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IHSDiagnosisICD-10
7.2.3Headache attributed to spontaneous (or idiopathic) low CSF pressure G44.820  
Previously used terms Spontaneous intracranial hypotension, primary intracranial hypotension, low CSF-volume headache, hypoliquorrhoeic headache

Diagnostic criteria:

  1. Diffuse and/or dull headache that worsens within 15 minutes after sitting or standing, with at least one of the following and fulfilling criterion D:
    1. neck stiffness
    2. tinnitus
    3. hypacusia
    4. photophobia
    5. nausea
  2. At least one of the following:
    1. evidence of low CSF pressure on MRI (eg, pachymeningeal enhancement)
    2. evidence of CSF leakage on conventional myelography, CT myelography or cisternography
    3. CSF opening pressure <60 mm H2O in sitting position
  3. No history of dural puncture or other cause of CSF fistula
  4. Headache resolves within 72 hours after epidural blood patching

Comments:

The underlying disorder may be low CSF volume. A history of trivial increase in intracranial pressure (eg, on vigorous coughing) is often elicited. In other cases a sudden drop in atmospheric pressure has occurred.

Postural headache resembling that of low CSF pressure has been reported after coitus. Such headache should be coded here because it is due to CSF leakage.

Many patients with spontaneous low CSF pressure headache respond to epidural blood patching, epidural saline infusion or pharmacological therapies such as intravenous caffeine or conventional analgesics. Some have spontaneous resolution of their headache, while others relapse after initial successful treatment. Cases of dural sleeve herniation, particularly in the thoracic area, have been reported and have been successfully treated surgically.

Dural puncture should be avoided in patients with positive MRI signs such as meningeal enhancement with contrast.