7.2 Headache attributed to low cerebrospinal fluid (CSF) pressure

Description:

Orthostatic headache caused by low cerebrospinal fluid (CSF) pressure (either spontaneous or secondary), or CSF leakage, usually accompanied by neck pain, tinnitus, changes in hearing, photophophia and/or nausea. It remits after normalization of CSF pressure or successful sealing of the CSF leak.

Diagnostic criteria:
  1. Any headache1 fulfilling criterion C
  2. Either or both of the following:
    1. low cerebrospinal fluid (CSF) pressure (<60 mm CSF)
    2. evidence of CSF leakage on imaging2
  3. Headache has developed in temporal relation to the low CSF pressure or CSF leakage, or led to its discovery3
  4. Not better accounted for by another ICHD-3 diagnosis.
Notes:
  1. 7.2 Headache attributed to low cerebrospinal fluid pressure is usually but not invariably orthostatic. Headache that significantly worsens soon after sitting upright or standing and/or improves after lying horizontally is likely to be caused by low CSF pressure, but this cannot be relied upon as a diagnostic criterion.
  2. Brain imaging showing brain sagging or pachymeningeal enhancement, or spine imaging (spine MRI, or MRI, CT or digital subtraction myelography) showing extradural CSF.
  3. Evidence of causation may depend upon onset in temporal relation to the presumed cause, together with exclusion of other diagnoses.