• Title/Summary/Keyword: neuromyelitis optica spectrum disorder

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Autonomic dysfunction in multiple sclerosis and neuromyelitis optica spectrum disorder

  • Soonwook Kwon;Ju-Hong Min
    • Annals of Clinical Neurophysiology
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    • v.25 no.1
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    • pp.19-26
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    • 2023
  • Autonomic dysfunction occurs frequently in multiple sclerosis (MS) and neuromyelitis optica spectrum disorder (NMOSD). Patients with either condition may present with autonomic symptoms such as bladder, sexual, cardiovascular, thermoregulatory, and gastrointestinal dysfunction, and fatigue, but autonomic symptoms that affect quality of life are underrecognized in clinical practice. The immunopathogenesis of MS has been considered to be associated with autonomic dysfunction. Applying appropriate treatment strategies for autonomic dysfunction is important to improve the quality of life of patients. Here we review autonomic dysfunction and how this is managed in patients with MS and NMOSD.

Investigation of serum biomarkers for neuropathic pain in neuromyelitis optica spectrum disorder: a preliminary study

  • Hyun, Jae-Won;Kim, Yeseul;Kim, Ho Jin
    • Annals of Clinical Neurophysiology
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    • v.23 no.1
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    • pp.46-52
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    • 2021
  • Background: We aimed to investigate candidates for serological biomarkers of neuropathic pain in individuals with neuromyelitis optica spectrum disorder (NMOSD). Methods: We analyzed 38 sera samples from 38 participants with NMOSD in National Cancer Center. Neuropathic pain was evaluated using the painDETECT questionnaire. Pain with neuropathic components (painDETECT score ≥ 13) was observed in 22 participants, among whom 17 had definite neuropathic pain (painDETECT score ≥ 19). The remaining 16 participants had non-neuropathic pain (painDETECT score < 13). Serum glial fibrillary acidic protein (GFAP) levels were assessed using a single-molecule array assay. Several cytokines, including tumor necrosis factor-alpha (TNF-α), interleukin (IL)-6, IL-10, and IL-17A, were measured by a multiplex bead-based immunoassay. Results: In comparison of NMOSD participants with neuropathic pain components (or definite neuropathic pain) and those with non-neuropathic pain, the absolute values of serum GFAP, TNF-α, IL-6, and IL-10 levels were higher in participants with neuropathic pain components (or definite neuropathic pain), but these findings did not reach statistical significance. Conclusions: Further larger-scale investigations to find reliable serological biomarkers for neuropathic pain in NMOSD are warranted.

Results of a Survey on Diagnostic Procedures and Treatment Choices for Neuromyelitis Optica Spectrum Disorder in Korea: Beyond the Context of Current Clinical Guidelines

  • Hye Lim Lee;Su-Hyun Kim;Jin Myoung Seok;Byung Jo Kim;Ho Jin Kim;Byoung Joon Kim
    • Journal of clinical neurology
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    • v.18 no.2
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    • pp.207-213
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    • 2022
  • Background and Purpose Neuromyelitis optica spectrum disorder (NMOSD) is a rare demyelinating disease of the central nervous system (CNS). We investigated the medical behaviors of experts in Korea when they are diagnosing and treating NMOSD. Methods An anonymous questionnaire on the diagnosis and treatment of NMOSD was distributed to experts in CNS demyelinating diseases. Results Most respondents used the 2015 diagnostic criteria for NMOSD and applied a cerebrospinal fluid examination, magnetic resonance imaging (MRI) of the brain and spine, and anti-aquaporin-4 antibody testing to all suspected cases of NMOSD. All respondents prescribed steroid pulse therapy as an first-line therapy in the acute phase of NMOSD, and 67% prescribed azathioprine for maintenance therapy in NMOSD. However, details regarding monitoring, the tapering period of oral steroids, second-line therapy use in refractory cases, management during pregnancy, and schedule of follow-up MRI differed according to the circumstances of individual patients. We analyzed the differences in response rates between two groups of respondents according to the annual number of NMOSD patients that they treated. The group that had been treating ≥10 NMOSD patients annually preferred rituximab more often as the second-line therapy (p=0.011) and had more experience with rituximab treatment (p=0.015) compared with the group that had been treating <10 NMOSD patients. Conclusions This study has revealed that NMOSD experts in Korea principally follow the available treatment guidelines. However, the differences in specific clinical practices applied to uncertain cases that have been revealed will need to be investigated further in order to formulate suitable recommendations.

Neuromyelitis optica spectrum disorders with an inverted V sign on spinal cord magnetic resonance imaging: anti-aquaporin-4 antibody and functional vitamin B12 deficiency (척수에 뒤집힌 V징후를 가진 시신경척수염: 항아쿠아포린-4항체와 비타민 B12 기능적 결핍)

  • Sung Jo Bang;Sohyeon Kim;Young Seok Jeong;Seo Hyeon Lee;Hung Youl Seok
    • Journal of Medicine and Life Science
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    • v.19 no.3
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    • pp.130-133
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    • 2022
  • Several studies have reported a possible link between anti-aquaporin-4 antibody and vitamin B12 deficiency in neuromyelitis optica spectrum disorder (NMOSD). Bilaterally symmetric hyperintense signals on magnetic resonance imaging (MRI) of the posterior columns, called the inverted V sign, are a characteristic feature of subacute combined degeneration associated with vitamin B12 deficiency. We report a patient with anti-aquaporin-4 antibody-positive NMOSD and an inverted V sign on MRI of the spinal cord and address the association between anti-aquaporin-4 antibody and functional vitamin B12 deficiency.

Myelin oligodendrocyte glycoprotein antibody-associated disorders: clinical spectrum, diagnostic evaluation, and treatment options

  • Lee, Yun-Jin;Nam, Sang Ook;Ko, Ara;Kong, JuHyun;Byun, Shin Yun
    • Clinical and Experimental Pediatrics
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    • v.64 no.3
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    • pp.103-110
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    • 2021
  • Inflammatory or immune-mediated demyelinating central nervous system (CNS) syndromes include a broad spectrum of clinical phenotype and different overlapping diseases. Antibodies against myelin oligodendrocyte glycoprotein (MOG-Ab) have been found in some cases of these demyelinating diseases, particularly in children. MOG-Ab is associated with a wider clinical phenotype not limited to neuromyelitis optica spectrum disorder, with most patients presenting with optic neuritis, acute disseminated encephalomyelitis (ADEM) or ADEM-like encephalitis with brain demyelinating lesions, and/or myelitis. Using specific cell-based assays, MOG-Ab is becoming a potential biomarker of inflammatory demyelinating disorders of the CNS. A humoral immune reaction against MOG was recently found in monophasic diseases and recurrent/multiphasic clinical progression, particularly in pediatric patients. This review summarizes the data regarding MOG-Ab as an impending biological marker for discriminating between these diverse demyelinating CNS diseases and discusses recent developments, clinical applications, and findings regarding the immunopathogenesis of MOG-Ab-associated disorders.