The role of anti-aquaporin 4 antibody in the conversion of acute brainstem syndrome to neuromyelitis optica

Cheng, Chen; Jiang, Ying; Lu, Xiaodong; Gu, Fu; Kang, Zhuang; Dai, Yongqiang; Lu, Zhengqi; Hu, Xueqiang

Abstract

Background: Acute brainstem syndrome (ABS) may herald multiple sclerosis (MS), neuromyelitis optica (NMO), or occur as an isolated syndrome. The aquaporin 4 (AQP4)-specific serum autoantibody, NMO-IgG, is a biomarker for NMO. However, the role of anti-AQP4 antibody in the conversion of ABS to NMO is unclear.

Methods: Thirty-one patients with first-event ABS were divided into two groups according to the presence of anti-AQP4 antibodies, their clinical features and outcomes were retrospectively analyzed.

Results: Fourteen of 31 patients (45.16 %) were seropositive for NMO-IgG. The 71.43 % of anti-AQP4 (+) ABS patients converted to NMO, while only 11.76 % of anti-AQP4 (-) ABS patients progressed to NMO. Anti-AQP4 (+) ABS patients demonstrated a higher IgG index (0.68 ± 0.43 vs 0.42 ± 0.13, p < 0.01) and Kurtzke Expanded Disability Status Scale (4.64 ± 0.93 vs 2.56 ± 0.81, p < 0.01) than anti-AQP4 (-) ABS patients. Area postrema clinical brainstem symptoms occurred more frequently in anti-AQP4 (+) ABS patients than those in anti-AQP4 (-) ABS patients (71.43 % vs 17.65 %, p = 0.004). In examination of magnetic resonance imaging (MRI), the 78.57 % of anti-AQP4 (+) ABS patients had medulla-predominant involvements in the sagittal view and dorsal-predominant involvements in the axial view.

Conclusions: ABS represents an inaugural or limited form of NMO in a high proportion of anti-AQP4 (+) patients.

Citation

Cheng, C., Jiang, Y., Lu, X., Gu, F., Kang, Z., Dai, Y., Lu, Z., & Hu, X. (in press). The role of anti-aquaporin 4 antibody in the conversion of acute brainstem syndrome to neuromyelitis optica. BMC Neurology, 16(1), https://doi.org/10.1186/s12883-016-0721-1

• Journal Article Type: Article
• Acceptance Date: Oct 16, 2016
• Online Publication Date: Oct 21, 2016
• Deposit Date: Oct 18, 2017
• Publicly Available Date: Oct 18, 2017
• Journal: BMC Neurology
• Electronic ISSN: 1471-2377
• Publisher: Springer Verlag
• Peer Reviewed: Peer Reviewed
• Volume: 16
• Issue: 1
• DOI: https://doi.org/10.1186/s12883-016-0721-1
• Keywords: Acute brainstem syndrome; Anti-aquaporin 4 antibody; Neuromyelitis optica; Magnetic resonance imaging
• Public URL: https://nottingham-repository.worktribe.com/output/822188
• Publisher URL: https://doi.org/10.1186/s12883-016-0721-1
• Contract Date: Oct 18, 2017