Chapter 25. Facial Pain

The diagnosis and management of patients with facial pain can be daunting even to experienced physicians. The causes are myriad, ranging from the mundane (sinus and dental disease) to the exotic (short-lasting unilateral neuralgiform headache with conjunctival injection and tearing, or SUNCT). Misdiagnosis and mismanagement are common. The goal of this chapter is to discuss some of the more important causes of facial pain, and to guide proper identification and treatment.

Simply because pain is felt in the face does not imply that it necessarily originates in facial structures. As elsewhere in the body, pain may be local in origin, or referred. The role of the trigeminovascular system and the spinal trigeminal nucleus as a point of anatomic and physiologic convergence is discussed in Chapter 19. Suffice it to say that the location of the pain may not be so important diagnostically as other features.

The approach to the evaluation of facial pain requires careful attention to detail. In the history, it is essential to obtain an accurate description of the nature of the pain, or pains, what may have incited it, and what currently provokes and ameliorates it. Are there associated phenomena, such as autonomic changes? Past medical history, including trauma and surgical or dental procedures may provide essential clues. Is there associated depression or any other psychiatric problem? What therapies have been tried, and with what outcomes?

After obtaining a detailed history, a thorough examination is necessary. In addition to the general physical examination, a thorough neurologic examination is essential. The examiner looks for signs of raised intracranial pressure (papilledema, diminished up gaze, sixth cranial nerve palsies) and cranial nerve dysfunction (particularly oculosympathetic paresis). The head and neck require careful attention. Are there trigger points? Is there dental or sinus pathology? Auscultation for bruits and palpation of the carotid artery are sometimes informative maneuvers.

Lastly, the results of prior diagnostic studies are reviewed, noting the timing of the studies. Were the appropriate studies performed? If the situation has changed, perhaps a study should be repeated. Sometimes a diagnosis may become apparent only after serial clinical examinations or diagnostic studies, or both.

After a careful history, examination, and review of the data, a tentative diagnosis may be rendered. Often further consultation is required. Treatment is offered based on the tentative diagnosis and may, in itself, sometimes be diagnostic. The thoughtful physician should always be willing to reconsider the diagnosis.

Facial pain clearly represents a diagnostic challenge. With attention to the fundamental approach outlined in the preceding paragraphs, the vast majority of patients may achieve a satisfying outcome.

Neuralgias are paroxysmal pain in the distribution of a particular nerve. The pain is typically maximal at onset and lancinating, and may be described as “electric shocks” or “jabbing.” There may be a single sharp pain, or repetitive pains in succession. The pain may be so brief as to last but an instant, or for several seconds. There is usually a refractory period after the severe pain, during which pain does not occur. Some neuralgic conditions have trigger zones (areas that, when stimulated, provoke an attack) or other triggers. Careful history taking often reveals other pain occurring as well, such as continuous aching, burning, or throbbing. Inquiry must be made about all the sensations that occur, because some patients mention only the severe exacerbations. Response to treatment may provide a clue to diagnosis, but can also be misleading. “Diagnostic blocks” in the setting of facial pain do not necessarily define the site from which the pain arises because of overlap of cranial nerves V, IX, and X (which converge on the spinal trigeminal nucleus and the tractus solitarius).

The best-known facial neuralgia is trigeminal neuralgia, which is discussed in detail in this chapter and, in many ways, serves as a model for understanding other neuralgias causing facial pain.

Trigeminal Neuralgia

Trigeminal neuralgia is a severe, (usually) unilateral facial pain, characterized by lancinating pains in the distribution of one or more divisions of the trigeminal nerve. Although onset may occur in the second and third decades, the majority of cases begin in middle and old age. With an annual incidence rate of 4 to 5 per 100,000, it is one of the most frequently seen neuralgias in the elderly.1 The pain may be so excruciating that facial muscle spasms can be seen, hence the older term, tic douloureux.

The facial pain tends to occur in paroxysms and is maximal at or near onset. The severe exacerbations tend to last from one to several seconds, but may occur in volleys. There may also be a coexisting continual deep or dull pain. Patients may or may not, therefore, have pain-free periods.

Trigeminal neuralgia most often involves the second and third divisions of the trigeminal nerve (V2 and V3), but can include or be limited to the first division, as well. Trigger zones may be present. Often, lightly touching these areas will trigger a paroxysm, and patients tend to protect these areas. Other triggers may include chewing, talking, brushing teeth, cold air, or smiling and grimacing. A refractory period of several minutes typically follows an episode, during which a paroxysm cannot be provoked. In occasional patients, the pain may be bilateral, but not on both sides simultaneously.

This condition may be idiopathic (primary), or symptomatic (secondary). Idiopathic implies no known underlying condition, but in point of fact most idiopathic cases are the result of vascular compression of the trigeminal nerve near its entry into the pons. Symptomatic causes include multiple sclerosis, tumors, and basilar artery aneurysm or ectasia.2–4

Unlike some other facial pain conditions, trigeminal neuralgia typically does not awaken patients at night. Some patients have a history of so-called pretrigeminal neuralgia, which is said to be dull, continuous, aching pain in the jaw, evolving eventually into trigeminal neuralgia. This brief, milder pain is sometimes suspected to have a dental origin, and dental procedures are performed. Because trigeminal neuralgia is sometimes precipitated by dental procedures (e.g., dental extraction), confusion about etiology has resulted.5

The pathophysiology of trigeminal neuralgia is not fully elucidated. Demyelinative lesions of trigeminal fibers appear to set up ectopic impulses and ephapses. This alteration of afferent input may disinhibit pain pathways in the spinal trigeminal nucleus. Evidence for a role of central pain mechanisms includes the presence of refractory periods after a triggered episode, trains of painful sensations after a single stimulus, and some latency from the time of stimulation to the onset of pain.6

Clinically, certain features of the history help in making the diagnosis. Paroxysms of pain in the distribution of the trigeminal nerve, especially if trigger points are present, are typical. The examination may reveal these trigger zones, which often are near the midline. If sensory loss is present, a mass lesion is more likely. In younger patients (20 to 40 years old) with trigeminal neuralgia, multiple sclerosis should be considered. A demyelinative lesion in the pons would explain bilateral symptoms.

Once the diagnosis is suspected on clinical grounds, a careful search for ipsilateral dental pathology should be undertaken (oral surgery consultation should be considered). Magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) should be performed to look for evidence of demyelinating lesions, a mass lesion in the cerebellopontine angle, or an ectatic blood vessel (rarely seen with MRA). The differential diagnosis includes SUNCT, cluster-tic syndrome, jabs and jolts syndrome, and other neuralgias, all of which are discussed later in this chapter.

Trigeminal neuralgia is usually successfully treated with medication (Table 25-1). Carbamazepine is felt to be the most effective medication. Baclofen, phenytoin, valproate, pimozide, and clonazepam have utility as well, alone or in various combinations.7 Misoprostol, a prostaglandin E analogue, has been reported as effective in trigeminal neuralgia caused by multiple sclerosis.8 Gabapentin and lamotrigine have also been reported to be useful, although there is less clinical experience with these agents.9,10

Neuroleptic medications, including chlorpromazine and perphenazine, have been used successfully in intractable cases, and novel neuroleptics such as olanzapine and quetiapine are being tried as well. Tizanidine has been used in selected cases. Topiramate has been suggested, because other anticonvulsant medication has been effective.

We have found carbamazepine to be the most useful of all the available drugs. If doses are started low, especially in the elderly (e.g., 50 mg b.i.d.) and advanced gradually, the drug is highly efficacious with few side effects, and few patients develop tolerance. If pain control is insufficient, baclofen may be added. If the patient is desperate for immediate pain relief, intravenous infusion of phenytoin or fosphenytoin (250 to 500 mg at no more than 50 mg/min, monitoring pulse and blood pressure) may be rapidly effective, although many patients quickly develop tolerance to the drug. Nonetheless, intravenous phenytoin may afford immediate temporary relief while oral carbamazepine therapy is begun. This maneuver may also enable the physician to better examine the patient with a sensitive face.11 Details of the use of these medications may be found elsewhere in this book (see Chaps. 62, 63, and 64). Rarely, there are spontaneous permanent remissions of trigeminal neuralgia. More often, the illness tends to wax and wane in terms of severity and frequency of exacerbations. Therefore, in patients achieving good relief of pain with medications, periodic attempts to gradually withdraw these drugs are warranted.

For patients refractory to therapy with drugs, various surgical procedures may have efficacy. Janetta has popularized microvascular decompression, the dissection away from the trigeminal nerve of various vascular structures, often an ectatic superior cerebellar artery.12 Long-term outcome is good (over 80% pain-free), but this procedure does involve an intracranial approach with a small morbidity and mortality rate. Other procedures directed against the trigeminal nerve include percutaneous radiofrequency rhizotomy, glycerol rhizolysis, and balloon compression. Complications include anesthesia dolorosa (a central pain disorder resulting from hypersensitization of the second-order spinal trigeminal nucleus neuron) and keratitis, as well as facial weakness caused by injury of the facial nerve. More recently, excellent results with minimal morbidity have been reported with gamma knife therapy.13 For patients unable or unwilling to tolerate more aggressive surgical procedures, peripheral nerve root avulsion may provide relief. Although often temporarily effective, nerves often regenerate and pain recurs.

Tenser has noted that after surgery for trigeminal neuralgia, herpes simplex virus reactivation occurs in 17% to 94% of patients. The speculation is that altered function of cranial nerve V (CNV) underlies the beneficial response to the various surgical manipulations (injury to the trigeminal root ganglion).14

In summary, patients suffering from trigeminal neuralgia deserve a careful evaluation, especially to exclude symptomatic causes such as multiple sclerosis or a cerebellopontine angle mass. Ipsilateral dental pathology should be sought. Pain relief can usually be achieved through aggressive pharmacologic therapy, surgery or both.

Cluster-Tic Syndrome

It has been reported that the pains of trigeminal neuralgia and cluster headache may coexist.15 In the cluster-tic syndrome, there are three types of pain. One component of the pain resembles trigeminal neuralgia—paroxysmal, extremely brief, and severe. The second component is more similar to cluster headache, although of variable length, with autonomic phenomena (lacrimation, rhinorrhea). The third type of pain is a mixture of the first two. This pain may be provoked by trigger points or moving the neck.

Cluster-tic syndrome usually afflicts patients between 20 and 70 years old. It may exist in chronic or episodic forms (remissions and recurrences). Medical therapy is usually unsuccessful, although when surgery (microvascular decompression or trigeminal rhizotomy) relieves the neuralgia, the cluster-like pain may be lessened and become more responsive to therapy.

The differential diagnosis includes SUNCT, which is discussed later in this chapter. Secondary (symptomatic) SUNCT has been reported as a result of arteriovenous malformation in the cerebellopontine angle, and secondary cluster-tic syndrome has been seen associated with an ectatic basilar artery running deep into the cerebellopontine cistern.16,17 We feel it is possible that so-called cluster-tic syndrome and SUNCT may actually be the same condition.

Glossopharyngeal Neuralgia

Glossopharyngeal neuralgia is defined as paroxysmal pain in areas supplied by cranial nerves IX and X (CN IX, X). There are numerous analogies to trigeminal neuralgia, with which it occasionally coexists.

This entity is much less common than trigeminal neuralgia. Age of onset ranges from childhood to old age, although middle age is most frequent. This condition usually occurs as paroxysmal, severe, unilateral pain involving the ear, larynx, tonsil, or tongue. It is almost never bilateral. Triggers include chewing, swallowing, coughing, speaking, yawning, certain tastes, and touching the neck or external auditory canal (rarely the pre- or postauricular areas).18

The pain typically radiates upward from the oropharynx toward the ear. The duration of the severe paroxysms is seconds to minutes, but there may also be a low-grade, constant, dull background pain. Up to several dozen attacks may occur each day, some of which awaken the patient from sleep. Some episodes are associated with strenuous coughing or hoarseness.

Similar to trigeminal neuralgia, glossopharyngeal neuralgia may occur in a pattern of bouts lasting weeks to months, alternating with longer periods of remission. Severe attacks may be associated with bradycardia and asystole, resulting in syncope.19 Presumably, in these cases, input from CN IX into the tractus solitarius has an effect on the dorsal motor nucleus of X.

Also, similar to trigeminal neuralgia, there are idiopathic and secondary (symptomatic) forms. Presumably, in the idiopathic form, peripheral demyelinization results in brainstem discharges. Alternatively, vascular compression of CN IX and X may occur at the nerve root entry zone by the vertebral artery or posterior inferior cerebellar artery. Symptomatic causes include cerebellopontine angle tumor, brainstem demyelinative lesions, peritonsillar abscess, carotid aneurysm, and Eagle’s syndrome (in which CN IX is compressed laterally against an ossified stylohyoid ligament).20

The evaluation of a patient suspected of suffering from glossopharyngeal neuralgia incudes a careful history, especially inquiring about the presence of trigger factors and nocturnal awakening. All aspects of an attack need to be recorded. MRI and MRA are appropriate, because approximately 25% of cases of glossopharyngeal neuralgia result from secondary causes, such as brainstem mass lesions. Plain skull films might reveal an ossified stylohyoid ligament in Eagle’s syndrome.

Medical therapy of glossopharyngeal neuralgia is essentially the same as for trigeminal neuralgia (see Table 25-1). Additionally, the application of local anesthetics to the oropharynx may be both diagnostic and therapeutic. Injection of local anesthetic into the region of the stylohyoid ligament can be diagnostic if Eagle’s syndrome is strongly considered. Patients failing drug therapy may be candidates for surgical treatment. Procedures include intracranial sectioning of CN IX along with the upper three to four rootlets of CN X at the jugular foramen, or vascular decompression.20

The differential diagnosis of glossopharyngeal neuralgia includes so-called geniculate neuralgia (nervus intermedius neuralgia of Hunt), which is discussed later in this chapter. The obvious overlap of clinical features between the two conditions fosters suspicions that they may actually be variations of the same condition.

Superior Laryngeal Neuralgia

The superior laryngeal nerve is a branch of CN X that runs adjacent to the carotid bifurcation and supplies the cricothyroid muscle of the larynx. A lesion of this nerve produces a weak, hoarse voice. The nerve may be involved by local disease of the carotid or injured by carotid endarterectomy.21

Clinically, the patient suffers paroxysmal pain that radiates from the throat to the ear or eye, similar to the pain of glossopharyngeal neuralgia. There is generally hoarseness. The episodes last seconds to minutes, and may occur spontaneously, or be triggered by coughing, swallowing, or speaking. During severe paroxysms, the patient temporarily may be rendered mute.

On examination, hoarseness of speech, and a trigger point superolateral to the thyroid cartilage may be noted. The differential diagnosis includes glossopharyngeal neuralgia, geniculate neuralgia, and carotidynia (all discussed elsewhere in this chapter). Local blockade of the nerve is diagnostic. Some patients respond to carbamazepine and possibly other drugs used for neuralgias. Neurectomy may be curative.21

Postherpetic Neuralgia

Acute herpes zoster (shingles) often causes facial pain, especially affecting V1. The varicella zoster virus persists in sensory nerve ganglia, and may cause a painful rash. Postherpetic neuralgia is variably defined as pain that persists (anywhere from 1 to 6 months) after the rash has healed. Although treatment of acute zoster with acyclovir, valacyclovir, famciclovir, steroids, pain medications, and even sympathetic nerve blocks may shorten the duration and alleviate the immediate pain, none has been clearly proven to prevent the occurrence of postherpetic neuralgia. Age is the major risk factor. Patients younger than 40 years of age rarely develop postherpetic neuralgia, whereas more than 75% of patients older than age 70 years are afflicted.22 Lesser risk factors include diabetes mellitus, V1 involvement, and other immunologic compromise.

Clinically, there is usually scarring and pigmentary changes in the affected dermatome. The pain is constant and variably aching, burning, lancinating, or itchy. The mechanism of the pain is likely deafferentation pain. Pathologically, there are degenerative changes in the involved axons, dorsal ganglion, and even the dorsal horn of the spinal cord. Patients complain of allodynia (pain with non-noxious stimulus) in the affected area.23

Because this condition preferentially affects the elderly, treatment can be difficult. Unlike trigeminal neuralgia, anticonvulsants are usually ineffective. Amitriptyline is the single most useful agent, although the anticholinergic side effects may be troublesome. Beginning with a low dose (e.g., 10 mg) and slowly escalating toward 75 mg at bedtime may improve tolerance. If side effects are troublesome, either nortriptyline or doxepin, which have fewer anticholinergic side effects, may be substituted (both are available in liquid form, allowing very gradual dose titration if necessary). Approximately 60% of patients obtain significant relief with these agents.24,25

Many other agents have been anecdotally reported to benefit patients with postherpetic neuralgia. These include controlled-release oxycodone, capsaicin cream, topical lidocaine gel, EMLA cream, and gabapentin. With persistence, pain relief can usually be achieved. Fortunately, for most patients, the pain eventually spontaneously remits, for the majority in less than 3 years.

Other Neuralgias

Numerous neuralgias have been described as causes of facial pain. However, some share many features with other conditions, and it is likely that some of these conditions simply share several names. Of these, Raeder’s paratrigeminal syndrome is the best recognized.

Raeder’s syndrome consists of constant, unilateral, burning facial pain with hypesthesia or dysesthesia, or both, in the distribution of the trigeminal nerve, most often V1, plus oculosympathetic paresis (ptosis and miosis). This syndrome may be caused by carotid artery dissection. It may also occur with trauma, mass or lesion of the middle cranial fossa, syphilis, and sinusitis. In the absence of these underlying conditions, it is generally a self-limited process, remitting in weeks to months.26

Sphenopalatine neuralgia (with numerous other synonyms, including greater superficial petrosal neuralgia) is described as unilateral, episodic, perinasal facial pain with nasal congestion.27 Most of these cases closely resemble cluster headache, and the existence of this neuralgia as a separate diagnostic entity is questionable.

The same may be said for geniculate neuralgia (or nervus intermedius neuralgia of Hunt). This condition has been described as lancinating pains deep in the ear with a trigger zone in the external auditory canal.28 The existence of geniculate neuralgia as a separate entity also has been called into question. The similarities with the better-accepted diagnosis—glossopharyngeal neuralgia—are obvious.

The carotid artery, like most arteries of its caliber, is sensitive to pain. Distention or inflammation of nociceptive fibers in its walls tends to produce pain, which can seem localized to the neck or, quite commonly, be referred to a number of areas including the jaw, face and periorbital regions, and sinus areas.

Carotidynia

Carotidynia is pain that appears to emanate from the carotid artery. Raskin has described two types: acute and chronic recurrent carotidynia.29

The acute form seems to be a self-limited problem. The patient suffers from unilateral or, less often, bilateral pain that may be provoked by swallowing, coughing, or moving the neck. The pain may be pounding, sharp, or dull. The duration is from a few days to (rarely) several months. Corticosteroids have been advocated for relief. The differential diagnosis includes giant cell arteritis and carotid dissection. The evaluation of such complaints should include an erythrocyte sedimentation rate (ESR) and MRI or MRA to evaluate the anatomy of the carotid arteries.

Chronic recurrent carotidynia is felt to be a manifestation of migraine. This condition, like migraine, seems to be more common in women. Pain occurs in the jaw, cheek, or periorbitally. The frequency of attacks is quite variable. Pain may be dull and continuous, or sharp, or throbbing. An association with dental extraction has been speculated. Drugs effective in the prophylaxis of migraine are effective in this condition. Examples include propranolol, methysergide, and amitriptyline. It should be noted that if one examines a migraineur during an attack, the carotid artery is usually found to be tender.

Carotid Artery Dissection

Arterial dissection is caused by penetration of blood into the arterial wall with resultant narrowing of the vascular lumen, sometimes even leading to complete occlusion. Carotid artery dissection may be spontaneous (no discernible inciting event), posttraumatic, or the result of underlying disease of the vessel (such as fibromuscular dysplasia, Marfan’s syndrome, cystic medial necrosis).

Carotid dissection is a frequently recognized cause of stroke in the young, especially in migraineurs.30 Clinically, patients complain of neck pain radiating to the cheek or periorbitally. The pain may be dull, sharp, or throbbing. Uncommonly, the headache is bilateral. Focal ischemic symptoms (transient ischemic attack or stroke) may ensue as a result of loss of blood flow or embolization from the tip of the clot. There may be an ipsilateral oculosympathetic paresis (ptosis, miosis) resulting from involvement of the internal carotid plexus. Bruits may be appreciated, either subjective or objective, or both.31 MRI and MRA may, in some cases, be as sensitive as angiography for the detection of this lesion.32 Carotid ultrasound and transcranial Doppler, although less effective for initial detection, are excellent noninvasive methods for studying the resolution of the dissection, which may occur over weeks to months.33 Treatment may involve antiplatelet agents, heparin, or warfarin. An urgent neurologic consultation is indicated.

Facial pain may occur after trauma. Although bullet wounds and other head injuries may trigger pain, surgery is also a cause. Facial pain may occur after maxillofacial surgery, orbital enucleations, sinus procedures, and dental procedures. Dental procedures may trigger a variety of syndromes, including neuralgia and facial migraine.

In all cases of posttraumatic facial pain, a careful search should be made for underlying pathology, especially dental. Some patients manifest constant burning pain, occasionally with tingling and intermittent stabbing. With trophic changes, edema, and redness, reflex sympathetic dystrophy (complex regional pain syndrome, type 1) should be suspected.34 In patients who complain of significant burning pain, sympathetic blockade of the stellate ganglion may be effective, even without obvious complex regional pain syndrome.

Treatment of posttraumatic facial pain can be challenging. Brief lancinating pains may respond to agents used in treatment of neuralgias (see Table 25-1). Amitriptyline may reduce pain and lessen associated depression. Other agents useful for treating migraine headaches have been employed empirically for symptomatic benefit. Fortunately, posttraumatic facial pain is a self-limited condition, generally resolving spontaneously within several years.

In some cases, direct trauma to superficial facial nerves, such as the supraorbital and supratrochlear nerves, can lead to persistent lancinating or, less typically, aching pain. Neuroma formation is a postulated mechanism, and local nerve block with lidocaine or bupivacaine can be both diagnostic and therapeutic. We have found percussion tenderness over the nerve in question to be particularly helpful in predicting response to local nerve block.

Acute sinusitis, maxillary sinusitis being the most common, may cause facial pain. The pain may be felt in the cheeks, upper teeth, or bifrontally. The discomfort may be dull and continuous, throbbing, or sharp. The differential diagnosis includes migraine, which is often misdiagnosed as “sinus headaches.”

To make a diagnosis of acute sinusitis as a cause of facial pain, positive findings should be present. On examination, there is tenderness to percussion, often with a purulent nasal discharge, and fever. Laboratory evaluation may show an elevated ESR and white blood count. Sinus imaging (x-rays or computed tomography scan) should be abnormal. Without these findings, alternate diagnoses should be sought. Sphenoid sinusitis can be less obvious, and referred pain can include periorbital and maxillary regions.

The acute condition often responds to decongestants and simple analgesics. More severe, progressive cases require antibiotics. Refractory cases are candidates for ENT consultation for surgery. Chronic sinusitis is rarely a cause of headaches and facial pain.

Oromandibular conditions as a cause of pain are discussed in detail in Chapter 26. Dental pains are an extremely common cause of facial pain. Specific inquiry regarding prior dental procedures should be made of all patients. Trigeminal neuralgia has been associated with ipsilateral dental pathology. The presence of provocative factors, such as chewing, or the effect of hot or cold liquids may provide useful clues. The teeth and temporomandibular joints should be carefully evaluated in cases of facial pain. Temporomandibular joint syndrome (limitation of jaw movement, crepitus in the joint, and pain with use) may cause pain in the temple, jaw, and neck. Dental or oral surgical consultation may be extremely useful.

Before declaring a diagnosis of dental or sinus disease as the etiology of facial pain, positive evidence must be sought. Many patients with primary headache syndromes have been subjected to numerous dental or surgical procedures, because of where their pain was located, not because of truly local disease.

The International Headache Society (IHS) definition of atypical facial pain is “persistent facial pain that does not have the characteristics of the cranial neuralgias classified above and is not associated with physical signs or a demonstrable organic cause.”35 Patients, usually women, present with unremitting facial pain, usually unilateral at onset, sometimes spreading bilaterally. The neurologic examination is, by definition, normal.

Raskin has justly pointed out that a better designation for this entity is “facial pain of unknown cause.”36 In some cases, serious underlying pathology may not declare itself initially. For example, nasopharyngeal carcinoma and other cancers have eventually been implicated in some cases. Only when a neurologic deficit appears (e.g., cranial nerve palsies) may the real diagnosis become evident. Lung cancer has been another reported cause (due to referred pain presumably via CN X).37 Occult dental pathology, such as infected cavities of maxillary and mandibular bones at the sites of previous extractions, is another consideration. Dental evaluation with injection of anesthetic followed, when effective, by curettage and antibiotics has cured some sufferers.38

Many cases have been labeled psychogenic. This may be appropriate if there is evidence for somatization disorder, conversion disorder, or somatic delusions, but probably “psychogenic” is an overused label. Certainly, many patients have concomitant depression, which should be addressed.

All patients presenting with idiopathic facial pain require a careful history and examination. Dental consultation to rule out occult pathology is important. An MRI scan of the head, with attention to the base of the brain is indicated. A chest x-ray, perhaps with a chest CT scan, may help rule out lung cancer, especially in smokers. Serial examinations may be required as the clinician looks for an occult process to declare itself.

The treatment of idiopathic facial pain is extremely challenging. Burning pain may respond to stellate ganglion blocks. Heterocyclic antidepressants may lessen pain as well as help coexistent depression. Behavioral medicine measures, such as biofeedback and cognitive behavioral therapy, may be tried.

(Clearly, “idiopathic facial pain” is a more appropriate designation than “psychogenic” or “atypical.” A multidisciplinary approach to both diagnosis and treatment yields the best results.)

Primary (Facial) Headache

It should be remembered that migraine, cluster headache, and other so-called headaches (such as chronic paroxysmal hemicrania) may present mainly in the face. Many patients diagnosed with “sinus headaches” are, in fact, migraineurs. Careful attention to details of the history and examination should clarify the diagnosis. Patient reports revealing a family history, trigger factors, or the presence of an aura point toward migraine as the etiology.

Likewise, cluster headache and other short-lasting headaches may present with pain mainly in the face rather than peri- or retro-orbitally. The clarification of the duration of the episodes, plus associated autonomic features (ptosis, rhinorrhea, lacrimation) is diagnostically helpful.

Jabs and Jolts

This head pain syndrome has many names; the IHS terms it idiopathic stabbing headache. Other authors refer to it as ice-pick headache. This is a primary headache syndrome with paroxysmal pain lasting up to 10 seconds, but usually less than 2 seconds. Like trigeminal neuralgia, it may occur in volleys.39 Pain is generally multifocal and can involve the face as well as the head, with the orbital region most commonly affected. The frequency of these sudden brief pains is quite variable, from rare isolated jolts, to 50 or more per day.

These pains may occur as a separate, primary head pain condition or in association with other disorders, including migraine, giant cell arteritis, cluster headache, chronic paroxysmal hemicrania, or even tension-type headache. The differential diagnosis of jabs and jolts syndrome includes SUNCT (see following section), and trigeminal neuralgia. Jabs and jolts syndrome usually responds to treatment with indomethacin. Trigeminal neuralgia often has trigger points and responds much more readily to carbamazepine or other appropriate therapies (see Table 25-1).

SUNCT

Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) is a rare syndrome. The brief pain (usually 15 to 120 seconds in duration) is typically near the eye, but may occur in the temple or face. Multiple (up to 100) episodes occur daily, sometimes with other autonomic associations (ptosis, rhinorrhea).

The pain is almost always unilateral, and far more common in men. Neck movements trigger the pain in some patients. There are primary and secondary (symptomatic) forms. Secondary causes of SUNCT include cerebellopontine angle arteriovenous malformation.40 Clinical similarities with trigeminal neuralgia and cluster-tic syndrome are apparent. Because secondary trigeminal neuralgia and SUNCT can be caused by cerebellopontine angle arteriovenous malformations, it may be that microvascular decompression could alleviate other SUNCT cases. MRI is indicated in the evaluation of SUNCT. To date, there is no known effective therapy of primary SUNCT.

Patients presenting with facial pain often represent a diagnostic and therapeutic challenge. The number of pain-sensitive structures in the face is impressive, and many different entities share similar presenting symptoms. However, close attention to the fundamentals of thorough history taking and examination maximize diagnostic accuracy, the foundation upon which rational treatment rests.