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
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.
Neurol Neuroimmunol Neuroinflamm. 2017 Apr 21;4(4):e343. doi: 10.1212/NXI.0000000000000343. eCollection 2017 Jul.
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).
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.
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.
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.
描述水通道蛋白4免疫球蛋白G（AQP4-IgG）阳性视神经脊髓炎谱系障碍（NMOSD）患者中，磁共振成像（MRI）钆增强病变所反映的软脑膜血脑屏障损害情况。
从5个中心收集了11例有软脑膜强化（LME）的AQP4-IgG阳性NMOSD患者的回顾性病例系列。对临床细节不知情的外部神经放射科医生评估MRI。
11例患者在对比剂增强后的T1加权像和液体衰减反转恢复像上显示LME，这是软脑膜血脑屏障破坏以及对比剂短暂漏入蛛网膜下腔的征象，其中6例在脑部，6例在脊髓。LME的形态为线性或广泛分布，5例伴有室管膜周围强化，所有病例均伴有脑实质内强化。脊髓LME的位置与脑实质内对比剂强化相邻，累及的椎体节段中位数为12个（范围5 - 17个）。在MRI显示LME时，所有患者均有临床发作，如脑病（36%）和/或脊髓病（70%），症状发作与LME之间的间隔中位数为12天（范围为2 - 30天）。LME与最初的最后区发作（44%）、全身感染迹象（33%）或脑脊液中的AQP4-IgG（22%）相关，随后出现临床进展。5例在初始临床表现时发现LME，6例在临床复发时发现LME，从而确诊为NMOSD。
本研究表明，在复发期间，AQP4-IgG阳性NMOSD患者的软脑膜血脑屏障改变可能伴有脑实质血脑屏障破坏。
