Headaches are not only one of the 19 different neuropsychiatric syndromes in SLE but also the most common chief complaint of patients with neuropsychiatric lupus. Approximately 50% of patients with SLE are presumed to have neuropsychiatric phenomena during their illness. There're no specific serological, radiological or histological biomarkers to confirm the clinical diagnosis of neuropsychiatric lupus. Therefore, physicians tend to try controlling lupus activity especially when the origins of the headache in patients with lupus are difficult to define. However, neuropsychiatric lupus can only be diagnosed after excluding other causes which is the point. A 47-year-old woman with lupus presented to the emergency department with the sudden onset of postural headache with nausea and vomiting. Through CSF tapping and CT myelography, intracranial hypotension with spontaneous CSF leakage was revealed. Her symptoms promptly improved after therapy using an epidural blood patch. Intracranial hypotension with spontaneous CSF leakage is rare disease, and it has never been reported in patients with SLE before. This case emphasizes the importance of finding out the origin of a headache in patients with lupus.

Keywords: Intracranial hypotension, Spontaneous CSF leakage, Systemic lupus erythematosus