100 research outputs found
Value of Area Postrema Syndrome in Differentiating Adults With AQP4 vs. MOG Antibodies
Objectives: To compare the frequency of area postrema syndrome (APS) in adults with anti-aquaporin-4 (AQP4) and anti-myelin oligodendrocyte glycoprotein (MOG) antibodies. Methods: APS is defined as acute or subacute, single or combined, episodic or constant nausea, vomiting, or hiccups, persisting for at least 48 h, which cannot be attributed to any other etiology. The presence of APS was investigated in 274 adults with AQP4 antibodies and 107 adults with MOG antibodies from 10 hospitals. Results: The study population comprised Korean adults (β₯18 years). At the time of disease onset, 14.9% (41/274) adults with AQP4 antibodies had APS, while none of the participants with MOG antibodies developed APS (p < 0.001). During the course of the disease, 17.2% (47/274) adults with AQP4 antibodies had APS in contrast to 1.9% (2/107) adults with MOG antibodies with APS (p < 0.001). Conclusions: APS, one of the core clinical characteristics of individuals with AQP4 antibodies, is an extremely rare manifestation in Korean adults with MOG antibodies.ope
A Case of Anti-Myelin Oligodendrocyte Glycoprotein Antibody-Positive Late-Onset Acute Disseminated Encephalomyelitis
ope
Anti-Myelin Oligodendrocyte Glycoprotein Antibody-Positive Encephalitis with Seizure and Unilateral Cortical Fluid-Attenuated Inversion Recovery-Hyperintense Lesions
ope
Whole-Body Muscle MRI in Patients with Hyperkalemic Periodic Paralysis Carrying the SCN4A Mutation T704M: Evidence for Chronic Progressive Myopathy with Selective Muscle Involvement
BACKGROUND AND PURPOSE: Hyperkalemic periodic paralysis (hyperKPP) is a muscle sodium-ion channelopathy characterized by recurrent paralytic attacks. A proportion of affected individuals develop fixed or chronic progressive weakness that results in significant disability. However, little is known about the pathology of hyperKPP-induced fixed weakness, including the pattern of muscle involvement. The aim of this study was to characterize the patterns of muscle involvement in hyperKPP by whole-body magnetic resonance imaging (MRI).
METHODS: We performed whole-body muscle MRI in seven hyperKPP patients carrying the T704M mutation in the SCN4A skeletal sodium-channel gene. Muscle fat infiltration, suggestive of chronic progressive myopathy, was analyzed qualitatively using a grading system and was quantified by the two-point Dixon technique.
RESULTS: Whole-body muscle MRI analysis revealed muscle atrophy and fatty infiltration in hyperKPP patients, especially in older individuals. Muscle involvement followed a selective pattern, primarily affecting the posterior compartment of the lower leg and anterior thigh muscles. The muscle fat fraction increased with patient age in the anterior thigh (r=0.669, p=0.009), in the deep posterior compartment of the lower leg (r=0.617, p=0.019), and in the superficial posterior compartment of the lower leg (r=0.777, p=0.001).
CONCLUSIONS: Our whole-body muscle MRI findings provide evidence for chronic progressive myopathy in hyperKPP patients. The reported data suggest that a selective pattern of muscle involvement-affecting the posterior compartment of the lower leg and the anterior thigh-is characteristic of chronic progressive myopathy in hyperKPP.ope
Prevalence and Incidence of Neuromyelitis Optica Spectrum Disorder in Korea: Population Based Study
Background: Although neuromyelitis optica spectrum disorder (NMOSD) is known to be a rare disease, its prevalence and incidence have not yet been studied in Korea. We performed a population-based study to examine the prevalence and incidence of NMOSD in Korea using data from the Korean National Health Insurance (NHI) claims database.
Methods: Data from 2013 to 2017 were obtained, with a washout period set as 2013 and 2014. The prevalence and incidence of NMOSD in 2016 and 2017 were calculated using population census data. Subjects were divided into 5 groups at 15-year intervals, depending on the age at which the diagnostic code was entered. The relative risk (RR) for each age group was compared with the oldest (β₯ 60 years) age group.
Results: The overall prevalence was estimated to be 3.36 and 3.56 per 100,000 individuals, with an incidence of 0.41 and 0.65 per 100,000 individuals-year in 2016 and 2017, respectively. The mean age was 43.08 (standard deviation, 14.56) years, and the ratio of male to females was 1:4.7. The incidence was higher in female individuals aged between 30 and 59 years (RR, 2.8-3.05; P < 0.05).
Conclusion: Nationwide prevalence of NMOSD in Korea was 3.36 and 3.56/100,000 and its incidence was 0.41 and 0.65/100,000-year in 2016 and 2017 respectively.ope
Early Electrodiagnostic Features of Upper Extremity Sensory Nerves Can Differentiate Axonal Guillain-BarrΓ© Syndrome from Acute Inflammatory Demyelinating Polyneuropathy
BACKGROUND AND PURPOSE: Serial nerve conduction studies (NCSs) are recommended for differentiating axonal and demyelinating Guillain-Barr? syndrome (GBS), but this approach is not suitable for early diagnoses. This study was designed to identify possible NCS parameters for differentiating GBS subtypes.
METHODS: We retrospectively reviewed the medical records of 70 patients with GBS who underwent NCS within 10 days of symptom onset. Patients with axonal GBS and acute inflammatory demyelinating polyneuropathy (AIDP) were selected based on clinical characteristics and serial NCSs. An antiganglioside antibody study was used to increase the diagnostic certainty.
RESULTS: The amplitudes of median and ulnar nerve sensory nerve action potentials (SNAPs) were significantly smaller in the AIDP group than in the axonal-GBS group. Classification and regression-tree analysis revealed that the distal ulnar sensory nerve SNAP amplitude was the best predictor of axonal GBS.
CONCLUSIONS: Early upper extremity sensory NCS findings are helpful in differentiating axonal-GBS patients with antiganglioside antibodies from AIDP patients.ope
Anti-titin antibody is associated with more frequent hospitalization to manage thymoma-associated myasthenia gravis
Background and purpose: Anti-titin antibodies are antistriational antibodies associated with thymoma-associated myasthenia gravis (MG). We evaluated whether the patients with anti-titin antibody are more frequently hospitalized to manage thymoma-associated MG than those patients without anti-titin antibody.
Methods: Patients with thymoma-associated MG who conducted the serological test for anti-titin antibody were retrospectively included. Disease severity, treatments, MG-related annual hospitalization rate, and MG-related emergency room (ER) visit rate were compared between the patients with anti-titin antibody and those patients without anti-titin antibody. Multivariate analysis was conducted to analyze the association between anti-titin antibody serostatus and multiple admissions (hospitalization or ER visit of β₯2 times).
Results: Of the 64 included patients, 31 (48.4%) patients were positive for anti-titin antibody (titin+ group) and 33 (51.6%) patients were negative for anti-titin antibody (titin- group). Both the annual rate of MG-related hospitalization and ER visit were significantly higher in the titin+ group [0.2 (0.1-0.6) and 0.1 (0-0.2) per year, respectively] than those in the titin- group [0 (0-0.2) and 0 (0-0) per year, p = 0.004 and p = 0.006, respectively]. In multivariate analysis, positive anti-titin antibody was still significantly associated with multiple admissions [odds ratio (OR) 4.11, 95% CI 1.05-16.03] compared to the titin- group as a reference after adjusting for sex, follow-up duration, age at onset, systemic chemotherapy, and the Masaoka staging.
Conclusion: The presence of anti-titin antibody is associated with more frequent hospital utilization. Personalized explanation and careful monitoring strategy could be required in patients with thymoma-associated MG with anti-titin antibody for the timely detection of relapses.ope
Autoantibody Testing in Neuromuscular Disorders
Autoantibodies are present in many autoimmune disorders, including diseases impacting the peripheral nerve, neuromuscular junction, and muscle. Some of these autoantibodies play a vital role in pathogenesis, whereas others are unlikely to be directly pathogenic, but may be useful biomarkers. The identification of autoantibodies is valuable in diagnosis, as well as in establishing a treatment plan in antibody-mediated neuromuscular disorders. This review briefly summarizes antibody, autoantibody, and methods of autoantibody testing for clinicians who treat patients with neuromuscular disorders.ope
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Abstract 95Maste
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