Tian Song

Background and ObjectivesLongitudinally extensive transverse myelitis (LETM) is a core feature of neuromyelitis optica spectrum disorder (NMOSD), which leads to spinal cord atrophy. We aim to deter...

Introduction: Spinal cord infarction (SCI) is a rare but devastating myelopathy, characterized by a high disability rate and an unfavorable prognosis. It has often been underdiagnosed and misdiagnosed as idiopathic transverse myelitis (ITM). This study aimed to describe the clinical features, radiological biomarkers, treatments, and functional outcome of SCI, distinguishing it from ITM. Methods: A retrospective observational cohort study included patients who met the diagnostic criteria of SCI and ITM from January 2019 to October 2024. The clinical, radiological data, and diagnosis were recorded, and the functional outcomes were reached via telephone and face‐to‐face evaluations. Univariate analysis was used to differentiate the two groups. Results: During the study period, a total of 22 SCI patients with a median age of 53.0 years (interquartile range (IQR): 41.8 to 60.2) were enrolled. Thirteen patients underwent the diffusion‐weighted imaging (DWI) and the apparent diffusion coefficient (ADC) sequence, among whom 12 were confirmed as having definite SCI. Compared with ITM, SCI has the following characteristics. The time from onset to nadir in SCI is much shorter, mostly within 6 h ( p < 0.001). On sagittal MRI, SCI often manifests as linear lesions, while ITM tends to present as patchy and fusiform lesions ( p < 0.001). We have also defined a lesion characteristic of SCI based on T2‐weighted sequences, termed the “eccentric sign”. Moreover, patients with SCI generally have a poorer prognosis and higher dependence. Conclusions: SCI can be diagnosed and differentiated from ITM based on clinical features and radiological signs.

Objective: The objective of this study was to compare clinical features and prognosis of late‐onset neuromyelitis optica spectrum disorder ( LO ‐ NMOSD , onset age ≥60 years) with adult‐onset NMOSD ( AO ‐ NMOSD , onset age 18–59 years), and to provide insights for individualized management in elderly patients. Methods: Data from 748 patients with NMOSD (diagnosed according to the 2015 International Panel for NMO Diagnosis criteria) in the China National Registry of Neuro‐Inflammatory Diseases ( CNRID ) were analyzed. Patients were stratified into AO ‐ NMOSD (18–59 years, n = 617) and LO ‐ NMOSD (≥ 60 years, n = 131). Demographics, clinical manifestations, imaging, treatments, and outcomes were compared using appropriate statistical methods including Kaplan–Meier survival curves and Cox proportional hazards regression. Results: LO ‐ NMOSD showed distinct traits: a lower female predominance (76.34% vs 86.55%), higher transverse myelitis ( TM ) incidence at onset (57.36% vs 40.17%), elevated annualized relapse rate ( ARR ; 0.52 ± 0.03 vs 0.38 ± 0.01), and accelerated disability (median Expanded Disability Status Scale [ EDSS] 4.75 vs 3.0). TM ‐predominant relapses (39 of 45, 86.67% in LO vs 96 of 148, 64.86% in AO ) contributed significantly to disability. Kaplan–Meier analysis showed LO ‐ NMOSD had a higher risk of relapse (hazard ratio [ HR] = 1.932, 95% confidence interval [CI] = 1.427–2.615), disability ( HR = 3.192, 95% CI = 1.932–5.274) and reaching visual acuity ( VA ) ≤20 of 30 ( HR = 3.523, 95% CI = 1.585–7.828). Cox regression confirmed that onset age ≥60 years was an independent risk factor for relapse ( HR = 2.05, 95% CI = 1.60–2.59), disability ( HR = 3.16, 95% CI = 2.14–4.62), and reaching VA ≤20 of 30 ( HR 3.26, 95% CI = 1.83–5.48). Interpretation: LO‐NMOSD is characterized by myelitis‐predominance with recurrent spinal cord involvement, high risk of relapses, and severe disability. It thus underscores the need for heightened clinical attention, with rigorous monitoring that balance safety and efficacy for elderly patients with NMOSD. ANN NEUROL 2026;99:1139–1151

Objective: Glial fibrillary acidic protein astrocytopathy (GFAP‐A) is a recently defined nosological form belonging to the class of autoimmune inflammatory disorders affecting the central nervous system (CNS). Here, we report the clinical and MRI characteristics, treatment, and prognosis of a GFAP‐A cohort from two centers in China.Methods: We retrospectively analyzed the data from 38 adult patients with positive GFAP antibodies and diagnosed as GFAP‐A between June 2019 and September 2024. Clinical features, semiquantitative antibody test results, MRI features, treatment approaches, and prognosis were collected.Results: Among the 38 patients, 24 were male, and the median age at disease onset was 49.5 years. The clinical phenotype included encephalomyelitis (28.9%), myelitis (23.7%), encephalitis (18.4%), meningoencephalomyelitis (18.4%), meningitis/spinal meningitis (7.9%), and peripheral neuropathy (2.6%). In enhanced MRI images, 4 (10.5%) of the patients showed enhancement of the cerebral meninges, 2 (5.3%) had enhancement of the ependyma, and 5 (13.2%) had enhancement of the spinal cord pia mater. 77.1% of the patients responded to the glucocorticoid treatment, while 65.8% had a monophasic course. Spearman correlation analysis showed that CSF‐specific oligoclonal bands were significantly correlated with 1‐year relapse (CI = 0.527, p = 0.003).Interpretation: The clinical manifestations of GFAP‐A are highly diverse, encompassing encephalitis, myelitis, and meningitis, including spinal meningitis. The enhancement of the spinal pia mater and ependyma on MRI was confirmed. Most patients exhibit a positive response to glucocorticoid therapy. The presence of CSF‐specific oligoclonal bands could potentially serve as an indicator for predicting recurrence.

Background: Optic neuritis (ON) is an important clinical manifestation of neuromyelitis optic spectrum disease (NMOSD). Myelin oligodendrocyte glycoprotein (MOG) antibody-related and aquaporin 4 (AQP4) antibody-related ON show different disease patterns. The aim of this study was to explore the differences in structure and function of the visual pathway in patients with ON associated with MOG and AQP4 antibodies.Methods: In this prospective study, we recruited 52 subjects at Beijing Tiantan Hospital, including 11 with MOG Ig+ ON (MOG-ON), 13 with AQP4 Ig+ ON (AQP4-ON), and 28 healthy controls (HCs). Fractional anisotropy (FA), mean diffusivity (MD), axial diffusivity (AD), and radial diffusivity (RD) of optic radiation (OR), primary visual cortex volume (V1), brain volume, and visual acuity (VA) were compared among groups. A multiple linear regression was used to explore associations between VA and predicted factors. In addition, we used optical coherence tomography (OCT) to examine thickness of the peripapillary retinal nerve fiber layer (pRNFL) and retinal ganglion cell complex (GCC) in a separate cohort consisting of 15 patients with ON (8 MOG-ON and 7 AQP4-ON) and 28 HCs.Results: Diffusion tensor imaging showed that the FA of OR was lower than controls in patients with AQP4-ON (p = 0.001) but not those with MOG-ON (p = 0.329) and was significantly different between the latter two groups (p = 0.005), while V1 was similar in patients with MOG-ON and AQP4-ON (p = 0.122), but was lower than controls in AQP4-ON (p = 0.002) but not those with MOG-ON (p = 0.210). The VA outcomes were better in MOG-ON than AQP4-ON, and linear regression analysis revealed that VA in MOG-ON and AQP4-ON was both predicted by the FA of OR (standard β = −0.467 and −0.521, p = 0.036 and 0.034). Both patients of MOG-ON and AQP4-ON showed neuroaxonal damage in the form of pRNFL and GCC thinning but showed no statistically significant difference (p = 0.556, 0.817).Conclusion: The structural integrity of OR in patients with MOG-ON, which is different from the imaging manifestations of AQP4-ON, may be a reason for the better visual outcomes of patients with MOG-ON.

Background Glucocorticoid (GC) is the first-line therapy in acute attacks of multiple sclerosis (MS), but its efficacy is individually variable and may be associated with glucocorticoid receptor (GR) gene. Objective To establish the association between GR gene sequence and clinical GC sensitivity in Chinese MS patients. And to investigate the expression differences of serum GRα and FK506 binding protein 5 (FKBP5) in GC responders and non-responders. Materials and methods Coding exons 2–9 of the GR gene from 97 MS patients were sequenced. We performed ELISA to detect serum GRα and FKBP5 before the GC impulse therapy in patients with different GC sensitivities (according to the EDSS changes before and after the GC medication). Results Seven new mutations were located in exon 2, but the presence or absence of mutations was not associated with the response to GC therapy (P = 0.416). The GC-sensitive patients had higher GRα (P = 0.011) but lower FKBP5 (P = 0.025) levels in the serum. Conclusions The GR mutations detected in our study were not associated with the response to GC in Chinese MS patients. Higher GRα and lower FKBP5 levels in the serum might predict the response to GC, which may provide potential therapeutic target for GC-resistant patients with acute MS attack.

Deep venous thrombosis (DVT) is a severe complication in longitudinally extensive transverse myelitis (LETM) patients. It may interfere with LETM treatment and delay the recovery of the spinal dysfunction. However, there is less data about the prevalence and risk factors of DVT in patients with LETM. We analyzed data retrospectively to ascertain the prevalence of DVT and the clinical risk factors for DVT. Clinical data on 255 LETM patients were collected from medical records. All patients were performed color Doppler ultrasound(US) to screen DVT in both lower extremities when admitted. Clinical characteristics of LETM patients with DVT were compared with those without DVT using corresponding statistical methods. Multivariate logistic regression was performed to identify risk factors related to DVT. DVT were found in 11.8% patients with LETM. Univariate analysis showed that age, muscle force and elevated baseline D-dimer were risk factors for DVT. After multivariate logistic regression, age, dyslipidemia, segments of lesions, and elevated baseline D-dimer remained significant independent risk factors. DVT is common in patients with LETM and related to patient’s age, dyslipidemia, segments of lesions, and elevated baseline D-dimer. Early recognition of DVT and thrombosis prophylaxis are appropriate in patients with LETM.