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Table of Contents
REVIEW ARTICLE
Year : 2015  |  Volume : 3  |  Issue : 1  |  Page : 8-14

Trigeminal autonomic cephalalgias


Professor, Department of Neurology, SBKS MI & RC, Piparia, Waghodia, Vadodara, Gujarat, India

Date of Web Publication3-Aug-2018

Correspondence Address:
Sanjay Prakash
Professor, Department of Neurology, SBKS MI & RC, Piparia, Waghodia, Vadodara, Gujarat
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2347-6486.238521

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  Abstract 


TACs are a group of primary headaches with pain and autonomic features in the distribution of trigeminal nerve on one side. They are also associated with marked restlessness/agitation. The TACs include the following 5 entities: cluster headache (CH), paroxysmal hemicranias (PH), short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT), Short lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA), and hemicranias continua (HC). All TACs are episodic headache disorders except HC. The TACs differ in attack duration and frequency. CH has the longest attack duration (15-180 minutes) and relatively low attack frequency. PH has intermediate duration (2-30 minutes) and intermediate attack frequency. SUNCT and SUNA have the shortest attack (1-600 seconds) duration and the highest attack frequency. Hemicrania continua are characterized by continuous strictly unilateral pain in trigeminal distribution with variable exacerbations. The most specific part of this group is that they all respond to highly ‘selective’ drug. The drugs commonly used for other headache disorders are largely ineffective here. CH responds to lithium and verapamil. SUNCT and SUNA show response to lamotrigine. HC and PH show dramatic response to indomethacin.

Keywords: Trigeminal autonomic cephalalgia, Cluster Headache, Paroxysmal hemicranias, hemicranias continua, SUNCT, SUNA, Indomethacin


How to cite this article:
Prakash S. Trigeminal autonomic cephalalgias. J Integr Health Sci 2015;3:8-14

How to cite this URL:
Prakash S. Trigeminal autonomic cephalalgias. J Integr Health Sci [serial online] 2015 [cited 2022 May 29];3:8-14. Available from: https://www.jihs.in/text.asp?2015/3/1/8/238521




  Introduction Top


Trigeminal autonomic cephalgias (TACs) are a group of primary headache disorders with pain and autonomic features in the distribution of trigeminal nerve. The TACs are listed in section III of the International Classification of Headache Disorders (ICHD-3). International Headache Society (IHS) recently published third edition of International Classification of Headache Disorders (ICHD-3).[1] There are total five different types of TACs. The recent classification of TACs (ICHD-3) is summarized [Table 1].
Table 1: Classification of Trigeminal Autonomic Cephalagias (ICHD-3)

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The aim of this article is to increase awareness and provide basic information regarding TACs, as misdiagnosis and mistreatments are quite common.

TACs are largely a treatable group but the drugs used for this group are highly “selective” and drugs commonly used for other headache disorders are largely ineffective here. These headaches disorders are known for their severity. They are associated with marked restlessness and agitation. A misdiagnosis is very common for this headache disorders.[2] Therefore, attempt should be made to minimize the delay in diagnosing these cases. We discuss briefly each of the TACs, specifically their clinical characteristics, diagnosis and management.

[Table 2] provide a comparative view of diagnostic criteria of all TACs.
Table 2: A comparative view of ICHD-3 diagnostic criteria of all four TACs

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Cluster Headache

Cluster headache (CH) occurs in one per thousand in the population. The male: female ratio was 7-9:1 in earlier studies. However, recent studies suggest that this could be 2-3: 1. This fall in ratio may be because of more recognition (i.e. under reporting in the past) and changing life style of women including smoking habit.[3] The diagnosis is based mainly on the history of the patient and is diagnosed according to ICHD-3 criteria [Table 2].

Cluster headache consists of recurrent, unilateral attacks of headache of great intensity and brief duration. The attacks are commonly accompanied by local symptoms and signs of autonomic dysfunction. Attacks occur in series or clusters (so called cluster periods) that are separated by headache-free interval remission periods. On average, a cluster period lasts 6±12 weeks while remissions last for 12 months (episodic CH). A minority of patients (10-15%) have attacks for >1 year without remission or with remissions lasting <14 days (chronic cluster headache).[3],[4],[5] Periodicity is a characteristic feature of episodic CH. In this, attacks of pain tend to recur at the same hour each day (usually between 1-3 AM, 1-2 PM, and 9-10 PM). A cluster period tends to start at the same month of the year (usually during spring or autumn).

CH has a strong association with the habit of smoking. Most CH patients, up to 90% males and about 70% females, are smokers. The smoking habit is associated with a more severe phenotype of CH than that of patients who never smoke. Smokers may have longer active periods and a higher number of attacks per day.[6] Alcohol also has strong association w ith CH.[4] During a cluster period, alcohol brings on pain within an hour of drinking (in contrast to migraine where headache starts after a few hours of alcohol intake). Alcohol triggers attacks during a cluster period but not in remission.[7]

Individual attack: Pain, is located behind and around the eye and spreads to ipsilateral parts of the head or face. Maximum pain is normally retro-orbital in greater than 70% of patients. Attacks are strictly unilateral. In 15% of patients the headaches shift to the other side of the head at the next cluster period.[3],[4],[5]

The attacks last between 15- 180 minutes, with greater than 75% attacks being less than 60 minutes. Attack frequency is one attack every other day to eight attacks per day. Most patients experience two or less headaches in a day. Cluster headache intensity is always severe or very severe and never mild,

The signature feature of cluster headache is the ipsilateral cranial autonomic (CAS) and it is reported in more than 93% patients.[3],[4] However, each attack may not have CAS. Only 47-52 % CH patient report CAS in each attack. CAS is related with the intensity and some less severe attack may not have autonomic features. Lacrimation is the most common associated symptom, occurring in 70-90% of patients followed by conjunctival injection in 50-60%, nasal congestion in 30-50%, and nasal rhinorrhea in 30-50%.

Another characteristic feature of CH is restlessness or agitation during headache attacks.[8] The patient feels inability to lie still during attacks. Restlessness or agitation may be in the form of walking, pacing, rocking, rolling on the ground, head banging, pressing aching part, putting something on aching part, crying, etc. Only 3% patients can lie still during attack. This is in contrast to migraine where patients have a tendency to move as little as possible and it is one of the important clinical features to differentiate CH with migraine.

Migrainous features (nausea, vomiting, photophobia, and phonophobia) are common with CH. Photophobia, and phonophobia may be present in more than 50% patients with CH. Nausea is also reported in about 40% patients. The presence of these ‘migrainous features’ may create diagnostic with migraine.[3],[4],[5] The presence of nausea, vomiting, or photophobia should not rule out a diagnosis of cluster headache.

Management

Pharmacological management is divided into abortive, transitional, and prophylactic therapies [Table 3].[9],[10] The transitional medications are used mostly in high frequency CH and help in stopping the headache cluster in early period, while prophylactic medication dosages are being increased to therapeutic levels. Transitional therapies are generally given for 1-3 weeks.
Table 3: Drugs used for CH

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Surgery is a last -resort measure in drug- refractory patients. Procedures may be destructive, such as pterygopalatine ganglion or trigeminal ganglion procedures, or trigeminal nerve root section.

Neurostimulation therapies such as deep brain stimulation of the posterior hypothalamic region or occipital nerve stimulation can also be tried in treatment resistant cases.[10]

Paroxysmal Hemicrania

Paroxysmal hemicrania, like cluster headache, is characterised by strictly unilateral, brief, excruciating headaches that occur in association with cranial autonomic features.[1]

PH is considered as a rare disease entity. However, in a review, we noted that PH may be highly under diagnosed and under reported primary headache disorder.[11] The prevalence of PH may be more than 15% of the prevalence of CH. The chronic form (80%) of PH is more prevalent than the episodic form (20%).[12],[13]

Like CH, the patients with PH have cranial autonomic features (lacrimation, conjunctival injection, rhinorrea, nasal congestion etc). Although, restlessness or agitation is not included in ICHD-3 criterion for PH, it is quite frequent even in patients with PH (up to 90%). PH attacks occur regularly at all hours of the day and there is no preponderance for nocturnal attacks. Periodicity and clustering are also not noted with PH.[11],[14]

Paroxysmal hemicrania differs from cluster headache mainly in the high frequency and shorter duration of individual attacks. The hallmark of PH is absolute cessation of the headache with indomethacin. However, there are marked overlap in frequency and duration of the attacks between PH and CH. The overlap in duration and frequency of attacks of cluster headache and PH are also evident in the diagnostic criteria of the ICHD-3. Considerable overlap is also noted in treatment. Patients with CH may show complete response to indomethacin. Drugs effective in patients with CH have also been shown effective in patients with PH. Because of these overlaps, misdiagnosis of PH as CH and vice versa is very common.[15]

Indomethacin is usually started at the dose of 25 mg three times in day. The drug is gradually escalated (25 mg tid every three days) up to 100 mg tid or until the patient gets complete relief. [11] Although response to indomethacin is a must to diagnose a case with PH, many patients will respond to other drugs. Other NSAIDs such as acetyl salicilic acid, naproxen, diclofenac, and ketoprofen may be effective in a few patients with PH. The COX-2 inhibitors, rofecoxib and celecoxib, have also been reported to be effective in PH. Calcium channel blockers such as verapamil, flunarizine and nicardiapine have also been reported to be effective in patients with PH. There are several case reports of a partial or complete response to various other drugs, such as: verapamil, topiramate, acetazolamide, etc [Table 4]. Greater occipital nerve (GON) injection with lidocaine and methylprednisolone is helpful in some patients with PH.[16]
Table 4: Drugs other than indomethacin that may be effective in PH and HC

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Short-lasting unilateral neuralgiform headache attacks (SUNHA)

In ICHD-3, two subtypes of primary headaches are described under this heading: (1) Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and (2) Short lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA). [2]

SUNCT/SUNA is relatively rare TAC that is characterized by very short-lasting attacks of severe unilateral head pain associated with prominent cranial autonomic features and is usually triggerable without refractory period. Triggers factors may be noted in about 80% patients. Triggers may be like touching the face, washing, shaving, eating, chewing, brushing teeth, talking, coughing or neck movement.[17],[18]

An individual attack in both SUNCT and SUNA may last between1-600 sec (1 sec to 10 minutes). Pain is accompanied by ipsilateral conjunctival injection and lacrimation in SUNCT. In SUNA, there may be cranial autonomic symptoms other than conjunctival injection and lacrimation, or, indeed, only one of those symptoms may be present. The most important differential diagnosis for SUNCT/SUNA is trigeminal neralalgia. Prominent autonomic features, more prolonged attack duration, lack of refractory period to trigger factors and relative resistance to carbamazepine are cited as distinguishing features separating SUNCT from trigeminal neuralgia.[17],[18]

Medical treatment of SUNCT/SUNA may be disappointing. Lamotrigine is considered most effective drug for SUNCT/SUNA and may show complete response in more than 50% patients. Topiramate and gabapentine are two other drugs which may be effective in about one third patients.[19]

Hemicrania continua

Hemicrania continua (HC), like paroxysmal hemicrania (PH), is an indomethacin-responsive headache. HC is different from other TACs in that it has continuous course (not episodic). Strictly unilateral continuous head pain of moderate intensity with superimposed exacerbations of more severe pain is the central feature of HC. Periods of exacerbations are accompanied by cranial autonomic features, agitation and migrainous features.[20],[21] The frequency and duration of the exacerbations are highly variable. The duration of exacerbations may vary from a few minutes to a few days. In the same way, frequency may vary from a single attack over a few days to many attacks in a day. These all create diagnostic confusion. If patients and / or physician fail to recognize the background and focus only on the exacerbations, the diagnosis could be either migraine or cluster headache or any other primary headache disorders (depending on the duration, frequency and other characteristic features of the exacerbations). Therefore asking for “background continuous pain” is vital for making the diagnosis of HC. A few recent observations have demonstrated that interictal pain may be present in up to 50% patients with CH and PH. This may create diagnostic confusion. However, this interictal pain usually persists for less than half of the time of interictal period. However, interictal pain of CH or PH is usually described as ‘mild’. Moreover, wide variation in frequency and duration favor the diagnosis of HC.[19]

Like PH, indomethacin is started at the dose of 25 mg three times in day. The drug is gradually escalated (25 mg tid every three days) up to 100 mg tid or until the patient gets complete relief. A response to indomethacin is a must to fulfill the ICHD-3 criteria for PH. However, many patients will respond completely to other drugs.[22] COX-2 inhibitor (rofecoxib and celecoxib), acetyl salicylic acid, ibuprofen, piroxicam derivaties, topiramate, gabapentine, melatonin, vaerapamil, etc may be effective in a few patients with HC [Table 4]. Anesthetic blocks of the greater occipital nerve (GON) or supra orbital nerve (SON) and trochlear steroid injections might be helpful in patients with HC who are intolerant of indomethacin.



 
  References Top

1.
Headache Classification Subcommittee of the International Headache Society, The international classification of headache disorders, 3rd edn. Cephalalgia. 2013;33(9):629–808.  Back to cited text no. 1
    
2.
Viana M, Tassorelli C, Allena M, Nappi G, Sjaastad O, Antonaci F. Diagnostic and therapeutic errors in trigeminal autonomic cephalalgias and hemicrania continua: a systematic review. J Headache Pain. 2013 Feb 18;14(1):14  Back to cited text no. 2
    
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Ferrari A, Zappaterra M, Righi F, Ciccarese M, Tiraferri I, Pini LA, Guerzoni S, Cainazzo MM. Impact of continuing or quitting smoking on episodic cluster headache: a pilot survey. J Headache Pain. 2013 Jun 6;14(1):48.  Back to cited text no. 6
    
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Evans RW, Schürks M. Alcohol and cluster headaches. Headache: The Journal of Head and Face Pain. 2009 Jan 1;49(1):126-9.  Back to cited text no. 7
    
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Torelli P, Manzoni GC. Pain and behaviour in cluster headache. A prospective study and review of the literature. Functional neurology. 2003 Oct 1;18(4):205-10.  Back to cited text no. 8
    
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Becker WJ. Cluster headache: conventional pharmacological management. Headache: The Journal of Head and Face Pain. 2013 Jul 1;53(7):1191-6.  Back to cited text no. 9
    
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Prakash S, Belani P, Susvirkar A, Trivedi A, Ahuja S, Patel A. Paroxysmal hemicrania: a retrospective study of a consecutive series of 22 patients and a critical analysis of the diagnostic criteria. J Headache Pain. 2013 Mar 20; 14(1):26.  Back to cited text no. 11
    
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Goadsby PJ, Cittadini E, Cohen AS. Trigeminal autonomic cephalalgias: paroxysmal hemicrania, SUNCT/SUNA, and hemicrania continua. Semin Neurol. 2010; 30(2):186-91.  Back to cited text no. 12
    
13.
Prakash S, Patell R. Paroxysmal hemicrania: an update. Current pain and headache reports. 2014 Apr 1;18(4):1-8.  Back to cited text no. 13
    
14.
Cittadini E, Matharu MS, Goadsby PJ. Paroxysmal hemicrania: a prospective clinical study of 31 cases. Brain. 2008 Apr 1;131(4):1142-55.  Back to cited text no. 14
    
15.
Prakash S, Shah ND, Chavda BV. Cluster headache responsive to indomethacin: case reports and a critical review of the literature. Cephalalgia. 2010 Aug 1;30(8):975-82.  Back to cited text no. 15
    
16.
Cohen AS, Matharu MS, Goadsby PJ. Trigeminal autonomic cephalalgias: current and future treatments. Headache: The Journal of Head and Face Pain. 2007 Jun 1;47(6):969-80.  Back to cited text no. 16
    
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Cohen AS, Matharu MS, Goadsby PJ. Short- lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) or cranial autonomic features (SUNA)—a prospective clinical study of SUNCT and SUNA. Brain. 2006 Oct 1;129(10):2746-60.  Back to cited text no. 17
    
18.
Williams MH, Broadley SA. SUNCT and SUNA: clinical features and medical treatment. Journal of Clinical Neuroscience. 2008 May 31;15(5):526- 34.  Back to cited text no. 18
    
19.
Lambru G, Matharu MS. SUNCT and SUNA: medical and surgical treatments. Neurological Sciences. 2013 May 1;34(1):75-81.  Back to cited text no. 19
    
20.
Prakash S, Golwala P. A proposal for revision of hemicrania continua diagnostic criteria based on critical analysis of 62 patients. Cephalalgia. 2012 Aug 1;32(11):860-8.  Back to cited text no. 20
    
21.
Cittadini E, Goadsby PJ. Hemicrania continua: a clinical study of 39 patients with diagnostic implications. Brain. 2010 Jul 1;133(7):1973-86  Back to cited text no. 21
    
22.
Prakash S, Husain M, Sureka DS, Shah NP, Shah ND. Is there need to search for alternatives to indomethacin for hemicrania continua? Case reports and a review. Journal of the neurological sciences. 2009 Feb 15;277(1):187-90.  Back to cited text no. 22
    



 
 
    Tables

  [Table 1], [Table 2], [Table 3], [Table 4]



 

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