6.8.3 Headache attributed to Moyamoya angiopathy (MMA)


Chronic recurrent headache, which may be migraine-like, caused by and associated with the other clinical features of Moyamoya angiopathy.

Diagnostic criteria:
  1. Recurrent headache fulfilling criterion C
  2. Neuroimaging evidence of Moyamoya angiopathy (MMA)
  3. Evidence of causation demonstrated by both of the following:
    1. headache has developed in close temporal relation to other symptoms and/or clinical signs and/or imaging evidence of MMA, or led to its discovery
    2. either or both of the following:
      • a) headache has significantly worsened in parallel with other symptoms and/or clinical and/or radiological signs of worsening of MMA
      • b) headache has significantly improved after revascularisation surgery
  4. Not better accounted for by another ICHD-3 diagnosis.

Moyamoya angiopathy (MMA) is characterized by bilateral progressive narrowing and occlusion of the intracranial portion of the internal carotid, middle cerebral and anterior cerebral arteries. Several susceptibility genes have been identified or localized for MMA. In some other patients, MMA is associated with other conditions (sickle cell anaemia, Down syndrome and radiation therapy amongst others) and is referred to as Moyamoya syndrome.

MMA usually presents early in childhood or adolescence with ischaemic or haemorrhagic stroke that can cause acute headache. Outside of these acute vascular events, headache is highly common in both children and adults with MMA, phenotypically most commonly resembling 1.1 Migraine without aura, 1.2 Migraine with aura, 1.2.3 Hemiplegic migraine or 2. Tension-type headache; cluster headache-like attacks have been rarely reported.

Revascularization surgery has variable effects on headache in MMA, with improvement in some patients, persistence in others, and postoperative new-onset headache in another subset.