Sign Up for a Free Account
  • Updated 11.20.2023
  • Released 03.10.1994
  • Expires For CME 11.20.2026

Headache associated with spontaneous spinal CSF leak



In this article, the author reviews new concepts in spontaneous spinal CSF leak. Spontaneous spinal CSF leak can present therapeutic challenges far beyond the expected management of post-lumbar puncture CSF leaks or even post-epidural catheterization CSF leaks. This article is updated with a discussion on a nine-point brain MRI-based score that stratifies the likelihood of finding a spinal CSF leak and alternative treatments for managing spinal CSF venous fistulas.

Key points

• Spontaneous spinal CSF leak is characterized by an orthostatic headache often accompanied by neck stiffness, tinnitus, hypacusia, photophobia, or nausea. However, leaks can also present with a second-half-of-the-day headache and, rarely, no headache.

• The spinal location of spontaneous spinal CSF leak is important. Its pathophysiology is thought to be linked to reduced spinal dural compliance, resulting in a downward shift of the hydrostatic indifferent point and intracranial hypotension.

• It is important not to exclude spontaneous intracranial hypotension simply because the patient experiences a nonorthostatic headache or because neuroimaging and lumbar puncture results appear normal.

• The two main imaging tools used to localize spinal CSF leaks are fat-suppressed heavily T2-weighted magnetic resonance myelography and computed tomographic (CT) myelography.

• The treatment of choice is an autologous epidural blood patch, preferably delivered at the level of the spinal CSF leak. Fibrin sealant injection and surgical repair are reserved for intractable cases.

Historical note and terminology

The history of how spontaneous spinal CSF leaks have been described over the years reflects clinical controversy regarding the underlying pathophysiology of this disorder.

Schaltenbrand, a German neurologist, introduced the term aliquorrhea, meaning lacking or absence of to describe a disorder associated with low, unobtainable, or even negative CSF pressures. He noted that clinically, the disorder was marked by orthostatic headache and other features (115). This description provided the first understanding of what is now known as a spontaneous spinal CSF leak.

From the 1960s to the 1990s, with the advent of radionuclide cisternography (48; 68), conventional myelography, and MRI, CSF leaks and CSF dynamics could finally be studied (112; 56; 47; 99). Imaging findings, such as venous sinus engorgement, evoked the Monroe-Kellie doctrine, suggesting that changes in these structures occurred to compensate for lack of CSF in the brain space. For this reason, in the late 1990s, spontaneous spinal CSF leak was called CSF hypovolemia or CSF volume depletion (86). However, patients were also noted to have low CSF pressure, and a competing term spontaneous intracranial hypotension emerged (28; 83). No data have ultimately supported the theory of low volume or even consistently low CSF pressure. Even the concept of the Monroe-Kellie doctrine, or the constancy of brain volume, has been challenged after a study revealed a decrease in brain tissue volume in patients with spontaneous intracranial hypotension (162).

The preferred descriptive term is spontaneous spinal CSF leak (118). This term captures the importance of the CSF leak being spontaneous or occurring with minimal or no clear precipitant. The word spontaneous is important because patients with spontaneous leaks can have a very different prognosis and workup than patients with iatrogenic and traumatic CSF leaks. The term also specifies that the CSF leak should be from a spinal source. This term is important because most people with leaks from the cranium, ie, skull-based leaks, rarely, if ever, develop symptoms or brain imaging findings consistent with spontaneous spinal CSF leak (118; 131).

This is an article preview.
Start a Free Account
to access the full version.

  • Nearly 3,000 illustrations, including video clips of neurologic disorders.

  • Every article is reviewed by our esteemed Editorial Board for accuracy and currency.

  • Full spectrum of neurology in 1,200 comprehensive articles.

  • Listen to MedLink on the go with Audio versions of each article.

Questions or Comment?

MedLink®, LLC

3525 Del Mar Heights Rd, Ste 304
San Diego, CA 92130-2122

Toll Free (U.S. + Canada): 800-452-2400

US Number: +1-619-640-4660



ISSN: 2831-9125