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Front Immunol 2022 19;13:864664. Epub 2022 May 19.

Department of Neurology, Tongde Hospital of Zhejiang Province, Hangzhou, China.

Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune-mediated idiopathic inflammatory demyelinating disease with a typical clinical presentation of optic neuritis, acute myelitis, and area postrema syndrome. Most NMOSD patients are seropositive for disease-specific and pathogenic aquaporin-4 (AQP4) antibodies, which are key markers for the NMOSD diagnosis. Herein, we report an atypical case of a 41-year-old man who complained of intractable hiccups and vomiting at disease onset, followed by fever, headache, back pain, progressive paresthesia, and weakness of extremities later on. Read More

J Mol Neurosci 2022 Jun 30;72(6):1374-1401. Epub 2022 May 30.

Department of Health Sciences, Faculty of Applied Health Sciences, and Department of Biological Sciences, Faculty of Mathematics and Science, Brock University, ON, L2S 3A1, St. Catharine's, Canada.

A change in visual perception is a frequent early symptom of multiple sclerosis (MS), the pathoaetiology of which remains unclear. Following a slow demyelination process caused by 12 weeks of low-dose (0.1%) cuprizone (CPZ) consumption, histology and proteomics were used to investigate components of the visual pathway in young adult mice. Read More

Eur J Neurol 2022 May 26. Epub 2022 May 26.

Department of Neuro-Ophthalmology, Adolphe de Rothschild Foundation Hospital, Paris, France.

Background And Purpose: This study was undertaken to determine the role of optical coherence tomography (OCT) in predicting the final visual and structural outcome, and to evaluate the correlation between functional eye outcome and retinal changes, in patients with a first episode of optic neuritis (ON).

Methods: In this prospective study, consecutive adult patients with acute ON underwent ophthalmological evaluation at baseline and at 1 and 12 months, including OCT measurements of peripapillary retinal nerve fiber layer (pRNFL), macular ganglion cell and inner plexiform layer, and inner nuclear layer thicknesses; high- and low-contrast visual acuity; visual field assessment; and baseline brain magnetic resonance imaging. Univariate and multivariate linear regressions were used to assess predictive factors of outcome. Read More

J Clin Neurol 2022 May;18(3):280-289

Department of Neurology, Research Institute and Hospital of National Cancer Center, Goyang, Korea.

Antibodies against myelin oligodendrocyte glycoprotein (MOG-IgG) have recently been established as a biomarker for MOG-antibody-associated disease (MOGAD), which is a distinct demyelinating disease of the central nervous system. Among the diverse clinical phenotypes of MOGAD, myelitis is the second-most-common presentation in adults, followed by optic neuritis. While some features overlap, there are multiple reports of distinctive clinical and radiological features of MOG-IgG-associated myelitis, which are useful for differentiating MOGAD from both multiple sclerosis and neuromyelitis optica spectrum disorder. Read More

Front Immunol 2022 2;13:887041. Epub 2022 May 2.

Department of Pediatrics, The Second Xiangya Hospital of Central South University, Changsha, China.

Systemic lupus erythematosus (SLE) is an autoimmune connective tissue disease (CTD), the main features of which are multiple serum autoantibodies and extensive involvement of multiple systems. The onset age of patients varies from childhood to middle age, with nearly 1/5 in childhood. Sjogren's syndrome (SS) is also an autoimmune disease characterized by high-degree lymphocytic infiltration of exocrine glands, usually occurring in middle-aged and older women, and rarely in childhood. Read More

BMC Neurol 2022 May 18;22(1):185. Epub 2022 May 18.

Department of Neurology with Institute of Translational Neurology, University Hospital Münster, Albert-Schweitzer-Campus 1, 48149, Münster, Germany.

Background: Since the beginning of the COVID-19 pandemic and development of new vaccines, the issue of post-vaccination exacerbation or manifestation of demyelinating central nervous system (CNS) disorders has gained increasing attention.

Case Presentation: We present a case of a 68-year-old woman previously diagnosed with multiple sclerosis (MS) since the 1980s who suffered a rapidly progressive severe sensorimotor paraparesis with loss of bladder and bowel control due to an acute longitudinal extensive transverse myelitis (LETM) after immunization with the mRNA Pfizer-BioNTech COVID-19 vaccine. Detection of Aquaporin-4-antibodies (AQP4) in both serum and CSF led to diagnosis of AQP4-antibody positive neuromyelitis optica spectrum disorder (NMOSD). Read More

J Clin Neurosci 2022 Jul 12;101:89-93. Epub 2022 May 12.

Department of Neurology, Shengjing Hospital of China Medical University, Shenyang, China. Electronic address:

The neutrophil-to-lymphocyte ratio (NLR) is a biomarker for evaluating disease activity in systemic autoimmune diseases. However, few studies have discussed NLR changes in myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). This study aimed to explore the NLR difference between MOGAD, aquaporin-4 antibody (AQP4-Ab)-positive neuromyelitis optica spectrum disorders (NMOSD), and healthy controls (HCs) and evaluate the clinical value of NLR in the differential diagnosis. Read More

Mult Scler Relat Disord 2022 May 25;61:103772. Epub 2022 Mar 25.

Department of Neurology, Wakayama Medical University, 811-1 Kimiidera, Wakayama, Wakayama 641-8509, Japan.

Background: Neuromyelitis optica spectrum disorder (NMOSD) is a rare, chronic, autoimmune disease, characterized by astrocytopathic lesions in the central nervous system (Beekman et al., 2019; Fujihara et al., 2020). Read More

Eur J Med Res 2022 May 9;27(1):66. Epub 2022 May 9.

Department of Neurology, The Second Affiliated Hospital and Yuying Children's Hospital of Wenzhou Medical University, Zhejiang Province, Wenzhou, 325027, China.

Background: Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis can coexist with neuromyelitis optica spectrum disorder (NMOSD). Patients with overlapping anti-NMDAR encephalitis with positive NMDAR antibodies and aquaporin 4 immunoglobulin G (AQP4-IgG)-seropositive NMOSD are rare but should not be ignored.

Case Presentation: This report describes a unique case of anti-NMDAR encephalitis coexisting with NMOSD. Read More

Am J Case Rep 2022 May 1;23:e936283. Epub 2022 May 1.

Division of Academic Affairs and Research, Orlando Regional Healthcare, Orlando, FL, USA.

BACKGROUND Neuromyelitis optica (NMO) is a rare neurological disease characterized by attacks of transverse myelitis and optic neuritis, contiguous spinal cord lesions on more than 3 vertebral segments on magnetic resonance imaging (MRI), and seropositivity for AQP-4 Ab. The tissue destruction from NMO is immune mediated and results in demyelination and axonal damage. Optic and spinal nerve involvement can eventually lead to blindness, weakness, and altered consciousness, and bladder and bowel involvement in some cases. Read More

J Neurol Sci 2022 Jun 16;437:120262. Epub 2022 Apr 16.

Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, MA, United States of America; Department of Neurology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, United States of America. Electronic address:

Objective: To investigate the clinical and magnetic resonance imaging (MRI) characteristics of patients with varicella zoster virus (VZV) reactivation involving the cranial nerves and central nervous system (CNS).

Methods: This is a retrospective, multi-center case-series of 37 patients with VZV infection affecting the cranial nerves and CNS.

Results: The median age was 71 years [IQR 51. Read More

Rinsho Shinkeigaku 2022 May 26;62(5):351-356. Epub 2022 Apr 26.

Department of Neurology, Kobe City Nishi-Kobe Medical Center.

We report a case of a patient with neuromyelitis optica spectrum disorder (NMOSD) who was originally treated for multiple sclerosis (MS) due to a negative anti-aquaporin-4 (AQP4) antibody test, but later the antibody titer became positive. A 58-year-old woman without prior medical history developed acute left facial pain, vomiting, and hiccups. MRI showed an intraparenchymal lesion extending from the medulla oblongata to cervical cord with high T-weighted signal intensity. Read More

Sci Transl Med 2022 04 20;14(641):eabe9726. Epub 2022 Apr 20.

The Feinstein Institutes for Medical Research, Northwell Health, Manhasset NY 11030, USA.

The fetal brain is constantly exposed to maternal IgG before the formation of an effective blood-brain barrier (BBB). Here, we studied the consequences of fetal brain exposure to an antibody to the astrocytic protein aquaporin-4 (AQP4-IgG) in mice. AQP4-IgG was cloned from a patient with neuromyelitis optica spectrum disorder (NMOSD), an autoimmune disease that can affect women of childbearing age. Read More

J Neuroophthalmol 2022 06 25;42(2):239-245. Epub 2022 Mar 25.

Background: Neuromyelitis optica spectrum disease (NMOSD) and multiple sclerosis (MS) share clinical presentations including optic neuritis and brainstem syndromes. Internuclear ophthalmoplegia (INO) is characterized by slowed ipsilateral adduction saccades and results from a lesion in the medial longitudinal fasciculus (MLF). Although INO is a common clinical finding in MS, its prevalence in NMOSD is unknown. Read More

BMC Ophthalmol 2022 Apr 14;22(1):168. Epub 2022 Apr 14.

Department of Ophthalmology, Kaohsiung Veterans General Hospital, 386, Dazhong 1st Rd., Zuoying Dist, 81362, Kaohsiung City, Taiwan, Republic of China.

Background: Posterior ischemic optic neuropathy (PION) is a rare cause of visual loss, especially in young patients who are more prone to inflammatory demyelinating optic neuritis (ON) compared to other types of optic neuropathy. The diagnosis of PION is usually a diagnosis of exclusion; however, the emergence of modern neuroimaging technique with diffuse-weighted image (DWI) and apparent diffusion coefficient (ADC) sequences in Magnetic Resonance Imaging (MRI) provides more evidence for accurate diagnosis and management.

Case Presentation: A 30-year-old man with a history of hypertension and chronic renal failure secondary to glomerulonephritis presented with sudden onset of blurred vision, dyschromatopsia, pain, and positive relative afferent pupillary defect (RAPD) in the left eye for 1 week. Read More

Expert Opin Emerg Drugs 2022 03 30;27(1):91-98. Epub 2022 Mar 30.

Centro de Esclerosis Múltiple de Buenos Aires (CEMBA), Buenos Aires, Argentina.

Introduction: Neuromyelitis optica spectrum disorders (NMOSD) are rare but often devastating neuroinflammatory autoimmune diseases of the central nervous system. Acute treatment is critically important and it should be initiated early and aggressively, as relapses result in severe residual disability. Acute treatments are still based on clinical experience and observational studies. Read More

Mult Scler Relat Disord 2022 May 21;61:103750. Epub 2022 Mar 21.

Public Health Department, Faculty of Medicine, Universidad Catolica del Norte, Larrondo 1281, Coquimbo 1780000, Chile. Electronic address:

Background: There is a scarcity of information on the prevalence of demyelinating diseases in Chile and other Latin American countries. The aim of this study was to determine the prevalence of multiple sclerosis (MS) and neuromyelitis optica spectrum disorder (NMOSD) in a region of central-northern Chile.

Methods: A cross-sectional study was performed. Read More

J Neurosci Res 2022 Jul 22;100(7):1395-1412. Epub 2022 Mar 22.

Department of Radiology, Juntendo University Graduate School of Medicine, Tokyo, Japan.

Herein, we combined neurite orientation dispersion and density imaging (NODDI) and synthetic magnetic resonance imaging (SyMRI) to evaluate the spatial distribution and extent of gray matter (GM) microstructural alterations in patients with relapsing-remitting multiple sclerosis (RRMS) and neuromyelitis optica spectrum disorder (NMOSD). The NODDI (neurite density index [NDI], orientation dispersion index [ODI], and isotropic volume fraction [ISOVF]) and SyMRI (myelin volume fraction [MVF]) measures were compared between age- and sex-matched groups of 30 patients with RRMS (6 males and 24 females; mean age, 51.43 ± 8. Read More

Mult Scler Relat Disord 2022 Apr 20;60:103711. Epub 2022 Feb 20.

School of Nursing, Sun Yat-sen University, No. 74 Zhongshan 2nd Road, Guangzhou, Guangdong 510080, China. Electronic address:

Background: Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune inflammatory demyelinating disease of the central nervous system, which mainly involves the optic nerve and spinal cord and has high recurrence and disability rates. Patients with this condition are prone to social isolation, which has not been widely assessed. The purpose of this study was to investigate the experience of social isolation in patients with NMOSD. Read More

Cesk Slov Oftalmol 2022 ;78(1):31-34

Neuromyelitis Optica (NMO, Devics disease) is a rare demyelinating disease of the central nervous system, leading to optic neuritis and transverse myelitis. The clinical course of the disease and ocular changes in pregnancy are still not well known. Here we present the subclinical ophthalmological changes which were evaluated by spectral domain optical coherence tomography (SD-OCT) during the 39 weeks gestation and postpartum period in a pregnant woman with a diagnosis of NMO. Read More

BMC Neurol 2022 Mar 16;22(1):95. Epub 2022 Mar 16.

Post-Graduate Program in Health Sciences, Federal University of Bahia (UFBA), Salvador, Bahia, Brazil.

Introduction: Neuromyelitis optica spectrum disorders (NMOSD) is a rare inflammatory and demyelinating disease of the central nervous system (CNS) more frequent in women and Afro-descendants. No previous epidemiological or prognostic study has been conducted in the region of the state of Bahia, Brazilian Northeast.

Objective: To evaluate clinical and prognostic aspects in patients with NMOSD from a cohort in northeastern Brazil. Read More

Mult Scler Relat Disord 2022 Apr 27;60:103716. Epub 2022 Feb 27.

Department of Neurology, Massachusetts General Hospital, Boston, MA, United States of America; Harvard Medical School, Boston, MA, United States of America. Electronic address:

Background: Given the need for specialized laboratory techniques, diagnostic testing for serum antibodies to aquaporin-4, a protein associated with neuromyelitis optica spectrum disorder (NMOSD), is not globally accessible. We aimed to evaluate a novel point-of-care, filter paper-based test for serum AQP4 antibodies (AQP4-Ab).

Methods: Adults with AQP4-Ab seropositive NMOSD and seronegative controls (with other central nervous system demyelinating diagnoses) used lancets to place blood drops (∼1 mL) on filter paper cards. Read More

Rinsho Shinkeigaku 2022 Mar 25;62(3):190-197. Epub 2022 Mar 25.

Department of Neurology, Saiseikai Matsusaka General Hospital.

We report the case of a 51-year-old woman who developed neuromyelitis optica spectrum disorders (NMOSD) associated with primary biliary cholangitis (PBC). When she was 38 years old, she subacutely developed headache and urinary retention. A diffusion weighted image (DWI) on brain MRI showed high signal intensity in the left temporal white matter, and T weighted image (TWI) on spine MRI showed high signal intensities in the spinal cord. Read More

Mult Scler Relat Disord 2022 Apr 22;60:103709. Epub 2022 Feb 22.

Department of Ophthalmology, Seoul National University Hospital, Seoul, Republic of Korea; Department of Ophthalmology, Seoul National University College of Medicine, 101, Daehak-ro Jongno-gu, Seoul 03080, Republic of Korea. Electronic address:

Purpose: To investigate the ophthalmic and neurological features of myelin oligodendrocyte glycoprotein antibody seropositive optic neuritis (MOG-ON) in pediatric patients.

Methods: We analyzed the clinical data and orbital magnetic resonance images of patients aged below 15 years, diagnosed with MOG-ON at our institution (n = 40).

Results: The mean age at first ON onset was 7. Read More

Mult Scler Relat Disord 2022 Feb 18;58:103414. Epub 2021 Nov 18.

Siriraj Neuroimmunology Center, Faculty of Medicine Siriraj Hospital, Mahidol University, 2 Wanglang Road, Bangkok 10700, Thailand; Division of Neurology, Department of Medicine, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok 10700, Thailand. Electronic address:

Introduction: The pathogenesis of neuromyelitis optica spectrum disorder (NMOSD) has been vigorously illustrated, but triggers of the disease remain unclear. Viral infection and vaccination have been observed to precede certain cases of NMOSD. Amidst the Coronavirus disease 2019 (COVID-19) pandemic, mass vaccination takes place across the globe. Read More

Medicine (Baltimore) 2022 Feb;101(8):e28887

Department of Neurology, Jeonbuk National University Medical School and Hospital, Jeonju, South Korea.

Rationale: Patients with myasthenia gravis may also have comorbid autoimmune diseases. Since both myasthenia gravis and neuromyelitis optica spectrum disease are mediated by antibodies, they are likely to occur together. However, since multiple sclerosis is an autoimmune disease that is not mediated by a specific antibody, it has fewer immune mechanisms in common with myasthenia gravis than neuromyelitis optica spectrum disease. Read More

Front Immunol 2022 3;13:766099. Epub 2022 Feb 3.

Department of Neurology, The First Affiliated Hospital of Chongqing Medical University, Chongqing, China.

Objectives: This study sought to explore the expression patterns of repulsive guidance molecules a (RGMa) in neuromyelitis optica spectrum disorders (NMOSD) and to explore the correlation between RGMa and the clinical features of NMOSD.

Methods: A total of 83 NMOSD patients and 22 age-matched healthy controls (HCs) were enrolled in the study from October 2017 to November 2021. Clinical parameters, including Expanded Disability Status Scale (EDSS) score, degree of MRI enhancement, and AQP4 titer were collected. Read More

Neurologist 2022 May 1;27(3):147-150. Epub 2022 May 1.

Koc University Graduate School of Health Sciences.

Introduction: In the midst of the coronavirus disease of 2019 pandemic, active immunization by effective vaccination gained utmost importance in terms of global health. The messenger RNA (mRNA) vaccines are novel strategies requiring clinical surveillance for adverse events.

Case Report: We report a 43-year-old previously healthy female with an optic neuritis attack 24 hours following immunization with the second dose of coronavirus disease of 2019 mRNA BNT162b2 vaccine. Read More

Restor Neurol Neurosci 2022 ;40(1):35-42

Department of Adult Neurology, Medical University of Gdańsk, Gdańsk, Poland.

Background: Multiple sclerosis (MS) is associated with progressive brain atrophy, which in turn correlates with disability, depression, and cognitive impairment. Relapsing-remitting multiple sclerosis (RRMS) is a type of MS in which relapses of the disease are followed by remission periods. This is the most common type of the disease. Read More

Neuropathology 2022 Jun 15;42(3):212-217. Epub 2022 Feb 15.

Department of Neuropathology, Institute for Medical Science of Aging, Aichi Medical University, Nagakute, Japan.

We report an autopsy case of multiple sclerosis (MS) manifesting as a long spinal cord lesion. The patient was a Japanese woman. At the age of 59 years, she presented with a one-month history of progressive paraplegia, dysesthesia in the lower extremities, and urinary retention. Read More