6.3.5 Headache attributed to encephalotrigeminal or leptomeningeal angiomatosis (Sturge Weber syndrome)

Coded elsewhere:

Headache attributed to seizure secondary to Sturge Weber syndrome is coded as 7.6 Headache attributed to epileptic seizure.

Diagnostic criteria:
  1. Any new headache fulfilling criterion C
  2. Facial angioma is present, together with neuroimaging evidence of meningeal angioma ipsilateral to it
  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 and/or imaging evidence of the meningeal angioma
    2. headache has significantly worsened in parallel with other symptoms or clinical or radiological signs of growth of the meningeal angioma
    3. 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
  4. Not better accounted for by another ICHD-3 diagnosis.
Comments:

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).