M Netravathi1, Hari Krishna Bollampalli2, Maya Dattatraya Bhat3, Valakunja Harikrishna Ganaraja4, Shweta Prasad5, Anita Mahadevan6, Nitish Kamble7, Atchayaram Nalini8, Ravi Yadav9, Pramod Kumar Pal10, Parthasarthy Satishchandra11. 1. Department of Neurology, National Institute of Mental Health & Neurosciences (NIMHANS), Bangalore, India.. Electronic address: sundernetra@yahoo.co.in. 2. Department of Neurology, National Institute of Mental Health & Neurosciences (NIMHANS), Bangalore, India.; Department of Clinical Neurosciences, National Institute of Mental Health and Neurosciences (NIMHANS), Bangalore, India.. Electronic address: drharikrishna18@gmail.com. 3. Department of Neuroimaging and Interventional Neuroradiology (NIIR), National Institute of Mental Health and Neurosciences (NIMHANS), Bangalore, India.. Electronic address: mayabhat05@yahoo.co.in. 4. Department of Clinical Neurosciences, National Institute of Mental Health and Neurosciences (NIMHANS), Bangalore, India.. Electronic address: ganaraj.v.h.91@gmail.com. 5. Department of Neurology, National Institute of Mental Health & Neurosciences (NIMHANS), Bangalore, India.; Department of Clinical Neurosciences, National Institute of Mental Health and Neurosciences (NIMHANS), Bangalore, India.. Electronic address: mayabhat05@yahoo.co.in. 6. Department of Neuropathology, National Institute of Mental Health and Neurosciences (NIMHANS), Bangalore, India.. Electronic address: mayabhat05@yahoo.co.in. 7. Department of Neurology, National Institute of Mental Health & Neurosciences (NIMHANS), Bangalore, India.. Electronic address: nitishlk@gmail.com. 8. Department of Neurology, National Institute of Mental Health & Neurosciences (NIMHANS), Bangalore, India.. Electronic address: atchayaramnalini@yahoo.co.in. 9. Department of Neurology, National Institute of Mental Health & Neurosciences (NIMHANS), Bangalore, India.. Electronic address: docravi20@yahoo.com. 10. Department of Neurology, National Institute of Mental Health & Neurosciences (NIMHANS), Bangalore, India.. Electronic address: palpramod@hotmail.com. 11. Department of Neurology, National Institute of Mental Health & Neurosciences (NIMHANS), Bangalore, India.. Electronic address: drpsatishchandra@yahoo.com.
Abstract
BACKGROUND: Neuromyelitis Optica (NMO) is an autoimmune astrocytopathic disorder due to AQP4 antibodies. OBJECTIVES: To analyse clinical, neuroimaging features in NMO patients and assess the efficacy of various therapeutics. METHODS: AQP4+ve NMO patients were diagnosed based on consensus diagnostic criteria. RESULTS: 101 AQP4+ve NMO patients were seen with female (90) predominance. Adult population (71.3%) formed the larger group followed by pediatric (19.8%) and late-onset (8.9%). Myelopathy (36.2%) was most commonly seen followed by optic neuritis (19.1%), brainstem (17.1%), opticomyelopathy (16.1%), area postrema involvement (10.5%) and encephalopathy (1%). Encephalopathy and brainstem/cerebellar involvement were most common in pediatric population while opticomyelopathy was more common in late-onset patients. Hyperintensities of lower medulla was seen in 67.3% subjects and 49.5% had involvement of obex. Differential T2 hyperintensity of the long segment myelitis was found in 30.7%. Plasmapheresis was given in 71 subjects followed by maintenance therapy. Most of them showed significant improvement with EDSS score of 1 in 30.7%. CONCLUSIONS: Clinical manifestations in AQP4+ve NMO patients may vary depending on the age at onset of illness. MRI features affecting cervicomedullary junction, obex, differential T2 hyperintensities of the spinal cord may form a useful diagnostic clue. Plasmapheresis is helpful in achieving remission along with immunomodulation.
BACKGROUND:Neuromyelitis Optica (NMO) is an autoimmune astrocytopathic disorder due to AQP4 antibodies. OBJECTIVES: To analyse clinical, neuroimaging features in NMO patients and assess the efficacy of various therapeutics. METHODS:AQP4+ve NMO patients were diagnosed based on consensus diagnostic criteria. RESULTS: 101 AQP4+ve NMO patients were seen with female (90) predominance. Adult population (71.3%) formed the larger group followed by pediatric (19.8%) and late-onset (8.9%). Myelopathy (36.2%) was most commonly seen followed by optic neuritis (19.1%), brainstem (17.1%), opticomyelopathy (16.1%), area postrema involvement (10.5%) and encephalopathy (1%). Encephalopathy and brainstem/cerebellar involvement were most common in pediatric population while opticomyelopathy was more common in late-onset patients. Hyperintensities of lower medulla was seen in 67.3% subjects and 49.5% had involvement of obex. Differential T2 hyperintensity of the long segment myelitis was found in 30.7%. Plasmapheresis was given in 71 subjects followed by maintenance therapy. Most of them showed significant improvement with EDSS score of 1 in 30.7%. CONCLUSIONS: Clinical manifestations in AQP4+ve NMO patients may vary depending on the age at onset of illness. MRI features affecting cervicomedullary junction, obex, differential T2 hyperintensities of the spinal cord may form a useful diagnostic clue. Plasmapheresis is helpful in achieving remission along with immunomodulation.
Authors: Elham Khalilidehkordi; Laura Clarke; Simon Arnett; Wajih Bukhari; Sofia Jimenez Sanchez; Cullen O'Gorman; Jing Sun; Kerri M Prain; Mark Woodhall; Roger Silvestrini; Christine S Bundell; David Abernethy; Sandeep Bhuta; Stefan Blum; Mike Boggild; Karyn Boundy; Bruce J Brew; Matthew Brown; Wallace Brownlee; Helmut Butzkueven; William M Carroll; Celia Chen; Alan Coulthard; Russell C Dale; Chandi Das; Marzena J Fabis-Pedrini; David Fulcher; David Gillis; Simon Hawke; Robert Heard; Andrew P D Henderson; Saman Heshmat; Suzanne Hodgkinson; Trevor J Kilpatrick; John King; Chris Kneebone; Andrew J Kornberg; Jeannette Lechner-Scott; Ming-Wei Lin; Christopher Lynch; Richard A L Macdonell; Deborah F Mason; Pamela A McCombe; Jennifer Pereira; John D Pollard; Sudarshini Ramanathan; Stephen W Reddel; Cameron Shaw; Judith Spies; James Stankovich; Ian Sutton; Steve Vucic; Michael Walsh; Richard C Wong; Eppie M Yiu; Michael H Barnett; Allan G Kermode; Mark P Marriott; John Parratt; Mark Slee; Bruce V Taylor; Ernest Willoughby; Fabienne Brilot; Angela Vincent; Patrick Waters; Simon A Broadley Journal: Front Neurol Date: 2020-06-16 Impact factor: 4.003
Authors: Nishita Singh; Rohit Bhatia; Prerna Bali; V Sreenivas; M V Padma; Vinay Goyal; Rohit Saxena; Deepa Dash; Ajay Garg; S Leve Joseph Journal: Ann Indian Acad Neurol Date: 2020-01-06 Impact factor: 1.383