The production, absorption, and flow of cerebrospinal fluid (CSF) play key roles in the dynamics of intracranial pressure. Alterations in CSF pressure can lead to neurologic symptoms, the most common being headache. Most often, the headaches associated with low CSF pressure are orthostatic and occur after lumbar puncture, but similar headaches occur with spontaneous low CSF pressure due to spinal CSF leaks, and with CSF shunt overdrainage [1,2].
Headache attributed to spontaneous intracranial hypotension is being recognized with increasing frequency. Orthostatic headache, low CSF pressure, and diffuse meningeal enhancement on brain magnetic resonance imaging (MRI) are the major features of the classic syndrome. However, some cases have nonorthostatic headache, normal CSF pressure, or no evidence of diffuse meningeal enhancement . As our understanding of this syndrome is still evolving, the terms "low CSF pressure headache," "spontaneous intracranial hypotension," "spontaneous CSF leak," "CSF hypovolemia," and "CSF volume depletion" tend to be used interchangeably.
This topic will review the treatment and prognosis of headache attributed to spontaneous intracranial hypotension. Other aspects of this disorder are discussed separately. (See "Pathophysiology, clinical features, and diagnosis of spontaneous low cerebrospinal fluid pressure headache".)
Post-lumbar puncture headache is reviewed elsewhere. (See "Post-lumbar puncture headache".)
The diagnosis of headache attributed to spontaneous intracranial hypotension is reviewed here briefly and discussed in detail separately (see "Pathophysiology, clinical features, and diagnosis of spontaneous low cerebrospinal fluid pressure headache", section on 'Diagnosis'). The diagnosis should be considered in patients who present with positional orthostatic headache, with or without associated symptoms, perhaps in the setting of minor trauma, and in the absence of a history of dural puncture or other cause of CSF fistula. Confirmation of the diagnosis requires evidence of low CSF pressure by lumbar puncture, and/or evidence of CSF leakage on imaging with MRI (image 1 and image 2), computed tomographic myelography or radioisotope cisternography.