视神经脊髓炎谱系疾病与妊娠

视神经脊髓炎谱系疾病是一种中枢神经系统自身免疫性疾病,好发于育龄期女性,可影响妊娠计划、生育能力,对妊娠结局的影响尚不明确;妊娠可引起免疫系统和性激素水平变化,造成疾病复发和进展。合理的免疫抑制治疗可以有效避免妊娠相关疾病活动。妊娠期和分娩后应用小剂量糖皮质激素、硫唑嘌呤和利妥昔单抗可能是安全的。     

视神经脊髓炎谱系疾病与妊娠

视神经脊髓炎谱系疾病是一种中枢神经系统自身免疫性疾病,好发于育龄期女性,可影响妊娠计划、生育能力,对妊娠结局的影响尚不明确;妊娠可引起免疫系统和性激素水平变化,造成疾病复发和进展。合理的免疫抑制治疗可以有效避免妊娠相关疾病活动。妊娠期和分娩后应用小剂量糖皮质激素、硫唑嘌呤和利妥昔单抗可能是安全的。     

Neuromyelitis optica

Neuromyelitis optica (NMO) is a disabling inflammatory condition that targets astrocytes in the optic nerves and spinal cord. Neuro-ophthalmologists must be particularly aware of this disorder because about half of patients present as isolated unilateral optic neuritis months or years before a disease-defining and often crippling bout of myelitis. NMO is easily confused with multiple sclerosis because it is characterized by relapses that lead to stepwise accrual of deficits. The best predictor of conversion from optic neuritis to clinical definite NMO is the presence of a serum antibody to aquaporin-4 called NMO-IgG. However, this test is currently only about 75% sensitive. Suspicion of NMO should be high in patients who present with vision of light perception or worse or who are left with acuity of 20/50 or worse after optic neuritis and in those with simultaneous bilateral optic neuritis or recurrent attacks. Acute NMO relapses are generally treated with high-dose intravenous steroids, with plasma exchange often used as a rescue therapy for those who do not respond. Preventative strategies against relapses currently use broad-spectrum or selective B-lymphocyte immune suppression, but their use is based on small, generally uncontrolled studies. Hopefully, the future will bring more sensitive tools for defining risk and predicting outcome, as well as more targeted and effective forms of therapy.     

Neuromyelitis optica

Neuromyelitis optica (NMO), otherwise known as Devic's disease, is an idiopathic, severe, inflammatory disorder that preferentially affects the optic nerves and spinal cord. Clinical, laboratory and immunopathological evidence suggests that NMO is a humorally mediated disease distinct from MS. A spinal cord lesion extending contiguously over three or more vertebral segments is characteristic of NMO and, in combination with a cranial magnetic resonance imaging scan that does not meet radiological criteria for MS, is over 94% sensitive and 96% specific for NMO diagnosis. The serum autoantibody marker, neuromyelitis optica-immunoglobulin G (NMO-IgG), appears specific for NMO and suggests that the disease spectrum includes cases of recurrent longitudinally extensive transverse myelitis and Japanese opticospinal MS. Its target antigen is the water channel aquaporin-4, suggesting that NMO may represent a novel autoimmune channelopathy. Relapsing NMO has a poor prognosis; therapy, typically with immunosuppression, is necessary as early as possible in the disease course to prevent attack-related disability.     

Neuromyelitis optica

PURPOSE OF REVIEW: We review recent advances in neuromyelitis optica, an idiopathic inflammatory demyelinating disease of the central nervous system predominantly affecting optic nerves and spinal cord. We concentrate on a recently identified serum antibody biomarker, neuromyelitis optica immunoglobulin G (NMO-IgG), which distinguishes neuromyelitis optica from multiple sclerosis. RECENT FINDINGS: NMO-IgG is detected by indirect immunofluorescence. Its presence and specificity for neuromyelitis optica was confirmed in diverse populations. Seropositivity is now incorporated into new diagnostic criteria for neuromyelitis optica. Testing for this biomarker has suggested that the neuromyelitis optica spectrum is broader than previously recognized. Recently, the molecular target of NMO-IgG was identified as aquaporin-4. Immunopathologic studies suggest that loss of aquaporin-4 immunostaining is detectable in early lesions of neuromyelitis optica. A B-cell-specific monoclonal antibody, rituximab, may be an effective treatment even in patients not responding to other treatments. SUMMARY: Clinical, radiologic, and immunologic features distinguish neuromyelitis optica from other severe cases of multiple sclerosis. NMO-IgG is the first specific marker for a central nervous system demyelinating disease. The discovery of aquaporin-4 as the putative target of NMO-IgG, and recent data suggesting that aquaporin-4-specific antibodies are pathogenic may enhance our understanding of idiopathic inflammatory demyelinating diseases and their treatment.     

Neuromyelitis optica

Neuromyelitis optica (NMO, Devic syndrome) is a rare demyelinating disease of the central nervous system which mostly follows a relapsing course. Key features of this disorder include unilateral or bilateral optic neuritis and longitudinally extensive myelitis (> or = three segments). Brain lesions are rarely present at onset. They may however evolve during the course of disease but usually remain asymptomatic. The histopathology of NMO is suggestive of an underlying humoral autoimmune pathomechanism and indicates that NMO is a distinct entity rather than a variant of multiple sclerosis. The recent detection of NMO-specific serum autoantibodies against the water channel aquaporin-4 (Aqp4) is of significant diagnostic relevance and classifies NMO as the first inflammatory demyelinating disorder of the CNS with a defined autoantigen. More recent therapeutic strategies such as plasma exchange or pharmacological B-cell depletion are expected to improve long-term prognosis of NMO.     

Neuromyelitis optica

Whether neuromyelitis optica (NMO), the co-occurrence of myelitis and optic neuritis, is a variant of multiple sclerosis (MS) or a unique disease is controversial. Distinct neuropathological features and a fulminant clinical course argue in favor of NMO as a distinct disease. However, the combination of neurological impairments of myelitis and optic neuritis occurs in patients with several inflammatory disorders, including multiple sclerosis and collagen vascular diseases. NMO is also associated with certain infectious diseases. The fact that the NMO phenotype occurs in a variety of disease states suggests that NMO does not represent a specific clinical entity. To better understand NMO and its associations with recognized diseases, a systematic review of the literature using MEDLINE was conducted. The history of NMO, its nosology, associations with other diseases, and current concepts of its pathogenesis and treatment is reviewed in this article.     

Neuromyelitis optica and neuroregeneration

Totally three articles focusing on onset prediction of neuromyelitis optica and assessment of relevant clinical problems are published in three issues. We hope that our readers find these papers useful to their research.No. 1: Serum anti-aquaporin 4 anti-body in neuromyelitis patients is not correlated with the length of injured spinal cord segments Neural Regen Res. 2011;6(35):2725-2731.     

Corticoidbehandlung der Neuromyelitis optica

Zusammenfassung Die Neuromyelitis optica ist eine entzündliche Markscheidenerkrankung, deren Hauptsymptome eine ascendierende Querschnittslähmung, doppelseitige Erblindung, foudroyanter Verlauf bei schwerem Krankheitsgefühl und eine oft beschleunigte Senkungsgeschwindigkeit sind. Die Beobachtung ungewöhnlicher Häufung von Streßmomenten in den Anamnesen führte zur therapeutischen Anwendung von Corticoiden. Von 18 in 9 Jahren behandelten Fällen starben 2, die übrigen überlebten. Vergleichende Untersuchungen über Therapiedauer und Dosierungshöhe ergaben, daß Frühdiagnose und frühzeitige Behandlung mit genügender Menge von Corticoiden von besonderer Wichtigkeit für den Therapieerfolg sind. Ein Vergleich mit den Angaben der Literatur vermag diese Erfahrungen im allgemeinen zu bestätigen.Mit 6 TextabbildungenHerrn Professor Dr. G. Schaltenbrand zum 65. Geburtstag in Verehrung gewidmet.Referat, gehalten auf der Tagung der Deutschen Gesellschaft für Neurologie vom 17. bis 19. September 1962 in Köln.     

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 necrosis. The development of animal models and further analysis of the association of NMO-IgG with disease severity and treatment response will elucidate the pathobiology of NMO.     

Neuromyelitis optica spectrum disorder and menstruation

Background

Gender issues and the female preponderance in neuromyelitis optica spectrum disorder (NMOSD) have been investigated before, yet the interplay between NMOSD and menstrual characteristics has remained unknown. Thus, the aim was to compare menstrual cycle patterns and their symptoms in NMOSD patients and healthy women.

Neuromyelitis optica with hypothalamic involvement

We describe two cases of neuromyelitis optica (NMO) with clinical and radiographically confirmed features of hypothalamic involvement, in the absence of other parenchymal brain lesions. Their course is otherwise typical of Devic's form of NMO. A review of the literature identifies additional cases of NMO in which clinical features attributable to under-recognized dysfunction of the hypothalamic-pituitary axis were present. We propose that the currently accepted criteria for the diagnosis of NMO could be revisited to recognize the possibility of lesions developing within hypothalamic structures.     

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 demonstrated that AQP4 antibody plays a relevant pathogenetic role in NMO by inducing an increase of BBB permeability, complement cascade activation and astrocytic cytotoxicity. While corticosteroids and plasma exchange are better therapeutic options during NMO attacks, other treatments should be aimed at the prevention of recurrence, possibly by targeting autoantibody production and/or effector mechanisms. Rituximab and Mofetil Mycophenolate appear at the moment the most promising drugs. Since even the most sensitive AQP4 antibody tests fail to mark about 20-30% of the NMO cases, while 20-30% of positive patients have "atypical" or MS-like variants, it remains to be clarified if NMO, as a clinical entity, can still be considered a disease rather than a syndrome, with more possible pathogenetic mechanisms.