Incidence of spontaneous intracranial hypotension in Olmsted County, Minnesota: 2019–2021

Background: As the myriad clinical and radiographic presentations of spontaneous intracranial hypotension are increasingly recognized, disease prevalence appears to rise. True incidence data remain insufficient. We sought to calculate the incidence of spontaneous intracranial hypotension among the residents of Olmsted County, Minnesota. Methods: Our institution is a quaternary care center for spontaneous intracranial hypotension evaluation and provides primary neurologic care for local residents. All patients who were evaluated at our institution from 2019 to 2021 for spontaneous intracranial hypotension were reviewed, and cases of spontaneous intracranial hypotension with advanced imaging work-up using digital subtraction myelography or dynamic computed tomography myelography were identified. Patients who were residents of Olmsted County were identified. Results: Fourteen patients with spontaneous intracranial hypotension were identified in Olmsted County during the study period, all of whom were adults and underwent our institution's standard spontaneous intracranial hypotension work-up. In Olmsted County, the incidence of spontaneous intracranial hypotension was 3.8 per 100,000 adult population, including 3.2 per 100,000 adult females and 2.5 per 100,000 adult males. Seven of 14 patients (50%) had a cerebrospinal fluid-venous fistula. Conclusion: This study uniquely presents the incidence rate of spontaneous intracranial hypotension in the general population of Olmsted County, Minnesota. Spontaneous intracranial hypotension is more common in women than men and has an overall incidence roughly equivalent to that of aneurysmal subarachnoid hemorrhage in Olmsted County, a widely known neurologic condition. Approximately half the cases of spontaneous intracranial hypotension are due to cerebrospinal fluid-venous fistulas, which generally require specialty expertise for diagnosis and treatment.