurophysiological classification at the early stage. Serial NCS may be helpful for precise neurophysiological classification. This study suggests that follow-up NCSs should be performed within 3 weeks of symptom onset in patients with GBS in whom FN was performed within 10 days of symptom onset.

Effect of altered gene expression in lipid metabolism on cognitive improvement in patients with Alzheimer's dementia following fecal microbiota transplantation: a preliminary study.

Background: The brain-gut axis has emerged as a potential target in neurodegenerative diseases, including dementia, as individuals with dementia exhibit distinct gut microbiota compositions. Fecal microbiota transplantation (FMT), the transfer of fecal solution from a healthy donor to a patient, has shown promise in restoring homeostasis and cognitive enhancement. Objective: This study aimed to explore the effects of FMT on specific cognitive performance measures in Alzheimer's dementia (AD) patients and investigate the relationship between cognition and the gut microbiota by evaluating changes in gene expression following FMT. Methods: Five AD patients underwent FMT, and their cognitive function [Mini-Mental State Examination (MMSE), Montreal Cognitive Assessment (MoCA), and Clinical Dementia Rating Scale Sum of Boxes (CDR-SOB)] was assessed before and after FMT. The patients' fecal samples were analyzed with 16S rRNA to compare the composition of their gut microbiota. We also assessed modifications in the serum mRNA expression of patients' genes related to lipid metabolism using serum RNA sequencing and quantitative real-time polymerase chain reaction. Results: Significant improvements in cognitive function, as measured by the MMSE (pre- and post-FMT was 13.00 and 18.00) and MoCA were seen. The MoCA scores at 3 months post-FMT (21.0) were the highest (12.0). The CDR-SOB scores at pre- and post-FMT were 10.00 and 5.50, respectively. Analysis of the gut microbiome composition revealed changes via 16S rRNA sequencing with an increase in Bacteroidaceae and a decrease in Enterococcaceae. Gene expression analysis identified alterations in lipid metabolism-related genes after FMT. Conclusion: These findings suggest a link between alterations in the gut microbiome, gene expression related to lipid metabolism, and cognitive function. The study highlights the importance of gut microbiota in cognitive function and provides insights into potential biomarkers for cognitive decline progression. FMT could complement existing therapies and show potential as a therapeutic intervention to mitigate cognitive decline in AD.

U-shaped associations between glycated albumin and obesity and role of IL-10 in hyperacute ischemic stroke.

OBJECTIVE: There is growing interest in the use of new biomarkers such as glycated albumin (GA). In contrast to glycated hemoglobin (HbA1c), GA showed an inverse correlation with prestroke obesity status, but data are limited for ischemic stroke (IS). MATERIALS AND METHODS: We explored the association between GA and body mass index (BMI) and investigated inflammatory cytokines to support the academic background. In total, 155 patients with hyperacute IS (HIS) between 2011 and 2019 were included. To identify the association between GA and BMI, patients were divided into four groups according to BMI quartiles. Levels of inflammatory cytokines, including IL-1ß, IL-10, IL-6, TNF-α, and TNF-R1, were determined by ELISA using a ProcartaPlex multiplex immunoassay. RESULTS: The mean age of the 155 patients was 68 ± 12 years, and 67.1% were men. The lowest BMI group had higher GA levels (GA 2 T and 3 T = 80%) (p-value=0.017), and these U-shaped associations were maintained only for small vessel occlusion etiology (p-value= 0.004). Plasma IL-10 levels were positively correlated with BMI and showed a U-shaped pattern (p-value= 0.001). CONCLUSION: GA levels and BMI had U-shaped associations with HIS. IL-10, which acts as a protective cytokine for cardiovascular disease, may play a novel role in this association. Although GA is an emerging favorable clinical marker of cardiovascular outcomes, obesity status should be considered when interpreting these associations.

Chronic Hypoxemia Triggers a Neuropathic Process in Chronic Obstructive Pulmonary Disease: Insight From In Vivo Neurophysiological Assessments.

BACKGROUND AND PURPOSE: Peripheral neuropathies (PNs) are a common but poorly understood complication of chronic obstructive pulmonary disease (COPD). To clarify the initial trigger of a PN in COPD, we investigated the excitability of peripheral nerves in patients with COPD. METHODS: The automated nerve excitability test (NET) using the threshold-tracking paradigm was applied to 20 COPD patients. The recording protocol calculated the strength-duration time constant, threshold electrotonus (TE), current-threshold relationship, and recovery cycle (RC). Each NET parameter was compared with two control groups: normal controls group (NC group) and smokers without COPD group (smoker group). RESULTS: In the motor NETs, the change in the threshold in the mid-depolarizing phase of TE (40-60 ms) was smaller in the COPD group (50.7%±1.2%, mean±SEM; n=20) than in the NC group (54.5%±0.7%, n=25; p<0.01), as was the prominence of superexcitability in the RC (-22.6%±1.5% and -26.4%±1.1%, respectively; p=0.04). There were no significant differences in the sensory NETs. Comparisons between the COPD and smoker groups (n=25) also showed no differences in either the motor or sensory NETs. CONCLUSIONS: The pattern of excitability in COPD revealed a membrane depolarization attributable to Na+-K+-ATPase failure in the axolemma of distal motor nerves. This finding suggests that chronic hypoxemia and adaptative process can alter axonal excitability and trigger a resultant neuropathic process that is antecedent to PN in COPD.

Clinical pitfalls and serological diagnostics of MuSK myasthenia gravis.

BACKGROUND: We aimed to evaluate the diagnostic accuracy of enzyme-linked immunosorbent assay (ELISA) for anti-muscle specific tyrosine kinase (MuSK) antibody (Ab) in a large cohort of anti-acetylcholine receptor (AChR) Ab-negative generalized myasthenia gravis (MG), and also to investigate clinical contexts for the diagnosis of MuSK MG. METHODS: A retrospective study of 160 patients with a clinical suspicion of AChR Ab-negative generalized MG was performed. The serum samples were tested for anti-clustered AChR Ab by cell-based assay (CBA), anti-MuSK Ab by ELISA, CBA and/or radioimmunoprecipitation assay (RIPA). Clinical data were compared between anti-MuSK Ab-positive MG and double seronegative (AChR and MuSK) MG groups. RESULTS: After excluding non-MG and clustered AChR Ab-positive patients, we identified 89 patients as a cohort of AChR Ab-negative generalized MG. Anti-MuSK Ab was positive by ELISA in 22 (24.7%) patients. While CBA identified five additional anti-MuSK Ab-positive patients, the results of ELISA were mostly consistent with CBA and RIPA with Cohen's kappa of 0.80 and 0.90, respectively (p < 0.001). The most frequent differential diagnosis was motor neuron disease particularly of bulbar onset which showed remarkably overlapping clinical and electrophysiological features with MuSK MG at presentation. CONCLUSION: While confirming the highest sensitivity of CBA for detecting anti-MuSK Ab, our results highlight the clinical pitfalls in making a diagnosis of MuSK MG and may support a diagnostic utility of MuSK-ELISA in clinical practice.

The usefulness of global longitudinal peak strain and left atrial volume index in predicting atrial fibrillation in patients with ischemic stroke.

Introduction: Detection of atrial fibrillation (AF) is crucial for preventing recurrence in patients with ischemic stroke. We aimed to examine whether the left atrial volume index (LAVI) and global longitudinal peak strain (GLPS) are associated with AF in patients with ischemic stroke. Methods: We prospectively analyzed 678 consecutive patients with ischemic stroke. LAVI and GLPS were assessed using three-dimensional transthoracic echocardiography with speckle-tracking imaging. Multiple logistic regression was used to evaluate the association of AF with LAVI and GLPS. To evaluate the predictive value of LAVI and GLPS for the presence of AF, we used optimism-corrected c-statistics calculated by 100 bootstrap repetitions and the net reclassification improvement (NRI). Results: The mean patient age was 68 ± 13 years (men, 60%). Patients with AF (18%) were a higher LAVI (41.7 ml/m2 vs. 74.9 ml/m2, P < 0.001) and a higher GLPS than those without AF (-14.0 vs. -17.3, P < 0.001). Among the 89 patients classified with embolic stroke of unknown source, the probable cardioembolic group had higher GLPS (n= 17, -14.6 vs. -18.6, respectively; P= 0.014) than the other groups (n= 72). Adding GLPS to age, hypertension, and the LAVI significantly improved the NRI, with an overall NRI improvement of 6.1% (P= 0.03). Discussion: The LAVI andGLPS with speckle-tracking imaging echocardiography may help identify patients with AF.

Genotype and Phenotype Differences in CADASIL from an Asian Perspective.

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a hereditary cerebral small-vessel disease caused by mutations in the NOTCH3 gene. Classical pathogenic mechanisms are associated with cysteine gain or loss, but recent studies suggest that cysteine-sparing mutations might have a potential role as a pathogen. In comparison with CADASIL patients in Western countries, there are several differences in Asian patients: (1) prevalent locus of NOTCH3 mutations (exons 2-6 [particularly exon 4] vs. exon 11), (2) age at symptom onset, (3) prevalence of cerebral microbleeds and hemorrhagic stroke, (4) clinical symptoms, and (5) severity of white matter hyperintensities and typical involvement of the anterior temporal pole in magnetic resonance imaging. Both ethnicity and founder effects contribute to these differences in the clinical NOTCH3 spectrum in different cohorts. More functional investigations from diverse races are needed to clarify unknown but novel variants of NOTCH3 mutations. This review may broaden the spectrum of NOTCH3 variants from an Asian perspective and draw attention to the hidden pathogenic roles of NOTCH3 variants.

Bilateral Facial Weakness with Distal Paresthesia Following COVID-19 Vaccination: A Scoping Review for an Atypical Variant of Guillain-Barré Syndrome.

Background and Purpose: Recent population-based studies from the US and UK have identified an increase in the occurrence of Guillain-Barré syndrome (GBS) following coronavirus disease 2019 (COVID-19) vaccination. However, the localized variant of GBS might be underestimated due to its rarity and atypical features. We aimed to identify and characterize bilateral facial weakness with distal paresthesia (BFWdp) as a GBS variant following COVID-19 vaccination. Materials and Methods: Relevant studies published during the COVID-19 pandemic were searched and identified in the MEDLINE, Embase, and other databases. Results: This review found that 18 BFWdp cases presented characteristics similar to previous BFWdp cases as defined in the literature: male dominance, frequent albuminocytological dissociation, and acute inflammatory demyelinating neuropathy pattern. In contrast, facial nerve enhancement on brain MRI and antiganglioside antibody positivity were often observed in BFWdp following COVID-19 vaccination. Conclusions: The mechanism of BFWdp following COVID-19 vaccination appears to be somewhat different from that of sporadic BFWdp. Neurological syndromes with rare incidence and difficulty in diagnosis should be considered adverse events of COVID-19 vaccination.

Miller Fisher syndrome following COVID-19 vaccines: A scoping review.

BACKGROUND AND PURPOSE: Miller Fisher syndrome (MFS), a variant of Guillain-Barré Syndrome (GBS), could be underestimated in evaluations of its adverse events (AEs) following COVID-19 vaccination. We aimed to identify and characterize MFS following COVID-19 vaccination. MATERIALS AND METHODS: Relevant studies reported on during the COVID-19 pandemic were identified in the MEDLINE, Embase, and other databases. RESULTS: Nine cases of MFS following COVID-19 vaccination from various regions were included. Unlike MFS following COVID-19 infection, patients with MFS following COVID-19 vaccination frequently presented with anti-GQ1b antibody positivity (44%, 4/9). Unlike GBS following COVID-19 vaccination, only two of nine (22%) cases of MFS following COVID-19 vaccination had developed after viral-vector-related vaccine administration. CONCLUSIONS: Miller Fisher syndrome following COVID-19 vaccination seems to have a different pathophysiology from MFS following COVID-19 infection and GBS following COVID-19 vaccination. This neurological syndrome with a rare incidence and difficulty in diagnosis should be considered an AE of COVID-19 vaccination.

Does Essential Tremor Alter the Axonal Excitability Properties of Lower Motor Neurons?

PURPOSE: Automated nerve excitability testing has identified that the altered excitability of lower motor neuron (LMN) axons in central diseases is because of trans-synaptic plasticity. Essential tremor (ET) is considered a central disorder caused by an altered cerebellar circuit. This study aimed to identify alterations in the excitability of distal motor axons in subjects with ET, with the intention of clarifying whether a trans-synaptic mechanism or LMN adaptation for tremor affects the LMNs of subjects with ET. METHODS: Twenty-one consecutive patients diagnosed with ET underwent a clinical and electrophysiological evaluation. For the enrolled cases and 45 age- and gender-matched healthy controls, automated nerve excitability testing with threshold tracking techniques (QTRACS software with TRONDF multiple-excitability protocol) was used to evaluate multiple nerve excitability indices in distal median nerve motor axons. RESULTS: The automated protocol calculated the strength-duration time constant, parameters of threshold electrotonus and current-threshold relationship, and the recovery cycle of excitability. Comparisons of the automated nerve excitability testing parameters revealed no significant differences between the ET and control groups in any of strength-duration time constant, threshold electrotonus, current-threshold relationship, and recovery cycle, whereas the rheobase was higher in the ET group (3.4 ± 1.1 vs. 2.3 ± 1.1, mean ± standard error mean; P < 0.01). CONCLUSIONS: With the exception of an increased rheobase in ET subjects, no significant differences were observed in LMN excitability between the ET subjects and their controls. The extent of plasticity or adaptation in LMNs may be limited to a major change in central processes that exert marked effects on the pool of LMNs.

Bidirectional association between migraine and rheumatoid arthritis: two longitudinal follow-up studies with a national sample cohort.

OBJECTIVE: To investigate the bidirectional association between migraine and rheumatoid arthritis (RA). DESIGN: Two longitudinal follow-up studies. SETTING: Data collected from a national cohort between 2002 and 2013 by the Korean National Health Insurance Service-Health Screening Cohort. PARTICIPANTS: In cohort 1, matching resulted in the inclusion of 31 589 migraine patients and 126 356 control I participants. In cohort 2, matching resulted in the inclusion of 9287 RA patients and 37 148 control II participants. PRIMARY AND SECONDARY OUTCOME MEASURES: The HRs for RA in patients with migraine (cohort 1) and migraine in patients with RA (cohort 2) were analysed using stratified Cox proportional hazard models after adjusting for autoimmune disease, Charlson Comorbidity Index scores without rheumatoid diseases, obesity (body mass index), smoking and history of alcohol intake. Subgroup analyses stratified by age, sex, income and region of residence were also performed. RESULTS: The incidence of RA in the migraine group (2.0% (640/31 589)) was higher than that in the control I group (1.4% (1709/126 356), p<0.001). The adjusted HR for RA in the migraine without aura group was 1.48 (95% CIs=1.34 to 1.63, p<0.001).The incidence of migraine in the RA group (6.4% (590/9287)) was higher than that in the control II group (4.6% (1721/37 148), p<0.001). The adjusted HR for migraine without aura in the RA group was 1.35 (95% CI=1.23 to 1.49, p<0.001). CONCLUSION: Migraine increases the risk of RA, and RA is also associated with an increased risk of migraine.

Reversible Cerebral Vasoconstriction Syndrome Associated with Levonorgestrel-Releasing Intrauterine System.

Reversible cerebral vasoconstriction syndromes (RCVS) is a rare disease that is characterized by reversible multifocal stenosis of the cerebral arteries with various clinical manifestations. Though the pathomechanism of RCVS was unclear, we reported RCVS related to the levonorgestrel-releasing intrauterine system (IUS). A previous healthy 36-year-old woman had thunderclap headache after implanting the levonorgestrel-releasing IUS a year ago. In the serial angiography, we initially found left vertebra artery (VA), and then additionally new stenosis of both anterior cerebral arteries and middle cerebral arteries (MCA). Bilateral MCA stenosis improved but developed stenosis of right VA after a week. The mean flow velocities of both MCA increased in the first transcranial doppler (TCD), but normalized in the follow up TCD. Levonorgestrel might act as the vasoconstrictitve factor that increased the level of endothelin-1, diminished the release of NO and raised oxidative low-density lipoprotein (LDL). Although the exact pathological mechanisms for RCVS were not yet elucidated, this case might help clinicians understand the mechanisms of RCVS.

Personalized Consideration of Admission-Glucose Gap between Estimated Average and Initial Glucose Levels on Short-Term Stroke Outcome.

BACKGROUND: Poststroke hyperglycemia is associated with poor outcomes. Most prior studies used initial glucose as an indicator of poststroke hyperglycemia without considering glycemic control status at the time of stroke occurrence. We aimed to investigate the effect of an admission-glucose gap on short-term functional outcomes in acute ischemic stroke (AIS). METHODS: We enrolled patients with AIS or transient ischemic attack who had been admitted within 7 days of symptom onset to three stroke centers from May 2016 to December 2019. The admission-glucose gap between estimated average glucose levels (eAG) and initial glucose level (eAG-initial glucose) was categorized into four groups. The short-term functional outcome was evaluated using the modified Rankin Scale (mRS) score at 3 months after stroke onset and was dichotomized. RESULTS: Among 1332 included subjects, 548 (41.1%) had poor short-term functional outcomes. After adjusting for multiple variables, a severe negative glucose gap (eAG-initial glucose ≤ -50 mg/dL) was significantly associated with poor short-term functional outcome (OR, 1.573; 95% CI, 1.101-2.248). After dichotomizing glycemic control status, its significance was only maintained in the good glycemic control group (HbA1c < 6.5%) (OR, 1.914; 95% CI, 1.155-3.169). CONCLUSIONS: An elevated admission-glucose gap, in which the initial glucose level was much higher than the estimated glucose level was based on HbA1c, was associated with poor stroke prognosis. In addition to admission-glucose levels, glycemic control status at the time of stroke onset should be considered when predicting short-term stroke outcomes.

Guillain-Barré Syndrome and Variants Following COVID-19 Vaccination: Report of 13 Cases.

BACKGROUND: Amidst growing concern about an increased risk of Guillain-Barré syndrome (GBS) following COVID-19 vaccination, clinical and electrodiagnostic features have not been fully characterized. METHODS: We retrospectively reviewed medical records of the patients diagnosed with GBS and its variants following COVID-19 vaccination at four referral hospitals during the period of the mass vaccination program in South Korea (February to October 2021). RESULTS: We identified 13 patients with GBS and variants post COVID-19 vaccination: AstraZeneca vaccine (Vaxzevria) in 8, and Pfizer-BioNTech vaccine (Comirnaty) in 5. The mean time interval from vaccination to symptom onset was 15.6 days (range 4-30 days). Electrodiagnostic classification was demyelinating in 7, axonal in 4 and normal in 2 cases. Clinical manifestations were diverse with varying severity: classical GBS in 8 cases, paraparetic variant in 3, Miller-Fisher syndrome in 1 and acute cervicobrachial weakness in 1. Four patients developed respiratory failure, and 2 of them showed treatment-related fluctuations. CONCLUSION: Our observations suggest that COVID-19 vaccines may be associated with GBS of distinctive clinical features characterized by severe quadriplegia, disproportionately frequent bilateral facial palsy or atypical incomplete variants. Continuous surveillance and further studies using robust study designs are warranted to fully assess the significance of the association.

Comparison of MOG and AQP4 antibody seroprevalence in Korean adults with inflammatory demyelinating CNS diseases.

We aimed to compare seroprevalence of anti-myelin oligodendrocyte glycoprotein (MOG) and anti-aquaporin-4 (AQP4) antibodies in Korean adults with inflammatory demyelinating diseases (IDDs) of the central nervous system (CNS), based on a multicenter nationwide database. Sera were analyzed using a live cell-based assay for MOG and AQP4 antibodies. Of 586 Korean adults with IDDs of the CNS, 36 (6.1%) and 185 (31.6%) tested positive for MOG and AQP4 antibodies, respectively. No participant showed double positivity. Seroprevalence of MOG antibodies was about five times lower than that of AQP4 antibodies in a large cohort of Korean adults with IDDs of the CNS.

Finger drop sign as a new variant of acute motor axonal neuropathy.

We propose the finger drop sign as a new clinical variant of acute motor axonal neuropathy (AMAN) defined by immunological and radiological evidence. We identified eight consecutive patients who had AMAN. All of them developed prominent involvement of the finger extensors. We performed magnetic resonance imaging (MRI) of the extremity muscles and serological assays for antiganglioside antibodies and Campylobacter jejuni. Patients with AMAN showed characteristic and a markedly sustained weakness of the finger extensors with a distinctive pattern of the finger drop sign. Limb MRI revealed unevenly distributed abnormal signals in the muscles mainly innervated by the posterior interosseous nerve. All tested patients showed positivity for immunoglobulin G antibody against ganglioside complex of GM1 and phosphatidic acid. A pathophysiological understanding of this unique syndrome can provide further insight into antiganglioside-antibody-mediated axonal injury in Guillain-Barré syndrome.

Analysis of Altmetrics in Social Recognition of Neurology and Neurological Disorders.

This study used Altmetric analysis to rank neurological articles and assessed the implications in relation to the social recognition of neurology and neurological disorders. An Altmetric Explorer search was conducted on 25 May 2018 for articles published in the 91 journals included in the 2015 InCites™ Journal Citation Report®. We identified and analyzed the 100 articles with the highest Altmetric Attention Scores (AASs). A major proportion of the social impact (high AASs) was focused on neurodegenerative disorders such as dementia and neurodegenerative disorders. About half of the high-ranking articles provided academic information such as disease information (29 articles, 29%), new or advanced treatments (17%), and side effects of treatment (8%). The journal with largest number of top 100 articles was the New England Journal of Medicine (29 articles). Some of the data gathered via altmetrics can change a field of study, the public's health, or a larger society. This is the first report on the impact of academic articles in neurological disorder on the general public living in our altered information society.

Public Interest in Cognitive Impairment: An Analysis of the Top 50 Articles on Cognitive Impairment on Altmetric.

BACKGROUND: As the average life expectancy continues to increase, interest in cognitive impairment is increasing. Nowadays, as social media expands its reach, academic research is spreading through social media, changing the way and speed by which research is propagated and also who consumes this content. Therefore, using Altmetric, a new web-based set of metrics that analyzes the impact of content on social media platforms, we investigated the characteristics of influential research articles on the topic of cognitive impairment in social media. METHODS: An Altmetric Explorer search was performed on May 25, 2018, to extract the following information: (i) journal name, (ii) journal impact factor (IF), (iii) year of publication, (iv) article topic, (v) article type, and (vi) cognitive impairment subtype. RESULTS: The journal "Neurology" was the most cited journal for cognitive impairment articles shared on social media. Among the various types of cognitive impairment, most articles were related to dementia (all subtypes), Alzheimer's disease, and aging. The most common article type was original scientific paper, especially cohort study. The most popular topic was the identification of protective or risk factors for cognitive impairment. CONCLUSION: The characteristics of articles with a high Altmetric Attention Score were somewhat different from those of articles with a high number of traditional citations. Social media had the disadvantage that it was difficult to verify the authenticity of the primary source in question, but the advantage was that it could immediately determine the trends regarding how information about that source was being shared and consumed. Therefore, it may be advisable to use Altmetric analysis in combination with traditional methods of evaluating the research articles to understand the dissemination of scientific research and to direct future research.

Differences in nerve excitability properties between isolated bulbar palsy and bulbar-dominant amyotrophic lateral sclerosis.

Objectives: Isolated bulbar palsy (IBP) is a rare variant that can show a benign course, while progressive bulbar palsy (PBP) has been regarded as a bulbar-dominant type of classical amyotrophic lateral sclerosis (cALS). This study aimed to identify differences in the excitability properties between them.Methods: We consecutively collected data on 22 ALS patients: 13 with cALS, 5 with PBP, and 4 with IBP. An automated nerve excitability test (NET) was applied to measure the strength-duration time constant, threshold electrotonus (TE), current-threshold relationship, and recovery cycle. The axonal excitability properties were compared between the ALS groups and 25 controls.Results: Compared to controls, the cALS group showed a greater change in the depolarizing phase of TE of 90-100 ms after depolarizing current [TEd(90-100)] (53.3±1.3 [mean±SEM] for cALS and 49.0±0.7 for control, P<0.01) and lower S2 accommodation (19.6±0.8 and 22.6±0.7, respectively; P=0.01). There was a nonsignificant tendency for a high TEd(90-100) pattern to be less prominent in the IBP group than in the PBP group (51.5±4.22 and 48.8±1.5, respectively). In addition, all of the parameters of nerve excitability other than S2 accommodation in the PBP and IBP groups did not differ significantly from those in the controls.Conclusions: The excitability properties of IBP and PBP differ from those of cALS. The pattern of NET in PBP was more similar to that in cALS than that in IBP. These findings suggest that IBP is a different entity from bulbar-dominant ALS and PBP.

Top 100 Publications as Measured by Altmetrics in the Field of Central Nervous System Inflammatory Demyelinating Disease.

BACKGROUND: Altmetrics analyze the visibility of articles in social media and estimate their impact on the general population. We performed an altmetric analysis of articles on central nervous system inflammatory demyelinating disease (CIDD) and investigated its correlation with citation analysis. METHODS: Articles in the 91 journals comprising the "clinical neurology," "neuroscience," and "medicine, general, and internal" Web of Science categories were searched for their relevance to the CIDD topic. The Altmetric Explorer database was used to determine the Altmetric.com Attention Score (AAS) values of the selected articles. The papers with the top 100 AAS values were characterized. RESULTS: Articles most frequently mentioned online were primarily published after 2014 and were published in journals with high impact factors. All articles except one were dealt with the issue of multiple sclerosis. Most were original articles, but editorials were also common. Novel treatments and risk factors are the most frequent topics. The AAS was weakly correlated with journal impact factors; however, no link was found between the AAS and the number of citations. CONCLUSIONS: We present the top 100 most frequently mentioned CIDD articles in online media using an altmetric approach. Altmetrics can rapidly offer alternative information on the impact of research based on a broader audience and can complement traditional metrics.