Disruption of the leptomeningeal blood barrier in neuromyelitis optica spectrum disorder

Nasrin Asgari*, Eoin P. Flanagan, Kazuo Fujihara, Ho Jin Kim, Hanne P. Skejoe, Jens Wuerfel, Hiroshi Kuroda, Su Hyun Kim, Elisabeth Maillart, Romain Marignier, Sean J. Pittock, Friedemann Paul, Brian G. Weinshenker

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Objective: To describe leptomeningeal blood-barrier impairment reflected by MRI gadoliniumenhanced 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 LMEwere 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). LMEoccurred 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 patientswith AQP4-IgG-positive NMOSD during relapses.

OriginalsprogEngelsk
Artikelnummere343
TidsskriftNeurology: Neuroimmunology and NeuroInflammation
Vol/bind4
Udgave nummer4
Antal sider9
ISSN2332-7812
DOI
StatusUdgivet - 2017

Fingeraftryk

Neuromyelitis Optica
Area Postrema
Contrast Media

Citer dette

Asgari, Nasrin ; Flanagan, Eoin P. ; Fujihara, Kazuo ; Kim, Ho Jin ; Skejoe, Hanne P. ; Wuerfel, Jens ; Kuroda, Hiroshi ; Kim, Su Hyun ; Maillart, Elisabeth ; Marignier, Romain ; Pittock, Sean J. ; Paul, Friedemann ; Weinshenker, Brian G. / Disruption of the leptomeningeal blood barrier in neuromyelitis optica spectrum disorder. I: Neurology: Neuroimmunology and NeuroInflammation. 2017 ; Bind 4, Nr. 4.
@article{37011e10769c48dd81c255d2e315881b,
title = "Disruption of the leptomeningeal blood barrier in neuromyelitis optica spectrum disorder",
abstract = "Objective: To describe leptomeningeal blood-barrier impairment reflected by MRI gadoliniumenhanced 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 LMEwere 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). LMEoccurred 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 patientswith AQP4-IgG-positive NMOSD during relapses.",
author = "Nasrin Asgari and Flanagan, {Eoin P.} and Kazuo Fujihara and Kim, {Ho Jin} and Skejoe, {Hanne P.} and Jens Wuerfel and Hiroshi Kuroda and Kim, {Su Hyun} and Elisabeth Maillart and Romain Marignier and Pittock, {Sean J.} and Friedemann Paul and Weinshenker, {Brian G.}",
year = "2017",
doi = "10.1212/NXI.0000000000000343",
language = "English",
volume = "4",
journal = "Neurology: Neuroimmunology & Neuroinflammation",
issn = "2332-7812",
publisher = "Wolters Kluwer Health",
number = "4",

}

Asgari, N, Flanagan, EP, Fujihara, K, Kim, HJ, Skejoe, HP, Wuerfel, J, Kuroda, H, Kim, SH, Maillart, E, Marignier, R, Pittock, SJ, Paul, F & Weinshenker, BG 2017, 'Disruption of the leptomeningeal blood barrier in neuromyelitis optica spectrum disorder', Neurology: Neuroimmunology and NeuroInflammation, bind 4, nr. 4, e343. https://doi.org/10.1212/NXI.0000000000000343

Disruption of the leptomeningeal blood barrier in neuromyelitis optica spectrum disorder. / Asgari, Nasrin; Flanagan, Eoin P.; Fujihara, Kazuo; Kim, Ho Jin; Skejoe, Hanne P.; Wuerfel, Jens; Kuroda, Hiroshi; Kim, Su Hyun; Maillart, Elisabeth; Marignier, Romain; Pittock, Sean J.; Paul, Friedemann; Weinshenker, Brian G.

I: Neurology: Neuroimmunology and NeuroInflammation, Bind 4, Nr. 4, e343, 2017.

Publikation: Bidrag til tidsskriftTidsskriftartikelForskningpeer review

TY - JOUR

T1 - Disruption of the leptomeningeal blood barrier in neuromyelitis optica spectrum disorder

AU - Asgari, Nasrin

AU - Flanagan, Eoin P.

AU - Fujihara, Kazuo

AU - Kim, Ho Jin

AU - Skejoe, Hanne P.

AU - Wuerfel, Jens

AU - Kuroda, Hiroshi

AU - Kim, Su Hyun

AU - Maillart, Elisabeth

AU - Marignier, Romain

AU - Pittock, Sean J.

AU - Paul, Friedemann

AU - Weinshenker, Brian G.

PY - 2017

Y1 - 2017

N2 - Objective: To describe leptomeningeal blood-barrier impairment reflected by MRI gadoliniumenhanced 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 LMEwere 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). LMEoccurred 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 patientswith AQP4-IgG-positive NMOSD during relapses.

AB - Objective: To describe leptomeningeal blood-barrier impairment reflected by MRI gadoliniumenhanced 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 LMEwere 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). LMEoccurred 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 patientswith AQP4-IgG-positive NMOSD during relapses.

U2 - 10.1212/NXI.0000000000000343

DO - 10.1212/NXI.0000000000000343

M3 - Journal article

VL - 4

JO - Neurology: Neuroimmunology & Neuroinflammation

JF - Neurology: Neuroimmunology & Neuroinflammation

SN - 2332-7812

IS - 4

M1 - e343

ER -