Typical and Atypical NMOSD - Its Implications
Acta Scientific Neurology
Neuromyelitis optica (NMO) has evolved from Devic's classical description to a broader disease spectrum of NMO Spectrum Disorder (NMOSD), from monophasic illness to a polyphasic illness with multiple recurrences, disease confined to optic nerve and spinal cord to now brain stem, cerebrum and even with endocrinopathy due to hypothalamic involvement , coexisting infections  and a variety of autoimmune diseases, including non-organ specific autoimmune diseases and co-existent autoantibodies
... ent autoantibodies without diseases  . Objectives: To report, the epidemiological characteristics, clinical presentations, recurrence rate, treatment and response to therapy in 23 patients with NMO spectrum disorder among the Indian population. Materials and Methods : An observational, retrospective analysis of our prospectively maintained data base of patients during the period of May 2018 -June 2018 who satisfied International Panel for NMO Diagnosis (IPND) revised criteria  of NMOSD was done. Results: 1. 23 case records of NMOSD were retrieved. 14 cases were aquaporin4 positive and 9 were aquaporin4 negative. 2. Mean age is 39.6 years. Median age is 42 years (Range 17 -64). Female to male ratio is 1:2.8. 3. Clinical presentations included longitudinally extensive transverse myelitis being the most common followed by optic neuritis, area postrema syndrome, seizures and brainstem encephalitis. 4. Two had associated herpes simples virus encephalitis, one CSF VDRL positivity. Two had associated retro viral disease. 5. Two had ANA, Anti ds DNA antibody positive vasculitis. 6. One had CSF oligoclonal bands in CSF and an open ring enhancement on MRI suggestive of tumefactive demyelination. Discussion and Conclusion: Contrary to current literature there is unusual male preponderance in our study population. • Atypical lesions( Red flags) or course should suggest additional work up for associated diseases and prompt treatment ofwhich can lead to a significant recovery. • Aquaporin 4 antibody positivity, severity at presentation, associated diseases and relapse rate determine the prognosis.