7.3.3 Headache attributed to other non-infectious inflammatory intracranial disease


Headache caused by but not usually a presenting or prominent symptom of any of a variety of autoimmune disorders, and associated with other symptoms and/or clinical signs of the causative disorder. It remits after successful treatment of the autoimmune disorder.

Diagnostic criteria:
  1. Any headache fulfilling criterion C
  2. A non-infectious inflammatory disease known to be able to cause headache, other than those described above, has been diagnosed1
  3. Evidence of causation demonstrated by one or more of the following:
    1. headache has developed in temporal relation to the onset of the non-infectious inflammatory disease
    2. headache has significantly worsened in parallel with worsening of the non-infectious inflammatory disease
    3. headache has significantly improved in parallel with improvement in the non-infectious inflammatory disease
  4. Not better accounted for by another ICHD-3 diagnosis.

Headache can be causally associated with, but is not usually a presenting or prominent symptom of, acute demyelinating encephalomyelitis (ADEM), systemic lupus erythematosus (SLE), Behçet’s syndrome and other systemic or focal (eg, limbic encephalitis) autoimmune syndromes.