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.
GLOSSARY
- AP=
- area postrema syndrome/lesion;
- AQP4=
- aquaporin-4;
- BBB=
- blood-brain barrier;
- CS=
- cerebral syndromes;
- DWI=
- diffusion-weighted imaging;
- FLAIR=
- fluid-attenuated inversion recovery;
- Gd=
- gadolinium;
- IVMP=
- IV methylprednisolone;
- LETM=
- longitudinally extensive transverse myelitis;
- LME=
- leptomeningeal enhancement;
- MNL=
- mononuclear leukocyte;
- NMOSD=
- neuromyelitis optica spectrum disorder;
- ON=
- optic neuritis;
- PLEX=
- plasma exchange;
- PMNL=
- polymorphonuclear leukocyte;
- STIR=
- short tau inversion recovery;
- T1W=
- T1 weighted
Footnotes
↵* These authors contributed equally to this work as co-last authors.
Funding information and disclosures are provided at the end of the article. Go to Neurology.org/nn for full disclosure forms. The Article Processing Charge was funded by the authors.
Supplemental data at Neurology.org/nn
- Received January 2, 2017.
- Accepted in final form March 2, 2017.
- Copyright © 2017 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.
This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives License 4.0 (CC BY-NC-ND), which permits downloading and sharing the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal.
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