Headache attributed to spontaneous intracranial hypotension

Spontaneous intracranial hypotension (SIH) caused by a spontaneous cerebrospinal fluid (CSF) leak, results in CSF hypovolaemia. Owing to the variety of clinical presentations and numerous possible diagnostic investigations, diagnosis and appropriate treatment remains challenging in many patients. Although the typical presentation of SIH is an orthostatic headache, the clinical spectrum includes a variety of headache types, focal neurological symptoms, and even spinal manifestations. The underlying pathophysiology of SIH varies depending on the clinical scenario. However, a deeper understanding of the pathophysiology has led to the recognition of risk factors and an explanation for clinical symptoms and abnormalities on diagnostic investigations. Multiple diagnostic investigations can be used to determine if a CSF leak is present or not. Magnetic resonance imaging of the head with and without contrast is sensitive and non-invasive. However, computed tomography myelography remains the study of choice to locate the site of the leak. The rate of flow of the CSF leak can pose a challenge to standard diagnostic investigations. To date, a large-volume blind epidural blood patch is the mainstay of treatment, although more targeted approaches are used for more refractory cases.