neuromyelitis optica spectrum disorder
Also written as: NMOSD — neuromyelitis optica spectrum disorder
An autoimmune CNS disease characterized by severe optic neuritis and myelitis, often with AQP4 antibodies.
Full Definition
Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune inflammatory disease of the central nervous system that predominantly affects the optic nerves, spinal cord, and specific brain regions. The majority of patients have antibodies against aquaporin-4 (AQP4), a water channel protein highly expressed on astrocytes. NMOSD attacks tend to be more severe and less recoverable compared to multiple sclerosis, often causing blindness or paralysis. The disease can also affect the brainstem, hypothalamus, and cerebrum, particularly areas with high AQP4 expression. Early recognition is crucial as the treatment approach differs significantly from multiple sclerosis.
Usage
Usage note: Preferred term over 'neuromyelitis optica' as it encompasses the full clinical spectrum.
In Context
- "The patient was diagnosed with neuromyelitis optica spectrum disorder based on clinical presentation and positive AQP4 antibodies." — Clinical diagnosis
- "NMOSD attacks require aggressive immunosuppressive treatment to prevent permanent disability." — Treatment guideline