Headache associated with low CSF pressure

Yen-Feng Wang MD (Dr. Wang of National Yang-Ming University in Taipei, Taiwan received honorariums from Pfizer Taiwan and Eli Lilly Taiwan 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.)
Originally released March 10, 1994; last updated February 8, 2015; expires February 8, 2018

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 author reviews new concepts in spontaneous intracranial hypotension. He discusses the role of CSF volume depletion and addresses 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 advances of the imaging techniques to localize CSF leaks and treatment options is provided.

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 intracranial hypotension or “hypovolemia.”

 

• 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,” a disorder associated with low, unobtainable or even negative CSF pressures and clinically marked by orthostatic headaches and other features that are now recognized as the clinical picture of intracranial hypotension (Schaltenbrand 1938).

From the 1960s to the 1990s, radionuclide cisternography (Front and Penning 1973; 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 truly 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. It has been hypothesized that the main culprit in spontaneous CSF leaks is loss of CSF volume (Mokri 1999). Therefore, terms such as “CSF hypovolemia” or “CSF volume depletion” as well as “spontaneous CSF leaks” have appeared in the literature and have been used interchangeably with spontaneous intracranial hypotension (Mokri 1999; Chung et al 2000; Miyazawa et al 2003). It has been pointed out, however, that “CSF hypovolemia” is a misnomer because hypovolemia denotes a decrease in blood volume (Schievink 2008).

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