6.3.3 Headache attributed to dural arteriovenous fistula (DAVF)

Diagnostic criteria:
A. Any new headache fulfilling criterion C
B. A dural arteriovenous fistula (DAVF) has been diagnosed
C. Evidence of causation demonstrated by at least two of the following:

    1. headache has developed in close temporal relation to other symptoms and/or clinical signs of DAVF, or has led to the diagnosis of DAVF
    2. either or both of the following:

      a) headache has significantly worsened in parallel with other symptoms or clinical or radiological signs of growth of the DAVF
      b) headache has significantly improved after treatment of the DAVF

    3. at least one of the following:

      a) headache is accompanied by pulsatile tinnitus
      b) headache is accompanied by ophthalmoplegia
      c) headache is both progressive and worse in the morning and/or during coughing and/or bending over

    4. headache is localized to the site of the DAVF

D. Not better accounted for by another ICHD-3 diagnosis, and intracerebral haemorrhage and cerebral venous thrombosis have been excluded by appropriate investigations.

Studies devoted to 6.3.3 Headache attributed to dural arteriovenous fistula are lacking. A painful pulsatile tinnitus can be a presenting symptom, as well as headache with features of intracranial hypertension due to decrease in venous outflow and sometimes to sinus thrombosis. Carotidocavernous fistulae may present as painful ophthalmoplegia.