Compared with the patients with APS in AQP4 + NMOSD, patients with the APS in A-GFAP-A had a lower proportion of females (33.3% vs. 80%, p = 0.003), more hiccups (81% vs. 50%, p = 0.037), more leptomeningeal enhancement (69.1% vs. 5%, p = 0.000), higher CSF white blood cell count (median 120 vs. 10 cells/mm3, p = 0.000) and protein (median 0.949 vs. 0.407 g/L, p = 0.000), Furthermore, fewer patients with A-GFAP-A received long-term immunotherapy (19% vs. 65%, p = 0.003). Here, AQP4 is linked to autoimmune polyendocrinopathy.