A nine-year-old boy manifested with headache, progressive mild cognitive decline and hemiparesis, but without clinical epileptic seizures (with abnormal EEG waves). Brain magnetic resonance imaging (MRI) showed bilateral cortical lesions mainly on the right hemisphere, and new lesions developed in frontal, parietal, occipital and temporal lobes around the old lesions presenting as a lace-like or ring-like enhancement in T1 with contrast over a disease course of five years. A suspected diagnosis of primary angiitis of the central nervous system was initially considered. Treated with high-dose corticosteroids, intravenous immunoglobulins and monthly pulse cyclophosphamide, his symptoms worsened with the intracranial lesion progression. Brain biopsy of the right frontal lobe was performed nearly five years after onset; prominent neuronal loss, a microglial nodule, as well as parenchymal and perivascular lymphocytic infiltrate within the cortex were found, which coincided with RE pathology changes. Encouragingly, after a regimen of rituximab, lesions on the follow-up brain MRI tended to be stable. Apparently, it was immune-mediated, but did not strictly fit any known disease entity, although it was similar to RE. We summarize this unique case, including clinical characteristics, imaging and pathology findings. We also discuss the diagnosis and treatment, focusing on comparison to RE as well as other possible neurological diseases.

Neuronal intranuclear inclusion disease (NIID) is a rare and slowly progressive neurodegenerative disease characterized by the presence of eosinophilic neuronal intranuclear inclusions. The clinical manifestations of NIID are diverse, and the most common initial feature in cases of sporadic NIID is dementia. Herein, we report an adult female with keratitis as the initial presentation with subsequent bilateral limb tremor, gait disturbances, overemotional behavior, sweating and constipation. Diffusion-weighted imaging (DWI) showed hyperintensity in the bilateral fronto-parieto-occipital corticomedullary junction. Skin biopsy specimens revealed eosinophilic hyaline intranuclear inclusions in fibroblast cells, sweat gland cells and adipose cells. In vivo confocal microscopy of the cornea indicated the absence of corneal nerves in both affected eyes. The patient’s diagnosis of NIID was based on the presence of intranuclear inclusions in biopsied skin and the characteristic high-intensity signal in the corticomedullary junction obtained with DWI. This case report emphasizes that the clinical heterogeneity of NIID and an examination of the corneal nerves may offer valuable clues to its early diagnosis in some patients.

Objectives: To investigate the association between triglyceride-glucose-body mass index (TyG-BMI) and the 1-year stroke recurrence risk in patients with acute ischemic stroke (AIS) and hypertension. Methods: In this retrospective, multicenter cohort study, multivariable Cox proportional hazards models were constructed, and curve fitting and subgroup analyses were performed to evaluate the aforementioned association. TyG-BMI was analyzed as a continuous variable and in quartiles (Q1–Q4). Sex-specific stratified analyses were performed to explore potential effect modifications. Results: Among 1,620 enrolled patients (39.6% women; mean age 65.2 ± 11.5 years), elevated TyG-BMI was significantly associated with increased 1-year stroke recurrence risk after adjusting for potential confounding factors (hazard ratio [HR]=1.06, 95% confidence interval [CI]: 1.01–1.11, P = 0.032). This association was particularly prominent in women (HR = 1.18, 95%CI: 1.07–1.29, P<0.001). Women in the Q3 and Q4 TyG-BMI groups had significantly higher 1-year stroke recurrence risks (Q3: HR=8.81 95%CI: 2.22–34.97, P = 0.002; Q4: HR=5.79 95%CI: 1.49–22.56, P = 0.011) compared with those in the Q1 group. No significant association was observed in men (HR = 1.01, 95%CI: 0.94–1.08, P = 0.830). Segmented linear regression and curve fitting revealed a significant sex-specific nonlinear relationship between TyG-BMI and 1-year stroke recurrence risk. For women when TyG-BMI was below 221.97, each unit increase was associated with a more pronounced increase in the risk of 1-year stroke recurrence compared to patients above this threshold (adjusted HR = 1.04, 95% CI 1.01–1.07, P = 0.005). Conclusion: Elevated TyG-BMI is independently associated with a higher risk of 1-year stroke recurrence in patients with AIS and hypertension. A significant association was identified only in women, specifically among those with a TyG-BMI below 221.97, below which each unit increase in TyG-BMI was associated with a significantly greater risk of 1-year stroke recurrence compared to those above this threshold.

Introduction: Neuromyelitis optica spectrum disorder (NMOSD) and myelin oligodendrocyte glycoprotein antibody–associated disease (MOGAD) are among the leading causes of optic neuritis. This study aimed to examine differences in macular retinal structure and microvascular characteristics between affected and unaffected eyes in individuals with NMOSD and MOGAD. Method: This cross-sectional study enrolled both eyes of patients diagnosed with optic neuritis (ON)secondary to NMOSD (22 patients: 36 NMOSD-ON eyes and 8 NMOSD-NON eyes), MOGAD (23 patients: 34 MOG-ON eyes and 12 MOG-NON eyes), and 20 age- and sex-matched healthy controls (HCs, 40 eyes) recruited from the First Affiliated Hospital of Northwest University (Xi’an No.1 Hospital) between February 2023 and January 2025. Microvascular density (MVD), vascular density (VD), blood flow area (BFA), and macular ganglion cell–inner plexiform layer (GCIPL) thickness were measured and analyzed. Result: Both NMOSD-ON and MOG-ON eyes showed significant reductions in MVD of radial peripapillary capillary plexus (RPCP); MVD, VD, and BFA of superficial vascular complex (SVC); BFA of deep vascular complex (DVC); and GCIPL thickness compared with HCs ( P < 0.001). Compared with MOG-ON eyes, NMOSD-ON eyes demonstrated a greater reduction in BFA of choriocapillaris (CC) ( P = 0.040). In MOG-NON eyes, the MVD of RPCP; the MVD, VD, and BFA of SVC; the BFA of DVC; and the GCIPL thickness were significantly lower than those in HCs, but remained higher than in MOG-ON eyes. In NMOSD-ON eyes, all MVD and VD parameters, SVC BFA, and GCIPL thickness were inversely correlated with best-corrected visual acuity (BCVA) and Expanded Disability Status Scale (EDSS) scores ( P < 0.05). In MOG-ON eyes, SVC MVD, VD, and GCIPL thickness were inversely correlated with BCVA and disease duration, while RPCP MVD and SVC BFA were inversely correlated only with BCVA. Conclusion: Both NMOSD and MOGAD cause macular structural and microvascular damage associated with reduced BCVA. Decreased CC BFA may aid in distinguishing NMOSD from MOGAD.