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J Am Dent Assoc, Vol 139, No 12, 1616-1624.
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CLINICAL PRACTICE

JADA Continuing Education

Trigeminal autonomic cephalalgias

A review and implications for dentistry



Ramesh Balasubramaniam, BDSc, MS, Gary D. Klasser, DMD and Robert Delcanho, BDSc, MS, FFPMANZCA


   ABSTRACT
TOP
 ABSTRACT
CLINICAL CHARACTERISTICS
 PATHOPHYSIOLOGY OF TRIGEMINAL...
 DIAGNOSIS
 MEDICAL TREATMENT
 IMPLICATIONS FOR DENTISTRY
 CONCLUSION
 References
 
Background. The authors review the epidemiology, clinical features, pathophysiology, diagnosis, treatment, orofacial presentations and dental implications of trigeminal autonomic cephalalgias (TACs): cluster headache (CH), paroxysmal hemicrania (PH) and short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT).

Types of Studies Reviewed. The authors conducted PUBMED searches for the period from 1968 through 2007 using the terms "trigeminal autonomic cephalalgias," "cluster headache," "paroxysmal hemicrania," "short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing," "epidemiology," "pathophysiology," "treatment," "oral," "facial" and "dentistry." They gave preference to articles reporting randomized, controlled trials and those published in English-language peer-reviewed journals.

Results. TACs refers to a group of headaches characterized by unilateral head pain, facial pain or both with accompanying autonomic features. Although their pathophysiologies are unclear, CH, PH and SUNCT may be differentiated according to their clinical characteristics. Current treatments for each of the TACs are useful in alleviating the pain, with few refractory cases requiring surgical intervention. Patients with TACs often visit dental offices seeking relief for their pain.

Clinical Implications. Although the prevalence of TACs is small, it is important for dentists to recognize the disorder and refer patients to a neurologist. This will avoid the pitfall of administering unnecessary and inappropriate traditional dental treatments in an attempt to alleviate the neurovascular pain.

Key Words: Trigeminal autonomic cephalalgias; cluster headache; paroxysmal hemicrania; short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing

Abbreviations: CH: Cluster headache. • ICHD-II: International Classification of Headache Disorders II. • NSAID: Nonsteroidal anti-inflammatory drug. • PH: Paroxysmal hemicrania. • SUNA: Short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms. • SUNCT: Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing. • TACs: Trigeminal autonomic cephalalgias. • TMDs: Temporomandibular disorders. • V2/V3 > V1: Maxillary/mandibular division greater than ophthalmic division of the trigeminal nerve.

Trigeminal autonomic cephalalgias" (TACs) is a collective term that refers to a group of headaches characterized by unilateral head pain, facial pain or both, with accompanying autonomic features.1,2 The International Classification of Headache Disorders II (ICHD-II)2 classifies TACs as follows:

– episodic or chronic cluster headache (CH);
– episodic or chronic paroxysmal hemicrania (PH);
– short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT).

The aim of this article is to increase awareness and provide information regarding TACs, because patients often consult dentists regarding symptoms associated with these types of headaches.3 It is important for dentists to understand these disorders so that unnecessary and inappropriate invasive dental procedures will be avoided in this patient population.4 We discuss briefly each of the TACs, specifically their clinical characteristics, pathophysiology, diagnosis and treatment. In addition, we explore orofacial presentations and the dental implications of TACs.


   CLINICAL CHARACTERISTICS
TOP
 ABSTRACT
 CLINICAL CHARACTERISTICS
PATHOPHYSIOLOGY OF TRIGEMINAL...
 DIAGNOSIS
 MEDICAL TREATMENT
 IMPLICATIONS FOR DENTISTRY
 CONCLUSION
 References
 
Cluster headache The prevalence of CH is between 120 and 300 per 100,000 people in the general population.5–8 CH has a male preponderance, with a male-to-female ratio of 2.5–3.5:1.9,10 The age at onset of CH typically is between 20 and 29 years.11,12 The likelihood of a family history in a patient with CH is between 3 and 20 percent.11,13,14

CH is characterized by severe, mainly unilateral pain attacks usually localized to orbital, supraorbital or temporal sites or combinations of these sites, accompanied by ipsilateral autonomic features.1,2 These pains may radiate to the maxilla, nostril, gingiva, palate, jaws, teeth and neck region, thus confusing the dentist as to the true source of the pain.15–18

Pain The pain is excruciating, with patients often describing it as constant, boring and burning or with phrases such as "hot, burning poker in the eye" or "eye is being pushed out."10,19 Not surprisingly, approximately 93 percent of patients with CH report being restless during attacks, resulting in behaviors such as pacing, head banging and other physical activity.9,19,20 Patients with CH may find some relief from the pain by pressing on the superficial temporal arteries, applying heat or cold over the site of pain, pacing, isolating themselves from people or even getting fresh air.20–22 CH has a significant socioeconomic burden, which includes restrictions in daily living, social activities, family life and housework.23

Almost all CH attacks involve ipsilateral cranial autonomic features such as conjunctival injections, lacrimation, nasal congestion, rhinorrhea, forehead and facial sweating, miosis, ptosis, eyelid edema and facial flushing or pallor. These features are considered pathognomonic for CH.9–11,19,24 Apart from miosis, ptosis or both, which may persist after a CH attack, autonomic features tend to cease with the pain.25 Less commonly, migrainelike features such as an aura (visual, sensory or motor) may develop before a CH attack, and photophobia, phonophobia, osmophobia, nausea and vomiting may occur during an attack.9,10,26

Frequency of attacks The frequency of CH attacks can vary from one to three times daily (and in some cases up to eight times in a 24-hour period), with peaks at 1 a.m., 2 p.m. and 9 p.m.12,27 The attack usually begins abruptly without warning, lasting between 15 and 180 minutes (and in some cases up to eight hours) and terminating abruptly or dissipating slowly.10,22,28 As noted above, the International Headache Society’s ICHD-II has classified CH into two forms: episodic and chronic.2 The more common episodic form occurs in bouts, with periods of complete remission.1,2 The chronic form represents 10 percent of cases of CH and is either continuous or occurs with brief periods of remission.1,2,29

Paroxysmal hemicrania Approximately 1 in 50,000 people in the general population has some form of PH diagnosis.22 Contrary to CH, PH has a greater female preponderance, with a female-to-male ratio of 1.6–2.36:1.30,31 The typical age at onset of PH is between 20 and 30 years.31,32 A familial link for PH is rare, and further investigation into genetic predisposition is required.33

Pain PH is characterized by severe, short-lasting, strictly unilateral pain attacks localized to orbital, supraorbital or temporal sites or combinations of these sites, accompanied by one or more ipsilateral autonomic features.1,2 However, the pain also may involve the maxilla and frontal regions,2,22 neck and occiput,22,32 as well as orofacial structures,17,34–40 thereby making the separation from an odontogenic diagnosis somewhat difficult. The pain is excruciating, with patients typically describing the quality as boring or stabbing at peak intensity.1,41 One-third of patients with PH report experiencing pain or discomfort between PH attacks.31

Almost all PH attacks involve ipsilateral cranial and facial autonomic features, with lacrimation, conjunctival injection, nasal congestion and rhinorrhea observed most frequently.2,22,31 Similar to CH, migrainelike features, as described above, may occur during a PH attack.22,30,31

Most PH attacks are spontaneous; however, certain triggers such as glyceryl trinitrate, alcoholic drinks and mechanical rotation or manipulation of the head and/or neck may precipitate an attack.31,42,43 The attacks in PH are shorter-lasting (typically two to 30 minutes) than those in CH, but they can last up to two hours.1,2,31 PH attacks occur between one and 40 times per day,31 with a mean of six to 14 attacks per day30,44; they also may occur regularly at all hours of the day and night.

Forms of PH The International Headache Society’s ICDH-II has classified PH into two forms: episodic and chronic.2 Most patients (80 percent) experience the chronic form of PH, which essentially is unremitting. However, 20 percent of patients have the episodic form, which involves bouts of PH ranging from two weeks to 4.5 months, accompanied by periods of remission lasting between one and 36 months.31,45 Typically, episodic PH progresses to chronic PH.41 The hallmark of PH is absolute cessation of the headache with indomethacin therapy, and, hence, it is distinguishable from the other TACs.1,2

Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing SUNCT is extremely rare, with approximately 80 reported cases in the literature; however, because the classification is new and previously was not well-defined, it is likely to be more common than the literature suggests.46 It has a male-to-female ratio of 1.3:1 based on 50 patients with an onset at between 35 and 65 years of age (mean age, 50 years).47 To our knowledge, there has been only one report of SUNCT in a family and, hence, the possibility of a familial link is undetermined.48

Pain SUNCT is a syndrome characterized by strictly unilateral and intense pain attacks localized to orbital, supraorbital, temporal and frontal areas or combinations of these sites, and it is accompanied by cranial and facial autonomic features. The pain occasionally may involve the neck, other areas of the head, ear, nose, cheek, palate and throat.2,47,49–52 Of particular interest to dentists, 33 and 21 percent of patients with SUNCT reported experiencing pain localized to the maxillary branch of the trigeminal nerve and teeth, respectively.53 The pain intensity of SUNCT varies from moderate to excruciating47,49; however, 84 percent of patients with SUNCT reported experiencing pain as 10 of 10 (most severe pain imaginable) on a verbal rating scale.53

Features Almost all SUNCT attacks involve ipsilateral cranial and facial autonomic features, namely conjunctival injection (100 percent of patients) and tearing (94 percent),2,22,47 although rhinorrhea, nasal congestion, eyelid edema, ptosis, miosis and facial sweating or redness are reported infrequently.47 The autonomic features occur one to two seconds after the onset of an attack and abort within a few seconds of the pain’s ceasing.49 Unlike in CH and PH, migraine-like features are not associated with SUNCT attacks.47,53

The majority of SUNCT attacks occur spontaneously or are triggered by an innocuous mechanism, as in trigeminal neuralgia (TN).47 Common precipitants include touching or washing the face or scalp, shaving, eating, brushing teeth, talking and coughing.46,49 A SUNCT attack usually begins abruptly, with maximum intensity occurring within two to three seconds, and it may persist for between two and 600 seconds (mean duration, 49 seconds).49,51,54–56 Despite almost neuralgialike triggers in SUNCT, there are no refractory periods, as are seen in TN.49

The frequency of attacks varies from less than once a day to more than 60 attacks per hour, and they may last for days.55,57–59 SUNCT attacks have a bimodal distribution, occurring in the morning and afternoon or evening, but they rarely are nocturnal.53,60 The ICHD-II criteria do not distinguish between the chronic and episodic forms of SUNCT, as is the case for the other TACs2; however, reports suggest that a chronic form of SUNCT does exist.47,49,53


   PATHOPHYSIOLOGY OF TRIGEMINAL AUTONOMIC CEPHALALGIAS
TOP
 ABSTRACT
 CLINICAL CHARACTERISTICS
 PATHOPHYSIOLOGY OF TRIGEMINAL...
DIAGNOSIS
 MEDICAL TREATMENT
 IMPLICATIONS FOR DENTISTRY
 CONCLUSION
 References
 
Despite ongoing research, the exact pathophysiology of the various TACs remains unclear. The anatomical distribution of the pain suggests involvement of a central pain generator mediated by the first division of the trigeminal nerve and supported by neurogenic inflammatory mechanisms involving the intracranial and extracranial vasculature (trigeminovascular activation).61 Patients’ response to indomethacin therapy in PH, but not in CH and SUNCT, suggests a different pathophysiology between the subtypes. Indeed, although all three conditions can be considered phenotypically related in terms of symptomatology, they differ significantly in terms of frequency and duration of attacks, response to various medications and triggers. Furthermore, the associated autonomic phenomena, found both clinically and experimentally in subjects with different TACs, vary widely between patients, suggesting that a number of possible mechanisms are at play.

Drummond62 recently reviewed the autonomic mechanisms related to CH, which possibly are applicable to PH and SUNCT. These mechanisms include massive trigeminal-parasympathetic discharge63; centrally mediated hypothalamic disturbance64; and sympathetic dysfunction secondary to trigeminal parasympathetic activation during attacks and/or injury to the pericarotid plexus of sympathetic nerve fibers.65

Further evidence from animal studies65 documented that stimulation of trigeminal efferents can result in cranial autonomic outflow (that is, the trigeminal-autonomic reflex). This presumably occurs via brain-stem connections between the trigeminal nucleus caudalis and the superior salivatory nucleus.1,65,66 In fact, cranial autonomic symptoms appear as a normal physiological response to strong trigeminal nociceptive input.67,68 Benjamin and colleagues69 also suggested that cranial autonomic symptoms may be prominent in TACs owing to central disinhibition of the trigeminal-autonomic reflex by the hypothalamus. These processes or combinations of the above are responsible for the autonomic features manifested by patients with TACs. The finding of hypothalamic activation on functional imaging studies for all of the TACs points clearly to the hypothalamus as a generator or facilitator of these attacks and, therefore, a target for treatment.

Therefore, pain attacks in patients with TACs are thought to be initiated by hypothalamic discharge,70–72 although what actually triggers this discharge remains unknown. Clearly, further scientific research is required to elucidate the pathophysiology of the various TACs.


   DIAGNOSIS
TOP
 ABSTRACT
 CLINICAL CHARACTERISTICS
 PATHOPHYSIOLOGY OF TRIGEMINAL...
 DIAGNOSIS
MEDICAL TREATMENT
 IMPLICATIONS FOR DENTISTRY
 CONCLUSION
 References
 
The key to diagnosing a TAC is based on the patient’s history of experiencing attacks. These features include the rapid onset and location of the pain; quality, duration and temporal patterns of episodes; triggering factors; and associated autonomic features.2,9,19 Clinicians must make a differential diagnosis before establishing a working diagnosis for TACs, because many other conditions can mimic these headaches (Table 1Go). Because a dentist may be the first health care practitioner visited by a patient with a TAC, dentists should have the diagnostic skills to differentiate these symptoms from those of an odontogenic source. For example, a patient may report experiencing unilateral boring pain localized to the orbit that is triggered by alcohol consumption, which is highly suggestive of a CH rather than of odontogenic pain. Similarly, odontogenic pain typically is not associated with ipsilateral cranial autonomic features observed in patients with TACs, such as conjunctival injections, lacrimation, nasal congestion and rhinorrhea.


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  		TABLE 1 Medical conditions with symptoms similar to those of TACs.*

 
Dentists should refer a patient to a neurologist for a diagnostic workup if he or she has symptoms that are not representative of an odontogenic problem. Although the diagnosis of a TAC often is straightforward and usually does not require extensive diagnostic tests, a neurological examination supplemented with neuroimaging studies, such as computed tomography and magnetic resonance imaging, may be warranted to rule out central nervous system pathology. Clinicians may need to address several "red flags" to rule out an underlying pathology. These include a chronic unremitting headache, poor response to standard treatment, presence of abnormal physical findings and abnormal cranial nerve examination results (except for miosis and ptosis).73,74


   MEDICAL TREATMENT
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 ABSTRACT
 CLINICAL CHARACTERISTICS
 PATHOPHYSIOLOGY OF TRIGEMINAL...
 DIAGNOSIS
 MEDICAL TREATMENT
IMPLICATIONS FOR DENTISTRY
 CONCLUSION
 References
 
Successful treatment of TACs includes various forms of therapy. These include nonpharmacological therapies that encompass behavioral and lifestyle interventions; pharmacological therapies involving abortive strategies, preventive strategies or both; and nonsurgical and surgical procedures. Clinicians may administer these therapies as single interventions or in combination. We present below brief descriptions of the various treatment options for each TAC.

Cluster headache Altering lifestyle habits,9,75 such as cigarette smoking10,76 and alcohol intake,10 and treating obstructive sleep apnea77 should be considered in CH management. The clinician directs abortive pharmacological treatment toward managing the attack. This is achieved by administering 100 percent oxygen, triptans, ergot derivatives or intranasal lidocaine (Table 2Go). In some cases, practitioners administer preventive pharmacological treatment, including corticosteroids, verapamil and lithium, to reduce the duration and frequency of the attacks12 (Table 3Go). Investigators have studied numerous medications to treat patients with refractory cases of CH, with varying results.22 Clinicians also have used peripheral nerve blocks78 and surgical approaches for refractory cases and medically complex patients.79–84


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  		TABLE 2 Abortive treatments for cluster headache.

 

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  		TABLE 3 Preventive treatments for cluster headache.

 
Paroxysmal hemicrania The medical treatment for patients with PH is prophylactic use of indomethacin, which is considered pathognomonic for this type of headache. However, although rare, cases of indomethacin-resistant PH have been reported.30,85 Anecdotal evidence and case reports86,87 suggest that patients whose symptoms are refractory or whose conditions are nonresponsive may be treated with other medications or anesthetic blockades (Table 4Go).


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  		TABLE 4 Treatments for paroxysmal hemicrania.

 
Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing SUNCT is considered to be relatively refractory to treatment.88 Nevertheless, clinicians have tried pharmacological approaches (Table 5Go), as well as several surgical approaches involving local anesthetic blockades, invasive procedures involving the trigeminal nerve and neurosurgical procedures. Some case reports51,89–93 indicate that the anticonvulsant drug lamotrigine has been highly effective in a number of patients with SUNCT. However, other case reports54,94,95 indicate a lack of effectiveness with lamotrigine.


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  		TABLE 5 Treatments for short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing.

 

   IMPLICATIONS FOR DENTISTRY
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 ABSTRACT
 CLINICAL CHARACTERISTICS
 PATHOPHYSIOLOGY OF TRIGEMINAL...
 DIAGNOSIS
 MEDICAL TREATMENT
 IMPLICATIONS FOR DENTISTRY
CONCLUSION
 References
 
Dentists often are faced with the difficult task of diagnosing odontogeniclike pain emanating from various anatomical structures. TACs may present a challenge for the dentist because of their often overlapping and similar presentations to true odontogenic pain. Dentists need to keep in mind that the oral cavity is not the source of all odontogenic pain but may merely be a site of pain.96 The key to discerning source versus site of pain is to take a thorough medical history and perform a comprehensive clinical examination. Failure to complete these steps increases the risk of misdiagnosis, which may lead to unnecessary, inappropriate and irreversible dental procedures.

Patients often describe the pain due to CH as emanating from the midfacial region, which may be interpreted as pain originating from the teeth, jaws or temporomandibular joints.15–17 Therefore, it is not uncommon for people to seek care from dentists. Bahra and Goadsby4 reported that 230 (45 percent) of 511 subjects with CH were examined by a dentist before receiving the correct diagnosis. (We should point out that subjects also visited a mean of three physicians before receiving a correct diagnosis.) These authors also found that misdiagnosis often led to unnecessary and inappropriate dental procedures.

In another study, Bittar and Graff-Radford97 found that 14 (42 percent) of 33 subjects with CH underwent some form of invasive and irreversible dental procedures. Some investigators have postulated that dental extractions may be a precipitating factor for CH. In a study of 54 subjects with CH, Penarrocha and colleagues98 found that tooth extraction or endodontic treatment had been performed in the pain-affected quadrant in 31 (57 percent) of the subjects and in the contralateral quadrant in 18 (33 percent) of the subjects. In addition, they found that in 24 (44 percent) of the 54 subjects, tooth extraction had been performed after the onset of pain in an attempt to resolve the problem; however, only one subject reported having experienced improvement. These authors concluded that the onset of pain after a dental procedure might suggest a relationship between nerve damage and the development of CH, but we also must consider the possibility that dental extraction and endodontic procedures were performed in response to CH.

Because of the short duration of attacks, frequent recurrences, excruciating intensity and pulsatile pain quality found in patients with PH, it is possible that this disorder may be mistaken for dental pulpitis.40 It also is not uncommon for PH to manifest in the maxillary region, thereby being mistaken for tooth pain.31 Benoliel and Sharav35 reported on seven cases of PH, four of which had been confused with pain of dental origin. Two of these patients received irreversible dental treatments. Other studies have reported similar occurrences whereby the spectrum of failed dental treatment ranged from pharmacological approaches to full-mouth reconstruction.17,34,36,38

The literature also contains case reports in which patients with SUNCT, in addition to experiencing facial pain, complained of pain radiating to adjacent teeth.99–101 This resulted in clinicians’ delivering therapeutic interventions for tooth pain such as extraction, occlusal splints and incorrect drug treatment. PH also may be misdiagnosed as pain associated with temporomandibular disorders. The manifestation of PH in the temporal, maxillary and ear regions along with ipsilateral masticatory muscle tenderness may contribute to the misdiagnosis.35,36,38,40 Again, this may lead to unnecessary treatments such as inappropriate pharmacological approaches and surgical interventions.40

Differences in the quality and intensity of pain described for PH (throbbing and excruciating) and in musculoskeletal pain (mild-to-moderate aching pain) should assist practitioners in making the correct diagnosis. Another pain condition that may be confused with PH or SUNCT is TN. The common features of all three disorders, such as the excruciating pain, intermittent temporal pattern, unilaterality, frequency of attacks and lancinating nature, may be partially responsible for the misdiagnoses. In addition, there are reports in the literature of the coexistence of TN and SUNCT, suggesting a pathophysiological relationship between these short-lasting unilateral disorders.100,102,103

Because of these similarities, it is important for dentists to recognize and understand some differentiating features among these disorders. A major difference between TN, PH and SUNCT is that a TAC always is associated with autonomic features, whereas TN usually occurs without any such features,104 except in rare circumstances and only in the ophthalmic branch.105 In addition, it is rare for TN attacks to disturb the patient’s sleep, whereas sleep disturbance may occur in patients with PH, but it is rare in patients with SUNCT. Another differentiating feature between PH and TN involves the much shorter duration of the attack in TN, which often is triggered by an innocuous stimulation. However, this manifestation, although not consistent for patients with PH, is similar to that observed in patients with SUNCT. Finally, treatment of TN with carbamazepine often is considered pathognomonic for this disorder. Similarly, treatment of PH with indomethacin is considered pathognomonic; however, indomethacin will not alleviate TN nor will carbamazepine alleviate PH. SUNCT is relatively refractory to most medications, with the possible exception of lamotrigine and intravenous lidocaine. Table 6Go summarizes the clinical characteristics of TACs, TN and odontogenic pain.


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  		TABLE 6 Differentiating features of trigeminal autonomic cephalalgias, trigeminal neuralgia and odontogenic pain.

 

   CONCLUSION
TOP
 ABSTRACT
 CLINICAL CHARACTERISTICS
 PATHOPHYSIOLOGY OF TRIGEMINAL...
 DIAGNOSIS
 MEDICAL TREATMENT
 IMPLICATIONS FOR DENTISTRY
 CONCLUSION
References
 
TACs are painful and disabling primary headaches. Dentists may be the first practitioners visited by patients with these disorders, and, therefore, it is essential that they recognize and understand the characteristics of TACs to avoid making an incorrect diagnosis. This will prevent unnecessary and inappropriate traditional dental treatments. Ultimately, with this knowledge, dentists will be in a position to refer patients for appropriate care.


   FOOTNOTES
 

When this review was conducted, Dr. Balasubramaniam was a fellow, Department of Oral Medicine, University of Pennsylvania, School of Dental Medicine, Philadelphia. He now is a clinical associate professor, School of Dentistry, University of Western Australia, Perth, and codirector, Perth Orofacial Pain and Oral Medicine Centre, St. John of God Hospital, Subiaco Clinic, Suite 319, 25 McCourt St., Subiaco, Western Australia 6008, Australia, e-mail "ramesh.balasubramaniam{at}uwa.edu.au". Address reprint requests to Dr. Balasubramaniam.


Dr. Klasser is an assistant professor, Department of Oral Medicine and Diagnostic Sciences, University of Illinois at Chicago, College of Dentistry, Chicago.


Dr. Delcanho is a clinical associate professor, School of Dentistry, University of Western Australia, Perth, and codirector, Perth Orofacial Pain and Oral Medicine Centre, St. John of God Hospital, Subiaco Clinic, Western Australia, Australia


Disclosure. The authors did not report any disclosures.


   References
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 ABSTRACT
 CLINICAL CHARACTERISTICS
 PATHOPHYSIOLOGY OF TRIGEMINAL...
 DIAGNOSIS
 MEDICAL TREATMENT
 IMPLICATIONS FOR DENTISTRY
 CONCLUSION
 References
 

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