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Neurol Neuroimmunol Neuroinflamm. 2017 Apr 21;4(4):e343. doi: 10.1212/NXI.0000000000000343. eCollection 2017 Jul.

Disruption of the leptomeningeal blood barrier in neuromyelitis optica spectrum disorder.

Author information

1
Department of Neurobiology (N.A.), Institute of Molecular Medicine, University of Southern Denmark; Department of Neurology (E.P.F., S.J.P., B.G.W.), Mayo Clinic, Rochester, MN; Department of Multiple Sclerosis Therapeutics (K.F.), Fukushima Medical University School of Medicine; Multiple Sclerosis and Neuromyelitis Optica Center (K.F.), Southern TOHOKU Research Institute for Neuroscience, Koriyama, Japan; Department of Neurology (H.J.K., S.H.K.), Research Institute and Hospital of National Cancer Center, Goyang, Korea; Department of Radiology (H.P.S.), Aleris-Hamlet Hospital, Copenhagen, Denmark; Medical Image Analysis Center Basel (J.W.); Department of Biomedical Engineering (J.W.), University Basel, Switzerland; NeuroCure Clinical Research Center and Clinical and Experimental Multiple Sclerosis Research Center (J.W., F.P.), Department of Neurology, Charité Universitätsmedizin Berlin; Experimental and Clinical Research Center (J.W., F.P.), Max Delbrueck Center for Molecular Medicine and Charité Universitätsmedizin Berlin, Germany; Department of Neurology (H.K.), Tohoku University Graduate School of Medicine, Sendai, Japan; Department of Neurology (E.M.), Hôpital Pitié-Salpêtrière, APHP, Paris, France; Service de Neurologie A and Eugène Devic EDMUS Foundation against Multiple Sclerosis (R.M.), Observatoire Français de la Sclérose en Plaques (OFSEP), Hôpital Neurologique Pierre Wertheimer, Hospices Civils de Lyon, Bron; and Lyon Neurosciences Research Center (R.M.), FLUID team, Inserm U 1028/CNRS 5292, France.

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.

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