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IHS Diagnosis ICD-10
6.3.2 Headache attributed to arteriovenous malformation (AVM) [Q28.2] G44.811  

Diagnostic criteria:

  1. Any new acute headache fulfilling criteria C and D
  2. Neuroimaging evidence of arteriovenous malformation
  3. Evidence exists of causation by the arteriovenous malformation
  4. Headache resolves within 72 hours
  5. Subarachnoid haemorrhage, intracerebral haemorrhage and other causes of headache ruled out by appropriate investigations

Comment:

Cases have been reported highlighting the association of AVM with a variety of headaches such as cluster headache, chronic paroxysmal hemicrania (CPH) and short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT), but these cases had atypical features. There is no good evidence of a relationship between AVM and these primary headaches when they are typical.

Migraine with aura has been reported in up to 58% of women with AVM. A strong argument in favour of a causal relationship is the overwhelming correlation between the side of the headache or of the aura and the side of the AVM. There is thus a strong suggestion that AVM can cause attacks of migraine with aura (symptomatic migraine). Yet in large AVM series, migraine as a presenting symptom is rare, much less common than haemorrhage, epilepsy or focal deficits.

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