Neuromyelitis optica and neuromyelitis optica spectrum disorders

Abstract

Neuromyelitis optica or neuromyelitis optica spectrum disorders (NMOSD) are autoimmune diseases associated with a disease-specific autoantibody directed against the water channel protein aquaporin-4. While almost all patients with NMOSD show a relapsing-remitting course, just 2% of patients present with a progressive course, suggesting that preventing acute attacks can lead to stable remission and avoid progression of the condition. Standard immunotherapy, immunosuppressive agents, and corticosteroids can prevent acute attacks and maintain remission in the majority of patients with NMOSD. However, there is a strong need for alternative options for patients who are refractory to standard treatments. Emerging therapies targeting specific molecules related to the pathogenicity of NMOSD are currently being developed. In addition to standard intravenous high-dose corticosteroid and plasma exchange/plasma-pheresis, therapies targeted at inhibiting granulocytes, complement, and vas-cular endothelial growth factor are anticipated for acute attacks. With regard to preventive treatment of NMOSD, randomized clinical trials using mono-clonal immunoglobulin G antibody targeting CD19 and CD20 on B cells, inter-leukin-6, and complement protein C5 are underway. There are many preclinical therapeutic agents that target aquaporin-4 and the pathogenic anti-aqua-porin-4 antibody itself: complement inhibitor and T helper 17 cells based on the specific NMOSD pathology. The future goal of immunotherapies for NMOSD would be to select suitable therapies for the patient's pathological condition among off-target and molecular-target agents.

‘Leukodystrophy‐like’ phenotype in children with myelin oligodendrocyte glycoprotein antibody‐associated disease

“…The reduced susceptibility in the fully myelinated adult brain is consistent with the overall benign phenotype in adult cases of MOG antibody-Positive neuromyelitis optica spectrum disorder. [17][18][19] In a cohort of 56 adults with MOG antibody, the clinical course was favourable, with good clinical outcomes and minimal residual disability. 20 Although only two out of seven patients with 'leukodystrophy-like' phenotype demonstrated these radiological features at onset it is possible that the MRI was not performed at nadir or that there is a lag between clinical and radiological features.…”

Neuromyelitis optica spectrum disorder

“…Neuromyelitis optica spectrum disorder (NMOSD) is a potentially life-threatening neuroinflammatory disease targeting the optic nerve, spinal cord, and brain. [1][2][3][4] Relapses result in cumulative neurologic disabilities, are unpredictable, and are interspersed with remissions. Increased diagnostic accuracy and increased health care provider awareness have resulted in increased prevalence up to 10/100,000 in some geographic regions.…”

Spastic Paraplegias Due to Non-Traumatic Spinal Cord Disorders

“…The characteristic finding is multiple lesions in the brain and spinal cord observed on MRI [20]. Neuromyelitis optica (NMO) is a similar disease to MS, but usually long cord lesions (>3 vertebral body) are observed on spinal MRI, and autoantibodies against aquaporin 4 are usually detected in patients' sera [21,22].…”