Cranial and Facial Pain

Types of Headaches

Many individuals have more than one type of headache. It is valuable to establish this information at the beginning of the interview so that each type of pain can be carefully delineated. For example, both migraine headaches and tension-type headaches often occur in the same individual. Additionally, change in an established headache pattern can indicate a new condition.

Onset of Headaches

A stable headache disorder of many years’ duration is almost always of benign origin. Migraine headaches often begin in childhood, adolescence, or early adulthood. A headache of recent onset obviously has many possible causes, including the new onset of either a benign or serious condition. “Recent onset” has been defined differently by various authors; the typical range is from 1 to 12 months. In general, the more recent the onset of headache, the greater the risk of a possible secondary cause. The “worst ever” headache, an increasingly severe headache, or change for the worse in an existing headache pattern all raise the possibility of an intracranial lesion. Headaches of rapid (over seconds to minutes) onset suggest an intracranial hemorrhage, usually in the subarachnoid space, but they also can be caused by intracerebral hemorrhage, cerebral venous thrombosis, intra- and extracranial arterial dissection, pituitary apoplexy, spontaneous intracranial hypotension, reversible cerebral vasoconstriction syndrome (RCVS), acute hypertensive crisis, and other conditions that must be excluded with the first occurrence of “thunderclap headache” ( ). If no underlying cause is discovered despite an expedited and thorough investigation, a diagnosis of primary thunderclap headache can be considered. Onset of a new headache in patients older than 50 years raises suspicion of an intracranial lesion (e.g., subdural hematoma) or giant cell (temporal or cranial) arteritis (GCA). A history of antecedent head or neck injury should be sought; even a relatively minor injury can be associated with subsequent development of subdural hemorrhage (especially in the elderly) and posttraumatic dissection of the carotid or vertebral arteries ( ). However, posttraumatic headaches can occur following head injury in the absence of any demonstrable pathology.

Frequency and Periodicity of Episodic Headaches

Migraine may be episodic or chronic. Chronic migraine is defined as headache occurring on more than 15 days per month for 3 or more months that has the features of migraine headache on 8 or more days per month. Chronic migraine usually develops in individuals with a history of episodic migraine headaches. Chronic migraine may occur with or without medication overuse. Some patients with medication overuse do not improve after drug withdrawal.

Episodic cluster headaches typically occur daily for several weeks or months and are followed by a lengthy headache-free interval. Chronic cluster headaches occur at least every other day for more than 1 year or with remissions lasting less than 3 months. If there is no regular periodicity, it is useful to inquire about the longest and shortest periods of freedom between headaches. Having the patient monitor headache frequency, duration, intensity, headache triggers, and medication use on a headache calendar or in a diary is helpful in diagnosis and measuring response to treatment. Tension-type headaches can be episodic (infrequent, <12 days per year) or frequent (≥12 and <180 days per year) or chronic (≥15 days per month on average for more than 3 months).

Thunderclap headache recurring over a few days or weeks raises the possibility of RCVS, a diagnosis that requires the exclusion of aneurysmal subarachnoid hemorrhage ( ).

Temporal Profile

A chronic daily headache without migrainous or autonomic features is likely to be a chronic tension-type headache. Untreated migraine pain usually peaks within 1–2 hours of onset and lasts 4–72 hours. Cluster headache is typically maximal immediately (if the patient awakens with the headache in progress) or peaks within minutes (if it begins while the patient is awake). Cluster headaches are more common in men and are infrequently inherited. They last from 15 to 180 minutes (usually 45–120 minutes). Headaches similar to cluster but lasting only 2–30 minutes and occurring several or many times a day are typical of episodic or chronic paroxysmal hemicrania, both of which are more common in women and are prevented by indomethacin ( ). Primary stabbing headaches (“ice-pick pains”) are momentary, typically lasting seconds. Stabbing headaches are more common in patients with migraine, tend to vary in location, and do not have identifiable triggers. Tension-type headaches commonly build up over hours and last hours to days to years. Headache that is daily and unremitting from onset, usually in patients without prior headaches, is classified as new daily persistent headache and may have features suggestive of migraine or tension-type headache. A chronic, continuous, unilateral headache of moderate severity with superimposed attacks of more intense pain associated with autonomic features suggests the diagnosis of hemicrania continua, an indomethacin-responsive syndrome. Occipital neuralgia and trigeminal neuralgia manifest as brief shock-like pains, often triggered by stimulation in the territory served by the affected nerve. Occasionally a dull pain in the same nerve distribution persists longer, often after a series of brief, sharp pains. S hort-lasting u nilateral n euralgiform headache with c onjunctival injection and t earing (SUNCT) is a rare syndrome consisting of paroxysms of first-division trigeminal nerve pain lasting 5–240 seconds but occurring 3–200 times per day with the associated autonomic symptoms for which it is named ( ). SUNA (short-lasting neuralgiform headache attacks with autonomic symptoms) is a related disorder and differs from SUNCT only in that not more than one of the following occurs ipsilateral to the pain: (1) conjunctival injection or (2) lacrimation (tearing).

Time of Day and Precipitating Factors

Cluster headaches often awaken patients from a sound sleep and may occur at the same time each day in an individual person. Hypnic headaches usually begin after age 50 years and regularly awaken the patient at a particular time of night. Unlike cluster headaches, they are usually bilateral and not associated with autonomic phenomena ( ). Migraine headaches can occur at any time but often begin in the morning. A headache of recent onset that disturbs sleep or is worse on waking may be caused by increased intracranial pressure. Tension-type headaches can be present during much of the day and are often more severe later in the day. Obstructive sleep apnea may be accompanied by the frequent occurrence of headache on awakening, as might medication overuse headache (“rebound headache”) and headache due to caffeine withdrawal. If headache begins while the patient is asleep, it is impossible to tell whether the onset was gradual or abrupt.

Patients with chronic recurrent headaches often recognize factors that trigger an attack. Migraine headaches may be precipitated by bright light, menstruation, weather changes, caffeine withdrawal, fasting, alcohol, sleeping more or less than usual, stress and release from stress, certain foods and food additives, perfume and smoke, and others. Alcohol can trigger a cluster headache within minutes of ingestion. If bending, lifting, coughing, or Valsalva maneuver brings on a headache, an intracranial lesion, especially one involving the posterior fossa, must be considered. Exertional headache and headache associated with sexual activity are both worrisome. Although either can occur as a primary headache disorder unassociated with structural disease or can be associated with migraine, both types can also be due to subarachnoid hemorrhage, arterial dissection, and RCVS, which must be excluded with the first occurrence of such headaches. Intermittent headaches that are worsened by sitting or standing and improved by lying down are characteristic of a cerebrospinal fluid (CSF) leak, although other headache patterns may occur. If there is no history of lumbar puncture, head trauma, or neurosurgical intervention, a spontaneous CSF leak may be the cause ( ). Lancinating face pain triggered by facial or intraoral stimuli occurs with trigeminal neuralgia. Glossopharyngeal neuralgia typically causes throat or ear pain that is triggered by chewing, swallowing, or talking, although cutaneous trigger zones in and about the ear are occasionally present.

Location

Asking the patient to outline the location of the pain with his or her finger can be very helpful. Trigeminal neuralgia is confined to one or more branches of the trigeminal nerve. The patient may be able to localize one or more trigger points on the face or in the mouth and then show how the pain spreads. Pain in the throat may be due to a local process or to glossopharyngeal neuralgia. Carotid artery dissection commonly presents with unilateral neck, face, and head pain; is frequently associated with an ipsilateral Horner syndrome; and often follows head or neck trauma.

Migraine is commonly unilateral, can be confined to the front or back of the head, and can affect the neck. Migraine pain can start on one side and spread to the other or be bilateral from onset. Cluster headaches are unilateral during an attack and typically are centered in, behind, or around one eye. Some patients’ cluster headaches switch sides with different cluster periods, and a smaller number of patients experience side shifts within a cluster period. The typical tension-type headache is generalized, although it may begin in the neck muscles and affect chiefly the occipital region or predominate frontally. When pain is localized to the eye, mouth, or ear, local processes involving these structures must be considered. Otalgia may be caused by a process involving the tonsil and posterior tongue. With chronic unilateral facial pain, an underlying lesion often cannot be identified. Occasionally facial pain may be a symptom of nonmetastatic lung cancer ( ).

Quality and Severity

The character and quality of the patient’s pain can have significance. In most cases, the type of pain can be designated as sharp, aching, or burning. Headaches may be steady or throbbing (pulsating) in character. It may be helpful to ask the patient to grade the severity of pain on a scale of 1–10. Patients who report that their pain is at level 20 are hurting but may be prone to exaggerate. Another useful approach is to ask the patient to indicate the severity of their headache pain as follows: mild = no impact on function, moderate = able to function with difficulty, severe = unable to function. Migraine pain often has a pulsating quality that may be superimposed on a more continuous pain. The pain of cluster headache is characteristically severe, boring, and steady and often described as a “hot poker.” SUNCT produces moderately severe pain in the orbital or temporal region and may be described as sharp and stabbing or (rarely) pulsatile. Tension-type headaches are often described as a steady feeling of fullness, tightness, or pressure or like a cap, band, or vise. Headaches caused by meningeal irritation, whether related to infectious meningitis or blood, are typically severe. Trigeminal neuralgia pain is severe, brief, sharp, electric shock–like, or stabbing; pains can occur up to several times per minute, and a milder ache may persist between paroxysms of pain. Glossopharyngeal neuralgia pain is similar in character to that of trigeminal neuralgia.

Premonitory Symptoms, Aura, and Accompanying Symptoms

Some patients have premonitory symptoms that precede a migraine headache by hours. These can include psychological changes (e.g., depression, euphoria, irritability) or somatic symptoms (e.g., constipation, diarrhea, abnormal hunger, fluid retention, increased urination). The term aura refers to focal cerebral symptoms associated with a migraine attack. These symptoms can last 5–60 minutes but usually last 10–30 minutes. Aura symptoms typically precede the headache but can continue into the headache phase or begin during the headache. Visual symptoms are most common and may consist of either positive (scintillating lights, spots, or zig-zag lines) or negative (scotomas or visual field loss) phenomena or both. The visual symptoms characteristically affect both eyes simultaneously but can rarely affect one eye alone. Less common hemispheric symptoms, such as unilateral somatosensory disturbances (tingling and/or numbness) or dysphasic language disturbance, may occur with or without visual symptoms. Aura symptoms usually have a gradual onset and offset; they typically increase and decrease over minutes. If more than one symptom occurs (e.g., visual plus somatosensory), the onsets usually are staggered and not simultaneous. Patients can experience migraine aura without an associated headache. Positive symptoms, the slow spread of symptoms, and staggered onsets help differentiate migraine aura from focal symptoms caused by cerebrovascular disease.

Symptoms originating from the brainstem or both cerebral hemispheres simultaneously—such as vertigo, dysarthria, ataxia, auditory symptoms, diplopia, bilateral visual symptoms in both eyes, bilateral numbness and/or paresthesias, and decreased level of consciousness—may accompany migraine with brainstem aura (formerly called basilar-type migraine) ( ). Vertigo without other brainstem signs can also accompany migraine headaches. Migraine with aura that includes motor weakness can be due to familial hemiplegic migraine if there is a family history in at least one first- or second-degree relative or due to sporadic hemiplegic migraine if there is no family history. It can be difficult for the patient to differentiate sensory loss from true weakness, but this must be carefully delineated. Nausea, vomiting, photophobia, phonophobia, and osmophobia characteristically accompany migraine attacks. In addition, lacrimation, rhinorrhea, and nasal congestion can accompany migraine headache and mimic headache of sinus origin ( ). Ipsilateral miosis, ptosis, lacrimation, conjunctival injection, and nasal stuffiness commonly accompany cluster headache; sweating and facial flushing on the side of the pain are much less common. Similar autonomic features also accompany episodic and chronic paroxysmal hemicrania and hemicrania continua. Very short attacks (5–240 seconds) with ipsilateral conjunctival injection and tearing suggest SUNCT ( ). Horner syndrome is common in carotid artery dissection. In the setting of acute transient or persistent monocular visual loss, GCA and carotid dissection and stenosis should be considered. Temporomandibular joint dysfunction includes jaw pain precipitated or aggravated by movement of the jaw or clenching of the teeth and is associated with reduced range of jaw movement, joint clicking, and tenderness over the joint. Headache accompanied by fever suggests an infection. Headache associated with persistent or progressive diffuse or focal central nervous system (CNS) symptoms, including seizures, implies a structural cause. Purulent or bloody nasal discharge suggests an acute sinus cause for the headache. Likewise, a red eye raises the possibility of an ocular process such as infection or acute glaucoma. A history of polymyalgia rheumatica, jaw claudication, or tenderness of the scalp arteries in an older person strongly suggests GCA. Transient visual obscurations upon standing, usually pulsatile tinnitus, diplopia (especially for objects in the distance), and papilledema may be associated with increased intracranial pressure from any cause, including idiopathic intracranial hypertension (pseudotumor cerebri).

Aggravating and Mitigating Factors

Worsening of headache as a result of a cough or physical jolt suggests an intracranial component to the pain. Sufferers of cluster headache tend to endure their pain in an agitated state, pacing and moving about, whereas patients with migraine typically prefer to lie still. Precipitation or marked worsening of headache in the upright position and/or relief with recumbency suggests intracranial hypotension. Routine physical activity, light, sound, and smells typically aggravate migraine headaches. Rest, especially sleep, and avoidance of light and noise as well as the application of cold tend to benefit the migraineur. Massage, ice, or heat may reduce the pain associated with tension-type headache. Local application of pressure over the affected eye or ipsilateral temporal artery, local application of heat or cold, and (rarely) brief intense physical activity may alleviate the pain of cluster headache.

Family History of Headaches

Migraine is often an inherited disorder, and a family history of migraines (sometimes referred to as “sick headaches”) is present in about two-thirds of patients. Tension-type headaches can also be familial. In individuals with cluster headaches, studies have shown a family history of cluster headaches in 5%–20%. Familial hemiplegic migraine is a rare autosomal dominant variant of migraine with aura, wherein the aura includes hemiparesis lasting from minutes to 24 hours.