Secondary Headaches Due to Organic Causes

Introduction

The International Classification of Headache Disorders (ICHD-3) classification of headache disorders divides headaches into primary and secondary, with the latter being headaches caused by an identifiable underlying (secondary) cause. Nine secondary headache categories are given in ICHD-3 (see Table 1-1), each with further subdivisions covering the wide spectrum of secondary causes. An older classification distinguished between secondary headaches due to organic (i.e., non-mechanical or injury-associated) headaches and post-traumatic headache (PTH). In this section, we survey some of the more common types of secondary headache due to organic causes, while PTH is discussed in Post-traumatic Headache. Headaches discussed here include those due to:

• Space-occupying lesion (tumors, hematomata, intracranial hemorrhage, brain abscesses, arteriovenous malformations)
• Infection
• Low cerebrospinal fluid (CSF) pressure
• Cranial arteritis
• Major neuralgias.

As with other types of headache, diagnosis of secondary headaches is based on:

• History
• Physical examination
• Laboratory results
• Radiologic examinations.

All patients must undergo careful and thorough examinations to rule out these possibly morbid causes. Selection of therapy is entirely dependent on the cause of the headaches.

Physicians should always be alert to recent onset of headaches starting in a patient age 40 or older. They should also be vigilant in evaluating patients who have experienced a recent change in a headache pattern that had been consistent for many years. The physician should also be wary of the presentation of recent onset of headache in a patient with a prior history of cancer. To help physicians decide which patients require further work-up to diagnose potential secondary headaches with high morbidity and mortality, the concept of “red flags” – symptoms and conditions whose presence requires further imaging and/or laboratory testing. One proposed list of red flags – the SNOOP10 list – is shown in Table 6-1. A complementary concept of “green flags” – symptoms and conditions whose presence suggests no further work-up is necessary – has also been developed, but has not yet been validated as a useful approach.

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Headaches Due to Space-Occupying Lesions

A headache produced by an intracranial lesion is usually attributed to:

• Inflammation
• Traction
• Displacement of the pain-sensitive structures of the head, most often the blood vessels.

The term “traction headache” is based on the displacement of these structures as a result of traction. The following pattern is demonstrated in headaches resulting from intracranial lesions:

• A steady, nonthrobbing, deep, dull ache
• The headache can awaken the patient from a sound sleep
• The headache is intermittent, although it can be continuous in some patients
• The headache is rarely as intense as the headache associated with fever
• Exertion can trigger or exacerbate the headache
• The frequency and duration will progressively increase.

Tumor

Tumor-associated headaches are classified in the ICHD-3 as “Headache attributed to intracranial neoplasm” – the diagnostic criteria are presented in Table 6-2. These headaches are common, occurring in 32% to 71% of patients with intracranial tumors. Headache is always a cardinal sign of rapidly increasing intracranial pressure. However, a slow-growing tumor may cause a dull, transitory headache that is relieved by simple analgesics. Some generalizations can be made about headache due to brain tumor:

• In about one third of patients with headache due to brain tumor, the pain overlies the tumor; However, in the remaining patients, the headache may be referred from a distant intracranial source
• A tumor below the tentorium will frequently cause occipital pain and cervical muscle spasm
• A tumor above the tentorium will often manifest as a headache at the vertex or in the frontal regions
• If the tumor is at the midline, exertion such as coughing, straining, or sudden head movement can exacerbate the headache
• A tumor that is chiasmatic at the sella may cause the pain to be referred to the vertex
• A posterior fossa tumor usually manifests as a headache
• If the tumor is hemispheric, the headache is usually felt on the ipsilateral side
• A lesion in the pituitary fossa often causes a frontal and bitemporal headache, which is bursting in character
• Pain is often present behind the ear in a cerebellopontine angle lesion.

Headache rarely occurs as an initial symptom with several lesions, such as craniopharyngiomas and hypophysial adenomas. However, it can be an initial manifestation with other tumors, such as:

• Meningiomas
• Gliomas.

Meningiomas (which compress the brain from the outside) typically cause seizures, focal symptoms and/or progressive impairment of intellectual function before they will produce a headache. The slow growth and bone invasion of these lesions probably counter the effects of their proximity to pain-sensitive structures.

Gliomas and large infiltrating tumors can advance throughout one hemisphere without causing headache because the position of the large vessels is not disturbed. These lesions may cause headache early due to their rapid growth and possible occlusion of the lateral, third and fourth ventricle.

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Hematomas

In the ICHD-3, headaches associated with hematomas fall under the “Headache attributed to non-traumatic intracranial hemorrhage” category, a subcategory of “Headache attributed to cranial and/or cervical vascular disorder.” There are no characteristic symptoms that will help the physician differentiate an acute subdural hematoma from a cerebral contusion or laceration. Chronic subdural hematomas may occur after trivial or closed-head trauma. Although headache is a prominent symptom of subdural hematoma, the diagnosis will be difficult to establish if the patient presents in a confused state. Other symptoms of acute subdural hematoma are:

• Drowsiness
• Confusion
• Slowness in thinking
• Occasional agitation.

These symptoms will progressively intensify. Focal and lateralizing signs occur late and are less prominent than the disturbance in consciousness. In both the subacute and chronic types of subdural hematomas, the clinical picture resembles the presentation of progressive supratentorial mass. Its characteristics include:

• Hemiparesis
• Focal seizures
• Choked discs.

In distinguishing the types of subdural hematomas, the following should be considered:

• Acute subdural hematomas usually become clinically significant within 48 hours of injury.
• Subacute subdural hematomas will become clinically significant within 2 to 14 days after injury.
• Chronic subdural hematomas will become clinically significant ≥14 days after injury.

Headache is not an important clinical sign in acute subdural hematoma since the patient is often unconscious upon admission after severe head injury. Headache may or may not be present in patients with subacute subdural hematomas. Surgical intervention is usually indicated in these patients since these hematomas rarely absorb spontaneously.

Headache is the most common complaint of patients with the chronic form. The injury experienced by these patients is usually mild to moderate, and a history of injury may be absent. If the headache is not treated, progressive signs of brain compression may occur and are often nonlocalizing and nonspecific. These symptoms include:

• Apathy
• Inappropriate behavior
• Confusion.

Sudden head movements or jolts can exacerbate the headache. Head tapping can be used as a clinical sign. In elderly patients, chronic subdural hematoma may present as mental deterioration without headache.

Epidural hematoma most often presents after injury to the middle meningeal artery on the undersurface of the temporal bone. As the injured patient progresses from a complaint of headache to restlessness, the clinician should be concerned about the possibility of expanding intracranial mass. As the lesion increases in size and produces brain compression and decreasing levels of consciousness, the patient may be combative and restless and not necessarily complain of headache.

Subarachnoid Hemorrhage

After head injury, bleeding into the subarachnoid space may occur. It is the most common cause of intracranial hemorrhage and may be secondary to intracerebral hemorrhage. The bleeding may also occur spontaneously from:

• Preexisting aneurysms
• Vascular malformations
• Rarely:
  • Blood dyscrasia
  • Intracranial tumor
  • Some form of arteritis.

Headache due to subarachnoid hemorrhage from organic causes is classified in the ICHD-3 as “Acute headache attributed to non-traumatic subarachnoid hemorrhage,” a sub-category of “Headache attributed to cranial and/or cervical vascular disorder.”

The clinical presentation for all subarachnoid hemorrhages is:

• Acute onset of severe headache
• Frontal or diffuse pain radiating to the neck, back and even the lower extremities
• Pain described as “the worst headache ever” by the patient
• Within minutes, a variable degree of mental confusion may occur.

Blood in the subarachnoid space causes a chemical meningitis. The “meningeal signs” include:

• Stiff neck
• Kernig’s sign (inability to extend the leg with the thigh flexed)
• Mildly elevated temperature, pulse and blood pressure.

Signs and symptoms following a subarachnoid hemorrhage are largely due to vascular spasm resulting in brain ischemia, infarct and cerebral edema. The cause of a subarachnoid hemorrhage may be suggested by:

• A history of progressive neurologic deficits
• A history of seizure disorder
• Auscultating a cranial bruit.

These signs would suggest an intracranial vascular malformation. Aneurysms may be symptomatic before hemorrhage and cause varied degrees of headache or extraocular paresis by compression of the third cranial nerve. Easy bruising or prolonged bleeding from lumbar or venipuncture sites may indicate blood dyscrasia.

Generally, an unruptured aneurysm does require surgical intervention. The performance of invasive diagnostic procedures should be minimized in cases of unruptured aneurysms.

Brain Abscess

In the ICHD-3 classification, headache due to brain abscess falls under the “Headache attributed to localized brain infection” category, which also includes other infections, including subdural empyema and infectious granuloma. The signs and symptoms of brain abscess are comparable to those of an expanding intracranial lesion. Cerebral edema is significant in the pathophysiology. Early symptoms include:

• Headache
• Nausea
• Vomiting
• Seizures.

Brain abscess may be due to:

• Active or acute bacterial infection involving extracranial sites
• Extracranial fungal and parasitic infections.

Early diagnosis can expedite treatment measures and be established by specific procedures, including:

• Chest X-ray to rule out lung abscess
• Electroencephalogram (EEG) to rule out characteristic focal high-voltage waves
• Complete blood count
• Computed tomography (CT) scan or magnetic resonance imaging (MRI).

Parenchymal abscess is often associated with disease of adjacent nasal and aural structures, and the patient may experience headache from the latter source before brain tissue becomes involved. A headache will not occur until the abscess is of adequate size to cause traction and displacement, usually 2 months after the parenchymal breakdown has started. The abscess will then be of maximal size, walled off and will have become a cystic tumor that gradually developed in size from accumulation of fluid. The headache of brain abscess is usually associated with:

• Leukocytosis
• Fever
• Pleocytosis.

Brain abscess from ear infections may occur either above or below the tentorium. Evidence of brainstem embarrassment occurs early in the illness and includes:

• Hiccuping
• Vomiting
• Occipital headache.

Epidural abscess is often present in headache associated with sinus disease and osteomyelitis of the adjacent bony wall, and which persists after drainage of the sinus. These epidural abscesses occur in the frontal region adjacent to the diseased sinus, which may be:

• Frontal sinus
• Ethmoid sinus
• Sphenoid sinus.

Also, the abscess may occur after osteomyelitis of the mastoid bone in the postauricular occipital region.

Arteriovenous Malformations

Arteriovenous malformations (AVMs) vary in size from a mass of tortuous vessels occupying most of one cerebral hemisphere to barely visible blemishes involving any part of the brain. Although an AVM may be present at birth, the symptoms may not manifest until adolescence or early adulthood. Occasionally, the symptoms may never occur. In the ICHD-3, “Headache attributed to AVM” is a subtype of “Headache attributed to unruptured vascular malformation.”

The AVMs attain clinical significance depending on the major neurologic complications associated with or caused by the malformation, including:

• Subarachnoid hemorrhage
• Seizure disorder
• Progressive neurologic deficits.

The presence of AVMs can be established by Computed tomography (CT) scanning or Magnetic resonance imaging (MRI).

Infections

Headaches are a common symptom of both intracranial (brain or meningeal) and systemic (typically viral) infection. In the ICHD-3, “Headache attributed to infection” is a major type of secondary headache, divided into “Headache attributed to intracranial infection” and “Headache attributed to systemic infection,” both of which are further subdivided into specific infection types (e.g., bacterial meningitis or meningoencephalitis, viral encephalitis, systemic viral infection, etc.).

Headache associated with fever and stiff neck should always alert the clinician to the possibility of meningitis. Meningitis may be caused by a variety of sources:

• Viral infection
• Bacterial infection
• Fungal infection
• Blood disorder
• Disease that metastasizes to the meninges, such as lymphoma.

The symptoms of meningitis are determined by:

• Type of structure involved
• Degree of inflammation
• Location of the inflammatory process.

The headache associated with meningitis is:

• Severe and global
• Throbbing in nature
• Associated with:
• Nausea
• Vomiting
• Photophobia
• Stiff neck
• An alteration in the level of consciousness (occasionally)
• Occasional occurrence of a rash.

It may be due to the extreme reflex spasm of the cervical musculature. In the early stages, with mild to moderate headache and minimal neck stiffness, the cause may be attributed to influenza. Lumbar puncture will establish the diagnosis. A morbid outcome of this disorder may be prevented with:

• Early diagnosis
• Aggressive antibiotic therapy.

Four other conditions may be mistaken for meningitis. The common element in these disorders is severe headache and/or the presence of resistance to anterior flexion of the neck. These conditions include:

• Retropharyngeal abscess, particularly in children
• Superior longitudinal sinus thrombosis
• Subarachnoid hemorrhage
• Meningism that may accompany certain infections, such as typhoid fever.

Low Cerebrospinal Fluid–Pressure Headache

In the ICHD-3, “Headache attributed to low cerebrospinal fluid (CSF) pressure” is a major subtype of “Headache attributed to non-vascular intracranial disorder.” The most common form of low CSF-pressure headache is the postpuncture headache. This headache may be mild or severe and occur within a few hours to several days after lumbar puncture. The headache duration varies from a few days to several weeks. One in four patients undergoing lumbar puncture will experience these headaches. The pain of these headaches is described as:

• A dull, deep ache or throbbing
• Bifrontal or suboccipital
• May be associated with moderate neck stiffness.

The most recognized characteristic of these headaches is their occurrence when the patient is erect and their disappearance when the patient is horizontal. Pain may be exacerbated by shaking of the head. These headaches are usually resistant to all forms of treatment except bed rest in the horizontal position and the passage of time. The cause is attributed to the loss of CSF secondary to leakage through the dural hole.

Other causes of low CSF–pressure headache have been identified:

• Primary intracranial hypotension
• CSF rhinorrhea
• Inappropriate ventricular shunt.

The appropriate therapy for these disorders is correction of the cause.

Chronic or Marked Increases in Intracranial Pressure

Two disorders are identified as causing these headaches:

• Acute hydrocephalus
• Idiopathic intracranial hypertension.

Acute hydrocephalus occurs in patients with ventricular obstruction or with shunt malfunction in a treated hydrocephalic patient. The associated headache – classified in the ICHD-3 as “Headache attributed to intracranial hypertension secondary to hydrocephalus” – is severe and followed by visual disturbances. Emergency ventricular drainage must be completed rapidly to prevent permanent neurologic deficit or death.

No specific cause can be established for idiopathic intracranial hypertension (IIH), previously also known as benign intracranial hypertension, although there is evidence of increased intracranial pressure. In the ICHD-3, “Headache attributed to IIH” is a subtype of “Headache attributed to increased CSF pressure.” Because this disorder can sometimes resolve spontaneously, it is described as benign. However, permanent visual loss may also occur. Etiologic factors that may be involved include:

• Menstrual dysfunction
• Adrenal deficiency
• Corticosteroid therapy
• Hypoparathyroidism
• Vitamin A intoxication
• Tetracycline administration in infants
• Poisons
• Insecticides.

The one finding common to all cases of IIH is papilledema. Other symptoms may include:

• Generalized headache
• Giddiness
• Vomiting
• Blurred vision in some cases
• Rarely, seizures
• Striking appearance and feeling of well-being.

Pseudotumor is one of the syndromes associated with IIH. The symptoms include:

• Nonspecific, intermittent headache
• Condition present for several weeks or months before medical advice is sought.

There is a high incidence of this disorder in young women with obesity. The resting spinal pressures vary from 220 to 600 mm of water. The spinal fluid is always:

• Clear
• Colorless
• Exhibiting no abnormality of cellular or chemical constituents
• Protein count of the CSF is unusually low.

Most patients respond well to acetazolamide. Some patients will require shunt placement.

Cranial Arteritis

Headaches due to cranial arteritis are related to inflammatory processes of undetermined cause, which are limited to the cranial arteries. In the ICHD-3, “Headache attributed to arteritis” is classified as a subtype of “Headache attributed to cranial and/or cervical vascular disorder,” a major secondary headache category. In the inflammatory process associated with these headaches, the elasticity of the arterial walls seems to fade as the tissues appear frayed or fragmented. Giant cells within the vessel walls are most numerous in the region of the deranged internal elastic lamina. The pain is evoked by inflammatory responses that include those of the pain-sensitive structures of the head. The headaches occur coincidentally with the inflammatory process and once the disease is managed, the headaches do not recur.

Giant cell arteritis (GCA) used to also be referred to as temporal arteritis. The ICHD-3 criteria require, among other criteria (such as diagnosis of GCA) two of the following criteria to establish the diagnosis of “Headache attributed to GCA”:

• Headache has developed in close temporal relation to other symptoms and/or clinical or biological signs of onset of GCA, or has led to the diagnosis of GCA
• Headache has either (or both): a) significantly worsened in parallel with worsening of GCA; b) significantly improved or resolved within three days of high-dose steroid treatment
• Headache is associated with scalp tenderness and/or jaw claudication.

Diagnosis of GCA is confirmed with a temporal artery biopsy that will demonstrate giant cell arteritis.

The clinician should be alert to this disorder in patients over age 50 who are experiencing a recent onset of headache. The clinician should obtain a sedimentation rate by the Westergren method in all patients over age 50. In patients with GCA, the sedimentation rate is usually >60 mm/hour, although some patients may present with normal sedimentation rates. Early diagnosis and treatment are essential to prevent the irreversible blindness associated with this disorder.

The headache is probably the most prominent presenting complaint. Patients will describe it as:

• Very severe in intensity
• Often unilateral, localized over one temple; however, the pain may be bilateral
• Throbbing or boring
• Occasionally, a stabbing sensation across the temporal area
• Often having a burning component.

The headache is often worse when the patient lies flat in bed and decreases in severity when the patient is sitting upright. Applying pressure to the common carotid artery may diminish the pain, and stooping may exacerbate the pain.

Many patients will present with a history similar to that of rheumatoid arthritis, and giant cell arteritis is often associated with polymyalgia rheumatica. Other symptoms include:

• Night sweating
• Aching of the joints
• Fever
• Weight loss.

The patient may experience pain or discomfort on opening the mouth as well as pain and stiffness in the area of the temporomandibular joint. Intermittent claudication of the jaw may be triggered by excessive chewing, thus resulting in weight loss. Facial swelling and red nodules may be observed over the temporal region. The visual symptoms may develop ≥5 months after the symptoms manifest. These symptoms are believed to be caused by a decrease in the blood supply to the optic nerve, and include:

• Ophthalmoplegia
• Diplopia
• Ptosis
• Other symptoms of ocular motor paralysis.

Resultant ischemia of the optic nerve occurs in about 50% of untreated patients. Many patients with untreated giant cell arteritis may experience transient visual blurring before the onset of the irreversible blindness.

Because imminent treatment is essential to prevent the blindness, therapy with corticosteroids should be initiated before receiving the results of the biopsy. The usual starting dose is prednisone 40 mg to 60 mg daily. Maintenance dose is 10 mg to 20 mg per day, with the sedimentation rate used as a guide in administering the drug. The disease process usually disappears 6 to 8 months after initiation of therapy.

Before the major manifestation of giant cell arteritis, pain may occur in other areas including:

• Teeth
• Jaw
• Ear
• Zygoma
• Nuchal area
• Occiput.

These symptoms indicate involvement of other branches of the external carotid artery, particularly the external and internal maxillary arteries. Other arteries may be involved, including:

• Major vessels of the aorta
• Coronary arteries
• Arteries of the limbs.

The large and medium-size arteries are the principal sites of the inflammatory process.

Major Neuralgias

Major neuralgias include trigeminal neuralgia (previously also known as tic douloureux) and glossopharyngeal neuralgia. Characteristically, trigeminal neuralgia:

• Consists of episodic, recurrent pain
• Is unilateral in location, more common on the right side of the face
• Rarely begins before age 50
• Has a female-to-male ratio of 2:1.

The ICHD-3 classifies trigeminal neuralgia as a subtype of “Painful lesions of the cranial nerves and other facial pain” and divides it into three subtypes – classical (developing without a cause other than neurovascular compression), secondary (caused by an underlying disease, such as multiple sclerosis) and idiopathic (no significant abnormalities on electrophysiological tests or MRI). Trigeminal neuralgia rarely occurs before age 30 unless the patient has concomitant multiple sclerosis.

The pain is of high intensity and particularly affects trigger zones that are increased areas of sensitivity on the face, especially above the nares and mouth. When stimulated, these areas will trigger the attacks, often by trivial stimulation. Because of these trigger zones, the patient will demonstrate the “avoidance mechanism.” This mechanism is a major diagnostic clue. The patient with trigeminal neuralgia will typically avoid washing the face, shaving, chewing or any other action that will stimulate a trigger zone. Because many trigger zones are located in or near the oral cavity, patients with trigeminal neuralgia will lose a great deal of weight.

The distribution of trigeminal neuralgia affects the second or third divisions of the fifth cranial nerve and may radiate to the first division late in the disease. Patients have described the pain as follows:

• The occurrence of short, sharp, momentary bursts, like electric shock or a rapid repeating rifle
• Excruciating and severe enough to cause the patient to cry out or twitch
• Periods of remission are interrupted by attacks caused by stimulation of the trigger zones
• High-intensity jabs last <20 to 30 seconds, with short-term remission periods lasting a few seconds, followed by another jab of pain.

Because attacks of trigeminal neuralgia last only briefly, management is focused on preventive rather than abortive treatment. The treatment of choice for trigeminal neuralgia is anticonvulsant therapy – preferably carbamazepine or oxcarbazepine as first-line agents. These drugs will reduce the sensitivity of the trigger zones as well as relieve the pain, often dramatically, within hours after initiation of therapy. Table 6-3 reviews the agents used for trigeminal neuralgia. Initial therapy with carbamazepine is 200 mg two to four times per day. If this dose is well tolerated and if the pain is quickly relieved, the drug may be continued for an indefinite period, depending on the course of the disease. Severity of the pain will determine the titration of the drug. Maintenance dose is usually 200 mg per day. For oxcarbazepine, the initial dose is 300 mg (equivalent in efficacy to carbamazepine 200 mg) four times per day. Lamotrigine has been shown to be useful as add-on therapy to increase the efficacy of carbamazepine and oxcarbazepine, or for patients who cannot tolerate these agents. Gabapentin and pregabalin may also be used, but are known from clinical experience to be less effective than the first-line agents, although they are associated with fewer adverse reactions.

If the symptoms persist, phenytoin may be added to the regimen in doses up to 400 mg per day. Chlorphenesin should be considered if the patient is refractory to the first two agents. This drug is prescribed in doses of 400 mg three to four times daily. Surgical intervention may be considered if the patient has reached a three-drug treatment level. Baclofen has been used successfully in refractory trigeminal neuralgia at doses of 15 mg to 90 mg (75 mg as initiating dose) per day, and is especially useful for patients with concomitant multiple sclerosis. Finally, botulinum toxin A, at 25-100 units applied over the pain area, has been shown to improve pain outcomes in randomized controlled trials.

The final stage of therapy may be surgical intervention (Table 6-4). Surgical procedures should only be considered after other methods have failed and include:

• Invasive non-ablative methods (microvascular decompression)
• Invasive ablative methods (balloon compression, radiofrequency thermocoagulation, glycerol rhizolysis)
• Non-invasive ablative methods (stereotactic radiosurgery).

In general, microvascular decompression is the procedure of choice for classical trigeminal neuralgia, i.e., when the cause if neurovascular compression, since it has the best long-term efficacy in these cases. However, microvascular decompression is a major procedure that involves a craniotomy, and is therefore not indicated for patients with significant comorbidities. For idiopathic trigeminal neuralgia, ablative procedures are preferred. Of these, stereotactic radiosurgery (which focuses a radiation beam on the trigeminal root entry zone) is the non-invasive option. Invasive options include percutaneous ablation of the trigeminal ganglion or root by balloon compression (mechanically), radiofrequency thermocoagulation (thermally), and by glycerol injection (chemically), or internal neurolysis – a microsurgical procedure that dissects the cisternal portion of the trigeminal nerve into multiple nerve fascicles. The percutaneous ablative procedures appear to have equal efficacy, but often require repetitive treatments. Internal neurolysis has good long-term efficacy, but carries a high complication risk.

Glossopharyngeal neuralgia is another subtype of the ICDH-3 “Painful lesions of the cranial nerves and other facial pain” category. It is similar to trigeminal neuralgia, except that the symptoms manifest from the anatomic base of the glossopharyngeal nerve. This pain is usually located in the:

• Pharynx
• Tonsils
• Ear.

It can be triggered by swallowing, yawning, or eating. Treatment is similar to that of trigeminal neuralgia.

Certain atypical neuralgias may present that cannot be categorized according to the symptomatology and are not associated with trigger zones. The pain does not occur as jabs but rather as continuous pain. Atypical facial pain can be described as:

• Steady diffuse aching, not neuritic
• Continuous for hours, days, months
• No trigger zones
• Often localized tenderness
• May have a vascular component
• May radiate to other regions of the face, neck, or cranium.

Autonomic symptoms may also occur and include:

• Cutaneous pallor
• Sweating
• Flushing
• Rhinitis.

To alter the responses to autonomic stimuli, the patient may respond to β-blockers, including propranolol. Due to the chronic nature of atypical facial pain, the patient has the potential to develop depression or habituation problems. A multidisciplinary approach to this patient, possibly in an inpatient facility, may be indicated. Psychological testing and counseling may be required in patients with atypical facial pain. Treatment measures include:

• Nonhabituating analgesics
• Antidepressants
• Nerve blocks
• Transcutaneous electrical stimulation
• Biofeedback
• Psychological counseling.

Some patients will experience postinfectious neuralgia, particularly as a complication of herpes zoster of the face and head. This neuralgia most often affects the elderly. Most of these affected patients will demonstrate pain with involvement of the Gasserian (trigeminal) ganglion. In some patients, the ophthalmic division of the nerve is infected or present with a herpetic rash in the external auditory canal with facial palsy (Ramsay Hunt syndrome). Unfortunately, the pain of postherpetic neuralgia usually dominates the remaining years of these patients. The pain of postherpetic neuralgia has been described as:

• Steady and sustained
• Almost always unilateral
• Burning and aching
• Frequently interrupting sleep.

Diagnosis is usually obvious as the scars of the herpetic eruption are apparent, and trophic changes to the skin may result from this disorder. The pain of herpes zoster usually abates within 2 to 3 weeks, although the neuralgia may persist for months or years.

These patients also tend to develop depression and dependency problems. Tricyclic antidepressants may be beneficial, although dosage increases should be undertaken cautiously in elderly patients. The addition of a phenothiazine may be considered. These patients require reassurance and patience, and may need psychological counseling. Surgical intervention is not indicated in these patients.

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Temporomandibular Joint Disorder

Temporomandibular joint (TMJ) disorder is one of the more overly publicized and overly diagnosed disorders. In the ICHD-3, it is classified in the “Headache or facial pain attributed to disorder of the cranium, neck, eyes, ears, nose, sinuses, teeth, mouth or other facial or cervical structure” category of secondary headache. Symptoms of TMJ disorder include:

• Localized facial pain
• Limitation of motion of the jaw
• Muscle tenderness
• Joint crepitus.

The pain of TMJ disorder is usually localized in front of and behind the ear on the affected side. This pain may radiate over the cheek and face. X-rays of the jaw are usually normal. Due to the localized pain, the patient will almost always use the opposite side of the mouth for chewing, thus splinting the painful side. There is no evidence that hearing loss, damage to the cranial nerves, disturbances of equilibrium, development of Meniere’s disease, or difficulty with the eustachian tube is associated with TMJ disorder.

Pain relief of TMJ disorder should be directed toward abating muscle spasm. Mouth reconstructions are usually not indicated, and extensive oral surgery should not be undertaken unless other measures have failed. Simple analgesics and muscle-relaxant agents, such as chlorphenesin, may be beneficial. Nondrug methods, such as heat, hot packs and massage, may be beneficial. Dental splints and similar procedures may prove helpful.