Neuromyelitis optica spectrum disorder in a tertiary hospital in the Philippines: a case series

Objective:To describe leptomeningeal blood-barrier impairment reflected by MRI gadolinium-enhanced lesions in patients with aquaporin-4 immunoglobulin G (AQP4-IgG)–positive neuromyelitis optica spectrum disorder (NMOSD).Methods:A retrospective case series of 11 AQP4-IgG–positive NMOSD patients with leptomeningeal enhancement (LME) were collected from 5 centers. External neuroradiologists, blinded to the clinical details, evaluated MRIs.Results:LME was demonstrated on postcontrast T1-weighted and fluid-attenuated inversion recovery images as a sign of leptomeningeal blood-barrier disruption and transient leakage of contrast agent into the subarachnoid space in 11 patients, 6 in the brain and 6 in the spinal cord. The patterns of LME were linear or extensive and were accompanied by periependymal enhancement in 5 cases and intraparenchymal enhancement in all cases. The location of LME in the spinal cord was adjacent to intraparenchymal contrast enhancement with involvement of a median number of 12 (range 5–17) vertebral segments. At the time of LME on MRI, all patients had a clinical attack such as encephalopathy (36%) and/or myelopathy (70%) with median interval between symptom onset and LME of 12 days (range 2–30). LME occurred in association with an initial area postrema attack (44%), signs of systemic infection (33%), or AQP4-IgG in CSF (22%) followed by clinical progression. LME was found at initial clinical presentation in 5 cases and at clinical relapses leading to a diagnosis of NMOSD in 6 cases.Conclusion:This study suggests that altered leptomeningeal blood barrier may be accompanied by intraparenchymal blood-brain barrier breakdown in patients with AQP4-IgG–positive NMOSD during relapses.

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Pediatric Neuromyelitis Optica Spectrum Disorder: Case Series and Literature Review

Life ◽

10.3390/life12010019 ◽

2021 ◽

Vol 12 (1) ◽

pp. 19

Author(s):

Michela Ada Noris Ferilli ◽

Roberto Paparella ◽

Ilaria Morandini ◽

Laura Papetti ◽

Lorenzo Figà Talamanca ◽

...

Keyword(s):

Neuromyelitis Optica ◽

Pediatric Patients ◽

Demyelinating Disease ◽

Area Postrema ◽

Case Series ◽

Myelin Oligodendrocyte Glycoprotein ◽

Spectrum Disorder ◽

Neuromyelitis Optica Spectrum Disorder ◽

Periaqueductal Gray Matter ◽

Aquaporin 4 Antibody

Neuromyelitis Optica Spectrum Disorder (NMOSD) is a central nervous system (CNS) inflammatory demyelinating disease characterized by recurrent inflammatory events that primarily involve optic nerves and the spinal cord, but also affect other regions of the CNS, including hypothalamus, area postrema and periaqueductal gray matter. The aquaporin-4 antibody (AQP4-IgG) is specific for NMOSD. Recently, myelin oligodendrocyte glycoprotein antibodies (MOG-IgG) have been found in a group of AQP4-IgG negative patients. NMOSD is rare among children and adolescents, but early diagnosis is important to start adequate therapy. In this report, we present cases of seven pediatric patients with NMOSD and we review the clinical and neuroimaging characteristics, diagnosis, and treatment of NMOSD in children.

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Interleukin-6 in neuromyelitis optica spectrum disorder pathophysiology

Neurology Neuroimmunology & Neuroinflammation ◽

10.1212/nxi.0000000000000841 ◽

2020 ◽

Vol 7 (5) ◽

pp. e841 ◽

Cited By ~ 2

Author(s):

Kazuo Fujihara ◽

Jeffrey L. Bennett ◽

Jerome de Seze ◽

Masayuki Haramura ◽

Ingo Kleiter ◽

...

Keyword(s):

Neuromyelitis Optica ◽

Interleukin 6 ◽

Case Series ◽

Autoimmune Disorder ◽

Multiple Roles ◽

Spectrum Disorder ◽

Neuromyelitis Optica Spectrum Disorder ◽

Neurologic Disability ◽

Recent Phase

Neuromyelitis optica spectrum disorder (NMOSD) is a rare autoimmune disorder that preferentially affects the spinal cord and optic nerve. Most patients with NMOSD experience severe relapses that lead to permanent neurologic disability; therefore, limiting frequency and severity of these attacks is the primary goal of disease management. Currently, patients are treated with immunosuppressants. Interleukin-6 (IL-6) is a pleiotropic cytokine that is significantly elevated in the serum and the CSF of patients with NMOSD. IL-6 may have multiple roles in NMOSD pathophysiology by promoting plasmablast survival, stimulating the production of antibodies against aquaporin-4, disrupting blood-brain barrier integrity and functionality, and enhancing proinflammatory T-lymphocyte differentiation and activation. Case series have shown decreased relapse rates following IL-6 receptor (IL-6R) blockade in patients with NMOSD, and 2 recent phase 3 randomized controlled trials confirmed that IL-6R inhibition reduces the risk of relapses in NMOSD. As such, inhibition of IL-6 activity represents a promising emerging therapy for the management of NMOSD manifestations. In this review, we summarize the role of IL-6 in the context of NMOSD.

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P-OT003. A case series of familial neuromyelitis optica spectrum disorder (NMOSD)

Clinical Neurophysiology ◽

10.1016/j.clinph.2021.02.304 ◽

2021 ◽

Vol 132 (8) ◽

pp. e123

Author(s):

Chai Liang Ang ◽

Jie Ping Schee ◽

Eng Kian Ng ◽

Yuen Kang Chia

Keyword(s):

Neuromyelitis Optica ◽

Case Series ◽

Spectrum Disorder ◽

Neuromyelitis Optica Spectrum Disorder

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Adverse events of rituximab in neuromyelitis optica spectrum disorder: a systematic review and meta-analysis

Therapeutic Advances in Neurological Disorders ◽

10.1177/17562864211056710 ◽

2021 ◽

Vol 14 ◽

pp. 175628642110567

Author(s):

Hao Wang ◽

Juanping Zhou ◽

Yi Li ◽

Lili Wei ◽

Xintong Xu ◽

...

Keyword(s):

Adverse Events ◽

Neuromyelitis Optica ◽

Meta Analysis ◽

Case Series ◽

Spectrum Disorder ◽

Cochrane Library ◽

Neuromyelitis Optica Spectrum Disorder ◽

Duration Of Illness ◽

Evidence Quality ◽

Study Designs

Background: The adverse events (AEs) of rituximab (RTX) for neuromyelitis optica spectrum disorder (NMOSD) are incompletely understood. Aim: To collate information on the reported the AEs of RTX in NMOSD and assess the quality of evidence. Methods: PubMed, EMBASE, Web of Science, Cochrane Library, Wanfang Data, CBM, CNKI, VIP, clinicaltrials.gov, and so on were searched for studies with control groups as well as for case series that had assessed the RTX-associated AEs. The incidence of AEs and the comparison of AE risks among different therapies were pooled. The GRADE was developed for evidence quality. Results: A total of 3566 records were identified. Finally, 36 studies (4 RCTs, 6 crochet studies, 2 NRCTs, and 24 case series), including 1542 patients (1299 females and 139 males), were included for final analyses. Rates of patients with any AEs, any serious AEs (SAEs), infusion-related AEs, any infection, respiratory infection, urinary infection, and death were 28.57%, 5.66%, 27.01%, 17.36%, 4.76%, 4.76%, and 0.17%, respectively. The results from subgroup analysis showed that AE rates were most likely not associated with covariates such as duration of illness and study designs. Very low-quality evidence suggested that the risk ratios (RR) of any AEs (0.84, 95% CI = 00.42–1.69, p = 0.62) and any infections (1.24 95% CI = 0.18–8.61) of RTX were similar to that of azathioprine, and the RR of any AEs of RXT was akin to that of mycophenolate mofetil (0.66, 95% CI = 0.32–1.35 p = 0.26). Evidence of low to high quality showed the lower RR of RTX in other AEs, but not in infusion-related AEs. Strategies to handle AEs focused on symptomatic treatments. Conclusions: RTX is mostly safer than other immunosuppressants in NMOSD: the incidence of RTX-associated AEs was not high, and when present, the AEs were usually mild or moderate and could be well controlled. Given its efficacy and safety, RTX could be recommended as a first-line treatment for NMOSD.

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Seropositive neuromyelitis optica spectrum disorder in Emirati patients: A case series

Journal of Clinical Neuroscience ◽

10.1016/j.jocn.2019.11.045 ◽

2020 ◽

Vol 72 ◽

pp. 185-190

Author(s):

Antonia Ceccarelli ◽

Victoria Ann Mifsud ◽

Amna Dogar ◽

Syed Irteza Hussain

Keyword(s):

Neuromyelitis Optica ◽

Case Series ◽

Spectrum Disorder ◽

Neuromyelitis Optica Spectrum Disorder

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Recent progress in maintenance treatment of neuromyelitis optica spectrum disorder

Journal of Neurology ◽

10.1007/s00415-020-10235-5 ◽

2020 ◽

Author(s):

Trygve Holmøy ◽

Rune Alexander Høglund ◽

Zsolt Illes ◽

Kjell-Morten Myhr ◽

Øivind Torkildsen

Keyword(s):

Clinical Trials ◽

Monoclonal Antibodies ◽

Neuromyelitis Optica ◽

Randomized Clinical Trials ◽

Inflammatory Diseases ◽

Case Series ◽

Spectrum Disorder ◽

Neuromyelitis Optica Spectrum Disorder ◽

Retrospective Case ◽

Superior Efficacy

Abstract Background Treatment of neuromyelitis optica spectrum disorder (NMOSD) has so far been based on retrospective case series. The results of six randomized clinical trials including five different monoclonal antibodies targeting four molecules and three distinct pathophysiological pathways have recently been published. Methods Literature search on clinical trials and case studies in NMOSD up to July 10. 2020. Results We review mechanism of action, efficacy and side effects, and consequences for reproductive health from traditional immunosuppressants and monoclonal antibodies including rituximab, inebilizumab, eculizumab, tocilizumab and satralizumab. Conclusion In NMOSD patients with antibodies against aquaporin 4, monoclonal antibodies that deplete B cells (rituximab and inebilizumab) or interfere with interleukin 6 signaling (tocilizumab and satralizumab) or complement activation (eculizumab) have superior efficacy compared to placebo. Tocilizumab and rituximab were also superior to azathioprine in head-to-head studies. Rituximab, tocilizumab and to some extent eculizumab have well-known safety profiles for other inflammatory diseases, and rituximab and azathioprine may be safe during pregnancy.

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