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 each type of pain can be carefully delineated. A 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, the more worrisome. 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 instantaneous onset suggest an intracranial hemorrhage, usually in the subarachnoid space, but also can be caused by intracerebral hemorrhage, cerebral venous thrombosis, arterial dissection, pituitary apoplexy, spontaneous intracranial hypotension, benign angiopathy of the central nervous system (CNS), acute hypertensive crisis, and idiopathic primary thunderclap headache (Ju and Schwedt, 2010). 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 epidural, subdural, subarachnoid, or intraparenchymal hemorrhage and posttraumatic dissection of the carotid or vertebral arteries (Dziewas et al., 2003). However, posttraumatic headaches can occur following head injury in the absence of any significant pathology.

Frequency and Periodicity of Episodic Headaches

Migraine may be episodic or chronic. Chronic migraine, occurring 15 or more days per month, usually develops in individuals with a history of episodic migraine headaches. Episodic migraine may become chronic with or without medication overuse.

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. 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, triggers, and medication use on a headache calendar or in a diary is helpful in diagnosis and measuring response to treatment.

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 to 2 hours of onset and lasts 4 to 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 awake). Cluster headaches can last 15 to 180 minutes (usually 45 to 120 minutes). Headaches similar to cluster but lasting only 2 to 30 minutes and occurring several times a day are typical of episodic or chronic paroxysmal hemicrania, both of which are more common in women and are prevented by indomethacin (Goadsby et al., 2010). Primary stabbing headaches (“ice-pick pains”) are momentary, lasting only seconds. Stabbing pains are more common in patients with migraine and cluster headaches. 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 shocklike 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. Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) is a rare syndrome consisting of paroxysms of first-division trigeminal nerve pain, lasting 5 to 240 seconds but occurring 3 to 200 times per day with the associated autonomic symptoms for which it is named (Goadsby et al., 2010)

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 typically affect older patients and regularly awaken the patient at a particular time of night. Unlike cluster headaches, they are usually diffuse and not associated with autonomic phenomena (Donnet and Lanteri-Minet, 2009). 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 typically are present during much of the day and often are more severe later in the day. Obstructive sleep apnea may be accompanied by the frequent occurrence of headache on awakening, as can medication-overuse headache (“rebound headache”).

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 (particularly beer and wine), 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 both are 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 and arterial dissection, both of 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. If there is no history of lumbar puncture, head trauma, or neurosurgical intervention, a spontaneous CSF leak may be the cause (Schievink, 2008). Lancinating face pain triggered by facial or intraoral stimuli occurs with trigeminal neuralgia. Glossopharyngeal neuralgia typically 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 his or her pain with their 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 commonly is unilateral and can be confined to the front or back of the head. Alternatively, the pain can start on one side and spread to the other or be global 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 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, however, facial pain may be a symptom of nonmetastatic lung cancer (Eross et al., 2003).

Quality and Severity

The character and quality of the patient’s pain can carry 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 to 10. Patients who report their pain level is 20 are hurting but may be prone to exaggerate. Migraine pain often has a pulsating quality that may be superimposed on a more continuous pain. The pain of cluster headache characteristically is severe, boring, and steady and often is 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 usually are 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 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 typically last 20 to 30 minutes but can last 1 hour and usually precede the headache. At other times, the aura may continue into the headache phase or begin during the headache. Visual symptoms are most common and may consist of either positive (flickering lights, spots, or lines) or negative (scotomas or visual field loss) phenomena or both. The visual symptoms characteristically affect both eyes but can affect one eye alone. Other hemispheric symptoms, such as 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 increase 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 paresthesias, and decreased level of consciousness, may accompany basilar-type migraine. 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. 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 (Cady et al., 2005). 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. Even shorter attacks (5–240 seconds) with ipsilateral conjunctival injection and tearing suggest SUNCT (Goadsby et al., 2010). Horner syndrome is common in carotid artery dissection. In the setting of acute transient or persistent monocular visual loss, GCA and carotid dissection 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 reduction in the 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 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 Factors

The worsening of headache as a result of a cough or physical jolt suggests an intracranial element to the pain. Sufferers of cluster headache tend to endure their pain in an agitated state, pacing and moving about, whereas patients with migraine prefer to lie still. Precipitation or marked worsening of headache in the upright position suggests intracranial hypotension. Routine physical activity, light, sound, and smells typically aggravate migraine headaches.

Mitigating Factors

Rest, especially sleep, and avoidance of light and noise tend to benefit the migraineur. Massage, ice, or heat may reduce the pain associated with a tension-type headache. Local application of pressure over the affected eye or ipsilateral temporal artery, the local application of heat or cold, and (rarely) brief intense physical activity may alleviate the pain of cluster headache. Headache due to intracranial hypotension typically is helped by recumbency.

Family History of Headaches

Migraine often is an inherited disorder, and a family history of migraine (sometimes referred to as “sick headaches”) is present in about two-thirds of patients. Tension-type headaches also can be familial. Cluster headache characteristically is not inherited. Familial hemiplegic migraine is a rare autosomal dominant variant of migraine with aura, wherein the aura includes hemiparesis lasting minutes to 24 hours.

Prior Evaluation

The patient should be asked about prior consultations and testing. If appropriate, the records and actual imaging studies can be obtained for review.

Prior Treatment

Response to treatment should be sought, including agents used to treat individual headache attacks and those used prophylactically. The dose and duration of each treatment should be reviewed. This information provides an opportunity to determine whether acute medications have been overused and whether prophylactic medications were optimized. A history of the use of caffeine-containing substances also should be elicited because they may cause or aggravate headaches through rebound withdrawal.

Disability

Baseline and follow-up assessment of headache-related disability is helpful in judging the effects of treatment and guiding headache therapy. The Migraine Disability Assessment Scale (MIDAS) is one useful validated clinical tool (Andrasik et al., 2005).

Patient Concerns and Reasons for Seeking Help

Headache pain can produce significant fear and anxiety regarding serious disease. The patient should be allowed to express any concerns so that each can be appropriately addressed.

The question of why the patient is seeking help may be obvious if the problem is of recent onset. If the problem is chronic, however, it can be useful to inquire why the patient has come for aid at this time. Red and yellow flags (see Box 18.3) help identify which patients are more likely to have a secondary cause of their pain.

Box 18.3

Headache Warning Flags

Red Flags

Head or neck injury

New onset or new type or worsening pattern of existing headache

New level of pain (e.g., worst ever)

Abrupt or split-second onset

Triggered by Valsalva maneuver or cough

Triggered by exertion

Triggered by sexual activity

Preorgasmic

Orgasmic

Headache during pregnancy or puerperium

Age > 50 years

Neurological signs or symptoms

Seizures

Confusion

Impaired alertness

Weakness

Papilledema

Systemic illness

Fever

Nuchal rigidity

Weight loss

Scalp artery tenderness

Secondary risk factors

Cancer

Immunocompromised host

Human immunodeficiency virus (HIV)

On immunosuppressants

Recent travel

Domestic

Foreign

Yellow Flags

Wakes patient from sleep at night

New onset side-locked headaches

Postural headaches

From De Luca, G.C., Bartleson, J.D., 2010. When and how to investigate the patient with headache. Semin. Neurol. 30, 133–134. Used with permission.

Other Medical or Neurological Problems

A history of past and current medical and neurological conditions, injuries, operations, and medication allergies should be obtained. A list of all current medications and dietary supplements should be recorded. A number of medications can cause headache, including hormonal, cardiovascular, and gastrointestinal agents (De Luca and Bartleson, 2010).

Neuroimaging and Other Imaging Studies

Cerebrospinal Fluid Tests

CSF examination can diagnose or exclude meningitis, encephalitis, subarachnoid hemorrhage, and leptomeningeal cancer and lymphoma. It can also document increased or decreased intracranial pressure and confirm the diagnosis of “headache and neurological deficits with CSF lymphocytosis” (HaNDL) (Gomez-Aranda et al., 1997). Measurement of the opening CSF pressure should always be performed.

Electrophysiological Testing

Electroencephalography is not useful in the investigation of headache unless the patient also has a history of seizures, syncope, or episodes of altered awareness (Sandrini et al., 2004). There is no indication for use of evoked potentials in evaluating the patient with headache (Sandrini et al., 2004).

General Medical Tests

A few blood tests are important in the investigation of headache. Elevation of the erythrocyte sedimentation rate (ESR), often to 100 mm per hour or higher, is frequently seen in GCA. A normal ESR does not exclude the condition, because 4% of patients with positive findings on temporal artery biopsy have a normal ESR (Smetana et al., 2002). C-reactive protein and platelet count are also often elevated in patients with GCA. Rarely, episodic headaches associated with unusual behavior or impairment of consciousness can suggest an insulinoma, which is supported by elevated serum insulin and C-peptide levels in the face of a low or relatively low fasting glucose level. Levels of carboxyhemoglobin can be measured in patients complaining of early morning headaches during the home heating season, especially when several members of the same household are affected. Drug and alcohol screening may be helpful in certain patients. Thyroid function should be checked in patients with chronic headache, because hypothyroidism can present with headaches. Plasma and urine levels of catecholamines and metanephrines should be measured if a pheochromocytoma is suspected.

Rarely, cigarette smokers can present with face pain that includes the ear and is due to an underlying ipsilateral lung tumor without CNS involvement. Chest radiograph or CT of the chest can confirm the diagnosis (Eross et al., 2003).

Special Examinations and Consultations

In patients who wake from sleep with headache without other reason for awakening, polysomnography can be performed to look for a treatable sleep disorder such as sleep apnea.

Formal visual field testing can be useful. Tonometry can document elevated intraocular pressure in glaucoma, but unless the eye is red or the cornea is cloudy, glaucoma is an uncommon cause of head or even eye pain. These tests are routinely done by ophthalmologists, who also have the equipment and expertise to perform slit-lamp and other specialized examinations. If pain of dental or temporomandibular joint origin is suspected, an oral surgeon or dentist skilled in the detection and treatment of these disorders should be consulted. Diagnosis of tumors of the sinuses, nasopharynx, and neck, as well as inflammation of the sinuses, is aided by consultation with an otorhinolaryngologist. Temporal artery biopsy is performed to confirm or exclude GCA. In some selected cases (e.g., headaches as a manifestation of a chronic pain disorder or a history of drug abuse), psychiatric consultation may be helpful in diagnosis and management.