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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.
- Any new headache fulfilling criterion C
- A cavernous angioma has been diagnosed
- Evidence of causation demonstrated by at least two of the following:
- headache has developed in close temporal relation to other symptoms and/or clinical signs of cavernous angioma, or led to its discovery
- 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
- headache is localized to the site of the cavernous angioma
- Not better accounted for by another ICHD-3 diagnosis1.
In particular, intracerebral haemorrhage has been excluded by appropriate investigations.
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.