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Immunopathogenesis of neuromyelitis optica.

Neuromyelitis optica (NMO, Devic's syndrome) is a clinical syndrome characterized by optic neuritis and (mostly longitudinally extensive) myelitis. If untreated, NMO usually takes a relapsing course and often results in blindness and tetra- or paraparesis. The discovery of autoantibodies to aquaporin-4, the most abundant water channel in the CNS, in 70-80% of patients with NMO (termed NMO-IgG or AQP4-Ab) and subsequent investigations into the pathogenic impact of this new reactivity have led to the recognition of NMO as an autoimmune condition and as a disease entity in its own right, distinct…

Biomarkers for neuromyelitis optica.

Neuromyelitis optica (NMO) is an acquired, heterogeneous inflammatory disorder, which is characterized by recurrent optic neuritis and longitudinally extensive spinal cord lesions. The discovery of the serum autoantibody marker, anti-aquaporin 4 (anti-AQP4) antibody, revolutionizes our understanding of pathogenesis of NMO. In addition to anti-AQP4 antibody, other biomarkers for NMO are also reported. These candidate biomarkers are particularly involved in T helper (Th)17 and astrocytic damages, which play a critical role in the development of NMO lesions. Among them, IL-6 in the peripheral blo…

The treatment of neuromyelitis optica.

Neuromyelitis optica (NMO) is an autoimmune disorder of the central nervous system directed against astrocytes. Initially diagnosed in individuals with monophasic or relapsing optic neuritis and transverse myelitis, NMO is now recognized as a demyelinating disorder with pleiotropic presentations due to the identification of a specific autoantibody response against the astrocyte water channel aquaporin-4 in the majority of individuals. As visual impairment and neurologic dysfunction in NMO are commonly severe, aggressive treatment of relapses and prophylactic immunomodulatory therapy are the fo…

Hydrocephalus in neuromyelitis optica.

A majority of patients with neuromyelitis optica (NMO) spectrum disorders (NMOSD) have MRI brain abnormalities, some of which are “NMO-typical” with localization in aquaporin 4 (AQP4)–rich circumventricular and periaqueductal regions.1 Although uncommon in adult patients, symptomatic brain involvement occurs in approximately 50% of NMO–immunoglobulin G (IgG) seropositive children. Here we report the clinical characteristics, type, and frequency of hydrocephalus in NMOSD.

The history of neuromyelitis optica.

The discovery of a novel serum autoantibody (termed NMO-IgG or AQP4-Ab) in a subset of patients in 2004 has revived interest in neuromyelitis optica (NMO). While the history of classical multiple sclerosis has been extensively studied, only little is known about the history of NMO. In the present article, we provide a comprehensive review of the early history of this rare but intriguing syndrome. We trace the origins of the concept of NMO in the 19th century medical literature and follow its evolution throughout the 20th and into the 21st century. Finally, we discuss recent proposals to revise…

[Treatment of neuromyelitis optica].

Neruomyelitis optica (NMO) is an inflammatory disease of the central nervous system characterized by severe optic neuritis and transverse myelitis, usually with a relapsing course. It is crucial that treatment is started as early as possible to avoid new relapses and further disability. Treatment of NMO spectrum disorders is divided into two objectives: one is to control the inflammatory damage in acute attacks and the other one is a maintenance treatment to avoid relapses. The former is based on high-dose intravenous corticosteroids and plasmapheresis, the latter is based on low-dose corticos

Neuromyelitis optica: an update.

Neuromyelitis optica (NMO) is an inflammatory condition characterized by the selective involvement of the optic nerves and spinal cord, and by a frequent relapsing course. Many clinical, laboratory and neuroimaging studies have provided useful means to distinguish NMO from multiple sclerosis (MS). The detection of aquaporin-4 (AQP4) antibodies has broadened the spectrum of the disorder, which now includes limited variants (either recurrent myelitis or optic neuritis), Asian opticospinal MS, and "atypical" forms with brain involvement. Many in vitro and in vivo evidence have recently demonstrat…

Neuromyelitis optica: an overview.

Neuromyelitis optica (NMO) is a relapsing inflammatory disorder of the central nervous system that closely resembles multiple sclerosis. This review discusses recent understanding of NMO with reference to epidemiology, clinical spectrum, immunopathology, diagnostic evaluation, clinical course and management. Within the clinical spectrum of NMO, the classical form (with relapsing opticomyelitis) and limited forms (either recurrent myelitis or optic neuritis), as well as its association with other autoimmune disorders, have been recognised in recent years. Further, symptomatic or asymptomatic ce…

[Pathology of neuromyelitis optica].

Understanding of the pathogenesis of neuromyelitis optica (NMO) is rapidly growing. In our immunohistochemical studies from 2006, the loss of AQP4 was evident in about 90% of NMO lesions, especially in perivascular areas of acute inflammatory lesions where immunoglobulins and complements were deposited. Glial fibrillary acidic protein (GFAP) was also weak or lost in those lesions. In contrast, myelin basic protein (MBP)-stained myelinated fibers were relatively preserved in those lesions where AQP4 was completely lost. In contrast to NMO lesions, AQP4 and GFAP were preserved or increased in de…

Neuromyelitis optica in the elderly.

Acute transverse myelitis and monolateral optic neuritis developed within a few weeks of each other in a 68-year-old woman. CSF findings con … em examination, clinical, laboratory and neuroimaging findings are consistent with the diagnosis of neuromyelitis optica (NO) related to multiple sclerosis. This case serves to document that NO can occur initially at an advanced age.

Neuromyelitis Optica Spectrum Disorder.

OBJECTIVE: This article reviews the clinical features of and diagnostic and treatment approaches to neuromyelitis optica spectrum disorder (NMOSD), with additional focus on rare phenotypes and specific contexts. Pathogenesis and pathology, differential diagnoses, and the potential use of novel biomarkers are also discussed. LATEST DEVELOPMENTS: A major advancement in the characterization of NMOSD was the discovery of serum antibodies targeting the water channel aquaporin-4 in the majority of patients, which also improved the characterization of disease pathogenesis. A proper diagnosis is fund…

Нейромиэлит Оптика Спектрум.

Нейромиелитное расстройство спектра (NMOSD) представляет собой серьезное аутоиммунное заболевание, которое в основном затрагивает оптические нервы и спинной мозг, что представляет собой отличный процесс заболевания от множественного склероза (MS) и заболевания миэлина олигодендроцита гликпротеина (MOG), связанного с антителами. Наиболее распространенным нейро-офтальмологическим проявлением NMOSD является атипичный оптический неврит (ON), характеризующийся серьезной потерей зрения с большей склонностью к двустороннему участию, продольным усилением зрительного нерва и хиазменным участием, чем оп

Совпадение в аннотации оригинала
Neuromyelitis optica spectrum disorder (NMOSD) is a severe autoimmune disease that predominantly affects the optic nerves and spinal cord, which is a distinct disease process from multiple sclerosis (MS) and myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease. The most common neuro-ophthalmologic manifestation of NMOSD is atypical optic neu…

Neuromyelitis Optica Spectrum Disorder.

Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune condition characterized by recurrent episodes of optic neuritis (ON) and transverse myelitis. This case report aims to highlight the importance of considering atypical presentations of NMOSD when confronted with MRI-detected Wernicke's encephalopathy. The primary target in NMOSD is the aquaporin-4 (AQP4) protein, predominantly located on astrocyte surfaces. Antibodies binding to AQP4 can lead to astrocyte dysfunction and damage, contributing to NMOSD's distinctive pathology. The associated immune response and inflammation can caus…

[Neuromyelitis Optica Spectrum Disorders].

Neuromyelitis optica spectrum disorders (NMOSD) are inflammatory autoimmune disorders of the central nervous system, that primarily cause optic neuritis and myelitis. Aquaporin-4 (AQP4) antibody is the key in NMOSD pathophysiology, which causes astrocytopathy, demyelination, and neuropathy through complement activation and cell-mediated immunity. Currently, biopharmaceutical agents are introduced for preventing relapse with high efficacy, expected to reduce side effects derived from long-term steroid therapy, and improve patients' quality of life.

Neuromyelitis Optica Spectrum Disorders.

PURPOSE OF REVIEW: This article provides a practical approach for providers caring for patients with neuromyelitis optica (NMO) spectrum disorders. Clinical and imaging features, diagnostic criteria, treatment of acute exacerbations, chronic preventive therapy, and symptom management in NMO spectrum disorders are discussed. RECENT FINDINGS: The rapid pace of research in NMO spectrum disorders has led to many recent advances. A broader understanding of the clinical spectrum of the disease as well as improvements in anti-aquaporin-4 antibody assays have led to recent revision of the diagnostic …

Neuromyelitis optica: changing concepts.

Neuromyelitis optica (NMO; Devic's disease) and the NMO spectrum disorders are idiopathic inflammatory demyelinating disorders that affect the central nervous system and have a predilection for optic nerves and spinal cord. The identification of NMO-IgG as a disease-specific marker and aquaporin 4 as the target antigen has renewed interest in NMO. Based on current data, we suspect that autoantibodies arising from peripheral B cells bind to aquaporin 4 expressed on astrocyte foot processes on the abluminal surface of microvessels, activate complement and initiate inflammatory demyelination and …

Neuromyelitis optica: current concepts.

Neuromyelitis optica (NMO; Devic's syndrome or Devic's disease) is a clinically defined disorder consisting of optic neuritis (ON) in combination with myelitis. Its nosological relationship to other central nervous system demyelinating diseases remains uncertain. Advances in understanding of its natural history and pathophysiology may eventually allow determination of whether idiopathic NMO represents a specific disease. This article summarizes recent progress in these areas of NMO research.