Chapter 69 Headache and Other Craniofacial Pain
A survey of a sample of 20,000 households estimated 27.9 million migraine patients in the United States. More than 90% of patients report an impaired ability to function during migraine attacks, and 53% report severe disability requiring bed rest. Approximately 31% of patients with migraine missed at least 1 day from work or school in the preceding 3 months due to migraine (Lipton et al., 2001). Indirect costs of migraine related to decreased productivity and lost days of work have been calculated to be $13 billion per year; estimates are that the equivalent of 112 million bedridden days per year are due to migraine (Hu et al., 1999). The World Health Organization declared migraine to be among the most disabling medical conditions experienced worldwide.
Classification
In 1988, the Headache Classification Committee of the International Headache Society introduced a detailed classification of headaches. The classification, revised in 2004, contains 14 main headache types (Box 69.1), and each headache type is further subclassified (Headache Classification Committee, 2004). Careful definition of migraine subtypes and other primary headache disorders is expected to rid future research and clinical publications of the confusing and often poorly defined terminology of earlier work. The 2004 classification has undergone a few revisions since its publication, and further refinement over the next few years is expected.
Box 69.1
Classification of Headache Disorders
Part Two: The Secondary Headaches
5. Headache attributed to head and/or neck trauma
6. Headache attributed to cranial or cervical vascular disorder
7. Headache attributed to non-vascular intracranial disorder
8. Headache attributed to a substance or its withdrawal
9. Headache attributed to infection
10. Headache attributed to disorder of homeostasis
11. Headache or facial pain attributed to disorder of cranium, neck, eyes, ears, nose, sinuses, teeth, mouth, or other facial or cranial structures
Adapted from Headache Classification Committee of the International Society, 2004. International Classification of Headache Disorders, second ed. Cephalalgia 24, 9-195.
Headache Attributed to Nonvascular, Noninfectious Intracranial Disorders
Tumors
Approximately 50% of patients with brain tumors report headaches; in one-third to one-half of these patients, headache is the primary complaint. In a prospective study of over 200 patients with intracranial tumors, 47.6% had headache at the time of presentation (Valentinis et al., 2009). Generalized headache is usual, but in approximately a third of patients, it overlies the tumor and is referred to the scalp near the lesion. Rapidly growing tumors are more likely to produce headache than indolent lesions, but slowly enlarging lesions can eventually produce pain by compromising the ventricular system or exerting direct pressure on a pain-sensitive structure. When the CSF circulation is partially obstructed, headache often becomes generalized and worse in the occipitonuchal area. This type of headache, which is a manifestation of raised intracranial pressure (ICP), is often worse on awakening, aggravated by coughing and straining, and often associated with nausea and vomiting. A prospective study showed that only 5.1% of patients presented with this pattern of headache; in most cases, the headache pattern was nonspecific (Valentinis et al., 2009). In children particularly, the vomiting may be precipitate and without nausea (projectile vomiting).
Supratentorial masses generally produce frontal or temporal head pain because of the trigeminal nerve supply to the anterior and middle cranial fossae. The superior surface of the tentorium cerebelli is supplied by the meningeal branches of the first division of the trigeminal cranial nerve, so an occipital lesion can cause pain referred to the fronto-orbital region. A posterior fossa mass generally causes occipitonuchal pain, because the meningeal nerve supply is largely through the upper cervical nerves which also supply the occipital and cervical dermatomes. CNs VII, IX, and X also provide sensory innervation of the posterior fossa, and therefore pain referral can be more widespread. Posterior fossa tumors result in headache earlier than their supratentorial counterparts, because the greater likelihood of compromise of the ventricular system leads to rapidly developing hydrocephalus and raised ICP (Edmeads, 1997).
Pituitary tumors and tumors near the optic chiasm commonly cause a frontotemporal headache, but they may cause referred pain near the vertex. However, patients with tumors of the sellar and parasellar regions do not often present with headache as the initial symptom, because the visual and endocrine symptoms are typically noted first (Edmeads, 1997).
Features that should serve as warnings that a patient’s headaches may not be of benign origin and raise the possibility of an intracranial mass lesion are (Purdy, 2001): subacute and progressive; new onset in adults; change in pattern; associated with nausea or vomiting; nocturnal or upon awakening in the morning; precipitated or worsened by changes in posture or Valsalva maneuver; associated with confusion, seizures, weakness, and/or abnormal neurological examination.
Obstruction of the Cerebrospinal Fluid Pathways
Congenital obstruction of the foramina of Luschka and Magendie (the Dandy-Walker syndrome) can lead to ballooning of the fourth ventricle and deformity of the cerebellum. Minor degrees of this malformation can remain asymptomatic until later in life and then manifest with obstructive hydrocephalus and headache. Similarly, the Chiari malformation in its various forms can obstruct the free circulation of CSF and lead to hydrocephalus and headache (Taylor and Larkins, 2002). This malformation can result in an occipital-suboccipital headache worsened or even initiated by a Valsalva maneuver during lifting, straining, or coughing. Thus, the Chiari malformation is one of the causes of an exertional or Valsalva maneuver–induced headache.
In communicating hydrocephalus, free communication exists between the ventricular system and the subarachnoid space, but CSF circulation or absorption is impaired. Obstruction in the basal cisterns or at the arachnoid granulations may follow subarachnoid hemorrhage and meningitis. Venous sinus occlusion can impair absorption of CSF. Headache may be a prominent symptom of both obstructive and communicating hydrocephalus, except in the case of normal-pressure hydrocephalus, which is generally painless (see Chapter 59).
Low–Cerebrospinal Fluid Pressure Headache
An identical low–CSF pressure headache can occur when a tear develops in the spinal theca. This is usually in the midthoracic region and may result from lifting or coughing or, at times, occurs spontaneously. It can also occur with a crush injury to the chest or abdomen and in patients with overdraining CSF shunts. When it occurs, in the absence of a significant history of trauma, it may not be considered as a cause of daily headache. A history of a headache rapidly responding to recumbency suggests a tear. However, in some patients whose headaches are long standing, a persistent headache may be noted and the postural feature of the headache may become less prominent. Nausea or emesis, neck pain, dizziness, horizontal diplopia, changes in hearing, photophobia, upper-limb paresthesias, vision blurring, and dysgeusia may also occur, particularly when the headache first develops. As additional cases have been reported in the literature, the clinical picture of CSF leaks has been found to take many forms (Mokri, 2004).
When a CSF leak is possible, the initial diagnostic test is MRI with gadolinium. MRI has become an invaluable diagnostic tool in this syndrome, with the cardinal features being diffuse pachymeningeal thickening with gadolinium enhancement, subdural collections of fluid, and evidence of brain descent (Figs. 69.1 to 69.3). This evidence includes cerebellar tonsillar descent (resembling a Chiari type I malformation); reduction in size or effacement of the prepontine, perichiasmatic, and subarachnoid cisterns; inferior displacement of the optic chiasm; and descent of the iter (the opening of the aqueduct of Sylvius as seen on a midsagittal MRI scan).
If the clinical and MRI findings are typical, determination of the CSF opening pressure may not be necessary. Measurement of the opening pressure is warranted in patients with normal MRI studies. However, in only 50% of patients is the CSF pressure less than 40 mm H2O. Because the opening pressure may be normal, the term CSF volume depletion best identifies the core of the problem in this disorder (Mokri, 2004). These patients may have a variable pleocytosis with up to 50 or more mononuclear cells/mm and a mild to modest increase in CSF protein (Mokri, 2004).
In patients with the typical clinical and radiographic features of low–CSF pressure headache, treatment may be conservative with bed rest and hydration for 1 to 2 weeks. If this is either impractical or ineffective, options include empirical treatment with a blood patch or further studies to identify the site of the CSF leak. In patients with spontaneous leaks, the leak is often at the level of the thoracic spine or cervicothoracic junction. Myelography with CT of the spine is more sensitive than radioisotope cisternography or MRI of the spine at localization of the source of the leak, but the latter procedures may serve as guides for obtaining multiple CT images at the appropriate levels. MR myelography may also be helpful in some patients to reveal a leak that is not demonstrable on conventional MRI sequences (Katramados et al., 2006). Most leaks are stopped with either conservative therapy or blood patches. Although an epidural blood patch is effective in the majority of patients, most require more than one blood patch, and some require as many as four to six blood patches (Mokri, 2004). For resistant leaks, surgical repair of the dural tear may be necessary. Even after a protracted duration, surgical repair of the causative dural tear can be quite effective (Mokri, 2004).
Idiopathic Intracranial Hypertension
Headache, transient visual obscuration, pulsatile tinnitus, and diplopia are the most common presenting symptoms of idiopathic intracranial hypertension (pseudotumor cerebri). The headache is rather nonspecific but tends to be worse on awakening and aggravated by activity. The visual obscurations are direct results of raised ICP leading to papilledema. Indeed, if papilledema is not present, the diagnosis is in doubt. Once determining by MRI that there is no intracranial mass, ventricular system obstruction, or thrombosis of a dural venous sinus, the high CSF pressure can be confirmed by lumbar puncture manometry. Removal of CSF to achieve a normal closing pressure relieves the headache and temporarily prevents visual obscurations. A discussion of long-term management of idiopathic intracranial hypertension is in Chapter 59.
Transient Syndrome of Headache with Neurological Deficits and Cerebrospinal Fluid Lymphocytosis
Although originally termed migrainous syndrome with CSF pleocytosis, several later reports used various terms including headache with neurological deficits and CSF lymphocytosis (Berg and Williams, 1995) and pseudomigraine with temporary neurological symptoms and lymphocytic pleocytosis (Gomez-Aranda et al., 1997). This self-limited syndrome consists of one to several episodes of variable neurological deficits accompanied by moderate to severe headache and sometimes fever. Each episode lasts hours, with total duration of the syndrome being from 1 to 70 days. CSF abnormalities have included a lymphocytic pleocytosis varying from 10 to more than 700 cells/mm, elevation of CSF protein, and in some patients, elevated opening pressure. MRI and CT are normal in the vast majority of reported cases. A single patient had MRI evidence of gray-matter swelling in addition to right temporal and occipital sulci CSF enhancement and hypoperfusion. Similar findings have been reported in hemiplegic migraine (Yilmaz et al., 2009). Another patient was found to have global hemisphere hypoperfusion on MRI perfusion imaging (Vallet et al., 2010). Results of microbiological studies are usually negative. A single patient was reported to have evidence of recent human herpesvirus-6 infection, but its pathogenic role in the syndrome is unclear (Emond et al., 2009). Accordingly, the cause of the syndrome is unclear, although an immune response to a viral infection is speculated. No treatment alters the self-limited course of this disorder. In contrast to this syndrome, episodes of Mollaret meningitis (see Chapters 53B and 73) are separated by months to years and are typically not accompanied by focal neurological symptoms.
Headache Attributed to Head or Neck Trauma
Posttraumatic Headache
Headaches, dizziness, difficulty concentrating, irritability, decreased libido, and fatigue are common complaints after head injury (Keidel and Ramadan, 2005). Many studies suggest that milder injuries are more frequently associated with posttraumatic headache then more severe injuries (Packard, 2005). This counterintuitive observation may result from the fact that seriously injured patients may have other symptoms of such severity that headache may be overshadowed by their other complaints; however, there is no definitive evidence as of yet to support this assumption. In civilian populations, the phenotypical appearance of headache attributed to traumatic brain injuries is most consistent with tension-type headache. In military populations, however, posttraumatic headache more commonly meets criteria for migraine. The underlying mechanisms behind this discrepancy are unclear. The variable latencies between injury and the onset of headache observed in combat veterans suggests the need to revise the diagnostic criteria for posttraumatic headaches; the currently accepted window of 7 days may not adequately capture the number of true cases (Vargas, 2009). Brain imaging rarely reveals abnormalities in the absence of an abnormal neurological examination, although unexpected subdural hematomas are occasionally found. MRI examination tends to be more sensitive for the detection of small occult extracerebral hematomas, cortical contusions, and indeterminate changes in the cerebral parenchyma. Treatment of posttraumatic syndrome and posttraumatic headache is difficult. Encouragement, patience, and a sympathetic attitude on the part of the physician are essential. Undoubtedly, a thorough physical examination and evaluation of appropriate imaging studies are important to reassure the patient that more sinister underlying disorders have been ruled out. As successful treatment can be challenging, reasonable expectations should be established. All the treatments useful for tension-type headaches, migraine, occipital neuralgia, and neck sprains may be needed. Physical therapy, biofeedback, and psychotherapy each have a place in treatment. Drug treatment may include analgesics (nonopioid), nonsteroidal antiinflammatory drugs (NSAIDs), and antidepressants. The tricyclic antidepressants and gabapentin can be particularly helpful.
Recovery from posttraumatic syndrome, including the headache, may be significantly delayed. Most patients who continue to have headaches for more than 2 months after the trauma continue to have them for 1 to 2 years (Ramadan and Láinez, 2005). If the history included a period of unconsciousness or if there is a simple skull fracture, a limited period of bed rest followed by a graduated return to full activity should be advised.
Headache Attributed to Infection
Inflammation of pain-sensitive structures such as the meninges and intracranial vessels produces the severe headache frequently associated with both meningitis and meningoencephalitis. Headache is the most common symptom in acute bacterial meningitis, occurring in nearly 90% of cases (van de Beek et al., 2004). Acute bacterial meningitis characteristically produces a severe holocephalic headache with neck stiffness and other signs of meningismus, including photophobia and irritability. Pain may be retro-orbital and may worsen with eye movement. The presence of the classic triad of fever, neck stiffness, and altered mental status has a low sensitivity for the diagnosis of meningitis; however, nearly all patients present with at least two of these symptoms and/or headache (van der Beek et al., 2004). Jolt accentuation of headache has a sensitivity of 100% and a specificity of 54% for meningitis diagnosis (Attia et al., 1999).
Mollaret meningitis is a rare and recurrent aseptic meningitis (see Chapters 53B and 73). The CSF cellular response includes large epithelioid cells (Mollaret cells). The pathogenesis is unknown but may relate to the herpes simplex virus (Jensenius et al., 1998). The condition may recur every few days or every few weeks for months or years. Headache, signs of meningismus, and low-grade fever accompany each attack. Treatment is mainly symptomatic.
Headache Attributed to Cranial or Cervical Vascular Disorders
Aneurysms and Arteriovenous Malformations and Thunderclap Headache
Parenchymal arteriovenous malformations (AVMs) rarely cause pain before rupture. Very large lesions can be associated with ipsilateral or bilateral throbbing cephalalgia, but they rarely cause a migraine-like headache. The presence of a cranial bruit or the classic triad of migraine, seizures, and focal neurological deficits may indicate an AVM. Numerous case reports and retrospective studies suggest migraine may be more prevalent in patients harboring AVMs versus the general population. Among these, the headache frequently occurs ipsilateral to the AVM, highlighting the need for cautious evaluation of patients with side-locked headaches (Monteiro et al., 1993). MR or CT angiography can usually exclude the presence of clinically significant aneurysms and AVMs.
The term thunderclap headache describes a severe headache of instantaneous onset (within seconds) and without warning, like a clap of thunder. Other conditions can also manifest with thunderclap headache: cerebral venous sinus thrombosis, cervicocephalic arterial dissection, pituitary apoplexy, acute hypertensive crisis, spontaneous intracranial hypotension, meningitis, embolic cerebellar infarcts, and reversible cerebral vasoconstriction syndromes (Calabrese et al., 2007; Schwedt et al., 2006). These entities are associated with significant neurological morbidity and not easily seen on the initial CT image, thus underscoring the frequent need for MRI and MRA/magnetic resonance venography (MRV) in this group if results of the initial workup are negative. Finally, for one category of primary thunderclap headache, no underlying cause is established.
Whether an unruptured cerebral aneurysm can cause a thunderclap headache is debated (Schwedt et al., 2006).
Carotid and Vertebral Artery Occlusion and Dissection
MRI or MRA usually confirm the diagnosis of arterial dissection. At the level of involvement, the lumen of the artery typically appears as a dark circle of flow void of smaller caliber than the original vessel, and the intracranial clot appears as a hyperintense and bright crescent or circle (in both T1- and T2-weighted images) surrounding the flow void (Fig. 69.4). Catheter angiography is rarely required (Mokri, 2002). The pain associated with cervicocephalic dissections is of variable duration and may require treatment with potent analgesics. Patients with evidence of distal embolization are usually treated with either antiplatelet agents or anticoagulation.
Giant-Cell Arteritis
Clinical Symptoms
The clinical manifestations of giant-cell arteritis result from inflammation of medium and large arteries. Table 69.1 summarizes clinical symptoms in 166 patients examined at the Mayo Clinic between 1981 and 1983. Headache was the most common symptom, experienced by 72% of patients at some time and the initial symptom in 33%. The headache is most often throbbing, and many patients report scalp tenderness. Headache is associated with striking focal tenderness of the affected superficial temporal or, less often, occipital artery. One-third of patients with headache may have no objective signs of superficial temporal artery inflammation.
Symptom | Patients with Symptom (%) | Patients in Whom It Was Initial Symptom (%) |
---|---|---|
Headache | 72 | 33 |
Polymyalgia rheumatica | 58 | 25 |
Malaise, fatigue | 56 | 20 |
Jaw claudication | 40 | 4 |
Fever | 35 | 11 |
Cough | 17 | 8 |
Neuropathy | 14 | 0 |
Sore throat, dysphagia | 11 | 2 |
Amaurosis fugax | 10 | 2 |
Permanent vision loss | 8 | 3 |
Claudication of limbs | 8 | 0 |
Transient ischemic attack/stroke | 7 | 0 |
Neuro-otological disorder | 7 | 0 |
Scintillating scotoma | 5 | 0 |
Tongue claudication | 4 | 0 |
Depression | 3 | 0.6 |
Diplopia |