Headache associated with low CSF pressure

Simy Parikh MD (Dr. Parikh of The Jefferson Headache Center at Thomas Jefferson University has no relevant financial relationships to disclose.)
Stephanie J Nahas MD (Dr. Nahas of Thomas Jefferson University received honorariums from Allergan, Amgen, Electrocore, Eli Lilly, and Supernus for consulting work, and from Amgen for speaking engagements.)
Shuu-Jiun Wang MD, editor. (Dr. Wang of the National Yang-Ming University School of Medicine and the Neurological Institute, Taipei Veterans General Hospital received consulting fees from Eli Lilly and Daichi-Sankyo.)
Originally released March 10, 1994; last updated June 5, 2018; expires June 5, 2021

This article includes discussion of headache associated with low CSF pressure, aliquorrhea, CSF hypovolemia, CSF volume depletion, hypoliquorrhea, intracranial hypotension headaches, low cerebrospinal fluid pressure headaches, Schaltenbrand headaches, and spontaneous intracranial hypotension. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

In this article, the authors review new concepts in spontaneous intracranial hypotension. The authors discuss the role of CSF volume depletion and address the substantial variability in the clinical aspects, imaging findings, and CSF abnormalities related to this disorder. Preexisting dural defects, likely related to disorders of the connective tissue matrix, are sometimes seen and contribute to the often complex anatomy of these spontaneous leaks, presenting therapeutic challenges far beyond the expected management of post-lumbar puncture or even post-epidural catheterization CSF leaks. An update with special emphasis on recent theories on orthostatic headache pathogenesis and advances of the imaging techniques to localize CSF leaks and treatment options is provided. The authors also briefly discuss posttraumatic CSF leak.

Key points

 

• Spontaneous intracranial hypotension is characterized by an orthostatic headache accompanied by neck stiffness, tinnitus, hypacusia, photophobia, or nausea.

 

• Spontaneous intracranial hypotension is caused by spontaneous spinal cerebrospinal fluid leaks, resulting in disruption of craniospinal CSF space elasticity and intracranial hypotension.

 

• Spinal CSF leaks can be localized with heavily T2-weighted magnetic resonance myelography, computed tomographic myelography, radionuclide cisternography, or gadolinium-enhanced magnetic resonance myelography.

 

• The treatment of choice is epidural blood patches, preferably delivered at the level of spinal CSF leaks; fibrin sealant injection and surgical repair are reserved for intractable cases.

Historical note and terminology

Schaltenbrand, a German neurologist, introduced the term “aliquorrhea,” to describe a disorder associated with low, unobtainable, or even negative CSF pressures, and the disorder was clinically marked by orthostatic headaches and other features (Schaltenbrand 1938). This description provided the first understanding of what is now known as spontaneous intracranial hypotension.

From the 1960s to the 1990s, radionuclide cisternography (Front and Penning 1974; Labadie et al 1976) and conventional myelography emerged as useful and reliable diagnostic tools to demonstrate CSF leaks and help with the study of CSF dynamics. Pachymeningeal enhancement and additional MRI abnormalities in intracranial hypotension were subsequently reported (Sable and Ramadan 1991; Hochman et al 1992; Fishman and Dillon 1993; Pannullo et al 1993). Magnetic resonance imaging of the head and spine has revolutionized the diagnosis of spontaneous intracranial hypotension and CSF leaks. Furthermore, it has been realized that an overwhelming majority of, if not all, cases of spontaneous intracranial hypotension result from spontaneous CSF leaks. Although at one point the etiology of spontaneous CSF leaks was thought to be secondary to loss of CSF volume (Mokri 1999), leading to terms such as “CSF hypovolemia” or “CSF volume depletion” appearing in the literature and being used interchangeably with spontaneous intracranial hypotension (Mokri 1999; Chung et al 2000; Miyazawa et al 2003), no data ultimately supported this theory. The current preferred descriptive term is “spontaneous spinal CSF leak” (Schievink 2008).

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