Spontaneous intracranial hypotension – a spinal disease

Charlotte Zander; Katharina Wolf; Amir El Rahal; Florian Volz; Jürgen Beck; Horst Urbach; Niklas Lützen

doi:10.1055/a-2318-8994

RöFo - Fortschritte auf dem Gebiet der Röntgenstrahlen und der bildgebenden Verfahren, Table of Contents

Rofo 2025; 197(01): 44-54
DOI: 10.1055/a-2318-8994

Review

Spontaneous intracranial hypotension – a spinal disease

Article in several languages: English | deutsch

Authors

Charlotte Zander

¹Dept. of Neuroradiology, University Medical Center Freiburg, Freiburg, Germany
Katharina Wolf

²Dept. of Neurosurgery, University Medical Center Freiburg, Freiburg, Germany (Ringgold ID: RIN14879)
Amir El Rahal

²Dept. of Neurosurgery, University Medical Center Freiburg, Freiburg, Germany (Ringgold ID: RIN14879)
Florian Volz

²Dept. of Neurosurgery, University Medical Center Freiburg, Freiburg, Germany (Ringgold ID: RIN14879)
Jürgen Beck

²Dept. of Neurosurgery, University Medical Center Freiburg, Freiburg, Germany (Ringgold ID: RIN14879)
Horst Urbach

¹Dept. of Neuroradiology, University Medical Center Freiburg, Freiburg, Germany
Niklas Lützen

¹Dept. of Neuroradiology, University Medical Center Freiburg, Freiburg, Germany

Abstract

Background

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.

Method

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.

Results and Conclusion

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.

Key Points

SIH is an underdiagnosed condition with a wide range of possible symptoms.
The first diagnostic step using MRI provides indications of the presence of SIH.
The second diagnostic step using (dynamic) myelography can identify the CSF leak.
Collaboration with a CSF center is advisable for further diagnosis and treatment.
Prompt detection and treatment of SIH improves the outcome.

Citation Format

Zander C, Wolf K, El Rahal A et al. Spontaneous intracranial hypotension – a spinal disease. Fortschr Röntgenstr 2024; DOI 10.1055/a-2318-8994

Keywords

spontaneous intracranial hypotension - CSF-venous fistula - myelography - cerebrospinal fluid leak - orthostatic headache

Full Text

References

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