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View Article and Find Full Text PDFAnn Clin Transl Neurol
April 2025
Cases of myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) co-occurring with neoplasms have been reported. In this international, retrospective cohort study in South Korea and the USA, 16 of 445 (3.6%) patients with MOGAD had concurrent neoplasm within 2 years of MOGAD onset, resulting in a standardized incidence ratio for neoplasm of 3.
View Article and Find Full Text PDFJAMA Neurol
October 2024
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.
View Article and Find Full Text PDFJ Neurol Neurosurg Psychiatry
July 2024
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.
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.
View Article and Find Full Text PDFThis review aimed to elucidate protein biomarkers in body fluids, such as blood and cerebrospinal fluid (CSF), to identify those that may be used for early diagnosis of multiple sclerosis (MS), prediction of disease activity, and monitoring of treatment response among MS patients. The potential biomarkers elucidated in this review include neurofilament proteins (NFs), glial fibrillary acidic protein (GFAP), leptin, brain-derived neurotrophic factor (BDNF), chitinase-3-like protein 1 (CHI3L1), C-X-C motif chemokine 13 (CXCL13), and osteopontin (OPN), with each biomarker playing a different role in MS. GFAP, leptin, and CHI3L1 levels were increased in MS patient groups compared to the control group.
View Article and Find Full Text PDFJ Neurol Neurosurg Psychiatry
October 2023
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.
Clin Neurophysiol
December 2022
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.
Parkinsonism Relat Disord
October 2022
Introduction: Hemifacial spasm (HFS) is an involuntary intermittent twitching of the facial muscles. Medical and surgical treatments can be considered for HFS. Among medical treatments, clonazepam is a benzodiazepine used to treat epilepsy, psychiatric symptoms, and movement disorders.
View Article and Find Full Text PDFMicrovascular 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.
View Article and Find Full Text PDFBackground: Whole-exome sequencing-based diagnosis of rare diseases typically yields 40%-50% of success rate. Precise diagnosis of the patients with neuromuscular disorders (NMDs) has been hampered by locus heterogeneity or phenotypic heterogeneity. We evaluated the utility of transcriptome sequencing as an independent approach in diagnosing NMDs.
View Article and Find Full Text PDFObjective: 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.
Neurol Neuroimmunol Neuroinflamm
January 2022
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).
Chemotherapy-induced peripheral neuropathy (CIPN) is a common and often dose-limiting side effect of many cancer drugs. Because the onset of neuronal injury is known, it is an ideal clinical target to develop neuroprotective strategies. Several years ago, we had identified ethoxyquin as a potent neuroprotective drug against CIPN through a phenotypic drug screening and demonstrated a novel mechanism of action, inhibition of chaperone domain of heat shock protein 90.
View Article and Find Full Text PDFLumbosacral 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.
View Article and Find Full Text PDFClin Neurophysiol Pract
February 2021
Objective: This study aimed to investigate the value of intraoperative neurophysiological monitoring (IONM) in anterior cervical spine discectomy with fusion (ACDF) for ossification of the posterior longitudinal ligament (OPLL).
Methods: Patients who underwent multimodal IONM (transcranial electrical motor-evoked potentials [tcMEP], somatosensory-evoked potentials, and continuous electromyography) for ACDF from 2009 to 2019 were compared to historical controls from 2003 to 2009. The rates of postoperative neurological deficits, neurophysiological warnings, and their characteristics were analyzed.
The aim of the present study was to investigate the frequency and clinical features of Guillain-Barré syndrome (GBS) with hyperCKemia. We retrospectively identified 139 patients with GBS at 2 teaching hospitals in South Korea. We excluded patients with Miller-Fisher syndrome (n = 19), acute bulbar palsy (n = 3), and those whose serum creatine kinase (CK) levels were not measured (n = 45).
View Article and Find Full Text PDFObjective: To investigate the prognostic and predictive value of intraoperative blink reflex (BR) monitoring during microvascular decompression (MVD) for hemifacial spasm (HFS).
Methods: We retrospectively reviewed 41 patients with HFS undergoing MVD with intraoperative BR and lateral spread response (LSR) monitoring. Facial spasm was evaluated for six months postoperatively.
Ther Adv Neurol Disord
September 2019
Background: The objective of this study was to evaluate the efficacy and safety of repeated low-dose rituximab treatment guided by monitoring circulating CD19+ B cells in patients with refractory myasthenia gravis (MG).
Methods: Patients with refractory MG who had received rituximab treatment at two teaching hospitals between September 2013 and January 2017 were reviewed retrospectively. The treatment protocol consisted of an induction treatment with low-dose rituximab (375 mg/m twice with a 2-week interval), followed by retreatment (375 mg/m once).
Objective: To identify factors associated with the failure to generate baseline muscle motor evoked potentials (mMEPs) during spinal surgery, and to determine the association between baseline mMEP generation and postoperative outcomes.
Methods: A total of 345 patients who underwent spine surgery with intraoperative mMEP monitoring were included, and we retrospectively reviewed their demographic/clinical parameters, and mMEP recording results according to lesion locations.
Results: Multivariable logistic regression analysis revealed that preoperative Medical Research Council grade of the weakest muscle <3 was significantly associated with failure of baseline mMEP generation in both cervical and thoracic lesions.
Mol Neurobiol
March 2019
Amyotrophic lateral sclerosis (ALS) is a fatal, adult-onset, progressive neurodegenerative disorder with no known cure. Cu/Zn-superoxide dismutase (SOD1) was the first identified protein associated with familial ALS (fALS). Recently, TAR DNA-binding protein 43 (TDP-43) has been found to be a principal component of ubiquitinated cytoplasmic inclusions in neurons and glia in ALS.
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