Spontaneous intracranial hypotension – a spinal disease

Abstract

Spontaneous intracranial hypotension (SIH) remains an underdiagnosed condition despite increasing awareness due to recent scientific advances. Diagnosis can be delayed by the broad clinical presentation and imaging pitfalls. This results in a high degree of physical impairment for patients, including social and psychological sequelae as well as long-term damage in the case of delayed diagnosis and treatment.The study is based on a selective literature search on PubMed including articles from 1990 to 2023 and the authors’ clinical experience from working in a CSF center.SIH mostly affects middle-aged women, with the primary symptom being position-dependent orthostatic headache. In addition, there is a broad spectrum of possible symptoms that can overlap with other clinical conditions and therefore complicate the diagnosis. The causative spinal CSF loss can be divided into three main types: ventral (type 1) or lateral (type 2) dural leak and CSF-venous fistula (type 3). The diagnosis can be made using a two-stage workup. As a first step, noninvasive MRI of the head and spine provides indicators of the presence of SIH. The second step using focused myelography can identify the exact location of the cerebrospinal fluid leak and enable targeted therapy (surgical or interventional). Intrathecal pressure measurement or intrathecal injection of gadolinium is no longer necessary for primary diagnosis. Serious complications in the course of the disease can include space-occupying subdural hematomas, superficial siderosis, and symptoms of brain sagging, which can lead to misinterpretations. Treatment consists of closing the dural leak or the cerebrospinal fluid fistula. Despite successful treatment, a relapse can occur, which highlights the importance of follow-up MRI examinations and emphasizes the chronic nature of the disease. This paper provides an overview of the diagnostic workup of patients with suspected SIH and new developments in imaging and therapy.