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| Item Type: | Article |
|---|---|
| Title: | Disruption of the leptomeningeal blood barrier in neuromyelitis optica spectrum disorder |
| Creators Name: | Asgari, N., Flanagan, E.P., Fujihara, K., Kim, H.J., Skejoe, H.P., Wuerfel, J., Kuroda, H., Kim, S.H., Maillart, E., Marignier, R., Pittock, S.J., 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 |
| 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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