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Trigeminal Autonomic Cephalalgias a Diagnostic and Therapeutic Overview

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Trigeminal Autonomic Cephalalgias a Diagnostic and Therapeutic Overview ACNRSO14_Layout 1 04/09/2014 22:14 Page 12 HEADACHE SERIES Trigeminal Autonomic Cephalalgias A Diagnostic and Therapeutic Overview Dr Anna Cohen he Trigeminal Autonomic Cephalalgias duration (2-30 minutes) and occur more is a Locum Consultant Neurologist at the Royal Free Hospital in London. (TACs) are a group of headache disorders frequently during the day (at least five attacks per Her specialist interest is headache. Tcharacterised by attacks of moderate to day for more than half the time). The ipsilateral Her PhD at the Institute of Neurology severe unilateral pain in the head or face, with autonomic features are similar to CH. A diag - addressed the clinical aspects and associated ipsilateral cranial autonomic features nostic criterion of PH is that the attacks are abol - functional imaging work in the TACs, particularly SUNCT and SUNA. She such as lacrimation, conjunctival injection, rhin - ished by Indomethacin. has published widely on the subject orrhoea, nasal congestion, eyelid oedema and Attacks can wake the patient from sleep, of headache, with numerous original ptosis. The syndromes vary in the duration and although much less frequently than in CH; 2 and research papers in cluster headache frequency of the attacks, with cluster headache there is less circadian and annual periodicity and the TACs. (CH) attacks being the longest and least frequent, than in CH. Triggers to attacks included stress, Correspondence to: through paroxysmal hemicrania (PH), to the relief from stress, and exercise (as with triggers to Dr Anna Cohen Short-lasting Unilateral Neuralgiform headache migraine), and also alcohol and neck move - Email: [email protected] attacks, with the most frequent and shortest ment. 2 As in CH, PH can occur as episodic or Conflict of interest statement: attacks. Hemicrania Continua has recently been chronic forms, although CPH is commoner than The author states that she has no included in the classification of TACs, although it EPH. conflict of interest, either financial or shares characteristics of both migraine and the otherwise, in respect to this article. TACs. Short-lasting Unilateral Neuralgiform Provenance and peer review: headache attacks (SUNCT and SUNA) Commissioned and externally Cluster headache These syndromes have attacks of the shortest reviewed. This is the commonest of the TACs, with an inci - duration (1-600 seconds) and most frequent (up dence of around 0.1%. There is a male: female to hundreds of times per day). Originally known To cite: Cohen A. ACNR 2014;14(4):12-15. predominance of 3:1. Attacks of severe to excruci - as SUNCT (Short-lasting Unilateral Neuralgiform ating pain occur in and around the eye, retro- headache attacks with Conjunctival injection and orbital region, and side of the head. Attacks last Tearing), it became apparent that any one or all 15-180 minutes, and can occur once every other of the full range of autonomic features could be day, up to eight times per day. 1 The pain is associ - present ipsilateral to the side of the attack; and ated with ipsilateral conjunctival injection, therefore the ICHD-3 beta classification distin - lacrimation, nasal congestion, rhinorrhoea, fore - guishes between SUNCT and SUNA (Short-lasting head and facial sweating, miosis, ptosis end/or Unilateral Neuralgiform headache attacks with eyelid oedema, forehead and facial flushing, a cranial Autonomic symptoms), where either sense of fullness in the ear, and/or with restless - conjunctival injection, or tearing, or neither, but ness and agitation. not both, are present. 1 Characteristically the pain can wake the Again, SUNCT/SUNA can occur as either patient from sleep at night, often at a set time episodic or chronic forms (the latter is more (such as 90 minutes after falling asleep). Attacks common). There is a slight male preponderance can be triggered by strong smells such as paints, in SUNCT. Multiple cutaneous stimuli have been perfumes or petrol fumes, and by ingestion of reported to trigger attacks of SUNCT/SUNA, alcohol – which will typically induce an attack including: 3 within a few minutes, as opposed to migraine • Touching the face or scalp attacks which are induced within hours of • Bathing or showering ingesting alcohol. • Washing or brushing hair • Shaving Episodic and Chronic Cluster Headache • Nose blowing In 85-90% of cases, CH occurs as Episodic Cluster • Chewing or eating Headache (ECH), in bouts (or ‘clusters’), lasting • Brushing teeth weeks or months at a time, separated by remis - • Talking sion periods of months or years. Patients with • Coughing attacks for more than a year’s duration without a • Exercise remission of a month have Chronic Cluster • Light (including sunlight and fluorescent Headache (CCH). CCH can arise de novo or can lights) develop from ECH. Attacks can be of three types: single stab attacks; groups of stabs; or a saw-tooth pattern, with a Paroxysmal Hemicrania group of stabs occurring in quick succession The attacks of Paroxysmal Hemicrania (PH) are such that the pain does not return to baseline similar to those of CH, but they are of shorter between stabs. The sawtooth attacks, made up of 12 > ACNR > VOLUME 14 NUMBER 4 > SEPTEMBER/OCTOBER 2014 ACNRSO14_Layout 1 04/09/2014 22:14 Page 13 HEADACHE SERIES Table 1: Differential Diagnoses of the TACs Headache Syndrome Differential Diagnoses Distinguishing Features CH Migraine with prominent autonomic features Agitation usually present in CH; also circadian and circannual periodicity PH CH PH responds absolutely to indomethacin SUNCT/SUNA 1)Trigeminal Neuralgia (TN) Autonomic features and agitation are more prominent in SUNCT/SUNA, plus no refractory period between attacks as in TN 2) CH or PH (groups of stabs of SUNCT/SUNA) Cutaneous triggering more common in SUNCT/SUNA; also characterisation of the attack- stab/group of stabs/sawtooth HC 1)CH with background pain HC responds absolutely to indomethacin 2)migraine with chronic background pain many single attacks of SUNCT/SUNA, can endure for minutes or hours, and can often be mistaken for longer-lasting TACs. 3 Figure 1 depicts the three different types of attacks of SUNCT/SUNA. Hemicrania Continua Hemicrania Continua (HC) is a syndrome of continuous unilateral head or facial pain, without a moment’s break, for at least three months’ duration. There is a background constant pain with exacerbations up to moderate or severe intensity that can last for hours or days. The exacerbations are associ - ated with ipsilateral cranial autonomic symp - Figure 1. The different types of attacks in SUNCT/SUNA. Reproduced with permission from OUP 2006 (Cohen A S et al. Brain toms, as in the other TACs, and also agitation 2006;129:2746-2760). or restlessness. The symptom of itching or grittiness in the eye has been often cited as an identifying feature of HC, but a recent case series suggested that it was in fact HC, which shares clinical features of both shown that first-degree relatives of CH another autonomic symptom referable to all migraine and TACs, has activation in both patients are more likely to have CH than in the TACs. 4 However HC also shares some hypothalamus and brainstem structures. 11 the general population. 15 The HCRTR2 1246G features of migraine, with some patients A striking feature of the TACs is the auto - > A and the ADH4 925A > G polymorphisms reporting aggravation of the pain by move - nomic component accompanying each have been associated with CH. ment. Migrainous symptoms such as photo - attack of pain. This is mediated by the trigem - Pharmacogenetic studies have suggested phobia and phonophobia can be seen in all inal autonomic reflex, where stimulation of that the GNB3 825C > T polymorphism may the TACs, 5 but are more common in HC. trigeminal efferents can result in cranial modify treatment response to triptans among Hemicrania continua is usually unremit - autonomic outflow. 12 Thus, some degree of CH patients by altering the signal transduc - ting, but can occur in the remitting subtype autonomic symptomatology is a normal tion cascade via G protein-coupled recep - where there are pain free periods lasting at physiological response to cranial and facial tors. 16 least a day without treatment. pain, and can be present in other headache syndromes such as migraine, especially in Treatment of the TACs Differential diagnosis of the TACs the paediatric population. 13 CH is the only TAC for which an acute Table 1 outlines the differential diagnoses of However in the TACs the autonomic symp - (abortive) therapy is indicated to treat an the TACs, and suggests some clinical points toms are more prominent, in addition to individual attack. The other TACs are too to differentiate between them. agitation during an attack (especially in CH short and too frequent for abortive therapy to and SUNCT), which suggest a common be of any practical use, and therefore the Symptomatic TACs secondary to pathophysiological link. It is suggested that a mainstay of treatment is preventive therapy. structural lesions central disinhibition of the trigeminal-auto - Short-term preventive therapies are useful in The TACs are generally thought of as primary nomic reflex, as well as hypothalamic direct CH and SUNCT, in order to allow a pain-free headache disorders. However there are an modulation of the trigeminovascular noci - window for titration of preventive medica - increasing number of reports of TAC mimics ceptive pathways, are responsible. tions. due to posterior fossa or pituitary lesions. In Experimental results suggest that stimulation particular, SUNCT and SUNA are dispropor - of the posterior hypothalamus significantly Cluster headache tionally over-represented in headaches due inhibited light and facial-skin evoked activity Abortive therapies in CH include to pituitary micro- or macroadenomata. 3,6 of neurons in the trigeminal caudalis and Sumatriptan 6mg to be given subcutaneously upper cervical regions, 14 which further imply at the start of the attack, and this should Pathophysiology of the TACs the role of the hypothalamus in trigeminal abort an attack within a few minutes.
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