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Background and Purpose: Neuromyelitis optica spectrum disorder (NMOSD) is a severe neurological inflammatory disease mainly caused by pathogenic aquaporin-4 antibodies (AQP4-IgG). The safety and efficacy of the neonatal Fc receptor antagonist batoclimab addition to conventional intravenous methylprednisolone pulse (IVMP) therapy in patients with NMOSD acute attacks was assessed., Methods: In an open-label, dose-escalation phase 1b study, NMOSD patients with acute myelitis and/or optic neuritis received four doses of weekly subcutaneous injections of either 340 mg or 680 mg batoclimab with concurrent IVMP and were followed up for 27 weeks. The primary end-points were safety and tolerability. Secondary end-points included pharmacodynamics and efficacy, with key efficacy assessment at week 4., Results: In total nine NMOSD patients were enrolled, including two and seven in the 340 and 680 mg groups. Five patients had acute myelitis, while the remaining four had unilateral optic neuritis. Batoclimab add-on therapy had an overall good safety profile without serious adverse events. In the 680 mg group, mean immunoglobulin G (IgG) reached its maximum reduction at the last dose (day 22). In the meantime, AQP4-IgG was undetectable in six of seven subjects whose baseline AQP4-IgG titers ranged from 1:32 to 1:320. Expanded Disability Status Scale score was reduced by 1.3 ± 0.4 at week 4 (2.7 ± 1.3) compared with baseline (4.0 ± 1.0)., Conclusions: Batoclimab add-on therapy to IVMP is safe and tolerated in patients with NMOSD. Preliminary evidence suggests a beneficial neurological effect. A randomized controlled trial would be needed to prove the efficacy., (© 2022 European Academy of Neurology.)

Background: Clinical data on male neuromyelitis optica spectrum (NMO) patients remain limited to date., Objective: To analyze clinical characteristics, therapeutic responses, and outcomes in Chinese male NMOSD patients., Methods: We retrospectively assessed clinical, demographic, treatment, and outcome data of male patients with NMOSD., Results: We identified 52 male patients among 471 NMOSD patients. Male patients had more frequent optic neuritis (ON) attacks and less frequent myelitis than female patients. No-remission rates of high-dose intravenous steroid therapy were higher in male patients than in female patients., Conclusion: Male NMOSD patients had different clinical characteristics and worse response to high-dose intravenous steroid therapy than female patients., (© 2020. Fondazione Società Italiana di Neurologia.)

Introduction: Previous studies have shown that serum uric acid (UA) modulates outcomes of neurological diseases, although little is known about cerebrospinal fluid (CSF) UA levels in neuromyelitis optica spectrum disorders (NMOSDs)., Methods: Cerebrospinal fluid and serum UA levels were measured in samples from 68 patients, including NMOSDs during relapse ( n  = 38) and controls with noninflammatory and non-neurodegenerative diseases (CTLs, n  = 30). Correlation analysis was performed between CSF UA and clinical characteristics, serum UA, and blood-brain barrier integrity in NMOSDs., Results: Cerebrospinal fluid UA levels in NMOSDs were significantly higher than in CTLs ( p  =   .002), while serum UA differences between NMOSDs and CTLs were not statistically significant. In NMOSDs, CSF UA levels were significantly higher in patients with an impaired blood-brain barrier than in patients with an intact one ( p  <   .001), and significantly higher in longer disease duration than in shorter disease duration patients ( p  =   .002). CSF UA levels were also significantly higher in active patients upon MRI than in inactive patients ( p  <   .001), and significantly higher in patients with brain lesions than without brain lesions ( p  =   .024). CSF UA was significantly associated with the serum UA levels ( r  = .454, p  =   .002), disease duration ( r  = .383, p  = .018), and blood-brain barrier index ( r  = .805, p  <   .001), but did not correlate with age, gender, annualized relapse rate, duration, or severity of NMOSD. Multiple regression analysis demonstrated that CSF UA was independent of the blood-brain barrier index (β = .765, p  <   .001) and serum UA levels (β = .01, p  =   .019) in NMOSDs., Conclusions: Cerebrospinal fluid UA levels were elevated in NMOSD patients during relapse, and were likely modified by serum UA levels and blood-brain barrier integrity.

Background: Early stage neuromyelitis optica spectrum disorders (NMOSD) with non-opticospinal manifestations as initial symptoms are easily misdiagnosed; however, data on the full symptom profile are limited. Moreover, the clinical characteristics and long-term outcomes of these patients remain unknown. We sought to analyze the clinical characteristics, imaging features, and long-term outcomes of NMOSD with non-opticospinal manifestations as initial symptoms.Methods: We retrospectively included relevant patients from our center. Clinical, demographic, magnetic resonance imaging, treatment, and outcome data were compared according to the non-opticospinal vs. opticospinal initial symptoms.Results: We identified 43 (9.13 %) patients with non-opticospinal initial symptoms among 471 patients with NMOSD. Of these, 88.37 % developed optic neuritis/myelitis during an average follow-up period of 6.33 years. All the non-opticospinal symptoms were brain/brainstem symptoms. Most of the symptoms and associated brain lesions were reversible. These patients had a younger onset age (P < 0.001), lower serum aquaporin-4 (AQP4) antibody titers (P = 0.030), and a lower Expanded Disability Status Scale (EDSS) score at onset (P < 0.001) and follow-up (P = 0.041) than NMOSD patients with opticospinal initial symptoms. In addition, EDSS scores reached 3.0 (indicating moderate disability) later than in patients with opticospinal initial symptoms (P = 0.028).Conclusions: Patients with NMOSD with non-opticospinal initial symptoms have a younger onset age, lower serum AQP4 antibody titers, and better clinical outcomes. [ABSTRACT FROM AUTHOR]

Introduction: The aim was to characterize the optical coherence tomography (OCT) angiography measures in patients with multiple sclerosis (MS) and neuromyelitis optica spectrum disorder (NMOSD) and to evaluate their disease discrimination capacity. Methods: Patients with MS (n = 83) and AQP4‐IgG‐seropositive NMOSD (n = 91) with or without a history of optic neuritis, together with healthy controls (n = 34), were imaged. The main outcome measures were peripapillary retinal nerve fiber layer (pRNFL) thickness, macular ganglion cell‐inner plexiform layer (GC‐IPL) thickness, macular vessel density (VD), and perfusion density (PD) in the superficial capillary plexus. Diagnostic accuracy was assessed using the area under the receiver operating characteristics curve. Results: Compared with patients with MS, those with NMOSD had a significantly smaller average thickness of the pRNFL and GC‐IPL (80.0 [59.0; 95.8] μm versus 92.0 [80.2; 101] μm, p <.001; 68.0 [56.0; 81.0] μm, versus 74.5 [64.2; 81.0] μm, p <.001) and significantly smaller whole VD and PD areas (15.6 [12.6; 17.0] mm−1 versus 16.7 [14.8; 17.7] mm−1, p <.001; 0.38 [0.31; 0.42] mm−1 versus 0.40 [0.37; 0.43] mm−1, p <.01). The combination of structural parameters (average thickness of the pRNFL and GC‐IPL) with microvascular parameters (temporal‐inner quadrant of VD, temporal‐inner, nasal‐inferior, and nasal‐outer quadrant of PD) was revealed to have a good diagnostic capability for discriminating between NMOSD and MS. Conclusions: OCT angiography reveals different structural and microvascular retinal changes in MS and AQP4‐IgG‐seropositive NMOSD. These combined structural and microvascular parameters might be promising biomarkers for disease diagnosis. [ABSTRACT FROM AUTHOR]