Current perspectives on the recognition and diagnosis of low CSF pressure headache syndromes

ABSTRACT

Introduction

Headaches occur when cerebrospinal fluid (CSF) pressure drops following dural puncture or trauma or spontaneously. As the features of these headaches and their accompanying symptoms might not be typical, low CSF pressure headache syndromes, and spontaneous intracranial hypotension in particular, are often misdiagnosed and underdiagnosed.

Areas covered

The aim of this narrative review is to summarize the most recent evidence regarding the clinical presentation and the diagnosis of low CSF pressure headache syndromes.

Expert opinion

The clinical spectrum low CSF pressure headache syndromes varies significantly and key signs might be missing. Low CSF pressure headache syndromes should be included in the differential diagnosis of any case of refractory headache, even when the headache is not orthostatic, or there are normal neuroimaging findings, and/or lumbar puncture opening pressure is within normal limits. Future research should focus on controlled interventional studies on the treatment of low CSF pressure headache syndromes, which are currently lacking.

KEYWORDS:

• CSF leak
• low CSF pressure headache
• orthostatic headache
• pachymeningeal enhancement
• post-dural puncture headache
• spontaneous intracranial hypotension

Article highlights

• Low CSF pressure headache syndromes are often misdiagnosed and underdiagnosed.

• Orthostatic headache, pachymeningeal enhancement on MRI and low CSF opening pressure are the classic triad, but the absence of these features does not exclude the diagnosis of a low CSF pressure headache syndrome.

• Diffuse, smooth, pachymeningeal enhancement is the most sensitive and specific finding for low CSF pressure headache syndromes on imaging.

• Post-dural puncture headache is usually benign, but a high index of suspicion for complications due to low CSF pressure should be maintained.

• Evidence-based recommendations should be followed when performing lumbar puncture.

• Spontaneous intracranial hypotension might have a protracted course.

• Spinal imaging is often needed to establish a diagnosis of spontaneous intracranial hypotension or to provide targeted treatment.

Declaration of interest

D Mitsikostas has received research support. Speaking fees and travel grants from Amgen, Biogen, Cefaly, Genesis-Pharma, Genzyme-Sanofi, Eli Lily, EleCtocore, Lundbeck, Merck-Serono, Merz, Mylan, Novartis, Roche, and Teva. He performs clinical trials as principal investigator sponsored by Amgen, Biogen, Cefaly, Eli-Lily, EleCtocore, Lundbeck, Merz, Novartis, and Teva. He is co-chair of the Headache Scientific Panel at the European Academy of Neurology, and President of the Hellenic Headache Society. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

This paper was not funded.