OBJECTIVE: Neuromyelitis optica (NMO) is an inflammatory disease associated with optic neuritis and myelitis. Although some studies have reported multiple sclerosis (MS)-like lesions in 10-30% of NMO patients, brain MRI is usually normal. Several studies have observed metabolic abnormalities on MR spectroscopy in MS, even in normal-appearing white matter (NAWM).
BACKGROUND: Cases of anti-aquaporin (AQP)-4 antibody-positive familial neuromyelitis optica (NMO) in mothers and daughters are described. PARTICIPANTS: The demographic, clinical, neuroimaging, and anti-AQP-4 antibody status were investigated in four patients from two Asian families with anti-AQP-4 antibody-positive NMO
The clinical course of immune mediated optic neuritis (ON) will depend on the specific underlying inflammatory disease. These disorders have traditionally been classified according to clinical and MRI findings. Aquaporin-4 (AQP4) autoantibodies (neuromyelitis optica-IgG (NMO-IgG)) may have diagnostic and prognostic value in patients who present with isolated ON.
A full-term female neonate was born with severe hypotonia and weakness. Her mother had been treated for neuromyelitis optica (Devic disease) for 6 years
Recently, a disease-specific antibody was found in serum from patients with neuromyelitis optica (NMO), and its target antigen was identified as aquaporin 4 (AQP4) water channel protein. There is no clinical picture of pediatric cases with anti-AQP4 antibody, except one report from North America. Here, we report the clinical features of 18 Japanese anti-AQP4 antibody -positive patients with childhood-onset of NMO
OBJECTIVE: To evaluate the efficacy and safety of repeated rituximab treatment based on the assessment of peripheral circulating memory B cells over 24 months in patients with relapsing neuromyelitis optica (NMO). DESIGN: Prospective open-label study.
Growing evidence suggests that interleukin (IL)-17 and IL-17-secreting CD4(+)T (Th17) cells are involved in the pathogenic mechanisms of multiple sclerosis (MS). IL-17-secreting CD8(+) T cells were recently identified as a novel subset of CD8(+)T cells.
BACKGROUND: Although overt involvement of the central nervous system (CNS) in myasthenia gravis (MG) is considered rare, hyperreflexia is a common and yet unexplained finding. Aquaporin 4 (AQP4), the target autoantigen in neuromyelitis optica, is expressed both in the CNS and in the neuromuscular junction
The detection of antibodies against aquaporin-4 (AQP4) has improved the diagnosis of neuromyelitis optica (NMO). We evaluated a recently established cell-based anti-AQP4 assay in 273 patients with inflammatory CNS demyelination. The assay had a specificity of 99% and a sensitivity of 56% to detect all NMO patients and of 74% to detect the recurrent NMO patients, similar to the initial studies reported.
Background: A new autoantibody (termed NMO-IgG, or AQP4-Ab) has recently been described in patients with neuromyelitis optica (NMO) and its formes frustes, longitudinally extensive transverse myelitis (LETM) and recurrent optic neuritis (rON). However, AQP4-Ab has been found also in patients with co-existing rheumatic diseases such as systemic lupus erythematosus (SLE) or Sjogren’s syndrome (SS), conditions which are characterized by broad, polyspecific B cell activation
Background: Myasthenia gravis (MG) and neuromyelitis optica (NMO, also known as Devic disease) are rare autoimmune disorders, with upper-limit prevalence estimates in the general population of 15 per 100 000 and 5 per 100 000, respectively. To our knowledge, an association between these diseases has not been previously reported.
BACKGROUND: They are severe inflammatory demyelinating diseases of the central nervous system, often called idiopathic inflammatory demyelinating disease (IIDD). These diseases are explosive or pseudotumoral multiple sclerosis (MS), acute disseminated encephalomyelitis (ADEM), transverse myelitis and neuromyelitis optica (NMO).