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Am J Case Rep 2021 Jun 13;22:e931665. Epub 2021 Jun 13.

Department of Medicine, St. George's University School of Medicine, St. Georges, Grenada.

BACKGROUND Since the outbreak of the SARS-CoV-2 infection, extensive research has been conducted on the pulmonary implications of this novel disease. However, there has been limited data on the extrapulmonary manifestations. There have been few documented causes of optic involvement and little is understood about the pathophysiology around its presentation and the possible treatments to prevent long-term complications. Read More

Medicine (Baltimore) 2021 May;100(19):e25865

Department of Ophthalmology, University of Yamanashi, Chuo, Yamanashi.

Rationale: Coronavirus disease 2019 (COVID-19) has spread worldwide. It involves multiple organs of infected individuals and encompasses diverse clinical manifestations. We report a case of acute optic neuritis (ON) associated with myelin oligodendrocyte glycoprotein (MOG) antibody possibly induced by COVID-19. Read More

Front Neurol 2021 20;12:679430. Epub 2021 May 20.

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

To evaluate the clinical differences between pediatric and adult patients with myelin oligodendrocyte glycoprotein antibody-associated encephalomyelitis (MOG-EM). We retrospectively reviewed the clinical features of pediatric and adult patients with MOG-EM in our center between November 2015 and October 2020. Twenty-eight pediatric patients and 25 adults were admitted to our study. Read More

Surg Neurol Int 2021 19;12:177. Epub 2021 Apr 19.

Department of Neurosurgery, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma, United States.

Background: Concurrent diagnosis of multiple sclerosis (MS) and the central nervous system (CNS) germinoma is rare. The diagnostic criteria for MS rely primarily on clinical presentation, and CNS germinoma can present as an MS mimic. These factors contribute to the rarity of dual diagnosis. Read More

Int J Mol Sci 2021 May 14;22(10). Epub 2021 May 14.

Laboratory of Neurobiology, Department of Neurology, Institute of Neurosciences, San Carlos Health Research Institute, Universidad Complutense, 28040 Madrid, Spain.

Introduction: AQP4 (aquaporin-4)-immunoglobulin G (IgG)-mediated neuromyelitis optica spectrum disorder (NMOSD) is an inflammatory demyelinating disease that affects the central nervous system, particularly the spinal cord and optic nerve; remyelination capacity in neuromyelitis optica is yet to be determined, as is the role of AQP4-IgG in cell differentiation.

Material And Methods: We included three groups-a group of patients with AQP4-IgG-positive neuromyelitis optica, a healthy group, and a sham group. We analyzed differentiation capacity in cultures of neurospheres from the subventricular zone of mice by adding serum at two different times: early and advanced stages of differentiation. Read More

J Neuroinflammation 2021 May 29;18(1):121. Epub 2021 May 29.

Department of Neurology, St. Josef-Hospital, Ruhr-University Bochum, Bochum, Germany.

Background: To investigate age-related severity, patterns of retinal structural damage, and functional visual recovery in pediatric and adult cohorts of myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) optic neuritis (ON).

Methods: All MOGAD patients from the 5 participating centers were included. Patients with initial manifestation <18 years were included in the pediatric (MOGAD) cohort and patients with ≥18 years in the adult (MOGAD) cohort. Read More

BMC Ophthalmol 2021 May 27;21(1):237. Epub 2021 May 27.

Department of Ophthalmology, Samsung Changwon Hospital, Sungkyunkwan University School of Medicine, 158, Paryong-ro, Masanhoewon-gu, 51353, Changwon, Korea.

Background: The objective of this study was to investigate the clinical features and outcomes of Tolosa-Hunt syndrome (THS).

Methods: A retrospective review of the medical records was performed on patients with THS between March 2016 and January 2020. A total of eleven patients fulfilling the International Classification of Headache Disorders (ICHD-3 beta) diagnostic criteria for THS were included in this study. Read More

Zhonghua Yi Xue Za Zhi 2021 May;101(19):1415-1420

Department of Neurology, Beijing Tongren Hospital, Capital Medical University, Beijing 100730, China.

To investigate the clinical characteristics and prognosis of myelin oligodendrocyte glycoprotein (MOG) antibody-positive optic neuritis (ON). The data of 39 patients with MOG antibody-positive ON in the Department of Neurology of Beijing Tongren Hospital, Capital Medical University from January 1, 2017 to October 31, 2019 were retrospectively collected. There were 25 males and 14 females, aged from 15 to 80 (40±16) years. Read More

BMJ Case Rep 2021 May 12;14(5). Epub 2021 May 12.

Department of Ophthalmology, University of Science Malaysia-Kesihatan Campus, Kubang Kerian, Kelantan, Malaysia.

We describe an uncommon cause of paraneoplastic optic neuropathy in adenocarcinoma of the lung. A 45-year-old healthy woman presented with seizure and encephalitis, followed by an acute visual loss in both eyes for 1 week. Her visual acuity was no perception of light in the right eye and hand movement in the left eye. Read More

J Clin Neurophysiol 2021 May;38(3):171-180

Section of Clinical Neurophysiology, Department of Neurology, Hospital of the University of Basel, Basel, Switzerland.

Summary: Evoked potentials (EPs) measure quantitatively and objectively the alterations of central signal propagation in multiple sclerosis and have long been used for diagnosis. More recently, their utility for prognosis has been demonstrated in several studies, summarizing multiple EP modalities in a single score. In particular, visual, somatosensory, and motor EPs are useful because of their sensitivity to pathology in the frequently affected optic nerve, somatosensory tract, and pyramidal system. Read More

Indian J Ophthalmol 2021 05;69(5):1167-1171

Prasad Netralaya, Udupi, Karnataka, India.

Purpose: The aim of this work was to study the ocular manifestations and its management in spotted fever and typhus group of rickettsial disease.

Methods: A retrospective analysis of 50 patients with serologically confirmed Rickettsial disease. In all patients, relevant history, investigations and treatment details were collected and they underwent complete ophthalmic evaluation including measurement of best-corrected visual acuity, anterior segment examination and dilated fundus examination. Read More

Ann Indian Acad Neurol 2021 Jan-Feb;24(1):69-77. Epub 2020 Mar 3.

Department of Neurology, Ruby Hall Clinic, Pune, Maharashtra, India.

Background: Discovery of serum myelin oligodendrocyte glycoprotein (MOG) antibody testing in demyelination segregated MOG-IgG disease from AQ-4-IgG positive NMOSD.

Aims: To study clinico-radiological manifestations, pattern of laboratory and electrophysiological investigations and response to treatment through follow up in MOG-IgG positive patients.

Method: Retrospective data of MOG-IgG positive patients was collected. Read More

Medicine (Baltimore) 2021 Apr;100(16):e25580

Department of Neurology.

Rationale: Neuromyelitis optica spectrum disorder (NMOSD) is a severe inflammatory disorder of the central nervous system with an autoantibody against aquaporin-4 protein (AQP4), and amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease. We report a female patient with ALS who had asymptomatic AQP4 antibody at the diagnosis of ALS, and NMOSD occurred 4 years later after the diagnosis of ALS.

Patient Concerns: She was already bedridden and had tracheostomy because of ALS which was diagnosed at her age of 55. Read More

J Clin Neurosci 2021 May 11;87:55-58. Epub 2021 Mar 11.

Department of Radiology, Juntendo University School of Medicine, 1-2-1, Hongo, Bunkyo-ku, Tokyo 113-8421, Japan.

Multiple sclerosis and neuromyelitis optica spectrum disorders are both neuroinflammatory diseases and have overlapping clinical manifestations. We developed a convolutional neural network model that differentiates between the two based on magnetic resonance imaging data. Thirty-five patients with relapsing-remitting multiple sclerosis and eighteen age-, sex-, disease duration-, and Expanded Disease Status Scale-matched patients with anti-aquaporin-4 antibody-positive neuromyelitis optica spectrum disorders were included in this study. Read More

Medicine (Baltimore) 2021 Apr;100(15):e24889

Department of Rheumatology.

Rationale: Anti-myelin oligodendrocyte protein antibody-associated disease (MOGAD) is a new disease entity with various clinical phenotypes. MOGAD often present with recurrent optic neuritis (ON), and it can also develop as a compartment of neuromyelitis optica spectrum disorder (NMOSD). Moreover, multiple autoantibodies such as an anti-myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA) had been reported in the serum of patients with NMOSD. Read More

BMC Neurol 2021 Apr 9;21(1):153. Epub 2021 Apr 9.

Department of Neurology, Chang Gung Memorial Hospital Linkou Medical Center and College of Medicine, Chang-Gung University, Linkou, Taoyuan, Taiwan.

Background: Neuromyelitis optica spectrum disorder (NMOSD) is a rare neuroinflammatory disorder of the central nervous system that typically involves the optic nerve, the spinal cord and other specific brain regions. In relapse of the disease, factors associated with clinical features and lesion severity are important for clinicians to predict disease-related disability.

Methods: We retrospectively analyzed 22 female patients with NMOSD who had spinal cord lesions. Read More

Neurology 2021 05 7;96(20):e2525-e2533. Epub 2021 Apr 7.

From the Department of Neurology (J.L., H.R., A.G.F., O.C.M., E.S.S., H.E., E.O., N.P., B.T., N.J.L., S.D., N.F., O.K., P.A.C., K.C.F., S.S.), Johns Hopkins University School of Medicine; and Department of Electrical and Computer Engineering (J.L.P.), Johns Hopkins University, Baltimore, MD.

Objective: To investigate the effects of rituximab on retinal atrophy in patients with relapsing-remitting multiple sclerosis (RRMS), we performed serial optical coherence tomography (OCT) scans among a cohort of patients with RRMS on rituximab and compared rates of ganglion cell + inner plexiform layer (GCIPL) atrophy to those observed among age- and sex-matched glatiramer acetate (GA)-and natalizumab-treated patients with RRMS and healthy controls (HCs).

Methods: In this observational study, patients with RRMS treated with a single disease-modifying therapy and HCs were followed with serial OCT for a median duration of 2.8 years. Read More

Nervenarzt 2021 Apr 31;92(4):317-333. Epub 2021 Mar 31.

AG Molekulare Neuroimmunologie, Neurologische Klinik, Universitätsklinik Heidelberg, Heidelberg, Deutschland.

Aquaporin 4 (AQP4) immunoglobulin (Ig)G-associated neuromyelitis optica spectrum disorders (NMOSD) and myelin oligodendrocyte glycoprotein immunoglobulin (Ig)G-associated encephalomyelitis (MOG-EM, also termed MOG antibody-associated disease, MOGAD) are important autoimmune differential diagnoses of multiple sclerosis (MS), which differ from MS with respect to optimum treatment and prognosis. AQP4 IgG-positive NMOSD take a relapsing course in virtually all cases and MOG-EM in at least 80% of adult cases. Both diseases can quickly lead to permanent disability if left untreated, although MOG-EM is associated with a better overall long-term prognosis. Read More

J Neurovirol 2021 04 23;27(2):359-363. Epub 2021 Mar 23.

Centro de Investigación en Enfermedades Infecciosas, Instituto Nacional de Enfermedades Respiratorias Ismael Cosío Villegas, Calzada de Tlalpan 4502, Col. Sección XVI, CP , 14080, Ciudad de Mexico, Mexico.

The most common neurologic symptoms in COVID-19 are headache, anosmia, and dysgeusia. Optic neuritis is an unusual manifestation of SARS-CoV-2 infection. We report a case of a patient who initially consulted for vision loss in the absence of respiratory symptoms. Read More

BMC Neurol 2021 Mar 9;21(1):109. Epub 2021 Mar 9.

Neuroscience Center, General Hospital of Ningxia Medical University, Key Laboratory of Craniocerebral Diseases of Ningxia Hui Autonomous Region, Yinchuan, 75004, China.

Background: Neuromyelitis optica spectrum disorders (NMOSD), a group of autoimmune neurological diseases, involve the optic nerve, spinal cord, and brain. Meningitis is rarely reported as the primary clinical manifestation of both anti-aquaporin-4 (AQP4)/ anti-myelin oligodendrocyte glycoprotein (MOG) antibody-negative NMOSD (NMOSD).

Case Presentation: A 30-year-old man initially presented with fever, headache, and neck stiffness. Read More

Arq Neuropsiquiatr 2021 03;79(3):229-232

Universidade de São Paulo, Faculdade de Medicina, Departamento de Neurologia, São Paulo SP, Brazil.

Background: Azathioprine is a common first-line therapy for neuromyelitis optica spectrum disorder (NMOSD).

Objective: The aim of this study was to determine whether long-term treatment (>10 years) with azathioprine is safe in NMOSD. Methods: We conducted a retrospective medical record review of all patients at the School of Medicine of the University of São Paulo (São Paulo, Brazil) who fulfilled the 2015 international consensus diagnostic criteria for NMOSD and were treated with azathioprine for at least 10 years. Read More

Drugs Today (Barc) 2021 Mar;57(3):209-218

Professor Emeritus of Pharmacology, University of Auckland, Auckland, New Zealand and Professor Emeritus of Oral Biology, University of Alberta, Edmonton, Alberta, Canada.

Neuromyelitis optica spectrum disorders (NMOSD) consist of a rare autoimmune disorder in which patients suffer from relapses that affect the optic nerve, spinal cord or brainstem. Few have a full recovery. NMOSD is more common in women, the age of onset being around 30-40 years of age depending on race. Read More

Nervenarzt 2021 Apr 16;92(4):307-316. Epub 2021 Mar 16.

Institut für klinische Neuroimmunologie, LMU Klinikum, Ludwig-Maximilians-Universität München, Marchioninistr. 15, 81377, München, Deutschland.

Neuromyelitis optica spectrum disorder (NMOSD), derived from NMO or Devic's disease, is considered as a distinct disease since the discovery of a novel and pathogenic serum autoantibody targeting aquaporin‑4 (AQP4-IgG) and is distinguished from classical multiple sclerosis (MS). With the continuous extension of knowledge on the clinical manifestations, the previously narrow diagnostic term NMO became NMOSD, which has also been used in the diagnostic criteria since 2015. The current diagnostic criteria enable the early diagnosis of NMOSD in patients with and without AQP4-IgG. Read More

Indian J Ophthalmol 2021 Apr;69(4):989-991

Department of Ophthalmology, Goa Medical College, Bambolim, Goa, India.

Ocular manifestations of COVID-19 are still being studied. Posterior segment involvement in viral entities is either direct viral involvement or a delayed immune response to the antigen. A 22-year-old woman presented with history of perceiving absolute inferior scotoma in the right eye for 4 days and history of fever and sore throat 10 days ago. Read More

Ann Neurol 2021 05 30;89(5):895-910. Epub 2021 Mar 30.

Department of Neurology, UCSF Weill Institute for Neurosciences, University of California San Francisco, San Francisco, CA.

Objective: Blood tests to monitor disease activity, attack severity, or treatment impact in neuromyelitis optica spectrum disorder (NMOSD) have not been developed. This study investigated the relationship between serum glial fibrillary acidic protein (sGFAP) concentration and NMOSD activity and assessed the impact of inebilizumab treatment.

Methods: N-MOmentum was a prospective, multicenter, double-blind, placebo-controlled, randomized clinical trial in adults with NMOSD. Read More

Zhonghua Yan Ke Za Zhi 2021 Mar;57(3):171-186

Demyelinating optic neuritis (DON) is an inflammatory demyelinating disease of the central nervous system, which can lead to visual loss in young adults as a major factor. The key points concerned by neuro-ophthalmologists are to reduce the rate of recurrence and improve the prognosis in the course of diagnosis and treatment of DON. Companied with the deep research of diagnostic markers, different classifications and therapies, and evidence-based medicine, there are more and more clinical evidences for the development and standardization of evidence-based guidelines for diagnosis and treatment. Read More

Ophthalmology 2021 Mar 11. Epub 2021 Mar 11.

Department of Ophthalmology, Emory University, Atlanta, Georgia; Department of Neurology, Emory University, Atlanta, Georgia. Electronic address:

Purpose: To prospectively examine diagnostic error of neuro-ophthalmic conditions and resultant harm at multiple sites.

Design: Prospective, cross-sectional study.

Participants: A total of 496 consecutive adult new patients seen at 3 university-based neuro-ophthalmology clinics in the United States in 2019 to 2020. Read More

Mult Scler Relat Disord 2021 May 25;50:102869. Epub 2021 Feb 25.

Pontificia Universidad Javeriana, Bogotá, Colombia; Department of Internal Medicine, Hospital Universitario San Ignacio, Bogotá, Colombia; Associate professor.

Background: Neuromyelitis Optica spectrum disorder (NMOSD) is an inflammatory disease, which manifests mostly as recurrent episodes of optic neuritis or myelitis that cause important disability. Early diagnosis and prompt initiation of immunosuppressive therapy are crucial in reducing relapses, disability, and mortality. Even though, there are few prospective randomized controlled trials, several drugs have proved to be both effective and safe. Read More

Neurology 2021 04 2;96(16):e2058-e2069. Epub 2021 Mar 2.

From the Department of Neurology (J.L., K.C.F., O.C.M., A.G.F., E.S.S., G.K., E.V., N.P., E.O., B.T., N.J.L., S.D., N.F., O.K., H.R., S.D.N., E.M.M., S.S., P.A.C.), Johns Hopkins University School of Medicine; and Departments of Biostatistics (C.M.C.) and Electrical and Computer Engineering (J.L.P.), Johns Hopkins University, Baltimore, MD.

Objective: To evaluate whether a retinal spectral-domain optical coherence tomography (SD-OCT) assessment at baseline is associated with long-term disability worsening in people with multiple sclerosis (PwMS), we performed SD-OCT and Expanded Disability Status Scale (EDSS) assessments among 132 PwMS at baseline and at a median of 10 years later.

Methods: In this prospective, longitudinal study, participants underwent SD-OCT, EDSS, and visual acuity (VA) assessments at baseline and at follow-up. Statistical analyses were performed using generalized linear regression models, adjusted for age, sex, race, multiple sclerosis (MS) subtype, and baseline disability. Read More

J Neuroimmunol 2021 04 20;353:577525. Epub 2021 Feb 20.

Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Rome, Italy; Istituto di Neurologia, Università Cattolica del Sacro Cuore, Rome, Italy.

Tumour Necrosis Factor alpha (TNFα) blockers are common and effective treatments for several autoimmune diseases but can be associated with neuroinflammatory events. We describe the disease course of ten patients who developed CNS demyelinating events while exposed to TNFα blockers. We divided them into two groups: eight patients with Relapsing Multiple Sclerosis and two isolated optic neuritis. Read More