Current Concepts in the Diagnosis and Management of Spontaneous Intracranial Hypotension

Spontaneous intracranial hypotension (SIH) is an underrecognized cause of secondary headache resulting from spinal cerebrospinal fluid (CSF) leakage. Although traditionally considered a benign and self-limiting condition, SIH is now known to be associated with substantial disability and potentially serious complications when diagnosis and treatment are delayed. The clinical presentation is heterogeneous, often leading to misdiagnosis and prolonged diagnostic latency. Recent advances have shifted the conceptual framework of SIH from a simple reduction in CSF pressure to a disorder of CSF volume depletion with distinct etiologic subtypes, including ventral dural tears, lateral dural defects associated with meningeal diverticula, and CSF-venous fistulas. Accordingly, diagnostic strategies have evolved toward a stepwise integration of clinical history with targeted imaging modalities. Contrast-enhanced brain magnetic resonance imaging (MRI) and whole-spine MRI serve as essential initial tools to establish the diagnosis and assess complications, while dynamic myelographic techniques such as digital subtraction myelography and dynamic or decubitus computed tomography myelography play a critical role in localizing the site and mechanism of CSF leakage. Management of SIH has similarly progressed toward subtype- based, individualized treatment. Epidural blood patching remains the mainstay of initial therapy, but its effectiveness varies depending on leak type and chronicity. In selected patients, surgical repair or endovascular embolization offers definitive treatment. This review summarizes current concepts in the diagnosis and management of SIH and proposes a practical diagnostic and therapeutic algorithm, while also addressing unmet needs and real-world limitations within the Korean healthcare system.