Trigeminal autonomic cephalgias

Abstract

1. Trigeminal autonomic cephalgias (TACs) are headaches/facial pains classified together based on:a suspected common pathophysiology involving the trigeminovascular system, the trigeminoparasympathetic reflex and centres controlling circadian rhythms;a similar clinical presentation of trigeminal pain, and autonomic activation. 2. There is much overlap in the diagnostic features of individual TACs. 3. In contrast, treatment response is relatively specific and aids in establishing a definitive diagnosis. 4. TACs are often presentations of underlying pathology; all patients should be imaged. 5. The aim of the article is to provide the reader with a broad introduction to, and an overview of, TACs. The reading list is extensive for the interested reader.

Keywords: Cluster headache; SUNCT; facial pain; headache; hemicrania continua; paroxysmal hemicrania.

Figure 1.

Photograph of a patient with cluster headache during a right-sided painful attack. Note the ipsilateral ptosis and miosis. Additionally there is obvious ipsilateral lacrimation and rhinorrhoea (see upper lip). Reprinted from Benoliel R and Sharav Y. Trigeminal autonomic cephalgias (TACs). In: Y Sharav and R Benoliel (eds) Orofacial pain and headache. Edinburgh: Mosby Elsevier, 2008; pp.223–254 with permission.

Figure 2.

Pain location in TACs and migraine. TACs are characterized by orbital and periorbital pain. In paroxysmal hemicrania and hemicrania continua there are large adjacent areas affected. Migraine is largely unilateral but may be bilateral in up to 30% of cases (this has been marked by a lighter-shaded area contralaterally). The two-headed arrow above the diagram indicates side shift, which occurs in specific headache.

Figure 3.

Frequency in neurovascular headaches and trigeminal neuralgia. The International Headache Society clearly defines pain frequency but there is considerable overlap. The short-lasting headaches (trigeminal neuralgia, SUNCT, paroxysmal hemicrania) are very frequent (more than eight per day, dotted line) with considerable overlap. Similarly, the long-lasting headaches overlap in the frequency of attacks. Trigeminal neuralgia (shown in double arrow) is often triggered but is usually of high frequency. SUNCT/A, short-lasting, unilateral, neuralgiform headache attacks with conjunctival injection and tearing/autonomic signs.

Figure 4.

Duration in neurovascular headaches and trigeminal neuralgia. The International Headache Society clearly defines pain duration but there is considerable overlap. Duration overlap occurs particularly in headaches lasting from 2 minutes to 4 hours; beyond these limits (dotted lines) diagnosis is relatively limited. It is important to note that migraines may occasionally last less than 4 hours (migraine in double arrow) and cluster headache has been reported to last up to 48 hours. Hem, hemicrania; SUNCT/A, short-lasting, unilateral, neuralgiform headache attacks with conjunctival injection and tearing/autonomic signs; TN, trigeminal neuralgia.

Figure 5.

Flow diagram for the diagnosis of headaches with autonomic signs. Preliminary diagnosis is based on location and accompanying autonomic signs. This is followed by duration, frequency and treatment response, particularly to oxygen and indomethacin. * High-frequency triggered facial pain may also be trigeminal neuralgia with autonomic signs. AS, autonomic signs; CH, cluster headache; HA, headache; O2, oxygen; PH, paroxysmal hemicrania; SUNCT, short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing.