The pathology of an autoimmune astrocytopathy: lessons learned from neuromyelitis optica

Brain Pathology
Claudia F LucchinettiTatsuro Misu

Abstract

Neuromyelitis optica (NMO) is a disabling autoimmune astrocytopathy characterized by typically severe and recurrent attacks of optic neuritis and longitudinally extensive myelitis. Until recently, NMO was considered an acute aggressive variant of multiple sclerosis (MS), despite the fact that early studies postulated that NMO and MS may be two distinct diseases with a common clinical picture. With the discovery of a highly specific serum autoantibody (NMO-IgG), Lennon and colleagues provided the first unequivocal evidence distinguishing NMO from MS and other central nervous system (CNS) inflammatory demyelinating disorders. The target antigen of NMO-IgG was confirmed to be aquaporin-4 (AQP4), the most abundant water channel protein in the CNS, mainly expressed on astrocytic foot processes at the blood-brain barrier, subpial and subependymal regions. Pathological studies demonstrated that astrocytes were selectively targeted in NMO as evidenced by the extensive loss of immunoreactivities for the astrocytic proteins, AQP4 and glial fibrillary acidic protein (GFAP), as well as perivascular deposition of immunoglobulins and activation of complement even within lesions with a relative preservation of myelin. In support of these path...Continue Reading

References

May 1, 1979·Journal of Neurology, Neurosurgery, and Psychiatry·I V AllenR K Shillington
Jan 1, 1974·The Journal of Cell Biology·D M Landis, T S Reese
Jan 1, 1969·Folia Psychiatrica Et Neurologica Japonica·H Shibasaki, Y Kuroiwa
Aug 1, 1993·Annals of Neurology·R N MandlerM Kornfeld
Aug 1, 1993·Proceedings of the National Academy of Sciences of the United States of America·S NielsenP Agre
Jan 7, 1998·Trends in Neurosciences·J L RidetF H Gage
Apr 6, 1999·Journal of Neurovirology·J W OhE N Benveniste
Sep 25, 1999·Neurology·D M WingerchukB G Weinshenker
Apr 5, 2001·The New England Journal of Medicine·M J Walport
Jun 22, 2002·Brain : a Journal of Neurology·Claudia F LucchinettiHans Lassmann
Oct 23, 2002·Brain : a Journal of Neurology·Tatsuro MisuYasuto Itoyama
Mar 22, 2003·Lancet·Brian G Weinshenker
Jul 10, 2003·Lancet Neurology·Jun-ichi Kira
Oct 3, 2003·Trends in Neurosciences·Bruce RansomMaiken Nedergaard
Jun 26, 2004·Journal of Leukocyte Biology·Huan-Zhong Shi
Nov 13, 2004·Glia·Pamela A CarpentierStephen D Miller
Dec 30, 2004·Neurology·Jorge Correale, Marcela Fiol
Aug 10, 2005·The Journal of Experimental Medicine·Vanda A LennonShannon R Hinson
Oct 1, 2005·Multiple Sclerosis : Clinical and Laboratory Research·Alexandre Y PoppeAmit Bar-Or
Mar 15, 2006·Archives of Neurology·Sean J PittockBrian G Weinshenker
Mar 23, 2006·Annual Review of Immunology·Marc E Rothenberg, Simon P Hogan
Apr 14, 2006·American Journal of Transplantation : Official Journal of the American Society of Transplantation and the American Society of Transplant Surgeons·M D Pescovitz
May 24, 2006·Neurology·D M WingerchukB G Weinshenker
Jun 17, 2006·The Tohoku Journal of Experimental Medicine·Tatsuro MisuYasuto Itoyama
Jul 13, 2006·Archives of Neurology·Sean J PittockVanda A Lennon
Aug 30, 2006·Trends in Neurosciences·Nancy Ann OberheimMaiken Nedergaard
Feb 6, 2007·Trends in Immunology·Cinthia FarinaEdgar Meinl
Feb 7, 2007·Brain : a Journal of Neurology·Shanu F RoemerClaudia F Lucchinetti
Apr 24, 2007·Brain : a Journal of Neurology·Toshiyuki TakahashiYasuto Itoyama
Jul 25, 2007·Neuropathology : Official Journal of the Japanese Society of Neuropathology·Jun-ichi SatohHidehiko Konno
Aug 21, 2007·Lancet Neurology·Dean M WingerchukBrian G Weinshenker
Oct 16, 2007·Journal of the Neurological Sciences·Jacques De KeyserMarcus W Koch
Feb 1, 2008·Journal of Molecular Neuroscience : MN·Jennifer L Orthmann-MurphySteven S Scherer
Feb 15, 2008·The New England Journal of Medicine·Stephen L HauserUNKNOWN HERMES Trial Group

❮ Previous
Next ❯

Citations

Jul 17, 2014·Current Neurology and Neuroscience Reports·Eoin P Flanagan, Brian G Weinshenker
Jul 30, 2014·Nature Reviews. Neurology·Monika BradlJürgen Sandkühler
Oct 31, 2015·The International Journal of Neuroscience·Dian HeLan Chu
Jun 17, 2015·Neuropathology : Official Journal of the Japanese Society of Neuropathology·Izumi Kawachi, Masatoyo Nishizawa
Oct 20, 2015·Journal of Neurology·Laura AzzopardiAlasdair J Coles
Sep 9, 2015·Multiple Sclerosis and Related Disorders·Shin IidaHirofumi Kusaka
Mar 31, 2016·Neurology. Neuroimmunology and Neuroinflammation·Klaus Lehmann-HornScott S Zamvil
Apr 30, 2014·Annals of Neurology·Lucas SchirmerBernhard Hemmer
Jun 3, 2014·Acta Neuropathologica Communications·Christopher A RobinsonBogdan F G H Popescu
Oct 2, 2015·Journal of Neuroinflammation·Margaret E Walker-CaulfieldCharles L Howe
Jun 3, 2015·Clinical Neuroradiology·M Nichtweiß, S Weidauer
May 29, 2015·Journal of Neurology·Lin-Jie ZhangLi Yang
Apr 14, 2016·Experimental Neurology·Ian D Duncan, Abigail B Radcliff
Jun 23, 2015·Annals of the New York Academy of Sciences·Sean J Pittock, Claudia F Lucchinetti
Jun 21, 2015·Neurology·Dean M WingerchukUNKNOWN International Panel for NMO Diagnosis
Jan 1, 2014·International Scholarly Research Notices·Leandro Castañeyra-RuizAgustín Castañeyra-Perdomo
Mar 10, 2015·Lancet Neurology·Achim GassUNKNOWN MAGNIMS Study Group
Apr 26, 2016·European Journal of Neurology : the Official Journal of the European Federation of Neurological Societies·S-H KimH J Kim
Aug 2, 2016·Lancet Neurology·Todd A HardyBrian G Weinshenker
Oct 21, 2016·Neurotherapeutics : the Journal of the American Society for Experimental NeuroTherapeutics·Russell C Dale
Aug 28, 2014·Multiple Sclerosis : Clinical and Laboratory Research·Georgina ArrambideXavier Montalban
Aug 9, 2016·Multiple Sclerosis : Clinical and Laboratory Research·Rufu JiaLinpei Jia
Aug 20, 2016·Multiple Sclerosis : Clinical and Laboratory Research·Manjunath NetravathiParthasarathy Satishchandra
Mar 25, 2017·Journal of Neuropathology and Experimental Neurology·Diana L ThomasClayton Wiley
Apr 8, 2017·Mayo Clinic Proceedings·Brian G Weinshenker, Dean M Wingerchuk
Dec 4, 2016·Journal of Neurology, Neurosurgery, and Psychiatry·Nicholas L ZalewskiEoin P Flanagan
May 22, 2016·Multiple Sclerosis : Clinical and Laboratory Research·Samuel K LudwinJack P Antel
Oct 3, 2017·Rheumatology·John S TzartosSocrates J Tzartos
Jan 4, 2018·The International Journal of Neuroscience·Li-Min LiLi Yang
Dec 2, 2017·European Journal of Neurology : the Official Journal of the European Federation of Neurological Societies·Y LongX Chen
Jun 21, 2018·Journal of Neurology, Neurosurgery, and Psychiatry·Frederike C OertelAlexander U Brandt
Jan 4, 2019·Journal of Neuropathology and Experimental Neurology·John W Prineas, Sandra Lee
Jun 9, 2018·Reviews in the Neurosciences·Reza RahmanzadehMohammad Ali Sahraian

❮ Previous
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