Special Considerations

Two thirds of women with migraine primarily experience them just before or during menses. These headaches are often associated with other premenstrual dysphoric symptoms. Estrogen withdrawal most likely influences this common migraine subgroup.

Migraine may worsen early in pregnancy but tends to improve during the second and third trimesters, particularly in women with migraine primarily related to their menstrual cycle. Women with migraines that begin earlier in life generally experience a significant decrease in episode frequency after menopause.

Great care must be used in the evaluation and treatment of “migraine” headache in the elderly. The incidence of headaches as a manifestation of other illnesses significantly increases with age. In addition, the use of migraine medication may be more hazardous in the elderly.

Management and Therapy

There are two primary steps in the care of migraine patients: treatment of the acute headache and prevention of subsequent events. The initial goal is prompt pain relief without recurrence and minimal, if any, adverse effects. For mild to moderately severe nondisabling pain, oral nonsteroidal anti-inflammatory drugs (NSAIDs), acetylsalicylic acid (aspirin) or acetaminophen, are recommended for short-term treatment. Caffeine may enhance the effect of these various medications. If needed, antiemetics are often useful in conjunction with analgesics.

However, in patients with more severe disabling migraines, oral, injectable, intranasal, or quick-dissolve sublingual serotonin 1B/1D receptor agonist (“triptan”) preparations are the medications of choice. Their success is attributed to the multiple sites of action triptans have on the migraine cascade that include decreasing cortical hyperexcitability, decreasing tissue leakage of neuropeptides and blocking their dural neurovascular effects, tempering trigeminal afferent input, suppressing or down-regulating brainstem activation, gating thalamic pain response, and finally countering progressive vasodilation (Fig. 11-4). The rapidly acting triptans include almotriptan, eletriptan, rizatriptan, sumatriptan, and zolmitriptan. The longer acting triptans include naratriptan and frovatriptan. These preparations are also recommended for patients with milder migraines that are not initially disabling but are refractory to simple analgesics. Butalbital, ergotamine, and isometheptene/dichloralphenazone preparations are also commonly used for abortive therapy. When the above-mentioned therapeutic options are ineffective in patients with the most severe migraines or if those treatments are contraindicated, nonnarcotic treatments, such as ketorolac and antiemetics, are attempted. When these fail, opiate-category medications are often used, primarily in the emergency room. However, the possibility of sedation and, more importantly, subsequent overuse and dependence must be considered.

Figure 11-4 Pathophysiology of Migraine and Triptan Site of Action.

Prophylactic treatment, the other aspect of migraine therapeutics, is indicated for patients with frequent headache. Other indications for preventive therapies include poor response or adverse effects to abortive treatments, frequent need for medications with potential for abuse and dependency, or patient preference when even infrequent migraines significantly interfere with daily activities or responsibilities. Tricyclic antidepressants, β-blockers, calcium channel blockers, sodium valproate, gabapentin, and topiramate are the most frequently used migraine prophylactic medications. Experimentation with different agents may be needed to determine which medication is the most effective for any individual patient. The initial choice may be directed by existing comorbidities or considerations. For instance, difficulty with sleep may prompt prescribing medications that are more sedating and help sleep initiation. Topiramate may be preferable over valproic acid for those in whom weight gain is a concern. A concomitant mood disorder may prompt the use of antidepressants or medications with mood-stabilizing properties. β-Blockers may help in controlling coexisting hypertension in some patients but should be avoided in those with reactive-airway disease or in athletes. Elucidating and avoiding potential dietary and environmental triggers is an important part of nonpharmacological migraine prevention. Sleep deprivation, dehydration, and erratic meals are strong triggers, and proper general health habits should be encouraged.

Future Directions

Half of migraine patients report dissatisfaction with therapy, despite the above options. A comprehensive approach to treatment of migraine headache is important, and must also address contributing factors such as hypertension or other medical problems, mood and sleep disorders, psychosocial stressors, and excessive use of caffeine, alcohol, and nicotine. Avoidance of rebound headache from overuse of analgesics is essential. Adjunct treatment with certain vitamins and supplements (riboflavin or vitamin B₂ and magnesium sulfate), exercise, biofeedback, cognitive–behavioral management training, and acupuncture can also be beneficial. Botulinum toxin type A is showing promise for migraine prevention as are clonazepam and some of the newer antiepileptic medications.

Clinical Vignette

A 34-year-old man presents to his internist for evaluation of severe pain above and behind his right eye. The pain began a few days ago and is intermittent. It occurs several times a day, usually lasting for 30–60 minutes, and often awakens him at night. The pain is associated with ipsilateral tearing, conjunctival injection, and nasal congestion. Alcohol triggers or exacerbates the pain. His wife reports that he has been irritable and agitated. On exam, he has right-sided periorbital edema and mild ptosis. He reports having similar symptoms 2 years ago and is concerned because that episode lasted for several weeks.

Cluster headaches are much less common than migraines, to which they are unrelated, affecting only 0.1% of adults. However, they are usually more severe and debilitating and have been referred to as the “suicide headache.” Although cluster headaches are very distinctive and stereotyped, they tend to be underdiagnosed. Cluster headaches usually respond well to the appropriate therapy and, therefore, a very careful history that aids in making the correct diagnosis is important. They usually first occur in the third decade, affecting men more often than women, although the ratio has decreased over the past several years (male-to-female ratio is 2–4 : 1).

The distinctive clinical features, as summarized in the clinical vignette, assist in diagnosing cluster headaches (Fig. 11-5). The underlying pathophysiology is related to activation of the trigeminal vascular and parasympathetic systems. The first two divisions of the trigeminal pathway are more commonly involved. Recent positron emission tomographic scan studies by Goadsby and colleagues revealed activation of the medial hypothalamic gray matter, an area involved in the control of circadian rhythms. It is felt that dysfunction of neurons in this area leads to activation of a trigeminal-autonomic loop in the brainstem. These pathophysiologic mechanisms would explain the cardinal symptoms of cluster headache that include the episodic/circadian nature of the attacks, the distribution and quality of pain, and associated autonomic symptoms.

Figure 11-5 Cluster Headache and Chronic Paroxysmal Hemicrania.

According to the International Headache Society’s revised classification of cluster headache, there must be recurrent attacks of at least severe unilateral pain lasting 15-180 minutes. The pain must be orbital, supraorbital, and/or temporal. The headaches must be accompanied by at least one of the following: restlessness or agitation, ipsilateral conjunctival injection and/or lacrimation, nasal congestion and/or rhinorrhea, eyelid edema, forehead and facial sweating, ipsilateral miosis and/or ptosis. Attacks have a frequency of one every other day to eight a day. Finally, other causes must be ruled out.

Management and Therapy

Like migraine, the treatment algorithm includes short-term and preventive therapy. The two most effective abortive therapies for cluster headache are sumatriptan 6 mg subcutaneous and high-flow oxygen inhalation at 7–10 L/min for 15–20 minutes. Other triptan preparations, oral indomethacin three times daily, ergotamines (particularly intravenous dihydroergotamine), and intranasal lidocaine are often beneficial. Melatonin and intranasal capsaicin are showing promise for treatment of episodic cluster headache. Preventive therapy must also be used. Verapamil 240 mg daily is the drug of choice for prophylaxis of cluster headaches. Other beneficial drugs include sodium valproate, lithium, topiramate, short-term corticosteroids, and methysergide, although now in short supply. Ten percent of cluster headache patients develop chronic or relentless symptoms, and combined-drug therapy may be needed. Very rarely, and only in well-selected medically refractory patients, is surgical ablation or radiofrequency rhizotomy of the trigeminal nerve necessary.

Paroxysmal Hemicranias

These are unusual primary headaches—unilateral and short-lived (2–45 minutes), which occur in a chronic or episodic manner. Typically, the pain has a severe throbbing or boring quality and often recurs several times during the same day. These headaches are associated with ipsilateral cranial autonomic dysfunction but, unlike cluster headaches, occur more often in women than in men. Furthermore, these headaches have daily recurrences and tend not to conglomerate over a few days such as in cluster headaches. They usually respond well to 25–50 mg indomethacin 2–3 times daily for at least 48 hours. These headaches by definition are “indomethacin responsive,” and a trial is always warranted if there are no medical contraindications to its use (Fig. 11-5). There are reports of good response as well to acetazolamide. Calcium channel blockers, such as verapamil, are used for long-term prophylactic treatment.

Short-Lasting Unilateral Neuralgiform Headache Attacks with Conjunctival Injection and Tearing

This is a syndrome of strictly unilateral headache attacks with the pain confined to the ocular/periocular area. Most episodes are moderate to severe in intensity with a burning, stabbing, or electrical quality. The duration of the paroxysms usually ranges from 10 to 120 seconds. Prominent, ipsilateral conjunctival injection and lacrimation are present. Nasal stuffiness or rhinorrhea and ipsilateral forehead perspiration may also be present. In contrast to paroxysmal hemicranias, this headache syndrome predominates in middle-aged men and is not responsive to indomethacin. In fact, treatment efforts with numerous medications have been frustrating, with little or inconsistent responses. Lately, lamotrigine promises to be of some benefit.

Clinical Vignette

A 45-year-old man complains of daily headache for 10 years. His headache lasts all day but is worse on awakening. He notes a dull, sometimes pulsatile, moderate, bifrontal head pain with mild nausea that responds to an over-the-counter combination of aspirin, acetaminophen, and caffeine. He currently takes two of these pills every 6 hours around the clock while awake. He has had numerous cranial scans and consultations for this problem. There is a longstanding history of intermittent headaches beginning in childhood. He is placed on a weaning schedule with eventual discontinuation of over-the-counter headache medications over 4 weeks and is advised to avoid all other forms of caffeine. His daily headache initially worsens, but then gradually improves. When seen in follow-up, he is improved and reports only intermittent, thrice-weekly tension-type headache. He is counseled on the hazards of medication and caffeine overuse and started on amitriptyline for prophylaxis.

The heterogeneous nature and numerous comorbidities associated with chronic daily headache represent a diagnostic and therapeutic challenge. The syndrome of chronic daily headache may evolve from a variety of primary and/or secondary headache types, with tension and migraine headaches being the most common. Frequent headaches of any type may lose their clinical distinctiveness and lead to an ill-defined vague head pain whose characteristics defy specific definition. The clinician must then uncover any previous history of intermittent or episodic headaches that may have transformed over time. Anxiety, mood, and sleep disorders are just a few of the common comorbid conditions. Medication overuse frequently contributes to chronic daily headache Almost any short-acting analgesic may lead to “rebound” headaches, but vasoactive medications such as caffeine, triptans, and ergotamines are the most likely to cause this phenomenon. Special care must be taken when patients overuse substances whose abrupt withdrawal may prove dangerous or life threatening, including butalbital, benzodiazepines, and opioids.

The treatment of chronic daily headache first requires that overused substances are weaned or discontinued. Many patients may resist this intervention out of fear that headaches will worsen. Careful education is necessary to explain the association between medication overuse and the chronic headaches. Support mechanisms to control anxiety and a clear management plan for headache recurrence are needed. Comorbid etiologic factors—depression, psychosocial stresses, and poor sleep—require attention. Nonpharmacologic interventions include emotional support, counseling, physical therapy, relaxation techniques, heat, and massage. The choice of prophylactic medication should be based on the underlying headache type and comorbidities. For example, the patient above suffers from transformed tension-type headache, and therefore amitriptyline is recommended. The use of botulinum toxin type A as a prophylactic agent for chronic daily headache has not been supported by randomized control trials. A recent trial has shown significantly decreased frequency of chronic headaches in patients receiving botulinum toxin injections compared to placebo and supports its future use as an adjuvant treatment for chronic headache syndromes in selected patients.

Exertional Headache

Physical exercise is always the precipitating factor in primary exertional headaches. They may develop during acute straining, such as weight lifting, or after sustained exercise, such as running. These headaches are characterized by throbbing pain for minutes or hours after discontinuation of activity. They often respond to 25–50 mg of indomethacin three times daily taken either after the exercise or prophylactically once a typical pattern is established. β-Blockers may also be used for prophylaxis. However, the possibility of serious disorders such as arterial dissection, aneurysmal rupture, and even an intracranial mass must be excluded. Computed tomography (CT), MRI, and MR angiography may be appropriate.

Headache Associated with Sexual Activity (Coital Headache)

Typically, these headaches develop as dull, generalized pain during sexual activity or as an acute, sometimes explosive, pain during orgasm. The headache lasts for minutes to hours after the cessation of sexual activity. Patients with coital headache often suffer from exertional headache as well. Clinicians may find patients suffering from this condition to be less than forthcoming about the circumstances surrounding the onset of headache. A careful history including questions about sexual symptoms is needed. Embarrassment and an unwillingness to address sexual concerns with a physician may lead patients with coital headache to go undiagnosed for years. As with other paroxysmal headaches, a search for underlying systemic or intracranial pathology, particularly a ruptured aneurysm, is needed before diagnosing a benign process. NSAIDs, particularly indomethacin (25–50 mg), may be helpful when administered before sexual activity.

Others

Hypnic headaches tend to occur more in women 50 years or older during rapid eye movement sleep and can recur several times at night. The pain is usually unilateral but can be bilateral and lasts 20–120 minutes after awakening. NSAIDs with caffeine or lithium have been used to control them. Cough headaches in contrast are sudden acute bilateral headaches occurring in older men and induced by straining or coughing. They last from seconds to 30 minutes and tend to be indomethacin responsive. Cough-induced headache is common in Arnold–Chiari type I malformations and this as well as cerebrovascular dissection or a rupture intracranial aneurysm should be excluded before making a diagnosis of a primary cough-induced headache.

Clinical Vignette

A 78-year-old man reports a constant, global headache for more than a month. He has felt generally unwell, with poor appetite, fatigue, and a 10-pound weight loss over the same time period. He experiences stiffness and pain in his shoulders and hips on awakening. A week prior to evaluation, he noticed a transient blurring of vision in his left eye for 20 minutes. His neurologic examination is normal, except for questionable temporal artery tenderness on the left. His sedimentation rate is found to be elevated, at 85 mm/hour. He is placed on prednisone 60 mg daily with rapid improvement of his symptoms. A left temporal artery biopsy, performed several days later, demonstrates findings typical for giant cell arteritis (transmural inflammatory response with occasional multinucleated giant cells and areas of internal elastic lamina disruption). Eight months later, he remains on 5 mg of prednisone to control his stiffness and pain.

Headache is the most common and prominent presenting symptom of giant cell or temporal arteritis, a serious disorder of the elderly with potentially devastating complications such as permanent blindness. As in the above vignette, early identification and prompt treatment prevents blindness from developing. The pain of temporal arteritis is usually bilateral and nonspecific, being throbbing or continuous, and with variable intensity, at times so mild that its potential significance is easily overlooked. Systemic complaints including anorexia, general malaise, myalgias, and arthralgias are common, and severe as important diagnostic clues. Polymyalgia rheumatica, a condition characterized by proximal musculoskeletal pain and morning stiffness, frequently accompanies the headache. Jaw or tongue claudication and rarely facial tissue ischemia have been described and reflect external carotid artery involvement. Patients may have subtle and intermittent visual blurring or frank episodes of monocular visual loss mimicking transient ischemic attacks. Although often present, temporal artery tenderness may be relatively minor (Fig. 11-7). Early diagnosis is paramount as arteritis may precipitously cause unilateral or sequential bilateral anterior ischemic optic neuropathy with permanent visual loss. Although posterior ciliary arteries are most commonly involved, visual loss may less commonly result from ophthalmic artery involvement and rarely retinal artery arteritis. Furthermore, the arteritis may be widespread with involvement beyond the temporal arteries to the aorta and its branches. Infrequently, ischemic stroke may occur as arteritis affects the extracranial carotid or vertebral arteries. The intracranial circulation is generally spared. Erythrocyte sedimentation rate (ESR) and C-reactive protein provide the laboratory means to support the diagnosis. Typically, the ESR is significantly increased to 60–110 mm/hour, although there are exceptions. Biopsy of a long temporal artery segment is indicated in every patient suspected of having temporal arteritis. Because the arteritis is patchy, a unilateral biopsy may fail to show the inflammatory changes of giant cell arteritis and bilateral biopsies may be required to make the diagnosis. A mixed infiltrate of neutrophils and T lymphocytes involves the media with concurrent intimal hyperplasia and gradual luminal narrowing. Granulomatous, inflammatory arteritis with giant cell formation and marked disruption of the internal elastic lamina are classic for temporal arteritis (see Fig. 11-7).

Figure 11-7 Giant-Cell (Temporal) Arteritis, Polymyalgia Rheumatica.

Treatment

Because temporal arteritis is a chronic disorder, it requires relatively long-term oral corticosteroid treatment. Prompt diagnosis and treatment are required to prevent serious complications. Treatment must not be delayed because corticosteroids do not alter the pathologic findings if the biopsy is done within a few days of initiating therapy. Prednisone is begun at 40–60 mg/day, followed by a very gradual taper. When transient visual or neurologic symptoms occur, higher initial dosages of corticosteroid may be indicated. Steroids may be needed for 1–2 years at smaller doses to control associated symptoms. Treatment must be individualized, with frequent monitoring of sedimentation rate and symptoms. Long-term steroid side effects, such as truncal weight gain, glucose intolerance, electrolyte imbalance, hypertension, osteoporosis, potential immunosuppression, and cataract formation, to name just a few, need to be followed closely.

Future Directions

Giant cell arteritis is a chronic disease that requires prolonged immunosuppression. In selected patients with longstanding disease, the use of corticosteroid-sparing drugs like methotrexate, azathioprine, and tumor necrosis factor-α inhibitors have been explored.

Clinical Vignette

A 38-year-old overweight woman presented with a recent onset of headaches and blurred vision. The headaches were increasingly severe and more bothersome to her when she bent forward. She noted intermittent double vision on lateral gaze.

Neurologic examination demonstrated limitation of lateral eye movements compatible with CN-VI paresis and modest papilledema. Brain imaging demonstrated diminished size of the lateral ventricles. Spinal fluid pressure was 350 mm CSF; its hematologic, cytologic, and chemical components were normal.

Idiopathic intracranial hypertension, also called pseudotumor cerebri, is a unique syndrome of relatively severe poorly defined and often progressive headaches with associated horizontal diplopia. In addition, transient visual obscurations and pulsatile tinnitus may be part of the clinical picture. It primarily presents in healthy, usually overweight, young women. Pseudotumor cerebri is associated with increased intracranial CSF pressure about 250 mm H₂O (Fig. 11-8).

Figure 11-8 Pseudotumor Cerebri.

Clinical Presentation and Diagnostic Studies

Neurologic examination demonstrates papilledema, with eventual optic nerve atrophy and visual field loss. Because of increased ICP, lateral rectus muscle weakness (pseudo–sixth nerve palsy) may be seen but patients are otherwise awake, alert, and have no focal neurologic deficits.

Hypervitaminosis A or various antibiotics such as tetracycline, minocycline, nitrofurantoin, ampicillin, or nalidixic acid may induce this syndrome. Other possible offending medications include oral contraceptives, corticosteroids, estrogen and progestin therapies, NSAIDs, amiodarone, perhexiline, and the anesthetic agents ketamine and nitrous oxide.

Neuroimaging studies are mandatory to exclude other causes of increased intracranial pressure, such as cerebral masses or dural sinus thrombosis. The CSF pressure is increased, usually >250 mm H₂O in the decubitus position, but the cell count and chemical profile are normal.

Treatment

With idiopathic pseudotumor cerebri, treatment generally consists of weight loss, low-salt diet, diuretics, and symptomatic headache control. Discontinuation of the offending medication often reverses the clinical picture. Frequent visual monitoring with formal visual field testing is essential. Chronic increased intracranial pressure causes loss of vision secondary to the optic nerve head swelling (i.e., papilledema) and eventual optic nerve fiber layer atrophy. The first sign of evolving optic nerve damage is inferonasal peripheral visual loss that gradually moves toward the center and forms a “nasal step.” Very close follow-up with Goldmann perimetry (more sensitive to peripheral vision out 50°) is essential. Loss of central visual acuity is unusual early on and only occurs with long-term papilledema after significant peripheral visual loss has occurred. Occasionally central visual blurring or waviness is reported and is due to macular wrinkling caused by pressure and retinal fluid collection from the adjacent swollen optic nerve head. If there is evidence of visual loss, then repeated lumbar punctures to decrease ICP and more aggressive treatment are needed. A trial of corticosteroids may, paradoxically, be helpful but is likely not to be effective in the long run. Optic nerve sheath fenestration just behind the globe theoretically decompresses the optic nerve head and allows CSF to be shunted into the orbit and absorbed. This has been found successful in up to 80% of patients in arresting visual loss but has little effect in controlling the headache. Fenestration of one side oddly has positive effects on both eyes. In recalcitrant cases CSF shunting procedures may also be considered and halts progressive visual loss in 30–50% of cases and controls the headache.

Clinical Vignette

A 28-year-old woman with a history of tension headaches presents to her physician with change in quality of her headaches. Unlike her prior headaches, these do not occur upon awakening but after arising and when straining. They are aggravated by routine physical activity, such as bending over and during light exercise, and would abate completely when lying down. These headaches are associated with nausea and vomiting, are more severe, and have lasted for 2 weeks, much longer than her usual headaches. She injured her neck in a car accident 3 weeks prior but has otherwise been feeling well.

An MRI of her brain and spine with contrast reveals marked leptomeningeal enhancement. A subsequent myelogram reveals a dural tear at the cervicothoracic junction. CSF analysis demonstrates slightly elevated protein and a low opening pressure.

Headache is often the presenting symptom in cases of intracranial hypotension. Precipitating events include lumbar puncture, CSF shunt placement, spinal surgery, and skull base and spinal tumors. As in the above vignette, symptoms can also develop after spinal trauma. Sometimes a simple Valsalva maneuver or coughing can precipitate the condition. However, some cases occur spontaneously or after only minor trauma, and therefore often go undiagnosed or are misdiagnosed.

Intracranial hypotension is due to a continuous leakage of CSF. Typically, the headaches develop soon after a lumbar puncture. Spontaneous cases may be less precipitous, evolving over days and are felt to be due to dural tears in the spine, most often in the cervical or thoracic regions along the nerve root dural sleeve. Generally, the headaches occur during the waking hours and are postural, worsening in the upright position, and improving or resolving with recumbence. Often, the headache is associated with nausea, vomiting, neck pain, and dizziness, which also clear on recumbence.

Leakage of CSF causes low pressure with sagging of the brain and traction on dural and vascular elements. This traction worsens in the upright position, explaining the postural component of the headache.

Diagnosis

MRI with gadolinium demonstrates diffuse pachymeningeal enhancement in most low–CSF pressure headaches. This is often accompanied by dural thickening that can be striking and may be confused with leptomeningeal inflammatory or neoplastic processes (Fig. 11-9). In more severe cases, subdural fluid collections and descent of the brain with downward displacement of the cerebellar tonsils can be seen. Lumbar puncture demonstrates decreased intracranial pressure, usually less than 50 mm H₂O. CSF analysis may be normal, but slight elevation of protein and a mild lymphocytic pleocytosis may be seen. Radioisotope cisternography or contrast myelography can be used to detect sites of CSF leakage.

Figure 11-9 Low-Pressure Headache.

Management and Therapy

Post–lumbar puncture headache usually subsides with bed rest and hydration within a few days. An abdominal binder and small doses of caffeine may also be of benefit. If it persists, an epidural autologous blood patch injected at the lumbar puncture site, theoretically sealing the leak, almost universally improves post–lumbar puncture headaches, providing credence to the theory that they are secondary to a CSF leak. Other options for treatment include prolonged bed rest and continuous intrathecal or epidural saline infusions. Surgical intervention is rarely necessary. In spontaneous cases, a blood patch in the lumbar region often provides relief despite no identifiable tear in that region. Occasionally, such headaches are resistant to therapy and become disabling.

In the above vignette, a diagnosis of traumatic leptomeningeal tear with CSF leak and subsequent low-pressure syndrome was made. A therapeutic trial of an autologous blood dural patch was successful, with relief of the headaches and associated symptoms within a week.

Trigeminal Neuralgia

Glossopharyngeal Neuralgia

Glossopharyngeal neuralgia is less common than trigeminal neuralgia and generally located in the ear, tonsillar area, or deep within the throat in the CN-IX sensory distribution. The pain is paroxysmal, recurrent, and severe, usually with swallowing as the primary triggering mechanism. Occasionally, patients experience bradycardia during the pain paroxysms and, sometimes, loss of consciousness. Medical treatment is similar to that of trigeminal neuralgia. Rhizotomy or decompression of CN-IX may be necessary in severe, medically intractable cases.

Occipital Neuralgia

Although similar in pain characteristics to trigeminal neuralgia, occipital neuralgia is differentiated by location to the posterior scalp innervated by the greater and lesser occipital nerves from the C2 dermatome (Fig. 11-10). Occipital neuralgia is probably underdiagnosed. Pain is generally localized to the base of the skull but may extend to the vertex, behind the ear or into the neck and upper back. Pain may be initially incited by hyperextension of the neck or whiplash injury. Patients are commonly seen after motor vehicle accident, sports trauma, or work-related injury. The neuralgia is usually unilateral, at times bilateral, and occurs in brief paroxysms of jabbing pain superimposed on a more chronic dull occipital ache.

Figure 11-10 Suboccipital Triangle.

Diagnosis is assisted by the identification of an occipital trigger point at the base of the skull between the mastoid process and the occipital protuberance. Although the pain and disability may be similar to trigeminal neuralgia, occipital neuralgia is more easily treated. Infiltration of the greater and lesser occipital nerves with a local anesthetic and steroid compounds may prove both diagnostic and therapeutic. When ineffective, carbamazepine and other drugs commonly used in the treatment of neuropathic pain are often employed.

Nasal Sinus Infection

This entity must be considered in all patients presenting with headache.

Often, a patient with tension headache presents with a self-diagnosis of “sinus headache” and a careful history and examination is needed. The typical patient with active nasal sinus infection experiences a deep boring discomfort in the maxillary or ethmoid facial region. With acute infections, there is often percussion tenderness, a purulent nasal discharge, and, if the infection is severe, fever. In contrast, sphenoid sinus infections are more easily masked, presenting with only deep-seated headaches, and pose the greatest risk for parameningeal seeding of the meninges and bacterial meningitis. Diagnosis depends on CT, MRI, or both. Appropriate antibiotic treatment, decongestants, and hydration are usually effective.

Dental Infection

An abscessed tooth, primarily in the upper jaw, is a relatively common cause of facial pain or headache. Although the diagnosis is usually obvious to patients, on occasion they may present to a physician first. The neurologist must consider a primary dental source as an unusual cause for some instances of head and facial pain. A careful dental evaluation may be diagnostic when no other mechanism is identified. Temporomandibular joint dysfunction causes referred periorbital, temporal, zygomatic, and mandibular pain and is often mistaken for a primary or secondary headache syndrome. Joint clicking or crepitance, focal tenderness, limited jaw movement, and an altered bite are hints to its diagnosis.