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Journal of Neurology Jul 2023The term 'neuromyelitis optica spectrum disorders' (NMOSD) is used as an umbrella term that refers to aquaporin-4 immunoglobulin G (AQP4-IgG)-positive neuromyelitis... (Review)
Review
Update on the diagnosis and treatment of neuromyelits optica spectrum disorders (NMOSD) - revised recommendations of the Neuromyelitis Optica Study Group (NEMOS). Part I: Diagnosis and differential diagnosis.
The term 'neuromyelitis optica spectrum disorders' (NMOSD) is used as an umbrella term that refers to aquaporin-4 immunoglobulin G (AQP4-IgG)-positive neuromyelitis optica (NMO) and its formes frustes and to a number of closely related clinical syndromes without AQP4-IgG. NMOSD were originally considered subvariants of multiple sclerosis (MS) but are now widely recognized as disorders in their own right that are distinct from MS with regard to immunopathogenesis, clinical presentation, optimum treatment, and prognosis. In part 1 of this two-part article series, which ties in with our 2014 recommendations, the neuromyelitis optica study group (NEMOS) gives updated recommendations on the diagnosis and differential diagnosis of NMOSD. A key focus is on differentiating NMOSD from MS and from myelin oligodendrocyte glycoprotein antibody-associated encephalomyelitis (MOG-EM; also termed MOG antibody-associated disease, MOGAD), which shares significant similarity with NMOSD with regard to clinical and, partly, radiological presentation, but is a pathogenetically distinct disease. In part 2, we provide updated recommendations on the treatment of NMOSD, covering all newly approved drugs as well as established treatment options.
Topics: Humans; Neuromyelitis Optica; Diagnosis, Differential; Myelin-Oligodendrocyte Glycoprotein; Aquaporin 4; Multiple Sclerosis; Immunoglobulin G; Autoantibodies
PubMed: 37022481
DOI: 10.1007/s00415-023-11634-0 -
Indian Journal of Ophthalmology Jul 2023Infections of orbit and periorbita are frequent, leading to significant morbidity. Orbital cellulitis is more common in children and young adults. At any age, infection... (Review)
Review
Infections of orbit and periorbita are frequent, leading to significant morbidity. Orbital cellulitis is more common in children and young adults. At any age, infection from the neighboring ethmoid sinuses is a likely cause and is thought to result from anatomical characteristics like thin medial wall, lack of lymphatics, orbital foramina, and septic thrombophlebitis of the valveless veins between the two. Other causes are trauma, orbital foreign bodies, preexisting dental infections, dental procedures, maxillofacial surgeries, Open Reduction and Internal Fixation (ORIF), and retinal buckling procedures. The septum is a natural barrier to the passage of microorganisms. Orbital infections are caused by Gram-positive, Gram-negative organisms and anaerobes in adults and in children, usually by Staphylococcus aureus or Streptococcus species. Individuals older than 15 years of age are more likely to harbor polymicrobial infections. Signs include diffuse lid edema with or without erythema, chemosis, proptosis, and ophthalmoplegia. It is an ocular emergency requiring admission, intravenous antibiotics, and sometimes surgical intervention. Computed tomography (CT) and magnetic resonance imaging (MRI) are the main modalities to identify the extent, route of spread from adjacent structures, and poor response to intravenous antibiotics and to confirm the presence of complications. If orbital cellulitis is secondary to sinus infection, drainage of pus and establishment of ventilation to the sinus are imperative. Loss of vision can occur due to orbital abscess, cavernous sinus thrombosis, optic neuritis, central retinal artery occlusion, and exposure keratopathy, and possible systemic sequelae include meningitis, intracranial abscess, osteomyelitis, and death. The article was written by authors after a thorough literature search in the PubMed-indexed journals.
Topics: Child; Young Adult; Humans; Orbital Cellulitis; Abscess; Orbit; Exophthalmos; Anti-Bacterial Agents
PubMed: 37417106
DOI: 10.4103/IJO.IJO_3283_22 -
Journal of Neurology Dec 2023Much has changed since our last review of recent advances in neuro-otology 7 years ago. Unfortunately there are still not many practising neuro-otologists, so that most...
Much has changed since our last review of recent advances in neuro-otology 7 years ago. Unfortunately there are still not many practising neuro-otologists, so that most patients with vestibular problems need, in the first instance, to be evaluated and treated by neurologists whose special expertise is not neuro-otology. The areas we consider here are mostly those that almost any neurologist should be able to start managing: acute spontaneous vertigo in the Emergency Room-is it vestibular neuritis or posterior circulation stroke; recurrent spontaneous vertigo in the office-is it vestibular migraine or Meniere's disease and the most common vestibular problem of all-benign positional vertigo. Finally we consider the future: long-term vestibular monitoring and the impact of machine learning on vestibular diagnosis.
Topics: Humans; Neurotology; Meniere Disease; Vestibular Diseases; Benign Paroxysmal Positional Vertigo; Vestibular Neuronitis; Dizziness
PubMed: 37592138
DOI: 10.1007/s00415-023-11922-9 -
Autoimmunity Reviews Dec 2023Neuromyelitis optica spectrum disorder (NMOSD) is a rare relapsing neuroinflammatory autoimmune astrocytopathy, with a predilection for the optic nerves and spinal cord.... (Review)
Review
Neuromyelitis optica spectrum disorder (NMOSD) is a rare relapsing neuroinflammatory autoimmune astrocytopathy, with a predilection for the optic nerves and spinal cord. Most cases are characterised by aquaporin-4-antibody positivity and have a relapsing disease course, which is associated with accrual of disability. Although the prognosis in NMOSD has improved markedly over the past few years owing to advances in diagnosis and therapeutics, it remains a severe disease. In this article, we review the evolution of our understanding of NMOSD, its pathogenesis, clinical features, disease course, treatment options and associated symptoms. We also address the gaps in knowledge and areas for future research focus.
Topics: Humans; Neuromyelitis Optica; Aquaporin 4; Prognosis; Autoantibodies
PubMed: 37852514
DOI: 10.1016/j.autrev.2023.103465 -
JAMA Neurology Sep 2023Differential diagnosis of patients with seronegative demyelinating central nervous system (CNS) disease is challenging. In this regard, evidence suggests that...
IMPORTANCE
Differential diagnosis of patients with seronegative demyelinating central nervous system (CNS) disease is challenging. In this regard, evidence suggests that immunoglobulin (Ig) A plays a role in the pathogenesis of different autoimmune diseases. Yet little is known about the presence and clinical relevance of IgA antibodies against myelin oligodendrocyte glycoprotein (MOG) in CNS demyelination.
OBJECTIVE
To investigate the frequency of MOG-IgA and associated clinical features in patients with demyelinating CNS disease and healthy controls.
DESIGN, SETTING, AND PARTICIPANTS
This longitudinal study comprised 1 discovery and 1 confirmation cohort derived from 5 centers. Participants included patients with suspected or confirmed demyelinating diseases and healthy controls. MOG-IgA, MOG-IgG, and MOG-IgM were measured in serum samples and cerebrospinal fluid (CSF) of patients, who were assessed from September 2012 to April 2022.
MAIN OUTCOMES AND MEASURES
Frequency and clinical features of patients who were seropositive for MOG-IgA and double-seronegative for aquaporin 4 (AQP4) IgG and MOG-IgG.
RESULTS
After the exclusion of 5 participants with coexisting AQP4-IgG and MOG-IgA, MOG-IgG, and/or MOG-IgM, 1339 patients and 110 healthy controls were included; the median follow-up time was 39 months (range, 0-227 months). Of included patients with isolated MOG-IgA, 11 of 18 were female (61%), and the median age was 31.5 years (range, 3-76 years). Among patients double-seronegative for AQP4-IgG and MOG-IgG (1126/1339; 84%), isolated MOG-IgA was identified in 3 of 50 patients (6%) with neuromyelitis optica spectrum disorder, 5 of 228 patients (2%) with other CNS demyelinating diseases, and 10 of 848 patients (1%) with multiple sclerosis but in none of the healthy controls (0/110). The most common disease manifestation in patients seropositive for isolated MOG-IgA was myelitis (11/17 [65%]), followed by more frequent brainstem syndrome (7/16 [44%] vs 14/75 [19%], respectively; P = .048), and infrequent manifestation of optic neuritis (4/15 [27%] vs 46/73 [63%], respectively; P = .02) vs patients with MOG-IgG. Among patients fulfilling 2017 McDonald criteria for multiple sclerosis, MOG-IgA was associated with less frequent CSF-specific oligoclonal bands (4/9 [44%] vs 325/351 [93%], respectively; P < .001) vs patients with multiple sclerosis who were MOG-IgG/IgA seronegative. Further, most patients with isolated MOG-IgA presented clinical attacks after recent infection or vaccination (7/11 [64%]).
CONCLUSION AND RELEVANCE
In this study, MOG-specific IgA was identified in a subgroup of patients who were double-seronegative for AQP4-/MOG-IgG, suggesting that MOG-IgA may be a novel diagnostic biomarker for patients with CNS demyelination.
Topics: Humans; Female; Male; Myelin-Oligodendrocyte Glycoprotein; Longitudinal Studies; Neuromyelitis Optica; Aquaporin 4; Brain Stem; Multiple Sclerosis; Autoantibodies; Immunoglobulin G; Immunoglobulin A; Immunoglobulin M
PubMed: 37548987
DOI: 10.1001/jamaneurol.2023.2523 -
Pathogens (Basel, Switzerland) Dec 2023Flaviviruses are a group of positive-sense, single-stranded RNA viruses predominantly transmitted by arthropods (mainly mosquitoes) that cause severe endemic infections... (Review)
Review
Flaviviruses are a group of positive-sense, single-stranded RNA viruses predominantly transmitted by arthropods (mainly mosquitoes) that cause severe endemic infections and epidemics on a global scale. They represent a major cause of systemic morbidity and death and are expanding worldwide. Among this group, dengue fever, the West Nile virus, yellow fever, Japanese Encephalitis, and, recently, the Zika virus have been linked to a spectrum of ocular manifestations. These manifestations encompass subconjunctival hemorrhages and conjunctivitis, anterior and posterior uveitis (inclusive of vitritis, chorioretinitis, and retinal vasculitis), maculopathy, retinal hemorrhages, and optic neuritis. Clinical diagnosis of these infectious diseases is primarily based on epidemiological data, history, systemic symptoms and signs, and the pattern of ocular involvement. Diagnosis confirmation relies on laboratory testing, including RT-PCR and serological testing. Ocular involvement typically follows a self-limited course but can result in irreversible visual impairment. Effective treatments of flavivirus infections are currently unavailable. Prevention remains the mainstay for arthropod vector and zoonotic disease control. Effective vaccines are available only for the yellow fever virus, dengue virus, and Japanese Encephalitis virus. This review comprehensively summarizes the current knowledge regarding the ophthalmic manifestations of the foremost flavivirus-associated human diseases.
PubMed: 38133340
DOI: 10.3390/pathogens12121457 -
Proceedings of the National Academy of... Aug 2023Although the visual system extends through the brain, most vision loss originates from defects in the eye. Its central element is the neural retina, which senses light,...
Although the visual system extends through the brain, most vision loss originates from defects in the eye. Its central element is the neural retina, which senses light, processes visual signals, and transmits them to the rest of the brain through the optic nerve (ON). Surrounding the retina are numerous other structures, conventionally divided into anterior and posterior segments. Here, we used high-throughput single-nucleus RNA sequencing (snRNA-seq) to classify and characterize cells in six extraretinal components of the posterior segment: ON, optic nerve head (ONH), peripheral sclera, peripapillary sclera (PPS), choroid, and retinal pigment epithelium (RPE). Defects in each of these tissues are associated with blinding diseases-for example, glaucoma (ONH and PPS), optic neuritis (ON), retinitis pigmentosa (RPE), and age-related macular degeneration (RPE and choroid). From ~151,000 single nuclei, we identified 37 transcriptomically distinct cell types, including multiple types of astrocytes, oligodendrocytes, fibroblasts, and vascular endothelial cells. Our analyses revealed a differential distribution of many cell types among distinct structures. Together with our previous analyses of the anterior segment and retina, the data presented here complete a "Version 1" cell atlas of the human eye. We used this atlas to map the expression of >180 genes associated with the risk of developing glaucoma, which is known to involve ocular tissues in both anterior and posterior segments as well as the neural retina. Similar methods can be used to investigate numerous additional ocular diseases, many of which are currently untreatable.
Topics: Humans; Transcriptome; Endothelial Cells; Optic Disk; Glaucoma; Optic Nerve; Sclera
PubMed: 37566633
DOI: 10.1073/pnas.2306153120