ABSTRACT
BACKGROUND AND PURPOSE: This study assessed the long-term outcomes of disease-modifying therapies (DMTs) in Korean multiple sclerosis (MS) patients treated in real-world clinical settings in Korea. METHODS: We retrospectively evaluated the medical records of 160 patients with an initial diagnosis of clinically isolated syndrome or relapsing-remitting MS who were treated for at least 2 years. A status of 3 for no evidence of disease activity (NEDA3) was defined as no relapse, disability progression, or active lesions in annual magnetic resonance imaging (MRI) evaluations. RESULTS: Patients who were initially treated with interferon β (n=152), glatiramer acetate (n=6), or teriflunomide (n=2) were included. The mean disease duration was 8.2 years. Compared to pretreatment, annualized relapse rates were significantly reduced after treatment [from 1.0±0.8 to 0.2±0.4 (mean±standard deviation), p < 0.001]. At the follow-up, 79 patients (49%) had changed their treatment regimen due to lack of efficacy (33%), side effects (14%), or other reasons (2%). Disability progression was observed in 18% of the patients over a mean treatment duration of 5.7 years. After 2 years, NEDA3 was observed in 38% of the patients. Loss of NEDA3 at 2 years was associated with long-term disability progression [odds ratio (OR)=17.975, p=0.003]. Poor response to first-line treatment was independently associated with a delay in treatment from disease onset (OR=1.238, p=0.049) and 10 or more brain lesions in the initial MRI (OR=3.648, p=0.047). CONCLUSIONS: This study has provided real-world evidence that DMTs are effective in reducing disease activity and disability progression in Korean MS patients.
Subject(s)
Humans , Brain , Diagnosis , Follow-Up Studies , Glatiramer Acetate , Interferons , Korea , Magnetic Resonance Imaging , Medical Records , Multiple Sclerosis , Recurrence , Retrospective StudiesABSTRACT
BACKGROUND AND PURPOSE: Alemtuzumab has shown high efficacy in clinical trials that primarily involved Western multiple sclerosis (MS) patients. To evaluate the therapeutic outcome of alemtuzumab in Korean patients with MS. METHODS: This study enrolled 23 consecutive patients who were treated with alemtuzumab from 2015 to 2018. Efficacy was evaluated using the annualized relapse rate (ARR), Expanded Disability Status Scale (EDSS), and radiological activity. No evidence of disease activity (NEDA) was defined as no clinical relapse, no worsening of the EDSS score, and no radiological activity. The safety profiles were also assessed. RESULTS: The mean age was 36 years and 16 of the patients were female. Seventeen and 12 of 23 patients were followed up for 1 year and 2 years, respectively. The ARR was markedly reduced from 1.52 during the 1-year period preceding alemtuzumab administration to 0.21 after initiating alemtuzumab (p<0.001). During the first and second years after initiating alemtuzumab, EDSS worsening was observed in 3 (18%) and 0 (0%) patients, respectively, and radiological activity was exhibited in 9 (53%) and 4 (33%). NEDA was observed in 6 (35%) patients during the first year and in 8 (67%) patients during the second year. Intriguingly, one patient experienced 2 severe clinical exacerbations, which occurred at 10 months after the first and 10 months after the second infusion of alemtuzumab. Nineteen of the 23 patients exhibited infusion-associated reactions and 3 patients exhibited herpes zoster infection. Thyroid dysfunction occurred in two patients at 18 and 20 months after initiating alemtuzumab. CONCLUSIONS: Consistent with observations in Western populations, alemtuzumab therapy in Korean MS patients led to marked reductions of disease activity without unexpected safety issues.
Subject(s)
Female , Humans , Follow-Up Studies , Herpes Zoster , Multiple Sclerosis , Recurrence , Thyroid GlandABSTRACT
Myasthenia gravis (MG) is an autoimmune disease caused by autoantibodies to the acetylcholine receptors of the neuromuscular junction characterized by weakness and abnormal fatigability of the muscles. Therefore, the diagnosis of MG depends on the recognition of this distinctive pattern of fatigable weakness. Previous studies presented the diagnostic efficacy of saccadic eye movements in patients with ocular MG. We here in report 2 patients of ocular MG showing the fatigue effects during repetitive sustained smooth pursuit, and the effects of the administration of edrophonium on myasthenic smooth pursuit. Changes in smooth pursuits reflecting peripheral and secondary central mechanisms were demonstrated.
Subject(s)
Humans , Autoantibodies , Autoimmune Diseases , Diagnosis , Edrophonium , Fatigue , Muscles , Myasthenia Gravis , Neuromuscular Junction , Pursuit, Smooth , Receptors, Cholinergic , SaccadesABSTRACT
Acute vestibular neuritis (VN) is characterized by acute/subacute vertigo with spontaneous nystagmus and unilateral loss of semicircular canal function. Vestibular system in human is represented in the brain bilaterally with functional asymmetries of the right hemispheric dominance in the right handers. Spatial working memory entails the ability to keep spatial information active in working memory over a short period of time which is also known as the right hemispheric dominance. Three patients (patient 1, 32-year-old female; patient 2, 18-year-old male; patient 3, 63-year-old male) suffered from acute onset of severe vertigo, nausea and vomiting. Patients 1 and 2's examination revealed VN on the right side showing spontaneous left beating nystagmus and impaired vestibular ocular reflex on the right side in video head-impulse and caloric tests. Patient 3's finding was fit for VN on the left side. We also evaluated visuospatial memory function with the block design test in these 3 VN patients which discovered lower scores in patients 1 and 2 and the average level in patient 3 compare to those of healthy controls. Follow-up block design test after resolved symptoms showed within normal range in both patients. Our cases suggest that the patients with unilateral peripheral vestibulopathy may have an asymmetrical effect on the higher vestibular cognitive function. The right VN can be associated with transient visuospatial memory dysfunction. These findings add the evidence of significant right hemispheric dominance for vestibular and visuospatial structures in the right-handed subjects, and of predominant dysfunction in the hemisphere ipsilateral to the peripheral lesion side.
Subject(s)
Adolescent , Adult , Female , Humans , Male , Middle Aged , Brain , Caloric Tests , Cognition , Follow-Up Studies , Memory , Memory, Short-Term , Nausea , Reference Values , Reflex , Semicircular Canals , Vertigo , Vestibular Neuronitis , VomitingABSTRACT
BACKGROUND AND PURPOSE: Patients treated with interferon-beta (IFN-β) can develop neutralizing antibodies (NAbs) against IFN-β that can negatively affect the therapeutic response. This study assessed the prevalence of NAbs and the impact of NAb positivity on the therapeutic response to IFN-β in Korean patients with multiple sclerosis (MS). METHODS: This was a multicenter study involving 150 MS patients from 9 Korean medical centers who were treated with IFN-β for at least 6 months. Sera that had not been influenced by acute treatment were assessed for NAbs using a luciferase reporter gene assay. To evaluate the association between persistent positivity for NAbs and disease activity, NAbs were tested at 2 different time points in 75 of the 150 patients. Disease activity was defined as the presence of clinical exacerbations and/or active MRI lesions during a 1-year follow-up after NAb positivity was confirmed. RESULTS: NAbs were found in 39 of the 150 (26%) MS patients: 30 of the 85 (35%) who were treated with subcutaneous IFN-β-1b, 9 of the 60 (15%) who were treated with subcutaneous IFN-β-1a, and 0 of the 5 (0%) who were treated with intramuscular IFN-β-1a. Thirty of the 39 patients exhibiting NAb positivity were tested at different time points, and 20 of them exhibited persistent NAb positivity. Disease activity was observed more frequently in patients with persistent NAb positivity than in those with transient positivity or persistent negativity [16/20 (80%) vs. 4/55 (7%), respectively; p < 0.001]. When disease activity was compared between patients with persistent and transient NAb positivity, the difference was unchanged and remained statistically significant [16/20 (80%) vs. 2/10 (20%), p=0.004]. CONCLUSIONS: These results further support that persistent NAb positivity is associated with disease activity in MS patients treated with IFN-β.
Subject(s)
Humans , Antibodies, Neutralizing , Follow-Up Studies , Genes, Reporter , Interferon-beta , Luciferases , Magnetic Resonance Imaging , Multiple Sclerosis , PrevalenceABSTRACT
BACKGROUND: Cervical (cVEMP) and ocular (oVEMP) vestibular-evoked myogenic potentials are short-latency manifestations of vestibulo-ocular and vestibulocollic reflexes that originate from the utricle and saccule. The aim of this study was to determine whether simultaneously recording cVEMP and oVEMP more reliably detects the reflex responses than when making separate recordings. METHODS: Both simultaneous recordings and conventional separate recordings of the cVEMP and oVEMP responses to air-conducted tone bursts were obtained in 39 healthy subjects. p13 and n10 latencies, and p13-n23 and n10-p16 amplitudes of cVEMP and oVEMP waveforms were measured, respectively. The asymmetry ratios (ARs) of the amplitudes were calculated. RESULTS: In cVEMP testing, the p13 latencies and p13-n23 amplitudes did not differ significantly between simultaneous and conventional separate recordings. In oVEMP testing, the n10 latencies measured in simultaneous recordings showed a small but statistically significant prolongation compared to the results in separate recordings. Moreover, the n10-p16 amplitudes were significantly larger in the simultaneous recordings. The AR of simultaneous recordings of cVEMP and oVEMP did not differ between the two recording methods. CONCLUSIONS: Simultaneously recording cVEMP and oVEMP provides a reliable test and could be a clinically useful and simplified diagnostic tool for evaluating dizzy patients.
Subject(s)
Humans , Reflex , Saccule and UtricleABSTRACT
BACKGROUND: Cervical (cVEMP) and ocular (oVEMP) vestibular-evoked myogenic potentials are short-latency manifestations of vestibulo-ocular and vestibulocollic reflexes that originate from the utricle and saccule. The aim of this study was to determine whether simultaneously recording cVEMP and oVEMP more reliably detects the reflex responses than when making separate recordings. METHODS: Both simultaneous recordings and conventional separate recordings of the cVEMP and oVEMP responses to air-conducted tone bursts were obtained in 39 healthy subjects. p13 and n10 latencies, and p13-n23 and n10-p16 amplitudes of cVEMP and oVEMP waveforms were measured, respectively. The asymmetry ratios (ARs) of the amplitudes were calculated. RESULTS: In cVEMP testing, the p13 latencies and p13-n23 amplitudes did not differ significantly between simultaneous and conventional separate recordings. In oVEMP testing, the n10 latencies measured in simultaneous recordings showed a small but statistically significant prolongation compared to the results in separate recordings. Moreover, the n10-p16 amplitudes were significantly larger in the simultaneous recordings. The AR of simultaneous recordings of cVEMP and oVEMP did not differ between the two recording methods. CONCLUSIONS: Simultaneously recording cVEMP and oVEMP provides a reliable test and could be a clinically useful and simplified diagnostic tool for evaluating dizzy patients.