Clinical efficacy of efgartigimod combined with intravenous methylprednisolone in the acute phase of neuromyelitis optica spectrum disorders.

Background: Neuromyelitis Optica Spectrum Disorders (NMOSD) comprise a group of autoimmune-mediated, inflammatory, demyelinating central nervous system diseases caused by aquaporin-4 (AQP4) IgG autoantibodies. Efgartigimod is a human IgG Fc fragment that reduces antibody titers by targeting the neonatal Fc receptor (FcRn). This study documents the efficacy of efgartigimod combined with intravenous methylprednisolone (IVMP) in the acute phase of NMOSD.

The epidemiology and clinical presentation of seropositive neuromyelitis optica spectrum disorder in a US population.

Objective: To define the epidemiology and clinical presentation of seropositive neuromyelitis optica spectrum disorder (NMOSD) in a large US health system.

Methods: We completed a retrospective observational study of adult patients in the University of Colorado Health System from 1 January 2011 to 31 December 2020, using Health Data Compass (HDC), a data warehouse that combines electronic health information with claims and public health data in Colorado. We screened HDC for patients with either (1) an abnormal aquaporin-4 IgG test or (2) any G36 ICD-10 code.

Visual quality of life in NMOSD and MOGAD: profiles, dynamics and associations with ageing and vision.

Objective: In this multicentric study, we were interested in the vision-related quality of life and its association with visual impairment in neuromyelitis optica spectrum disorders (NMOSD) and myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) in comparison to multiple sclerosis (MS) and healthy controls.

Methods: We analysed extracted data from the German NEMOS registry including National Eye Institute Visual Function Questionnaire (NEI-VFQ) scores, high and low contrast visual acuity (HCVA, LCVA), visually evoked potentials (VEP) and the scores for the expanded disability status scale (EDSS) and other neurological tests which assessed their disease-related impairment. The mean follow-up time of our patients was 1.

Optic neuritis in demyelinating diseases: study of 38 cases.

Background:  Optic neuritis is an inflammation of the optic nerve caused by genetic factors, external influences, and the activation of cross-reactive immune responses to infections.

Objective:  To describe the clinical and epidemiological characteristics of patients presenting optic neuritis as the initial symptom of some demyelinating diseases, divided among multiple sclerosis (MS), neuromyelitis optica spectrum disorder (NMOSD), and myelin oligodendrocyte glycoprotein-immunoglobulin G (MOG-IgG)-associated disorders (MOGADs).

Methods:  Thirty-eight patients who had optic neuritis as their first symptom and later developed MS, NMOSD, or MOGADs were analyzed.

One-year risk of multiple sclerosis after a first episode of optic neuritis according to modern diagnosis criteria.

Purpose: The last updates to diagnostic criteria for multiple sclerosis (MS) included a diagnostic category of 'possible MS'. However, no recent data is available to assess how much this distinction helps predict MS after isolated optic neuritis (ON). This study aimed to assess the global risk of developing MS one year after a first ON episode, and the specific risk according to the initial diagnosis of isolated ON or ON with possible MS.

Cerebellar Involvement in Attacks of Aquaporin-4-IgG Positive Neuromyelitis Optica Spectrum Disorder.

Objectives: To characterize the frequency and clinicoradiologic phenotype of cerebellar involvement in attacks of aquaporin-4-IgG positive neuromyelitis optica spectrum disorder (AQP4+NMOSD) which are incompletely captured in current diagnostic criteria.

Methods: Brain MRI scans from patients with AQP4+NMOSD in the Mayo Clinic database were reviewed, and those with cerebellar T2-hyperintense lesions ≤30 days from attack onset were included for clinical and radiologic characterization.

Results: From 432 patients with AQP4+NMOSD, we identified 17 (4%) with cerebellar attacks.

Complement Activation Profiles Predict Clinical Outcomes in Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease.

Background And Objectives: The role of the complement system in myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is not completely understood, and studies exploring its potential utility for diagnosis and prognosis are lacking. We aimed to investigate the value of complement factors (CFs) as diagnostic and prognostic biomarkers in patients with MOGAD.

Methods: Multicentric retrospective cohort study including patients with MOGAD, multiple sclerosis (MS) and aquaporin-4 seropositive neuromyelitis optica spectrum disorder (AQP4-NMOSD) with available paired serum and CSF samples.

Multimodal Longitudinal Optical Imaging Reveals Optic Neuritis Preceding Retinal Pathology in Experimental Autoimmune Encephalomyelitis.

Background And Objectives: Inflammatory demyelinating diseases of the CNS, chief among them multiple sclerosis (MS), are a major cause of disability in young adults. Early manifestations of MS commonly involve visual dysfunction, which is often caused by optic neuritis and is accompanied by quantifiable structural changes of the anterior visual pathway. Retinal optical coherence tomography (OCT) has emerged as an important tool for clinical assessment of these structural alterations, but the underlying pathobiological mechanisms and temporal dynamics are yet poorly understood at a cellular level.

Fewer relapses and worse outcomes of patients with late-onset myelin oligodendrocyte glycoprotein antibody-associated disease.

Background: To delineate the clinical characteristics and outcomes of late-onset myelin oligodendrocyte glycoprotein antibody-associated disease (LO-MOGAD) and compare them with those of early-onset MOGAD (EO-MOGAD).

Methods: This observational cohort study included 199 adult patients with MOGAD. We reviewed the patients' demographic and clinical data and performed comparative analyses between EO-MOGAD and LO-MOGAD (onset age 18-50 and ≥50 years, respectively).

Predictive model for converting optic neuritis to multiple sclerosis; decision tree in focus.

Background: Optic neuritis (ON) can be an initial clinical presentation of multiple sclerosis This study aims to provide a practical predictive model for identifying at-risk ON patients in developing MS.

Method: We utilized data from the Optic Neuritis Treatment Trial study, which enrolled 457 patients aged from 18 to 46 years, all diagnosed with acute ON. These patients underwent up to 15 years of neurological and ophthalmologic examinations and imaging.

CD22 blockade exacerbates neuroinflammation in Neuromyelitis optica spectrum disorder.

Background: Neuromyelitis optica spectrum disorder (NMOSD) is an autoantibody-triggered central nervous system (CNS) demyelinating disease that primarily affects the spinal cord, optic nerves and brainstem. Among the first responders to CNS injury, microglia are prominent players that drive NMOSD lesion formation. However, the key molecular switches controlling the detrimental activity of microglia in NMOSD are poorly understood.

Ocular Syphilis With Unilateral Optic Papillitis and Outer Retinopathy Complicated by Diabetic Retinopathy.

Syphilis has been increasing in adult infections in recent years, and ocular syphilis includes not only uveitis but also a variety of optic nerve and retinal lesions. We report a case of syphilis that caused unilateral optic papillitis and outer retinopathy complicated by diabetic retinopathy and improved with antibiotic treatment. The patient was a 61-year-old woman.

The real-world impact of biologics for NMOSD: A retrospective single-center study compared with natural course and conventional treatments in Japanese.

Background: Neuromyelitis optica spectrum disorder (NMOSD), a central nervous system inflammatory disease associated with aquaporin-4 immunoglobulin G (AQP4-IgG), is conventionally treated with oral steroids and immunosuppressants (IS) in Japan. Several biologics which show great efficacy in the clinical trials have been developed recently. However, studies on their efficacy, especially those comparing them with conventional treatments in real-world situations are lacking.

[Classic presentation of neuromyelitis optica: clinical case and review of the literature].

Background: Neuromyelitis optica (NMO or Devic's syndrome), characterized by acute attacks of optic neuritis and transverse myelitis, is associated with antiaquaporin 4 antibodies (anti-NMO-IgG) and it has a higher prevalence in non-Caucasian populations, where multiple sclerosis is less common.

Clinical Case: 41-year-old man, with no significant personal medical history, presented with clinical symptoms including paresthesias in the right hemibody and homonymous hemianopsia in the left eye. Three weeks later, he developed lumbalgia associated with paresthesias in both lower limbs up to the T7 sensory level, as well as acute urinary retention, followed by paraplegia.

MOGAD: A comprehensive review of clinicoradiological features, therapy and outcomes in 4699 patients globally.

Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is one of the most common antibody-mediated CNS disorders. Optimal diagnostic and prognostic biomarkers remain unclear. Our aim was to clarify these biomarkers and therapeutic outcomes internationally.

Epstein-Barr nuclear antigen 1 antibody-based indices are increased in patients with multiple sclerosis.

Background: Multiple sclerosis (MS) is a chronic central nervous system (CNS) disease, which is diagnosed by a combination of clinical symptoms and magnetic resonance imaging and measurement of an increased intrathecal antibody synthesis. Genetic as well as environmental factors influence onset of the disease, where especially Epstein-Barr virus (EBV) infection is directly involved in MS development. In this open retrospective study, we aimed to elaborate whether various serum and cerebrospinal fluid (CSF)-based EBV antibody indices may aid in the diagnosis of MS.

Cerebral cortical encephalitis in adults with myelin oligodendrocyte glycoprotein antibody-associated disease: A national case series.

Background And Purpose: Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) is a relatively recently described disease, most commonly presenting with optic neuritis and longitudinally extensive transverse myelitis. Cerebral cortical encephalitis is a rare manifestation of MOGAD.

Methods: We identified patients presenting with cerebral cortical encephalitis with positive MOG antibodies in serum across a large specialized service.