Headache attributed to seizure secondary to Sturge Weber syndrome is coded as 7.6 Headache attributed to epileptic seizure.
- Any new headache fulfilling criterion C
- Facial angioma is present, together with neuroimaging evidence of meningeal angioma ipsilateral to it
- 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 and/or imaging evidence of the meningeal angioma
- headache has significantly worsened in parallel with other symptoms or clinical or radiological signs of growth of the meningeal angioma
- headache is migraine-like, either bilateral or localized to the site of the angioma, and associated with aura contralateral to the site of the angioma
- Not better accounted for by another ICHD-3 diagnosis.
Sturge-Weber syndrome occurs exclusively sporadically, resulting from a somatic mosaic mutation in the GNAQ gene (Guanine nucleotide-binding protein, Q polypeptide).
6.3.5 Headache attributed to encephalotrigeminal or leptomeningeal angiomatosis (Sturge Weber syndrome) is poorly documented. More than 90% of cases of Sturge Weber syndrome have seizures, and half report post-seizure headaches, which should be coded accordingly. Isolated reports suggest that encephalotrigeminal or leptomeningeal angiomatosis may be a cause of symptomatic migraine, particularly of attacks with prolonged and/or motor auras (possibly related to chronic oligaemia).