Rui Wang1, Hong-Zhi Guan2, Hai-Tao Ren3, Wei Wang4, Zhen Hong5, Dong Zhou6. 1. Department of Neurology, West China Hospital, Sichuan University, Chengdu, Sichuan, People's Republic of China. Electronic address: 1046792011@qq.com. 2. Department of Neurology, Peking Union Medical College Hospital, People's Republic of China. Electronic address: guanhz@263.net. 3. Department of Neurology, Peking Union Medical College Hospital, People's Republic of China. Electronic address: rht20080808@163.com. 4. Department of Neurology, West China Hospital, Sichuan University, Chengdu, Sichuan, People's Republic of China. Electronic address: wangwei10102@aliyun.com. 5. Department of Neurology, West China Hospital, Sichuan University, Chengdu, Sichuan, People's Republic of China. Electronic address: Hongzhengoog@aliyun.com. 6. Department of Neurology, West China Hospital, Sichuan University, Chengdu, Sichuan, People's Republic of China. Electronic address: zhoudong66@yahoo.de.
Abstract
PURPOSE: Anti-NMDAR-encephalitis is a recently described form of autoimmune encephalitis. Here, we characterize CSF changes in Chinese patients with anti-NMDAR encephalitis, and explore the relationship between CSF findings and disease outcome. METHODS: The presence of NMDAR antibodies in serum or CSF samples was evaluated in patients diagnosed with encephalitis between October 1, 2010 and August 1, 2014 at the West China Hospital. All patients fulfilling our diagnostic criteria were included and CSF findings were analyzed. Patient outcome was assessed after 4, 8, 12, 16, 20, and 24 months using the modified Rankin scale (mRS). RESULTS: Out of 3000 people with encephalitis screened, 43 patients were anti-NMDAR antibody positive in CSF or serum and included in this study. 62.8% of the patients identified with positive CSFs had positive serum anti-NMDAR samples, while 100% patients with positive serum had positive CSF samples. In the CSF white cell counts were elevated in 58.1% of cases; protein was increased in 18.6%; QAlb>Qlim(Alb) of the blood-CSF barrier was found in 29.3%; intrathecal immunoglobulin synthesis was detected in 17.1%, and 39.5% patients exhibited increased CSF pressures. A longer follow-up period was associated with better outcomes. There was no relationship between changes in CSF findings and outcome. CONCLUSION: The sensitivity of NMDA receptor antibody testing is higher in CSF compared to serum. Other CSF abnormalities are present in some patients with Anti-NMDAR-encephalitis, however these changes do not appear to affect prognosis.
PURPOSE: Anti-NMDAR-encephalitis is a recently described form of autoimmune encephalitis. Here, we characterize CSF changes in Chinese patients with anti-NMDAR encephalitis, and explore the relationship between CSF findings and disease outcome. METHODS: The presence of NMDAR antibodies in serum or CSF samples was evaluated in patients diagnosed with encephalitis between October 1, 2010 and August 1, 2014 at the West China Hospital. All patients fulfilling our diagnostic criteria were included and CSF findings were analyzed. Patient outcome was assessed after 4, 8, 12, 16, 20, and 24 months using the modified Rankin scale (mRS). RESULTS: Out of 3000 people with encephalitis screened, 43 patients were anti-NMDAR antibody positive in CSF or serum and included in this study. 62.8% of the patients identified with positive CSFs had positive serum anti-NMDAR samples, while 100% patients with positive serum had positive CSF samples. In the CSF white cell counts were elevated in 58.1% of cases; protein was increased in 18.6%; QAlb>Qlim(Alb) of the blood-CSF barrier was found in 29.3%; intrathecal immunoglobulin synthesis was detected in 17.1%, and 39.5% patients exhibited increased CSF pressures. A longer follow-up period was associated with better outcomes. There was no relationship between changes in CSF findings and outcome. CONCLUSION: The sensitivity of NMDA receptor antibody testing is higher in CSF compared to serum. Other CSF abnormalities are present in some patients with Anti-NMDAR-encephalitis, however these changes do not appear to affect prognosis.
Authors: Marc Dürr; Gunnar Nissen; Kurt-Wolfram Sühs; Philipp Schwenkenbecher; Christian Geis; Marius Ringelstein; Hans-Peter Hartung; Manuel A Friese; Max Kaufmann; Michael P Malter; Marie Madlener; Franziska S Thaler; Tania Kümpfel; Makbule Senel; Martin G Häusler; Hauke Schneider; Florian Then Bergh; Christoph Kellinghaus; Uwe K Zettl; Klaus-Peter Wandinger; Nico Melzer; Catharina C Gross; Peter Lange; Jens Dreyhaupt; Hayrettin Tumani; Frank Leypoldt; Jan Lewerenz Journal: Neurol Neuroimmunol Neuroinflamm Date: 2021-10-25
Authors: Francesc Graus; Maarten J Titulaer; Ramani Balu; Susanne Benseler; Christian G Bien; Tania Cellucci; Irene Cortese; Russell C Dale; Jeffrey M Gelfand; Michael Geschwind; Carol A Glaser; Jerome Honnorat; Romana Höftberger; Takahiro Iizuka; Sarosh R Irani; Eric Lancaster; Frank Leypoldt; Harald Prüss; Alexander Rae-Grant; Markus Reindl; Myrna R Rosenfeld; Kevin Rostásy; Albert Saiz; Arun Venkatesan; Angela Vincent; Klaus-Peter Wandinger; Patrick Waters; Josep Dalmau Journal: Lancet Neurol Date: 2016-02-20 Impact factor: 44.182