Time to Treat First Acute Attack of Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease.

Importance: A proportion of people with myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) have a relapsing disease course and persistent anti-myelin oligodendrocyte glycoprotein immunoglobulin G (MOG-IgG) seropositivity. Few studies have investigated whether treatment of the first MOGAD attack is associated with the long-term disease course and/or MOG-IgG seronegative conversion. Objective: To investigate the association of time to treat the first acute MOGAD attack with relapse risk and MOG-IgG serostatus. Design, Setting, and Participants: This was a retrospective, nationwide, multicenter cohort study involving 14 secondary or tertiary hospitals in South Korea between November 2009 and August 2023. People with adult-onset MOGAD, who either had a relapse or were followed up for more than 12 months after disease onset and had a detailed medical record of their first attack, were included. Individuals were excluded for adolescent-onset MOGAD or short disease duration. Exposures: Patients were categorized based on the time to treat the first acute MOGAD attack: early (<5 days), intermediate (5-14 days), and late (not treated within 14 days). Main Outcomes and Measures: A multivariable analysis for clinical and treatment factors associated with relapsing disease course and/or MOG-IgG seronegative conversion. Further subgroup analyses were conducted among those without long-term nonsteroidal immunosuppressant (NSIS) maintenance treatment. Results: Among the 315 individuals screened, 75 were excluded. A total of 240 patients (median [IQR] age at onset, 40.4 [28.8-56.1] years; 125 female [52.1%]) with median (IQR) disease duration of 3.07 (1.95-6.15) years were included. A total of 110 of 240 patients (45.8%) relapsed after a median (IQR) of 0.45 (0.18-1.68) years, and 29 of 116 patients (25.0%) experienced a conversion to seronegative MOG-IgG. Both the time to treatment of the first MOGAD attack (late vs early: adjusted hazard ratio [aHR], 2.64; 95% CI, 1.43-4.84; P = .002; intermediate vs early: aHR, 2.02; 95% CI, 1.10-3.74; P = .02) and NSIS maintenance treatment (aHR, 0.24; 95% CI, 0.14-0.42; P < .001) were independently associated with the risk of relapse. In a subgroup without NSIS maintenance, the time to treat of the first MOGAD attack was still associated with higher risk of relapse (late vs early: aHR, 3.51; 95% CI, 1.64-7.50; P = .001; intermediate vs early: aHR, 2.68; 95% CI, 1.23-5.85; P = .01). Lastly, the time to treat of the first MOGAD attack was also associated with MOG-IgG seronegative conversion (early vs late: adjusted odds ratio, 7.04; 95% CI, 1.58-31.41; P = .01), whereas NSIS maintenance treatment was not. Conclusions and Relevance: Results of this cohort study suggest that early treatment of the first acute MOGAD attack was associated with a reduction in the proportion of relapsing disease course and an increase in the likelihood of MOG-IgG seronegative conversion. These data suggest that timing of acute phase treatment for the first MOGAD attack can be associated with the long-term prognosis and autoimmune status of patients.

Factors associated with adherence to noninvasive positive pressure ventilation in amyotrophic lateral sclerosis.

INTRODUCTION: This cohort study aimed to investigate the factors associated with noninvasive positive pressure ventilation adherence and assess the long-term effects of noninvasive positive pressure ventilation adherence in patients with amyotrophic lateral sclerosis (ALS). METHODS: The medical records of patients with ALS admitted to a tertiary hospital for noninvasive positive pressure ventilation initiation were retrospectively reviewed. Pulmonary function parameters, variables of blood gas analysis, the site of symptom onset, the time from onset and diagnosis to noninvasive positive pressure ventilation application, ALS Functional Rating Scale-Revised, neurophysiological index, and the length of hospital stay were evaluated. The adherence to noninvasive positive pressure ventilation was defined as the use of noninvasive positive pressure ventilation for ≥ 2 h/day or ≥ 4 h/day. The correlations between noninvasive positive pressure ventilation adherence or length of hospital stay and other clinical parameters were analyzed. RESULTS: Fifty-one patients with ALS were included in the study. The time from onset and diagnosis to NIPPV application was reduced by 16 months in the adherent group than that in the non-adherent group; however, the parameters of blood gas analysis and pulmonary function tests did not differ significantly between the groups. Furthermore, the neurophysiological index of the abductor digiti minimi muscle was higher by 4.05 in the adherent group than that in the non-adherent group. The adherence to noninvasive positive pressure ventilation prolonged tracheostomy-free survival compared to that of non-adherence. Desaturation events, lower forced vital capacity, last pCO2, bicarbonate, and base excess, and higher differences in pCO2, were associated with an increase in the length of hospital stay. CONCLUSIONS: Noninvasive positive pressure ventilation application shortly after symptom onset and ALS diagnosis in patients with CO2 retention and reduced forced vital capacity can be considered for successful adherence. Adherence to noninvasive positive pressure ventilation may result in reduced tracheostomy conversion rates and prolonged tracheostomy-free survival.

Intraoperative Brainstem Auditory Evoked Potentials and Postoperative Nausea and Vomiting After Microvascular Decompression.

BACKGROUND: We performed this study to investigate the effect of intraoperative brainstem auditory evoked potential (IBAEP) changes on the development of postoperative nausea and vomiting (PONV) after microvascular decompression (MVD) for neurovascular cross compression. METHODS: A total of 373 consecutive cases were treated with MVD. The use of rescue antiemetics after surgery was used as an objective indicator of PONV. IBAEP monitoring was routinely performed in all. RESULTS: The use of rescue antiemetics was significantly associated with female sex (OR = 3.427; 95% CI, 2.077-5.654; P < 0.001), PCA use (OR = 3.333; 95% CI, 1.861-5.104; P < 0.001), and operation time (OR = 1.017; 95% CI, 1.008-1.026; P < 0.001). A Wave V peak delay of more than 1.0 milliseconds showed a significant relation with the use of rescue antiemetics (OR = 1.787; 95% CI, 1.114-2.867; P = 0.016) and a strong significant relation with the use of rescue antiemetics more than 5 times (OR = 2.426; 95% CI, 1.372-4.290; P = 0.002). CONCLUSIONS: A wave V peak delay of more than 1.0 milliseconds might have value as a predictor of PONV after MVD. More detailed neurophysiological studies will identify the exact pathophysiology underlying PONV after MVD.

Advancing Intraoperative Neurophysiological Monitoring With Human Reflexes.

Human reflexes are simple motor responses that are automatically elicited by various sensory inputs. These reflexes can provide valuable insights into the functioning of the nervous system, particularly the brainstem and spinal cord. Reflexes involving the brainstem, such as the blink reflex, laryngeal adductor reflex, trigeminal hypoglossal reflex, and masseter H reflex, offer immediate information about the cranial-nerve functionality and the overall state of the brainstem. Similarly, spinal reflexes such as the H reflex of the soleus muscle, posterior root muscle reflexes, and sacral reflexes provide crucial information about the functionality of the spinal cord and peripheral nerves. One of the critical benefits of reflex monitoring is that it can provide continuous feedback without disrupting the surgical process due to no movement being induced in the surgical field. These reflexes can be monitored in real time during surgical procedures to assess the integrity of the nervous system and detect potential neurological damage. It is particularly noteworthy that the reflexes provide motor and sensory information on the functional integrity of nerve fibers and nuclei. This article describes the current techniques used for monitoring various human reflexes and their clinical significance in surgery. We also address important methodological considerations and their impact on surgical safety and patient outcomes. Utilizing these methodologies has the potential to advance or even revolutionize the field of intraoperative continuous monitoring, ultimately leading to improved surgical outcomes and enhanced patient care.

Prognostic factors of first-onset optic neuritis based on diagnostic criteria and antibody status: a multicentre analysis of 427 eyes.

BACKGROUND: Optic neuritis (ON) prognosis is influenced by various factors including attack severity, underlying aetiologies, treatments and consequences of previous episodes. This study, conducted on a large cohort of first ON episodes, aimed to identify unique prognostic factors for each ON subtype, while excluding any potential influence from pre-existing sequelae. METHODS: Patients experiencing their first ON episodes, with complete aquaporin-4 (AQP4) and myelin oligodendrocyte glycoprotein (MOG) antibody testing, and clinical data for applying multiple sclerosis (MS) diagnostic criteria, were enrolled. 427 eyes from 355 patients from 10 hospitals were categorised into four subgroups: neuromyelitis optica with AQP4 IgG (NMOSD-ON), MOG antibody-associated disease (MOGAD-ON), ON in MS (MS-ON) or idiopathic ON (ION). Prognostic factors linked to complete recovery (regaining 20/20 visual acuity (VA)) or moderate recovery (regaining 20/40 VA) were assessed through multivariable Cox regression analysis. RESULTS: VA at nadir emerged as a robust prognostic factor for both complete and moderate recovery, spanning all ON subtypes. Early intravenous methylprednisolone (IVMP) was associated with enhanced complete recovery in NMOSD-ON and MOGAD-ON, but not in MS-ON or ION. Interestingly, in NMOSD-ON, even a slight IVMP delay in IVMP by >3 days had a significant negative impact, whereas a moderate delay up to 7-9 days was permissible in MOGAD-ON. Female sex predicted poor recovery in MOGAD-ON, while older age hindered moderate recovery in NMOSD-ON and ION. CONCLUSION: This comprehensive multicentre analysis on first-onset ON unveils subtype-specific prognostic factors. These insights will assist tailored treatment strategies and patient counselling for ON.

Prognosis of amyotrophic lateral sclerosis patients after tracheostomy invasive ventilation in Korea.

Background: Tracheostomy invasive ventilation (TIV) is applied to a subset of amyotrophic lateral sclerosis (ALS) patients; however, its frequency and impact on prognosis vary across countries. Methods: We conducted a nationwide retrospective cohort study using Korean National Health Insurance claims data. All patients diagnosed with sporadic ALS from 2012 to 2017 were included, with the observation period until 2020. The survival time between the TIV and non-TIV groups was compared using propensity score matching analysis, and prognostic factors were assessed within the TIV group. Results: This study included 3484 ALS patients (mean [standard deviation] age, 62.4 [11.9] years, 60.4% male), among whom 1230 (35.3%) underwent TIV. After 1:1 propensity score matching, the survival duration between the two groups was not significantly different (28 vs. 25 months, p = 0.057). Cox regression indicated that older age (hazard ratios [HRs] for each decade compared to <40 years: 3.89, 3.83, 5.30, 6.78, and 8.40 [≥80 years]; p < 0.005 for all) and lower income (HR, 1.28; 95% confidence interval [CI], 1.09-1.52; p = 0.003) negatively impacted survival, while gastrostomy (HR, 0.57; 95% CI, 0.50-0.66; p < 0.001) and supportive care services (HR, 0.43; 95% CI, 0.32-0.59; p < 0.001) were associated with prolonged survival. Conclusions: TIV was administered to more than one-third of Korean ALS patients without significant survival prolongation. Older age, lower income, lack of gastrostomy, and insufficient supportive care were independent poor prognostic factors for survival, underscoring the importance of comprehensive management for ALS patients.

Therapeutic Outcomes and Electrophysiological Biomarkers in Anti-Myelin-Associated Glycoprotein Neuropathy: A Multicenter Cohort Study in South Korea.

BACKGROUND AND PURPOSE: Unlike other immune-mediated neuropathies, anti-myelin-associated glycoprotein (MAG) neuropathy is often refractory to immunotherapy. It is necessary to compare the relative efficacies of various immunotherapies and develop objective biomarkers in order to optimize its clinical management. METHODS: This study recruited 91 patients with high anti-MAG antibody titers from 7 tertiary hospitals in South Korea. We analyzed the baseline characteristics, therapeutic outcomes, and nerve conduction study (NCS) findings of 68 patients and excluded 23 false positive cases. RESULTS: The rate of positive responses to treatment was highest using zanubrutinib (50%) and rituximab (36.4%), followed by corticosteroids (16.7%), immunosuppressants (9.5%), intravenous immunoglobulin (5%), and plasma exchange (0%). Disability and weakness were significantly associated with multiple NCS parameters at the time of diagnosis, especially distal compound muscle action potential (CMAP) amplitudes. Moreover, the longitudinal trajectory of the average CMAP amplitudes paralleled the clinical courses, with a 16.2 percentile decrease as an optimal cutoff for predicting a clinical exacerbation (area under the receiver operating characteristic curve=0.792). CONCLUSIONS: Our study supports the use of NCS as an objective marker for estimating disease burden and tracking clinical changes in patients with anti-MAG neuropathy. We have described the beneficial effects of rituximab and a new drug, zanubrutinib, compared with conventional immunotherapies.

Different cutoff value of motor evoked potentials for the postoperative outcome in the ossification of the posterior longitudinal ligament surgery in the cervical and thoracic spine.

BACKGROUND CONTEXT: Transcranial muscle motor evoked potentials (Tc-mMEPs), a key component of intraoperative neurophysiologic monitoring (IONM), effectively reflect the changes in corticospinal tract integrity and are closely related to the occurrence of the postoperative motor deficit (PMD). Most institutions have applied a specified (fixed) alarm criterion for the heterogeneous groups in terms of etiologies or lesion location. However, given the high risk of PMD in ossification of the posterior longitudinal ligament (OPLL) surgery, it is essential to determine a tailored cutoff value for IONM. PURPOSE: We aimed to establish the intraoperative cutoff value of Tc-mMEPs reduction for predicting PMD in OPLL according to lesion levels. DESIGN: Retrospective analysis using a review of electrical medical records. PATIENT SAMPLE: In this study, we included 126 patients diagnosed with OPLL, who underwent surgery and IONM. OUTCOME MEASURES: The occurrence of PMD immediately and 1 year after operation, as well as the decrement of intraoperative Tc-mMEPs amplitude. METHODS: We analyzed OPLL surgery outcomes using Tc-mMEPs monitoring. Limbs with acceptable baseline Tc-mMEPs in the tibialis anterior or abductor hallucis were included in the final set. PMD was defined as a ≥1 decrease in Medical Research Council score in the legs, and it was evaluated immediately and 1year after operation. The reduction ratios of Tc-mMEPs amplitude compared with baseline value were calculated at the two time points: the maximal decrement during surgery and at the end of surgery. Receiver operating characteristic curve analysis was used to determine the cutoff value of Tc-mMEPs amplitude decrement for predicting PMDs. RESULTS: In total, 203 limbs from 102 patients with cervical OPLL and 42 limbs from 24 patients with thoracic OPLL were included. PMD developed more frequently in thoracic lesions than in cervical lesions (immediate, 9.52% vs 2.46%; 1 year, 4.76% vs 0.99%). The Tc-mMEPs amplitude cutoff point at the end of surgery for PMD (both immediate and 1-year) was a decrease of 93% in cervical and 50% in thoracic OPLL surgeries. Similarly, the Tc-mMEPs amplitude cutoff point at the maximal decrement during surgery for PMD (both immediate and 1 year) was a reduction of 97% in cervical and 85% in thoracic OPLL surgeries. CONCLUSIONS: The thoracic lesion exhibited a lower cutoff value than the cervical lesion for both immediate and long-term persistent PMD in OPLL surgery (Tc-mMEPs at the end of surgery measuring 93% vs 50%; and Tc-mMEPs at the maximal decrement measuring 97% vs 85% for cervical and thoracic lesions, respectively). To enhance the reliability of monitoring, considering the application of tailored alarm criteria for Tc-mMEPs changes based on lesion location in OPLL could be beneficial.

Membrane Protein Bcest Is Involved in Hyphal Growth, Virulence and Stress Tolerance of Botrytis cinerea.

Botrytis cinerea is a necrotrophic model fungal plant pathogen that causes grey mould, a devastating disease responsible for large losses in the agriculture sector. As important targets of fungicides, membrane proteins are hot spots in the research and development of fungicide products. We previously found that membrane protein Bcest may be closely related to the pathogenicity of Botrytis cinerea. Herein, we further explored its function. We generated and characterised ΔBcest deletion mutants of B. cinerea and constructed complemented strains. The ΔBcest deletion mutants exhibited reduced conidia germination and germ tube elongation. The functional activity of ΔBcest deletion mutants was investigated by reduced necrotic colonisation of B. cinerea on grapevine fruits and leaves. Targeted deletion of Bcest also blocked several phenotypic defects in aspects of mycelial growth, conidiation and virulence. All phenotypic defects were restored by targeted-gene complementation. The role of Bcest in pathogenicity was also supported by reverse-transcriptase real-time quantitative PCR results indicating that melanin synthesis gene Bcpks13 and virulence factor Bccdc14 were significantly downregulated in the early infection stage of the ΔBcest strain. Taken together, these results suggest that Bcest plays important roles in the regulation of various cellular processes in B. cinerea.

Early rituximab treatment reduces long-term disability in aquaporin-4 antibody-positive neuromyelitis optica spectrum.

BACKGROUND: Neuromyelitis optica spectrum disorder (NMOSD) causes relapsing inflammatory attacks in the central nervous system, leading to disability. As rituximab, a B-lymphocyte-depleting monoclonal antibody, is an effective in preventing NMOSD relapses, we hypothesised that earlier initiation of rituximab can also reduce long-term disability of patients with NMOSD. METHODS: This multicentre retrospective study involving 19 South Korean referral centres included patients with NMOSD with aquaporin-4 antibodies receiving rituximab treatment. Factors associated with the long-term Expanded Disability Status Scale (EDSS) were assessed using multivariable regression analysis. RESULTS: In total, 145 patients with rituximab treatment (mean age of onset, 39.5 years; 88.3% female; 98.6% on immunosuppressants/oral steroids before rituximab treatment; mean disease duration of 121 months) were included. Multivariable analysis revealed that the EDSS at the last follow-up was associated with time to rituximab initiation (interval from first symptom onset to initiation of rituximab treatment). EDSS at the last follow-up was also associated with maximum EDSS before rituximab treatment. In subgroup analysis, the time to initiation of rituximab was associated with EDSS at last follow-up in patients aged less than 50 years, female and those with a maximum EDSS score ≥6 before rituximab treatment. CONCLUSIONS: Earlier initiation of rituximab treatment may prevent long-term disability worsening in patients with NMOSD, especially among those with early to middle-age onset, female sex and severe attacks.

Intraoperative bulbocavernosus reflex monitoring in posterior lumbar fusion surgery.

OBJECTIVE: To assess the utility of intraoperative bulbocavernosus reflex (BCR) monitoring in posterior lumbar fusion surgery. METHODS: We retrospectively evaluated 153 patients undergoing posterior lumbar fusion with intraoperative BCR monitoring. Voiding function was assessed at discharge and two follow-ups. RESULTS: BCR was preserved in 151 patients and completely disappeared in two patients at the end of surgery. For patients in whom BCR was preserved, voiding difficulties at discharge and 1-month and 6-month follow-ups were noted in 16 (10.6%), 9 (6.0%), and 0 (0.0%) patients, respectively. However, patients with BCR loss experienced voiding difficulties at all three time-points. Statistical analysis showed a significant difference in voiding between those with preserved and disappeared BCRs at 6 months postoperatively. BCR disappeared during surgery but recovered before the end of surgery in six patients. Among these patients, one experienced transient voiding difficulties postoperatively but with good recovery. However, the other five patients did not experience postoperative voiding difficulties. CONCLUSIONS: BCR had low sensitivity for voiding dysfunction at discharge, but had high accuracy at 6-month follow-up examinations. BCR loss was associated with new voiding dysfunction. SIGNIFICANCE: Intraoperative BCR monitoring is a potentially useful tool for enhancing safety during posterior lumbar fusion by predicting postoperative voiding dysfunction.

Features of non-traumatic spinal cord infarction on MRI: Changes over time.

BACKGROUND AND PURPOSE: Spinal cord infarction (SCI) is difficult to diagnosis using MRI findings. We aimed to suggest the optimal timing of MRI studies for diagnosing SCI. MATERIALS AND METHODS: This retrospective study was approved by our institutional review board. The requirement for informed consent was waived. MRI scans of SCI patients diagnosed between January 2015 and August 2019 were enrolled in the SCI group and subdivided according to the interval between symptom onset and time of MRI scan (A, within 6 h; B, 6-12 hours; C, 12-24 hours; D, 24-72 hours; E, 3-7 days). Three radiologists analyzed the T2WI scans and evaluated the confidence level of diagnosing SCI using a five-point Likert scale: 1, certainly not; 2, probably not; 3, equivocal; 4, probably yes; 5, certainly yes. Scores of 4 and 5 were defined as "T2WI-positive SCI" and scores of 1-3 were defined as "T2WI-negative SCI". RESULTS: The SCI group included 58 MRI scans of 34 patients (mean age, 60.6 ± 14.0 years; 18 women). The T2WI positivity rate was 72.4% (42/58). In contrast to the other subgroups, subgroup A included fewer cases of T2WI-positive SCI (1/4, 25%) than T2WI-negative SCI. A confidence score of 5 was the most common in subgroup D (4/27, 14.8%). Among the 12 patients who underwent MRI studies more than twice, confidence scores increased with time. CONCLUSION: In patients with suspected SCI showing equivocal initial MRI findings, follow-up MRI studies are helpful, especially when performed between 24 and 72 hours after symptom onset.

Advances in Intraoperative Neurophysiology During Microvascular Decompression Surgery for Hemifacial Spasm.

Microvascular decompression (MVD) is a widely used surgical intervention to relieve the abnormal compression of a facial nerve caused by an artery or vein that results in hemifacial spasm (HFS). Various intraoperative neurophysiologic monitoring (ION) and mapping methodologies have been used since the 1980s, including brainstem auditory evoked potentials, lateral-spread responses, Z-L responses, facial corticobulbar motor evoked potentials, and blink reflexes. These methods have been applied to detect neuronal damage, to optimize the successful decompression of a facial nerve, to predict clinical outcomes, and to identify changes in the excitability of a facial nerve and its nucleus during MVD. This has resulted in multiple studies continuously investigating the clinical application of ION during MVD in patients with HFS. In this study we aimed to review the specific advances in methodologies and clinical research related to ION techniques used in MVD surgery for HFS over the last decade. These advances have enabled clinicians to improve the efficacy and surgical outcomes of MVD, and they provide deeper insight into the pathophysiology of the disease.

The utility of intraoperative neurophysiological monitoring in surgical treatment for spinal arteriovenous malformations: A historical control study.

OBJECTIVE: This study sought to investigate the utility of intraoperative neurophysiological monitoring (IONM) in the surgical treatment for spinal arteriovenous malformations (SAVMs). METHODS: We retrospectively reviewed the data of 39 patients who underwent surgical treatment for SAVMs. Twenty-eight patients who received multimodal IONM (transcranial electrical motor-evoked potentials [MEPs], somatosensory-evoked potentials, continuous electromyography, and bulbocavernosus reflex [BCR]) between 2011 and 2020 were compared to 11 historical controls between 2003 and 2011. The rates of postoperative neurological deficits (PNDs), neurophysiological warnings, and their characteristics were analyzed. RESULTS: PNDs were developed in 10.7% and 54.5% of patients in the IONM and historical control (non-IONM) groups, respectively (p = 0.008). Moreover, not applying IONM was the only significant risk factor for the development of PNDs in the logistic regression analysis (odds ratio 10.0, p = 0.007). In the IONM group, a total of three electrophysiological warnings were observed, and two of these were true positives; one patient complained of leg motor weakness after surgery with loss of the abductor halluces MEPs. The other patient experienced disappearance of the BCR during surgery and newly developed urinary retention. Overall, the sensitivity, specificity, positive predictive value, and negative predictive value of IONM warnings for detecting PNDs were 66.7%, 96.0%, 66.7%, and 96.0%, respectively. CONCLUSIONS: The neurological outcome of the IONM group was significantly better than that of the historical control group in the surgical treatments for SAVMs. SIGNIFICANCE: Multimodal IONM could be a useful tool to detect neurological damage with relatively high accuracy in this type of surgery.

Myelin Oligodendrocyte Glycoprotein-Immunoglobulin G in the CSF: Clinical Implication of Testing and Association With Disability.

BACKGROUND AND OBJECTIVE: To investigate the clinical relevance of CSF myelin oligodendrocyte glycoprotein-immunoglobulin G (MOG-IgG) testing in a large multicenter cohort. METHODS: In this multicenter cohort study, paired serum-CSF samples from 474 patients with suspected inflammatory demyelinating disease (IDD) from 11 referral hospitals were included. After serum screening, patients were grouped into seropositive myelin oligodendrocyte glycoprotein antibody associated disease (MOGAD, 31), aquaporin-4-IgG-positive neuromyelitis optica spectrum disorder (AQP4-IgG + NMOSD, 60), other IDDs (217), multiple sclerosis (MS, 45), and non-IDDs (121). We then screened CSF for MOG-IgG and compared the clinical and serologic characteristics of patients uniquely positive for MOG-IgG in the CSF to seropositive patients with MOGAD. RESULTS: Nineteen patients with seropositive MOGAD (61.3%), 9 with other IDDs (CSF MOG + IDD, 4.1%), 4 with MS (8.9%), but none with AQP4-IgG + NMOSD nor with non-IDDs tested positive in the CSF for MOG-IgG. The clinical, pathologic, and prognostic features of patients uniquely positive for CSF MOG-IgG, with a non-MS phenotype, were comparable with those of seropositive MOGAD. Intrathecal MOG-IgG synthesis, observed from the onset of disease, was shown in 12 patients: 4 of 28 who were seropositive and 8 who were uniquely CSF positive, all of whom had involvement of either brain or spinal cord. Both CSF MOG-IgG titer and corrected CSF/serum MOG-IgG index, but not serum MOG-IgG titer, were associated with disability, CSF pleocytosis, and level of CSF proteins. DISCUSSION: CSF MOG-IgG is found in IDD other than MS and also in MS. In IDD other than MS, the CSF MOG-IgG positivity can support the diagnosis of MOGAD. The synthesis of MOG-IgG in the CNS of patients with MOGAD can be detected from the onset of the disease and is associated with the severity of the disease. CLASSIFICATION OF EVIDENCE: This study provides Class II evidence that the presence of CSF MOG-IgG can improve the diagnosis of MOGAD in the absence of an MS phenotype, and intrathecal synthesis of MOG-IgG was associated with increased disability.

Actinomycosis of the nasal cavity.

INTRODUCTION: Actinomycosis of the nasal cavity is very rare. OBJECTIVE: The purpose of this study was to investigate the clinical features, treatment methods, and treatment results of actinomycosis of the nasal cavity in our hospital. METHODS: We retrospectively enrolled 11 patients with histopathologically identified actinomycosis of the nasal cavity from January 2010 to May 2020. RESULTS: This study included five males and six females. The most common symptom was purulent nasal discharge (36.4%). Nasal actinomycosis occurred in the maxillary sinus in 5 (45.5%) patients, the ethmoid sinus in two, the hard palate in two, the frontal sinus in one, and the nasal septum in one. After surgery, intravenous administration of antibiotics was performed on average for 7.4 days and oral antibiotics were prescribed for about 120.5 days. The clinical characteristics of the patients with nasal actinomycosis and the duration of antibiotic usage were not significantly different. Trauma was significantly associated with repeated nasal actinomycosis infections (p < 0.05). CONCLUSION: Actinomycosis of the nasal cavity should be suspected when a patient with chronic sinusitis does not respond to medical therapy and has a history of dental treatment, local surgery or radiation therapy. Nasal can be sufficiently treated with antibiotics and endoscopic surgery.

Efficacy of intraoperative bulbocavernosus reflex monitoring for the prediction of postoperative voiding function in adult patients with lumbosacral spinal tumor.

Lumbosacral spinal tumor surgery is associated with a relatively high risk of postoperative voiding dysfunction. This study aimed to investigate the correlation between intraoperative bulbocavernosus reflex (BCR) changes and postoperative voiding function in adult patients with lumbosacral spinal tumors. We retrospectively reviewed 63 patients who underwent intradural conus and cauda equina tumor surgeries with intraoperative BCR monitoring. We evaluated patients' voiding functions for 6 months postoperatively. BCR was maintained in 60 patients and disappeared in 3 patients at the end of the surgery. Among the patients in whom BCR was maintained, examinations conducted at discharge and at 1- and 6-month follow-ups revealed that 7 (11.7%), 4 (6.7%), and zero (0.0%) patients experienced voiding difficulty, respectively. However, all 3 (100%) patients without BCR experienced voiding difficulty at the three corresponding follow-ups. Data analysis indicated no significant difference in voiding between the maintained and disappeared BCR groups 6-months postoperatively. The sensitivity, specificity, positive predictive, and negative predictive values of intraoperative BCR monitoring for detecting new and worsening difficulty in voiding were all 100% 6 months postoperatively. Our results shows that intraoperative BCR monitoring is a reliable predictor of voiding function following surgery in adult patients undergoing lumbosacral spinal tumor surgery. Intraoperative BCR monitoring can be useful for assessing and monitoring the integrity of the voiding function during lumbosacral spinal tumor surgery.

The novel prognostic value of postoperative follow-up lateral spread response after microvascular decompression for hemifacial spasm.

OBJECTIVE: The lateral spread response (LSR) is an aberrant electrophysiological response in which a stimulus on one branch of the facial nerve spills over to other branches of the nerve, which can be captured by electrodes near each branch. The authors performed this study to evaluate the prognostic value of the follow-up LSR with a sufficient time interval from intraoperative LSR (IO-LSR) after microvascular decompression (MVD) for hemifacial spasm (HFS), excluding the interference of various intraoperative situations. METHODS: A total of 247 patients treated with MVD for HFS between June 2011 and March 2019 were enrolled in this study. The IO-LSR was routinely evaluated in all patients. The LSR was checked again on postoperative day (POD) 2 after surgery (POD2-LSR). A total of 228 patients (92.3%) were considered cured at the last clinical follow-up. RESULTS: The IO-LSR disappeared in 189 patients (76.5%), and among them, 181 patients (95.8%) were cured 1 year after surgery. The POD2-LSR disappeared in 193 patients (78.1%), and 185 patients (95.9%) among them were cured. Among the 189 patients in which the IO-LSR disappeared, the POD2-LSR reappeared in 26 patients (13.8%). In contrast, the POD2-LSR disappeared in 30 (51.7%) of 58 patients for whom the IO-LSR continued at the end of surgery. When classified into groups according to the status of the IO-LSR and POD2-LSR, in the group of patients in whom both LSRs disappeared, the cure rate was 98.2%, which was significantly higher than that of the other 3 groups (p < 0.05, Cochran-Armitage trend test). The use of both LSRs was found to be significantly associated with better predictability (p < 0.05, McNemar's test). CONCLUSIONS: Postoperative follow-up LSR examination may be beneficial in predicting clinical outcomes after MVD for HFS, especially when considered together with IO-LSR.