Trigeminal autonomic cephalgia

Last revised by Frank Gaillard on 8 Jul 2023

Trigeminal autonomic cephalgia is a relatively rare group of conditions consisting of cluster headaches, paroxysmal hemicrania, hemicrania continua, and short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (short lasting unilateral neuralgiform headache attacks syndrome1.

It is a group of primary headache disorders characterized by unilateral trigeminal distribution pain that occurs in association with prominent ipsilateral cranial autonomic features 1

Pathology

Although the precise pathophysiology of trigeminal autonomic cephalgias remains to be elucidated, there is a role of the ipsilateral trigeminal nociceptive pathways and the trigeminal-autonomic reflex 1-3. The ipsilateral autonomic features suggest cranial parasympathetic activation (lacrimation, rhinorrhea, nasal congestion, and eyelid edema) and sympathetic hypofunction (ptosis and miosis) 1

Radiographic features

Although no pathognomonic or typical findings have been defined in patients with trigeminal autonomic cephalgias, a wide variety of structural neurovascular abnormalities have been identified 3. Furthermore, no clinical features that suggest that structural pathology is likely to be present have been found 3.

As such, it has been proposed that imaging is worth performing in all patients with trigeminal autonomic cephalgias to exclude structural pathology 3.

Numerous putatively causative abnormalities have been reported including 3:

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