Content area
Full text
http://crossmark.crossref.org/dialog/?doi=10.1007/s00401-017-1682-1&domain=pdf
Web End = Acta Neuropathol (2017) 133:597612 DOI 10.1007/s00401-017-1682-1
ORIGINAL PAPER
http://crossmark.crossref.org/dialog/?doi=10.1007/s00401-017-1682-1&domain=pdf
Web End = Pathogenic implications of cerebrospinal uid barrier pathology in neuromyelitis optica
Yong Guo1 Stephen D. Weigand2 Bogdan F. Popescu3 Vanda A. Lennon1,4,5,6 Joseph E. Parisi1,4
Sean J. Pittock1,6 Natalie E. Parks1 Stacey L. Clardy1 Charles L. Howe1,5,6,7http://orcid.org/0000-0002-3889-8739
Web End = Claudia F. Lucchinetti1,6
http://orcid.org/0000-0002-3889-8739
Web End = Abstract Pathogenic autoantibodies associated with neuromyelitis optica (NMO) induce disease by targeting aqua-porin-4 (AQP4) water channels enriched on astrocytic end-feet at bloodbrain interfaces. AQP4 is also expressed at cerebrospinal uid (CSF)brain interfaces, such as the pial glia limitans and the ependyma and at the choroid plexus bloodCSF barrier. However, little is known regarding pathology at these sites in NMO. Therefore, we evaluated AQP4 expression, microglial reactivity, and complement deposition at pial and ependymal surfaces and in the fourth ventricle choroid plexus in 23 autopsy cases with clinically
Electronic supplementary material The online version of this article (doi:http://dx.doi.org/10.1007/s00401-017-1682-1
Web End =10.1007/s00401-017-1682-1 ) contains supplementary material, which is available to authorized users.
* Charles L. Howe [email protected]
* Claudia F. Lucchinetti [email protected]
1 Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA
2 Department of Health Sciences Research, Mayo Clinic, Rochester, MN, USA
3 Department of Anatomy and Cell Biology, Cameco MS Neuroscience Research Center, University of Saskatchewan, Saskatoon, SK, Canada
4 Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA
5 Department of Immunology, Mayo Clinic, Rochester, MN, USA
6 Center for Multiple Sclerosis and Autoimmune Neurology, Mayo Clinic, Rochester, MN, USA
7 Department of Neuroscience, Mayo Clinic, Rochester, MN, USA
Received: 6 August 2016 / Revised: 12 January 2017 / Accepted: 25 January 2017 / Published online: 9 February 2017 The Author(s) 2017. This article is published with open access at Springerlink.com
and/or pathologically conrmed NMO or NMO spectrum disorder. These ndings were compared to ve cases with multiple sclerosis, ve cases of choroid plexus papilloma, and ve control cases without central nervous system disease. In the NMO cases, AQP4 immunoreactivity was reduced relative to control levels in the pia (91%; 21/23), ependyma (56%; 9/16), and choroid plexus epithelium (100%; 12/12). AQP4 immunoreactivity was normal in MS cases in these regions. Compared to MS, NMO cases also...