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Trigeminal neuralgia caused by an underlying disease other than those described above.

Diagnostic criteria:
  1. Recurrent paroxysms of unilateral facial pain fulfilling criteria for 13.1.1 Trigeminal neuralgia, either purely paroxysmal or associated with concomitant continuous or near-continuous pain, but not necessarily unilateral
  2. Both of the following:
    1. a disorder other than those described above, but known to be able to cause trigeminal neuralgia, has been diagnosed1
    2. pain has developed after onset of the disorder, or led to its discovery
  3. Not better accounted for by another ICHD-3 diagnosis.

Recognized causes are skull-base bone deformity, connective tissue disease, arteriovenous malformation, dural arteriovenous fistula and genetic causes of neuropathy or nerve hyperexcitability.