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After the discovery of anti-AQP4 auto-antibodies there are two kinds of Optic-Spinal MS (OSMS): Anti-AQP4 positive OSMS or Neuromyelitis optica; Anti-AQP4 negative OSMS, currently idiopathic, considered inside the Inflammatory demyelinating diseases of the central nervous system spectrum.
Neuromyelitis optica (NMO) is a particular disease within the NMOSD spectrum. It is characterised by optic neuritis and longitudinally extensive myelitis. In more than 80% of NMO cases, the cause is immunoglobulin G autoantibodies to aquaporin 4 ( anti-AQP4 ), the most abundant water channel protein in the central nervous system.
Anti-AQP4 diseases, are a group of diseases characterized by auto-antibodies against aquaporin 4.. After the discovery of anti-AQP4 autoantibody in neuromyelitis optica, it was found that it was also present in some patients with other clinically defined diseases, including multiple sclerosis variants like optic-spinal MS.
The most common cause is multiple sclerosis (MS) or ischemic optic neuropathy due to thrombosis or embolism of the vessel that supplies the optic nerve. [13] [14] Up to 50% of patients with MS will develop an episode of optic neuritis, and 20–30% of the time optic neuritis is the presenting sign of MS.
Anti-MOG antibodies have been described in some patients with NMOSD [15] [16] who were negative for the aquaporin 4 (AQP-4) antibody. However, most NMOSD is an astrocytopathy, specifically an AQP4 antibody-associated disease, whereas MOG antibody-associated disease is an oligodendrocytopathy, suggesting that these are two separate pathologic entities. [2]
MS Multiple sclerosis NIDDM Non-insulin-dependent diabetes mellitus NRR Neuro-retinal rim NS Nuclear sclerosis: NTG Normal tension glaucoma: PDR Proliferative diabetic retinopathy PDT Photodynamic therapy: PK Penetrating keratoplasy: POAG Primary open-angle glaucoma PPDR Preproliferative diabetic retinopathy PRA Pan-retinal ablation PRK
MS is a clinically defined entity with several atypical presentations. Some auto-antibodies have been found in atypical MS cases, giving birth to separate disease families and restricting the previously wider concept of MS. Anti-AQP4 autoantibodies were found in neuromyelitis optica (NMO), which was
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