6.3.4 Headache attributed to cavernous angioma

Coded elsewhere:

Headache attributed to cerebral haemorrhage or seizure secondary to cavernous angioma is coded as 6.2.1 Acute headache attributed to non-traumatic intracerebral haemorrhage or 7.6 Headache attributed to epileptic seizure.

Diagnostic criteria:
  1. Any new headache fulfilling criterion C
  2. A cavernous angioma has been diagnosed
  3. 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 cavernous angioma, or led to its discovery
    2. either or both of the following:
      • -bheadache has significantly worsened in parallel with other symptoms or clinical or radiological signs of growth of the cavernous angioma
      • – headache has significantly improved or resolved after removal of the cavernous angioma
    3. headache is localized to the site of the cavernous angioma
    4. Not better accounted for by another ICHD-3 diagnosis1.
Note:

In particular, intracerebral haemorrhage has been excluded by appropriate investigations.

Comments:

Cavernous angiomas are increasingly recognised on MRI. Isolated case reports suggest that some cavernous angiomas may trigger cluster headache-like, SUNCT-like or migraine-like attacks. However, there is still no good study devoted to 6.3.4 Headache attributed to cavernous angioma.

In a series of 126 symptomatic patients with cavernous angiomas and KRIT 1 mutations, only 4% reported headache as a presenting symptom. On the contrary, headache is commonly reported as a consequence of cerebral haemorrhage or of seizures, which are the two main manifestations of cavernous angiomas; such headache should be coded to either of these accordingly.