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Disruption of the leptomeningeal blood barrier in neuromyelitis optica spectrum disorder

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Item Type:Article
Title:Disruption of the leptomeningeal blood barrier in neuromyelitis optica spectrum disorder
Creators Name:Asgari, N. and Flanagan, E.P. and Fujihara, K. and Kim, H.J. and Skejoe, H.P. and Wuerfel, J. and Kuroda, H. and Kim, S.H. and Maillart, E. and Marignier, R. and Pittock, S.J. and Paul, F. and Weinshenker, B.G.
Abstract:OBJECTIVE: To describe leptomeningeal blood-barrier impairment reflected by MRI gadolinium-enhanced lesions in patients with aquaporin-4 immunoglobulin G (AQP4-IgG)-positive neuromyelitis optica spectrum disorder (NMOSD). METHODS: A retrospective case series of 11 AQP4-IgG-positive NMOSD patients with leptomeningeal enhancement (LME) were collected from 5 centers. External neuroradiologists, blinded to the clinical details, evaluated MRIs. RESULTS: LME was demonstrated on postcontrast T1-weighted and fluid-attenuated inversion recovery images as a sign of leptomeningeal blood-barrier disruption and transient leakage of contrast agent into the subarachnoid space in 11 patients, 6 in the brain and 6 in the spinal cord. The patterns of LME were linear or extensive and were accompanied by periependymal enhancement in 5 cases and intraparenchymal enhancement in all cases. The location of LME in the spinal cord was adjacent to intraparenchymal contrast enhancement with involvement of a median number of 12 (range 5-17) vertebral segments. At the time of LME on MRI, all patients had a clinical attack such as encephalopathy (36%) and/or myelopathy (70%) with median interval between symptom onset and LME of 12 days (range 2-30). LME occurred in association with an initial area postrema attack (44%), signs of systemic infection (33%), or AQP4-IgG in CSF (22%) followed by clinical progression. LME was found at initial clinical presentation in 5 cases and at clinical relapses leading to a diagnosis of NMOSD in 6 cases. CONCLUSION: This study suggests that altered leptomeningeal blood barrier may be accompanied by intraparenchymal blood-brain barrier breakdown in patients with AQP4-IgG-positive NMOSD during relapses.
Source:Neurology Neuroimmunology & Neuroinflammation
ISSN:2332-7812
Publisher:American Academy of Neurology (U.S.A.)
Volume:4
Number:4
Page Range:e343
Date:July 2017
Official Publication:https://doi.org/10.1212/NXI.0000000000000343
PubMed:View item in PubMed

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