Systemic lupus erythematosus complicated by hydrocephalus and neuromyelitis optica spectrum disorder presenting with complex neuropsychiatric manifestations: a case report

A 59-year-old woman with 45 years history of systemic lupus erythematosus (SLE) and antiphospholipid syndrome was admitted to our hospital with progressive disturbances in consciousness, cognitive impairment and paraparesis following pneumonia and cerebral infarction. Diagnostic workup revealed anti-aquaporin-4 antibody positivity and a longitudinally extensive transverse myelitis lesion, leading to a diagnosis of neuromyelitis optica spectrum disorder (NMOSD). Additionally, ventricular enlargement and cerebrospinal fluid (CSF) findings raised suspicion of normal pressure hydrocephalus (NPH). Treatment with methylprednisolone pulse therapy, hydroxychloroquine, and mycophenolate mofetil resulted in rapid clinical improvement. We propose that systemic inflammation and blood-brain barrier disruption, triggered by infection and infarction, facilitated the entry of autoantibodies and potentially exacerbated CSF dynamics. This case illustrates that neuropsychiatric symptoms in SLE can arise from the convergence of multiple distinct pathologies. Clinicians should maintain a high index of suspicion for overlapping conditions like NMOSD and NPH to ensure appropriate intervention, even when systemic lupus activity is not overtly high.