Severe inflammatory demyelinating disorder distinct from multiple sclerosis
Once it was known as monophasic disease with co-occurance of optic neuritis and extensive myelitis but now is recognized as relapsing disorder in which these events can be separated by years or decades. Now a days it is known as NMO spectrum disorder
Pathogenesis-Autoimmune astrocytopathy with development of antibodies to aquaporin 4 (cell membrane water channel concentrated on glial foot processes at glial limiting membrane surrounding blood vessels and in association with pia mater)
Conventional MR imaging SPINE
Presence of longitudinally extensive (>/= 3 vertebrae) spinal cord lesion (LESCL) with predominant involvement of central gray matter
May acutely expand the cord and show patchy enhancement in their entire extent
Autoimmune myelitidis associated with conditions like Sjogren’s/SLE
Neurosarcoidosis and postinfectitious myelitidis-surface enhancement due to meningeal involvement is more common in these conditions. They may be indistinguishable from spinal NMO on radiological grounds
Long segment involvement the optic nerve associated with acute swelling and enhancement, particularly when bilateral and extending up to the optic chiasm, possibility of NMO should be considered in appropriate clinical settings
Typical of NMO brain lesions reflect AQP4 distribution. Nonspecific punctate or small lesions (< 3mms) in periependymal distribution surrounding third ventricle, cerebral aqueduct and forth ventricle and dorsal brainstem. Periventricular NMO lesions surrounding lateral ventricles tend to extend along the wall rather than perpendicular to it
Large confluent heterogenoushemispheric lesions are also known