Evaluation of Headache in Adults

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Evaluation of headache in adults
Authors: Zahid H Bajwa, MD, R Joshua Wootton, MDiv, PhD, Franz J Wippold II, MD, FACR
Section Editors: Jerry W Swanson, MD, MHPE, Jeffrey G Jarvik, MD, MPH
Deputy Editors: John F Dashe, MD, PhD, Susanna I Lee, MD, PhD
All topics are updated as new evidence becomes available and our peer review process is complete.
Literature review current through: Jul 2018. | This topic last updated: Apr 27, 2018.
INTRODUCTION — Headache is among the most common medical complaints. An overview of the approach to the patient with a chief complaint of headache is presented here. The
approach to adults presenting with headache in the emergency department is reviewed elsewhere. (See "Evaluation of the adult with nontraumatic headache in the emergency
department".)
The clinical features and management of specific primary headache syndromes are discussed separately. (See "Pathophysiology, clinical manifestations, and diagnosis of migraine in
adults" and "Tension-type headache in adults: Pathophysiology, clinical features, and diagnosis" and "Cluster headache: Epidemiology, clinical features, and diagnosis".)
EPIDEMIOLOGY AND CLASSIFICATION — As many as 90 percent of all primary headaches fall under a few categories, including migraine, tension-type, and cluster headache.
While episodic tension-type headache (TTH) is the most frequent headache type in population-based studies, migraine is the most common diagnosis in patients presenting to primary
care physicians with headache. The one-year prevalence of episodic TTH is approximately 65 percent (see "Tension-type headache in adults: Pathophysiology, clinical features, and
diagnosis", section on 'Epidemiology'), but most people with TTH do not present to clinicians for care. As an example, a study of two primary care units in Brazil found that migraine
was the most prevalent primary headache disorder, accounting for 45 percent of patients reporting headache as a single symptom [1]. Similarly, in the multinational Landmark study,
which evaluated 1203 patients visiting a primary practitioner with a complaint of episodic headache, migraine was present in 94 percent of the subjects, while TTH, by definition
episodic, was present in 3 percent [2].
Cluster headache typically leads to significant disability and most of these patients will come to medical attention. However, cluster headache remains an uncommon diagnosis in
primary care settings because of overall low prevalence in the general population (<1 percent). (See "Cluster headache: Epidemiology, clinical features, and diagnosis", section on
'Epidemiology'.)
Clinicians can easily become familiar with the most common primary headache disorders and how to distinguish them (table 1).
Migraine — Migraine is a disorder of recurrent attacks. The headache of migraine is often but not always unilateral and tends to have a throbbing or pulsatile quality. Accompanying
features may include nausea, vomiting, photophobia, or phonophobia during attacks (table 2). (See "Pathophysiology, clinical manifestations, and diagnosis of migraine in adults".)
Migraine trigger factors (table 3) may include stress, menstruation, visual stimuli, weather changes, nitrates, fasting, wine, sleep disturbances, and aspartame, among others. (See
"Pathophysiology, clinical manifestations, and diagnosis of migraine in adults", section on 'Precipitating and exacerbating factors'.)
Tension-type headache — The typical presentation of a TTH attack is that of a mild to moderate intensity, bilateral, nonthrobbing headache without other associated features. Pure
TTH is a rather featureless headache. (See "Tension-type headache in adults: Pathophysiology, clinical features, and diagnosis".)
Cluster headache — Cluster headache belongs to a group of idiopathic headache entities, the trigeminal autonomic cephalalgias (table 4), all of which involve unilateral, often severe
headache attacks and typical accompanying autonomic symptoms. Cluster headache is characterized by attacks of severe unilateral orbital, supraorbital, or temporal pain
accompanied by autonomic phenomena. Unilateral autonomic symptoms are ipsilateral to the pain and may include ptosis, miosis, lacrimation, conjunctival injection, rhinorrhea, and
nasal congestion. Attacks usually last 15 to 180 minutes. (See "Cluster headache: Epidemiology, clinical features, and diagnosis".)
Cluster headache may sometimes be confused with a life-threatening headache, since the pain from a cluster headache can reach full intensity within minutes. However, cluster
headache is transient, usually lasting less than one to two hours.
Secondary headache — Clinicians who evaluate patients with headache should be alert to signs that suggest a serious underlying disorder. (See 'Danger signs' below.)
In the Brazilian primary care study, 39 percent of patients presenting with headache had a headache that was due to a systemic disorder (most commonly fever, acute hypertension,
and sinusitis), and 5 percent had a headache that was due to a neurologic disorder (most commonly posttraumatic headache, headaches secondary to cervical spine disease, and
expansive intracranial processes) [1].
EVALUATION — The appropriate evaluation of headache complaints includes the following:
● Rule out serious underlying pathology and look for other secondary causes of headache (algorithm 1).
● Determine the type of primary headache using the patient history as the primary diagnostic tool (table 1). There may be overlap in symptoms, particularly between migraine and
tension-type headache (TTH) and between migraine and some secondary causes of headache such as sinus disease.
A systematic case history is the single most important factor in establishing a headache diagnosis and determining the future work-up and treatment plan. Imaging is not necessary in
the vast majority of patients presenting with headache. Nevertheless, brain imaging is warranted in the patients with danger signs suggesting a secondary cause of headache. (See
'Indications for imaging' below.)
History and examination — A thorough history can focus the physical examination and determine the need for further investigations and imaging exams. A systematic history should
include the following:
● Age at onset
● Presence or absence of aura and prodrome
● Frequency, intensity, and duration of attack
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● Number of headache days per month
● Time and mode of onset
● Quality, site, and radiation of pain
● Associated symptoms and abnormalities
● Family history of migraine
● Precipitating and relieving factors
● Exacerbation or relief with change in position (eg, lying flat versus upright)
● Effect of activity on pain
● Relationship with food/alcohol
● Response to any previous treatment
● Review of current medications
● Any recent change in vision
● Association with recent trauma
● Any recent changes in sleep, exercise, weight, or diet
● State of general health
● Change in work or lifestyle (disability)
● Change in method of birth control (women)
● Possible association with environmental factors
● Effects of menstrual cycle and exogenous hormones (women)
The examination of an adult with headache complaints should cover the following areas:
● Obtain blood pressure and pulse
● Listen for bruit at neck, eyes, and head for clinical signs of arteriovenous malformation
● Palpate the head, neck, and shoulder regions
● Check temporal and neck arteries
● Examine the spine and neck muscles
The neurologic examination should cover mental status testing, cranial nerve examination, funduscopy and otoscopy, and symmetry on motor, reflex, cerebellar (coordination), and
sensory tests. Gait examination should include getting up from a seated position without any support and walking on tiptoes and heels, tandem gait, and Romberg test.
Low-risk features — The following features can serve as indicators of patients who are unlikely to have serious underlying cause for headache [3,4]:
● Age ≤50 years
● Features typical of primary headaches (table 1)
● History of similar headache
● No abnormal neurologic findings
● No concerning change in usual headache pattern
● No high-risk comorbid conditions
● No new or concerning findings on history or examination
Patients with headache who meet these criteria do not require imaging.
The majority of patients with headache complaints have a completely normal physical and neurologic examination. However, some types of primary headache may be associated with
specific abnormalities:
● With TTH, there may be pericranial muscle tenderness.
● With migraine, there may be manifestations related to sensitization of primary nociceptors and central trigeminovascular neurons, such as hyperalgesia and allodynia.
● With hemicrania continua or one of the other trigeminal autonomic cephalalgias (cluster headache, paroxysmal hemicrania, and short-lasting unilateral neuralgiform headache
attacks), there may be evidence of autonomic activation.
Other abnormalities on examination should raise suspicion for a secondary headache disorder. Likewise, danger signs (ie, red flags) should prompt further evaluation, as discussed in
the sections below. (See 'Danger signs' below.)
Features suggesting migraine — The most common headache syndromes frequently present with characteristic symptoms (table 1). However, there may be considerable
symptom overlap; one population-based survey found that less than one-half of patients who complained of headaches that met criteria for migraine were properly diagnosed [5].
Migraine symptoms may also overlap with other causes of headache. As an example, a significant number of patients with migraine may have nasal symptoms that suggest sinus
disease [6]; in addition, a study of primary care patients with recurrent sinus headache found that 90 percent experienced attacks that met the International Headache Society criteria
for migraine [7]. (See 'Sinus symptoms' below.)
Given these pitfalls, a number of diagnostic instruments have been proposed, mainly to assist with the diagnosis of migraine, the most common primary headache syndrome in
patients presenting to primary care physicians. One such instrument (ID Migraine) preselects eligible subjects as those who had two or more headaches in the previous three months
and indicated either that they might want to speak with a health care professional about their headaches or that they experienced a headache that limited their ability to work, study, or
enjoy life [8]. The screen employs three questions:
During the last three months, did you have the following with your headaches?
● You felt nauseated or sick to your stomach.
● Light bothered you (a lot more than when you do not have headaches).
● Your headaches limited your ability to work, study, or do what you needed to do for at least one day.
The ID Migraine screen is positive if the patient answers "yes" to two of the three items. In a systematic review of 13 studies that involved over 5800 patients, the pooled sensitivity and
specificity of ID Migraine was 0.84 and 0.76, respectively [9]. A positive ID Migraine increased the pretest probability of migraine from 59 to 84 percent, whereas a negative ID Migraine
score reduced the probability of migraine from 59 to 23 percent.
Another simple and validated instrument, the brief headache screen, consists of three to six questions [10]. One version includes the following four questions:
● How often do you get severe headaches (ie, without treatment it is difficult to function)?
● How often do you get other (milder) headaches?
● How often do you take headache relievers or pain pills?
● Has there been any recent change in your headaches?
In one study, the presence of episodic disabling headache correctly identified migraine in 136 of 146 patients (93 percent) with episodic migraine, and 154 of 197 patients (78 percent)
with chronic headache with migraine, with a specificity of 63 percent [10]. Only 6 of 343 patients (2 percent) with migraine were not identified by disabling headache. Thus, virtually any
patient with severe episodic headaches can be considered to have migraine.
Among the questions above, the second on frequency of headache and the third on the need for pain pills may be helpful for identifying patients with medication overuse (eg, patients
who use symptomatic medications more than three days per week and/or who have daily headaches). The last question about recent changes in the headache is particularly helpful
for identifying patients who may have an important secondary cause of headache; a patient with a stable pattern of headache for six months is unlikely to have a serious underlying
cause.
Danger signs — Paying attention to danger signs is important since headaches may be the presenting symptom of a space-occupying mass or vascular lesion, infection, metabolic
disturbance, or a systemic problem. The following features in the history can serve as warning signs of possible serious underlying disease [11-13]. (See "Evaluation of the adult with
nontraumatic headache in the emergency department".)
The mnemonic SNOOP is a reminder of the danger signs ("red flags") for the presence of serious underlying disorders that can cause acute or subacute headache [14,15]:
● Systemic symptoms, illness, or condition (eg, fever, weight loss, cancer, pregnancy, immunocompromised state, including human immunodeficiency virus [HIV])
● Neurologic symptoms or abnormal signs (eg, confusion, impaired alertness or consciousness, papilledema, focal neurologic symptoms or signs, meningismus, or seizures)
● Onset is new (particularly for age >50 years) or sudden (eg, "thunderclap")
● Other associated conditions or features (eg, head trauma, illicit drug use, or toxic exposure; headache awakens from sleep, is worse with Valsalva maneuvers, or is precipitated
by cough, exertion, or sexual activity)
● Previous headache history with headache progression or change in attack frequency, severity, or clinical features
Any of these findings should prompt further investigation, including brain imaging with magnetic resonance imaging (MRI) or computed tomography (CT).
Specific features suggesting a secondary headache source — Other features that suggest a specific source of headache pain include the following:
● Impaired vision or seeing halos around light suggests the presence of glaucoma. Suspicion for subacute angle closure glaucoma should be raised by relatively short duration
(often less than one hour) unilateral headaches that do not meet criteria for migraine arising after age 50 [16].
● Visual field defects suggest the presence of a lesion of the optic pathway (eg, due to a pituitary mass).
● Sudden, severe, unilateral vision loss suggests the presence of optic neuritis. Optic neuritis typically presents with painful, monocular visual loss that evolves over several hours
to a few days. One-third of patients have visible optic nerve inflammation (papillitis) on funduscopic examination. (See "Optic neuritis: Pathophysiology, clinical features, and
diagnosis".)
● Blurring of vision on forward bending of the head, headaches upon waking early in the morning that improve with sitting up, and double vision or loss of coordination and balance
should raise the suspicion of raised intracranial pressure (ICP); this should also be considered in patients with chronic, daily, progressively worsening headaches associated with
chronic nausea. Idiopathic intracranial hypertension (pseudotumor cerebri) typically affects obese women of child-bearing age. Characteristic features are headache, papilledema,
vision loss or diplopia, elevated lumbar puncture (LP) opening pressure with normal cerebrospinal fluid (CSF) composition. (See "Evaluation and management of elevated
intracranial pressure in adults" and "Idiopathic intracranial hypertension (pseudotumor cerebri): Clinical features and diagnosis".)
● In patients who present with headache that is relieved with recumbency and exacerbated with upright posture, the diagnosis of headache attributed to spontaneous intracranial
hypotension should be considered. An additional major feature of this headache syndrome is diffuse meningeal enhancement on brain MRI. The accepted etiology is CSF
leakage, which may occur in the context of rupture of an arachnoid membrane. (See "Spontaneous intracranial hypotension: Pathophysiology, clinical features, and diagnosis".)
● The presence of nausea, vomiting, worsening of headache with changes in body position (particularly bending over), an abnormal neurologic examination, and/or a significant
change in prior headache pattern suggests the headache was caused by a tumor. (See "Overview of the clinical features and diagnosis of brain tumors in adults".)
● Intermittent headache with generalized sweating, tachycardia, and/or sustained or paroxysmal hypertension is suggestive of pheochromocytoma. (See "Clinical presentation and
diagnosis of pheochromocytoma".)
● Morning headache is nonspecific and can occur as part of a primary headache syndrome or may be secondary to a number of disorders including sleep apnea, chronic
obstructive pulmonary disease, and the obesity hypoventilation syndrome. (See "Clinical presentation and diagnosis of obstructive sleep apnea in adults" and "Chronic obstructive
pulmonary disease: Definition, clinical manifestations, diagnosis, and staging" and "Clinical manifestations and diagnosis of obesity hypoventilation syndrome".)
Need for emergency evaluation — A small proportion of patients present with serious or life-threatening headaches that require referral for emergency diagnosis and treatment.
These include:
● Sudden onset "thunderclap" headache – Severe headache of sudden onset (ie, that reaches maximal intensity within a few seconds or less than one minute after the onset
of pain) is known as thunderclap headache because its explosive and unexpected nature is likened to a "clap of thunder." Thunderclap headache requires urgent evaluation as
such headaches may be harbingers of subarachnoid hemorrhage and other potentially ominous etiologies (table 5). (See "Approach to the patient with thunderclap headache".)
● Acute or subacute neck pain or headache with Horner syndrome and/or neurologic deficit – Cervical artery dissection is usually associated with local symptoms including
neck pain or headache, and often results in ischemic stroke or transient ischemic attack. Horner syndrome is seen in approximately 39 percent of those with carotid and 13
percent of those with vertebral artery dissection [17,18].
● Headache with suspected meningitis or encephalitis – Fever, altered mental status, with or without nuchal rigidity may indicate central nervous system infection.
● Headache with global or focal neurologic deficit or papilledema – Headache is the primary symptom of increased ICP, which should be suspected when accompanied by
bilateral papilledema, focal neurologic deficit, or repeated episodes of nausea and vomiting.
● Headache with orbital or periorbital symptoms – Headache with visual impairment, periorbital pain, or ophthalmoplegia could indicate acute angle closure glaucoma, infection,
inflammation, or tumor involving the orbits.
● Headache and possible carbon monoxide exposure.
The evaluation of the adult with headache in the emergency department is described elsewhere. Laboratory tests, imaging, and LP for CSF analysis may be included in the evaluation.
(See "Evaluation of the adult with nontraumatic headache in the emergency department".)
Imaging — CT or MRI are the common modalities used to diagnose many causes of secondary headache. Choice of exact body part (eg, head, neck, face, etc) and use of contrast
varies with clinical scenario.
Indications for imaging — Patients with the danger signs or other features suggesting a secondary headache source will require imaging (See 'Danger signs' above and 'Specific
features suggesting a secondary headache source' above.).
Imaging is usually not warranted for patients with a stable migraine pattern and a normal neurologic examination, although a lower threshold for imaging is reasonable for patients with
atypical migraine features or in patients who do not fulfill the strict definition of migraine [19]. As an example, imaging is indicated for patients presenting with recent-onset headache
that is featureless (ie, bilateral, non-throbbing, without nausea and without sensitivity to light, sound, or smell) [20,21]. However, imaging for no other reason than reassurance is
sometimes performed in clinical practice. It is important that the clinician provide the patient with a clear explanation of both the diagnosis and the reason for imaging, especially if it is
being performed in someone suspected of having primary headache [20]. The patient should also be informed that incidental findings (eg, vascular lesion, small neoplasm) likely
unrelated to the headache can be seen in 1 to 2 percent of MRI exams and that there are few data providing guidance as to how they should be managed [22,23].
The vast majority of patients without danger signs do not have a secondary cause of headache [24,25]. As an example, in a study of 373 patients with chronic headache at a tertiary
referral center, all had one or more of the following characteristics that prompted referral for head CT scan: increased severity of symptoms or resistance to appropriate drug therapy,
change in characteristics or pattern of headache, or family history of an intracranial structural lesion [26]. Only two exams (less than 1 percent) showed potentially significant lesions
(one low grade glioma and one aneurysm); only the aneurysm was treated.
Choice of imaging exam — CT scan or MRI of the head is the preferred imaging exam for headache [27]. Choice of modality and need for intravenous (IV) contrast depends upon
the clinical indications. For imaging of the vessels, cerebral and cervical angiography using computed tomography (CTA) or magnetic resonance angiography (MRA) is performed as
an added exam to head CT or MRI and usually requires IV contrast administration. CTA or MRA exams image the arteries, veins, or both, depending on the indication. Exams tailored
for imaging the orbits and ear (encompassing the skull base and pituitary), face, and maxilla (encompassing the paranasal sinuses), or the temporomandibular joint (TMJ) are
sometimes added to the head imaging if an underlying diagnosis that localizes anatomically is suspected. Approximate effective radiation dose for a head CT is 2 mSv.
Factors to consider in imaging exam choice is diagnostic performance for suspected diagnosis, availability of the technology and radiologist expertise, and safety considerations. The
choice of when to image and with what modality for many suspected etiologies of headache are discussed here and in other related UpToDate topics. In addition, the ACR
Appropriateness Criteria provides general guidance for many common clinical scenarios of headache [28]. When the decision is not obvious, consultation with the radiologist is helpful
to facilitate patient referral.
Lumbar puncture — LP for CSF analysis is urgently indicated in patients with headache when there is clinical suspicion of subarachnoid hemorrhage in the setting of a negative or
normal head CT. In addition, LP is indicated when there is clinical suspicion of an infectious, inflammatory, or neoplastic etiology of headache. These issues are discussed elsewhere.
(See "Clinical manifestations and diagnosis of aneurysmal subarachnoid hemorrhage", section on 'Diagnosis of subarachnoid hemorrhage' and "Lumbar puncture: Technique,
indications, contraindications, and complications in adults".)
Common clinical scenarios — Patients with a chief complaint of headache accompanied by factors that suggest a serious but not immediately life-threatening diagnosis should be
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evaluated promptly in the outpatient or inpatient setting. Differences in patient demographics, comorbidities, and headache features can guide the evaluation to help ensure
appropriate diagnosis and management. (See 'Danger signs' above and 'Specific features suggesting a secondary headache source' above.)
New or recent onset headache — The absence of similar headaches in the past, when combined with high-risk features, suggests a possible serious disorder. Head MRI without
and with contrast should be obtained to evaluate for an intracranial mass lesion (eg, primary or metastatic neoplasm, abscess, hematoma), communicating or obstructive
hydrocephalus, or cerebral edema from ischemia or infarction (ie, stroke). If MRI is not available or contraindicated, head CT without and with contrast should be performed instead.
In patients with a new or recent onset of headache, high-risk features include:
● Older age – New headache in patients older than 50 years may suggest underlying pathology.
● Cancer – New headache type in a patient with cancer suggests metastasis. (See "Evaluation of the adult with nontraumatic headache in the emergency department", section on
'New headache in a cancer patient'.)
● Febrile or with Lyme disease – New headache associated with fever and altered mental status with or without nuchal rigidity can indicate meningitis. New headache in a patient
with Lyme disease suggests meningoencephalitis. (See "Evaluation of the adult with nontraumatic headache in the emergency department", section on 'New headache with
suspected meningitis or encephalitis' and "Nervous system Lyme disease", section on 'Lyme encephalomyelitis'.)
● Immunosuppression – New headache type in a patient with immunosuppression suggests an opportunistic infection or tumor.
Brain tumor is a rare cause of headache but should be considered in patients presenting with focal neurologic signs. It should also be considered when new-onset headaches occur in
adults older than 50 years. A prior history of headache does not rule out the possibility of brain tumor, and a change in headache pattern is a diagnostic "red flag." The features of brain
tumor headache are generally nonspecific and vary widely with tumor location, size, and rate of growth. The headache is usually bilateral but can be on the side of the tumor. Brain
tumor headache often resembles TTH but may resemble migraine or a variety of other headache types. (See "Brain tumor headache".)
In the absence of danger signs, patients who present with a new or recent onset headache and a normal neurologic examination are most likely to have primary headache, such as
migraine or TTH (table 1).
Older patients — Older patients are at increased risk for secondary types of headache (eg, giant cell arteritis, trigeminal neuralgia, subdural hematoma, acute herpes zoster and
postherpetic neuralgia, and brain tumors) and some types of primary headache (hypnic headache, cough headache, and migraine accompaniments) [29]. Need for imaging depends
on the suspected diagnosis. Diagnostic consideration include:
● Giant cell (temporal) arteritis (GCA) is a chronic vasculitis of large and medium sized vessels. The disease seldom occurs before age 50 years, and its incidence rises steadily
thereafter. A new type of headache occurs in two-thirds of affected individuals. The head pain tends to be located over the temporal areas but can be frontal or occipital in
location. The headaches may be mild or severe. Other common symptoms can include fever, fatigue, weight loss, jaw claudication, visual symptoms, particularly transient
monocular visual loss and diplopia, and symptoms of polymyalgia rheumatica. Laboratory testing may reveal an elevated erythrocyte sedimentation rate and/or serum C-reactive
protein, but these are not specific. The diagnosis is based on histopathology or imaging exams. Histopathologic evidence of GCA is most often acquired by temporal artery biopsy.
It may be possible to substitute for color Doppler ultrasound of the head, as performed by experienced operators, for temporal artery biopsy as a diagnostic procedure in the
appropriate clinical setting. (See "Clinical manifestations of giant cell arteritis" and "Diagnosis of giant cell arteritis".)
● Trigeminal neuralgia is defined by sudden, usually unilateral, severe, brief, stabbing or lancinating, recurrent episodes of pain in the distribution of one or more branches of the
fifth cranial (trigeminal) nerve. The incidence increases gradually with age; most idiopathic cases begin after age 50 years. Once the diagnosis is suspected on clinical grounds, it
is important to search for secondary causes. Patients with suspected trigeminal neuralgia or those with recurrent attacks of pain limited to one or more divisions of the trigeminal
nerve and no obvious cause (eg, herpes zoster or trigeminal nerve trauma) should undergo imaging to help distinguish classic trigeminal neuralgia from secondary causes. MRI
and MRA of the head without and with contrast tailored to evaluate the trigeminal nerve is the preferred imaging exam to evaluate for compression of the nerve by adjacent
vessels or other structures. (See "Trigeminal neuralgia".)
● Chronic subdural hematoma may present with the insidious onset of headaches, light-headedness, cognitive impairment, apathy, somnolence, and occasionally seizures. Imaging
with noncontrast CT or MRI is essential to confirm the diagnosis. (See "Subdural hematoma in adults: Etiology, clinical features, and diagnosis".)
● Acute herpes zoster and postherpetic neuralgia often involve cervical and trigeminal nerves. Pain is the most common symptom of zoster and approximately 75 percent of
patients have prodromal pain in the dermatome where the rash subsequently appears. The major risk factors for postherpetic neuralgia are older age, greater acute pain, and
greater rash severity. Acute herpes zoster is usually a clinical diagnosis based upon the characteristic vesicular lesions in a restricted dermatomal pattern. The diagnosis of
postherpetic neuralgia is made when pain persists beyond four months in the same distribution as a preceding documented episode of acute herpes zoster. (See "Clinical
manifestations of varicella-zoster virus infection: Herpes zoster" and "Postherpetic neuralgia".)
● Brain tumor should be considered as a possible cause of new-onset headaches in adults over age 50 years, as discussed above. (See 'New or recent onset headache' above and
"Brain tumor headache".)
● Hypnic headache, also known as "alarm clock headache," occurs almost exclusively after the age of 50 years and is characterized by episodes of dull head pain, often bilateral,
that awaken the sufferer from sleep. The diagnosis requires the exclusion of nocturnal attacks caused by other primary and secondary headaches. Therefore, imaging of the
brain, preferably by MRI without and with contrast, should be obtained to look for a structural cause. (See "Hypnic headache".)
● Primary cough headache most often affects people older than age 40 years and is provoked by coughing or straining in the absence of any intracranial disorder. Patients
presenting with de novo headache precipitated by coughing should have imaging, preferably brain MRI without and with contrast, to exclude a structural lesion. (See "Primary
cough headache".)
Pregnancy — New headache or change in headache during pregnancy may be due to migraine or another primary headache, but many other conditions can present with
headache at this time, particularly pre-eclampsia, postdural puncture headache, and cerebral venous thrombosis. Pre-eclampsia must be ruled in or out in every pregnant woman over
20 weeks of gestation with headache. (See "Preeclampsia: Clinical features and diagnosis".)
MRI without contrast is recommended when there is concern for a secondary headache, and MR venography without contrast should be included if cerebral venous sinus thrombosis
is a concern. If MRI is not immediately available or contraindicated, head CT without and with contrast can be used to evaluate for hemorrhage, mass effect or hydrocephalus. (See
"Headache in pregnant and postpartum women".)
Fever — Fever associated with headache may be caused by intracranial, systemic, or local infection, as well as other etiologies (table 6). Emergency evaluation is indicated if fever
is accompanied by symptoms suggestive of meningitis or encephalitis (eg, altered mental status, with or without nuchal rigidity). (See "Evaluation of the adult with nontraumatic
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or encephalitis'.)
Oromaxillofacial symptoms — TMJ disorders, trigeminal neuralgia and odontogenic conditions (eg, tooth impaction, dental abscess) may present as headache with facial pain.
Imaging tailored for the anatomic site of concern is usually indicated. (See "Overview of craniofacial pain" and "Temporomandibular disorders in adults" and "Trigeminal neuralgia" and
"Complications, diagnosis, and treatment of odontogenic infections".)
Head injury — Approach to diagnostic evaluation and imaging of headache following trauma and is discussed elsewhere. (See "Postconcussion syndrome", section on
'Headaches' and "Acute mild traumatic brain injury (concussion) in adults", section on 'Imaging'.)
Headache is variably estimated as occurring in 25 to 78 percent of persons following mild traumatic brain injury. Paradoxically, headache prevalence, duration, and severity is greater
in those with mild head injury compared with those with more severe trauma. Most often, the headache following head trauma can be clinically classified similarly to nontraumatic
headaches; migraine and TTH predominate.
Sinus symptoms — Although sinus headache is commonly diagnosed by clinicians and self-diagnosed by patients, acute or chronic sinusitis appears to be an uncommon cause
of recurrent headaches [30-32].
Headache of sinonasal origin usually does not require imaging for diagnosis as it is best evaluated with nasal endoscopy. If intracranial complications of sinus disease is suspected,
head MRI without and with contrast is indicated. (See "Acute sinusitis and rhinosinusitis in adults: Clinical manifestations and diagnosis".)
Autonomic features characteristically occur in trigeminal autonomic cephalalgias such as cluster headaches and are also common with migraine headache. These symptoms may
include nasal congestion, rhinorrhea, tearing, color and temperature change, and changes in pupil size. (See "Pathophysiology, clinical manifestations, and diagnosis of migraine in
adults".)
The prominence of sinus symptoms often leads to the misdiagnosis of "sinus headache" in patients who meet diagnostic criteria for migraine or, less often, TTH. This point is
illustrated by an observational study that enrolled 2991 patients with a history of clinician- or self-diagnosed sinus headache and no previous history of migraine; 88 percent of these
patients fulfilled criteria for migraine or migrainous headache, and 8 percent fulfilled criteria for TTH [33]. In the patients with migraine or migrainous headache, sinus pain, pressure,
and congestion commonly occurred in association with typical migraine features such as pulsing head pain and sensitivity to activity, light, and sound (figure 1).
Pain related purely to sinus conditions may have some features that aid in distinguishing it from migraine [34,35]. Sinus-related pain or headache is typically described as a pressure-
like or dull sensation that is usually bilateral and periorbital. However, it can be unilateral with deviated septum, middle or inferior turbinate hypertrophy, or unilateral sinus disease. In
addition, sinus-related pain is typically associated with nasal obstruction or congestion, lasts for days at a time, and is usually not associated with nausea, vomiting, photophobia, or
sonophobia. (See "Acute sinusitis and rhinosinusitis in adults: Clinical manifestations and diagnosis".)
The severity, extent, and location of sinus-related pain do not correlate with the extent or location of mucosal disease as revealed by imaging [35].
In general, the following principles apply to the relationship of rhinosinusitis and headache [34,36,37]:
● A stable pattern of recurrent headaches that interfere with daily function is most likely migraine.
● Recurrent self-limited headaches associated with rhinogenic symptoms are most likely migraine.
● Prominent rhinogenic symptoms with headache as one of several symptoms should be evaluated carefully for otolaryngologic conditions.
● Headache associated with fever and purulent nasal discharge is likely rhinogenic in origin.
Chronic headache — Chronic daily headache is not a specific headache type, but a syndrome that encompasses several primary and secondary headaches. The term "chronic"
refers either to the frequency of headaches or to the duration of the disease, depending upon the specific headache type. (See "Overview of chronic daily headache".)
In adults with chronic recurrent headaches, including those with migraine aura, with no recent change in headache pattern, no history of seizures, and no other focal neurologic signs
or symptoms, the routine use of imaging is not warranted. The yield of head CT or MRI in identifying potentially treatable lesions is <1 percent [38]. However, imaging to exclude a
secondary cause of headache is indicated in the initial evaluation of patients presenting with hemicrania continua, new daily persistent headache, cluster headache, paroxysmal
hemicrania, short-lasting unilateral neuralgiform headache attacks, and hypnic headache, described below.
With headache subtypes of long duration (ie, four hours or more), "chronic" indicates a headache frequency of 15 or more days a month for longer than three months in the absence of
organic pathology. These headache subtypes are:
● Chronic migraine headache (see "Chronic migraine")
● Chronic TTH (see "Tension-type headache in adults: Pathophysiology, clinical features, and diagnosis")
● Medication overuse headache, which is typically preceded by an episodic headache disorder (usually migraine or TTH) that has been treated with frequent and excessive
amounts of acute symptomatic medications (see "Medication overuse headache: Etiology, clinical features, and diagnosis")
● Hemicrania continua, a strictly unilateral, continuous headache with superimposed exacerbations of moderate to severe intensity accompanied by autonomic features and
sometimes by migrainous symptoms (see "Hemicrania continua")
● New daily persistent headache, characterized by headache that begins rather abruptly and is daily and unremitting from onset or within three days of onset at most, typically in
individuals without a prior headache history (see "New daily persistent headache")
With headache subtypes of shorter duration (ie, less than four hours), "chronic" refers to a prolonged duration of the condition itself without remission. The headache subtypes in this
category are the following:
● Chronic cluster headache (see "Cluster headache: Epidemiology, clinical features, and diagnosis")
● Chronic paroxysmal hemicrania, characterized by unilateral, brief, severe attacks of pain associated with cranial autonomic features that recur several times per day with
individual headache attacks that usually last 2 to 30 minutes (see "Paroxysmal hemicrania: Clinical features and diagnosis")
● Short-lasting unilateral neuralgiform headache attacks, characterized by sudden brief attacks of severe unilateral head pain in orbital, peri-orbital, or temporal regions,
accompanied by ipsilateral cranial autonomic symptoms (see "Short-lasting unilateral neuralgiform headache attacks: Clinical features and diagnosis")
● Hypnic headache, also known as "alarm clock headache," which occurs almost exclusively after the age of 50 years and is characterized by episodes of dull head pain, often
bilateral, that awaken the sufferer from sleep (see "Hypnic headache")
● Primary stabbing headache, characterized by sudden brief attacks of sharp, jabbing head pain in orbital, peri-orbital, or temporal regions (see "Primary stabbing headache")
Misconceptions — Several misconceptions may hinder headache evaluation and diagnosis.
● Although sinus headache is commonly diagnosed by clinicians and self-diagnosed by patients, acute or chronic sinusitis appears to be an uncommon cause of recurrent
headaches, and many patients presenting with sinus headache turn out to have migraine [30-32]. (See 'Sinus symptoms' above.)
● Patients frequently attribute headaches to eye strain. However, an observational study suggested that headaches are only rarely due to refractive error alone [39]. Nevertheless,
correcting vision may improve headache symptoms in some of patients with headache and refractive error.
● Some patients believe that hypertension is the cause of their headaches. While this can be true in the case of a hypertensive emergency, it is probably not true for typical migraine
or TTHs. As an example, a report from the Physicians' Health Study of 22,701 American male physicians ages 40 to 84 years analyzed various risk factors for cerebrovascular
disease and found no difference in the percentage of men with a history of hypertension in the migraine and nonmigraine groups [40]. Furthermore, a prospective study of 22,685
adults in Norway found that high systolic and diastolic blood pressures were actually associated with a reduced risk of nonmigrainous headache [41].
SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society
guideline links: Migraine and other primary headache disorders".)
INFORMATION FOR PATIENTS — UpToDate offers two types of patient education materials, "The Basics" and "Beyond the Basics." The Basics patient education pieces are written
in plain language, at the 5th to 6th grade reading level, and they answer the four or five key questions a patient might have about a given condition. These articles are best for patients
who want a general overview and who prefer short, easy-to-read materials. Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These
articles are written at the 10th to 12th grade reading level and are best for patients who want in-depth information and are comfortable with some medical jargon.
Here are the patient education articles that are relevant to this topic. We encourage you to print or e-mail these topics to your patients. (You can also locate patient education articles
on a variety of subjects by searching on "patient info" and the keyword(s) of interest.)
● Basics topic (see "Patient education: Headaches in adults (The Basics)")
● Beyond the Basics topics (see "Patient education: Headache causes and diagnosis in adults (Beyond the Basics)" and "Patient education: Headache treatment in adults (Beyond
the Basics)")
SUMMARY AND RECOMMENDATIONS
● While episodic tension-type headache (TTH) is the most frequent headache type in population-based studies, migraine is the most common diagnosis in patients presenting to
medical attention with headache. Clinicians can easily become familiar with the most common primary headache disorders and how to distinguish them (table 1). (See
'Epidemiology and classification' above.)
● Using the patient history as the primary diagnostic tool, the initial headache evaluation (algorithm 1) should determine whether the headache is primary or secondary and, if the
latter, whether the underlying cause is serious. (See 'Evaluation' above.)
● The following features can serve as indicators of patients who are unlikely to have serious underlying cause for headache:
• Age ≤50 years
• Features typical of primary headaches
• History of similar headache
• No abnormal neurologic findings
• No concerning change in usual headache pattern
• No high-risk comorbid conditions
• No new or concerning findings on history or examination
Patients with headache who meet these criteria do not require imaging. (See 'Low-risk features' above.)
● The mnemonic SNOOP is a reminder of the danger signs ("red flags") for the presence of serious underlying disorders that can cause acute or subacute headache:
• Systemic symptoms, illness, or condition (eg, fever, weight loss, cancer, pregnancy, immunocompromised state)
• Neurologic symptoms or abnormal signs (eg, confusion, impaired alertness or consciousness, papilledema, focal neurologic symptoms or signs, meningismus, or seizures)
• Onset is new (particularly for age >50 years) or sudden (eg, "thunderclap")
• Other associated conditions or features (eg, head trauma, illicit drug use, or toxic exposure; headache awakens from sleep, is worse with Valsalva maneuvers, or is
precipitated by cough, exertion, or sexual activity)
• Previous headache history with headache progression or change in attack frequency, severity, or clinical features
Patients with danger signs or other features of a secondary headache source require prompt evaluation and imaging (algorithm 1). (See 'Danger signs' above and 'Specific
features suggesting a secondary headache source' above and 'Common clinical scenarios' above.)
● Emergency diagnosis and treatment is indicated for sudden onset of severe headache (ie, thunderclap headache) and for new headache with suspected meningitis or
encephalitis, with neck pain with Horner syndrome suggesting cervical artery dissection, with focal neurologic deficit or papilledema suggesting increased intracranial pressure
(ICP), or with orbital or periorbital symptoms (algorithm 1). (See 'Need for emergency evaluation' above.)
● Computed tomography (CT) or magnetic resonance imaging (MRI) of the head is the preferred imaging exam for headache. Choice of modality and need for intravenous (IV)
contrast depends on the clinical indications (see 'Common clinical scenarios' above). For imaging of the vessels, cerebral and cervical angiography using computed tomography
(CTA) or magnetic resonance angiography (MRA) is performed as an added exam to head CT or MRI and usually requires IV contrast administration. (See 'Imaging' above.)
Use of UpToDate is subject to the Subscription and License Agreement.
REFERENCES
1. Bigal ME, Bordini CA, Speciali JG. Etiology and distribution of headaches in two Brazilian primary care units. Headache 2000; 40:241.
2. Tepper SJ, Dahlöf CG, Dowson A, et al. Prevalence and diagnosis of migraine in patients consulting their physician with a complaint of headache: data from the Landmark Study.
Headache 2004; 44:856.
3. American College of Emergency Physicians.. Clinical policy: critical issues in the evaluation and management of patients presenting to the emergency department with acute
headache. Ann Emerg Med 2002; 39:108.
4. Hainer BL, Matheson EM. Approach to acute headache in adults. Am Fam Physician 2013; 87:682.
5. Lipton RB, Diamond S, Reed M, et al. Migraine diagnosis and treatment: results from the American Migraine Study II. Headache 2001; 41:638.
6. Barbanti P, Fabbrini G, Pesare M, et al. Unilateral cranial autonomic symptoms in migraine. Cephalalgia 2002; 22:256.
7. Cady RK, Schreiber CP. Sinus headache: a clinical conundrum. Otolaryngol Clin North Am 2004; 37:267.
8. Rapoport AM, Bigal ME. ID-migraine. Neurol Sci 2004; 25 Suppl 3:S258.
9. Cousins G, Hijazze S, Van de Laar FA, Fahey T. Diagnostic accuracy of the ID Migraine: a systematic review and meta-analysis. Headache 2011; 51:1140.
10. Maizels M, Burchette R. Rapid and sensitive paradigm for screening patients with headache in primary care settings. Headache 2003; 43:441.
11. Edmeads J. Emergency management of headache. Headache 1988; 28:675.
12. Lipton RB, Bigal ME, Steiner TJ, et al. Classification of primary headaches. Neurology 2004; 63:427.
13. Lynch KM, Brett F. Headaches that kill: a retrospective study of incidence, etiology and clinical features in cases of sudden death. Cephalalgia 2012; 32:972.
14. Dodick D. Headache as a symptom of ominous disease. What are the warning signals? Postgrad Med 1997; 101:46.
15. Venkatesan A. Case 13: a man with progressive headache and confusion. MedGenMed 2006; 8:19.
16. Shindler KS, Sankar PS, Volpe NJ, Piltz-Seymour JR. Intermittent headaches as the presenting sign of subacute angle-closure glaucoma. Neurology 2005; 65:757.
17. Lyrer PA, Brandt T, Metso TM, et al. Clinical import of Horner syndrome in internal carotid and vertebral artery dissection. Neurology 2014; 82:1653.
18. Silbert PL, Mokri B, Schievink WI. Headache and neck pain in spontaneous internal carotid and vertebral artery dissections. Neurology 1995; 45:1517.
19. Silberstein SD, Rosenberg J. Multispecialty consensus on diagnosis and treatment of headache. Neurology 2000; 54:1553.
20. Goadsby PJ. To scan or not to scan in headache. BMJ 2004; 329:469.
21. Practice parameter: the utility of neuroimaging in the evaluation of headache in patients with normal neurologic examinations (summary statement). Report of the Quality
Standards Subcommittee of the American Academy of Neurology. Neurology 1994; 44:1353.
22. Morris Z, Whiteley WN, Longstreth WT Jr, et al. Incidental findings on brain magnetic resonance imaging: systematic review and meta-analysis. BMJ 2009; 339:b3016.
23. Vernooij MW, Ikram MA, Tanghe HL, et al. Incidental findings on brain MRI in the general population. N Engl J Med 2007; 357:1821.
24. Tsushima Y, Endo K. MR imaging in the evaluation of chronic or recurrent headache. Radiology 2005; 235:575.
25. You JJ, Gladstone J, Symons S, et al. Patterns of care and outcomes after computed tomography scans for headache. Am J Med 2011; 124:58.
26. Dumas MD, Pexman JH, Kreeft JH. Computed tomography evaluation of patients with chronic headache. CMAJ 1994; 151:1447.
27. Douglas AC, Wippold FJ 2nd, Broderick DF, et al. ACR Appropriateness Criteria Headache. J Am Coll Radiol 2014; 11:657.
28. America College of Radiology. ACR Appropriateness Criteria. https://acsearch.acr.org/list (Accessed on November 13, 2017).
29. Hale N, Paauw DS. Diagnosis and treatment of headache in the ambulatory care setting: a review of classic presentations and new considerations in diagnosis and
management. Med Clin North Am 2014; 98:505.
30. Cady RK, Schreiber CP. Sinus headache or migraine? Considerations in making a differential diagnosis. Neurology 2002; 58:S10.
31. Mehle ME. What do we know about rhinogenic headache? The otolaryngologist’s challenge. Otolaryngol Clin North Am 2014; 47:255.
32. Eross E, Dodick D, Eross M. The Sinus, Allergy and Migraine Study (SAMS). Headache 2007; 47:213.
33. Schreiber CP, Hutchinson S, Webster CJ, et al. Prevalence of migraine in patients with a history of self-reported or physician-diagnosed "sinus" headache. Arch Intern Med 2004;
164:1769.
34. Cady RK, Dodick DW, Levine HL, et al. Sinus headache: a neurology, otolaryngology, allergy, and primary care consensus on diagnosis and treatment. Mayo Clin Proc 2005;
80:908.
35. Tarabichi M. Characteristics of sinus-related pain. Otolaryngol Head Neck Surg 2000; 122:842.
36. Levine HL, Setzen M, Cady RK, et al. An otolaryngology, neurology, allergy, and primary care consensus on diagnosis and treatment of sinus headache. Otolaryngol Head Neck
Surg 2006; 134:516.
37. Marmura MJ, Silberstein SD. Headaches caused by nasal and paranasal sinus disease. Neurol Clin 2014; 32:507.
38. Frishberg BM. The utility of neuroimaging in the evaluation of headache in patients with normal neurologic examinations. Neurology 1994; 44:1191.
39. Gil-Gouveia R, Martins IP. Headaches associated with refractive errors: myth or reality? Headache 2002; 42:256.
40. Buring JE, Hebert P, Romero J, et al. Migraine and subsequent risk of stroke in the Physicians' Health Study. Arch Neurol 1995; 52:129.
41. Hagen K, Stovner LJ, Vatten L, et al. Blood pressure and risk of headache: a prospective study of 22 685 adults in Norway. J Neurol Neurosurg Psychiatry 2002; 72:463.
Topic 3349 Version 21.0
GRAPHICS
Characteristics of migraine, tension-type, and cluster headache syndromes
Symptom Migraine Tension-type Cluster
Location Adults: Unilateral in 60 to 70%, bifrontal or global in 30% Bilateral Always unilateral, usually begins around the eye or temple
Children and adolescents: Bilateral in majority
Characteristics Gradual in onset, crescendo pattern; pulsating; moderate or Pressure or Pain begins quickly, reaches a crescendo within minutes; pain is deep, continuous,
severe intensity; aggravated by routine physical activity tightness which excruciating, and explosive in quality
waxes and wanes
Patient Patient prefers to rest in a dark, quiet room Patient may remain Patient remains active
appearance active or may need
to rest
Duration 4 to 72 hours 30 minutes to 7 15 minutes to 3 hours

days
Associated Nausea, vomiting, photophobia, phonophobia; may have aura None Ipsilateral lacrimation and redness of the eye; stuffy nose; rhinorrhea; pallor;
symptoms (usually visual, but can involve other senses or cause speech or sweating; Horner syndrome; restlessness or agitation; focal neurologic symptoms
motor deficits) rare; sensitivity to alcohol
Graphic 68064 Version 7.0
Diagnostic criteria for migraine
Migraine without aura

A. At least five attacks fulfilling criteria B through D
B. Headache attacks lasting 4 to 72 hours (untreated or unsuccessfully treated)
C. Headache has at least two of the following characteristics:
Unilateral location
Pulsating quality
Moderate or severe pain intensity
Aggravation by or causing avoidance of routine physical activity (eg, walking or climbing stairs)
D. During headache at least one of the following:
Nausea, vomiting, or both
Photophobia and phonophobia
E. Not better accounted for by another ICHD-3 diagnosis
Migraine with aura

A. At least two attacks fulfilling criterion B and C
B. One or more of the following fully reversible aura symptoms:
Visual
Sensory
Speech and/or language
Motor
Brainstem
Retinal
C. At least three of the following six characteristics:
At least one aura symptom spreads gradually over ≥5 minutes
Two or more symptoms occur in succession
Each individual aura symptom lasts 5 to 60 minutes
At least one aura symptom is unilateral
At least one aura symptom is positive
The aura is accompanied or followed within 60 minutes by headache
D. Not better accounted for by another ICHD-3 diagnosis
Features of migraine in children and adolescents

Attacks may last 2 to 72 hours*
Headache is more often bilateral than in adults; an adult pattern of unilateral pain usually emerges in late adolescence or early adulthood
Photophobia and phonophobia may be inferred by behavior in young children
ICHD-3: International Classification of Headache Disorders, 3 rd edition.

* The evidence for untreated durations of less than two hours in children has not been substantiated.
Adapted from: Headache Classification Committee of the International Headache Society (IHS) The International Classification of Headache Disorders, 3rd edition. Cephalalgia 2018; 38:1.
Headache triggers
Diet Stress
Alcohol Let-down periods
Chocolate Times of intense activity
Aged cheeses Loss or change (death, separation, divorce, job change)
Monosodium glutamate Moving
Aspartame Crisis
Caffeine Changes of environment or habits

Nuts
Weather
Nitrites, nitrates
Travel (crossing time zones)
Hormones Seasons
Menses Altitude
Ovulation Schedule changes
Hormone replacement (progesterone) Sleeping patterns
Sensory stimuli Dieting
Strong light Skipping meals
Irregular physical activity

Flickering lights
Odors
Sounds, noise
Clinical features of the trigeminal autonomic cephalalgias
Cluster headache Paroxysmal hemicrania SUNCT and SUNA Hemicrania continua
Sex (female:male) 1:3 to 1:7 1:1 to 2.7:1 1:1.5 2:1
Pain
Type Stabbing, boring Sharp, stabbing, throbbing Burning, stabbing, sharp Throbbing, sharp, pressure, dull,
burning, aching, or stabbing
Severity Excruciating Excruciating Severe to excruciating Mild to severe
Site Orbit, temple Orbit, temple Periorbital Orbital, frontal, temporal; less often
occipital
Attack frequency 1 every other day to 8 per day 1 to 40 a day (>5 per day for more 1 to 200 per day Continuous pain with exacerbations
than half the time)
Duration of attack 15 to 180 minutes 2 to 30 minutes 1 to 600 seconds Months to years
Autonomic features Yes Yes Yes (prominent conjunctival Yes

injection and lacrimation with
SUNCT)
Restlessness and/or agitation Yes Yes Frequent Yes
Migrainous features (nausea, Yes Yes Rare Frequent

photophobia, or phonophobia)
Alcohol trigger Yes Occasional No Occasional
Cutaneous triggers No Rare Yes No
Indomethacin effect None Absolute None Absolute
Abortive treatment Sumatriptan injection or nasal spray Nil Lidocaine intravenous infusion Nil
Oxygen
Prophylactic treatment Verapamil Indomethacin Lamotrigine Indomethacin

Methysergide Topiramate
Lithium Gabapentin
SUNCT: short-lasting unilateral neuralgiform pain with conjunctival injection and tearing; SUNA: short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms.
Approach to the urgent evaluation of headache in an adult
Image
This is an overview of our approach to the urgent evaluation of headache in an adult. It should be used in conjunction with other UpToDate content on headache.
CO-Hgb: carboxyhemoglobin; CO: carbon monoxide; CT: computed tomography; HA: headache; CNS: central nervous system; MRA: magnetic resonance angiography; MRI: magnetic resonance imaging; MRV:
venography; LP: lumbar puncture; SAH: subarachnoid hemorrhage; c/w: consistent with.
* All patients with acute or subacute head trauma should have head CT scan; pregnant women >20 weeks gestation or women in early postpartum should be evaluated for preeclampsia.
¶ The main clinical features of these conditions are listed below:
Giant cell (temporal) arteritis typically affects people age >50 years and may be associated with systemic manifestations fever, fatigue, weight loss, jaw claudication, visual symptoms, particularly trans
and diplopia, or symptoms of polymyalgia rheumatic.
Acute angle-closure glaucoma may present with vision loss, headache, severe eye pain, light halos, nausea, and vomiting. Exam reveals a red eye, corneal cloudiness or edema, a shallow anterior cham
dilated pupil.
Optic neuritis typically presents with painful, monocular visual loss that evolves over several hours to a few days. One-third of patients have visible optic nerve inflammation (papillitis) on funduscopic exa
and orbits with gadolinium contrast confirms the diagnosis in most cases.
Idiopathic intracranial hypertension (pseudotumor cerebri) typically affects obese women of child-bearing age. Characteristic features are headache, papilledema, vision loss or diplopia, elevated lumb
pressure with normal cerebrospinal fluid composition. MRI with MR venography is the preferred study to exclude secondary causes of elevated intracranial pressure, particularly cerebral venous thrombosis
Pheochromocytoma is a rare condition that may present with episodic headache, sweating, and tachycardia. Sustained or paroxysmal hypertension is the most common sign, but 5 to 15% of patients pr
pressure.
Acute herpes zoster usually presents with a vesicular rash and acute painful neuritis. Prodromal pain may precede the rash by days to weeks. In unusual cases, pain may be related to herpes zoster with
herpete).
Postherpetic neuralgia usually affects the thoracic, cervical, and trigeminal nerves. In most cases, the diagnosis is made when pain persists beyond four months in the same distribution as a preceding d
herpes zoster.
Trigeminal neuralgia is defined by sudden, usually unilateral, severe, brief, stabbing or lancinating, recurrent episodes of pain in the distribution of one or more branches of the fifth cranial (trigeminal) n
Etiologies of thunderclap headache
Most common causes of thunderclap headache:

Subarachnoid hemorrhage
Reversible cerebral vasoconstriction syndromes (RCVS)
Conditions that less commonly cause thunderclap headache:

Cerebral infection (eg, meningitis, acute complicated sinusitis)
Cerebral venous thrombosis
Cervical artery dissection
Spontaneous intracranial hypotension
Acute hypertensive crisis
Posterior reversible leukoencephalopathy syndrome (PRES)
Intracerebral hemorrhage
Ischemic stroke
Conditions that uncommonly or rarely cause thunderclap headache:

Pituitary apoplexy
Colloid cyst of the third ventricle
Acute myocardial infarction
Aortic arch dissection
Aqueductal stenosis
Brain tumor
Giant cell arteritis
Pheochromocytoma
Pneumocephalus
Retroclival hematoma
Spinal epidural hematoma
Varicella zoster virus vasculopathy
Vogt-Koyanagi-Harada syndrome
Uncertain relationship with thunderclap headache:

Sentinel headache (unruptured intracranial aneurysm)
Primary thunderclap headache
Differential diagnosis of headache with fever
Intracranial infection
Meningitis
Bacterial
Fungal
Viral
Lymphocytic
Encephalitis
Brain abscess
Subdural empyema
Systemic infection
Bacterial infection
Viral infection
HIV/AIDS
Other systemic infection
Other causes
Familial hemiplegic migraine
Pituitary apoplexy
Rhinosinusitis
Subarachnoid hemorrhage
Malignancy of central nervous system
HIV/AIDS: human immunodeficiency virus/acquired immunodeficiency syndrome.
Sinus symptoms are common in patients with migraine
Image
In this observational study of patients with a history of sinus headache and

no previous history of migraine, sinus pain, pressure, and congestion
commonly occurred in association with typical migraine features such as
pulsing head pain and sensitivity to activity, light, and sound.
Reproduced with permission from: Schreiber CP, Hutchinson S, Webster CJ, et al.
Prevalence of migraine in patients with a history of self-reported or physician-
diagnosed "sinus" headache. Arch Intern Med 2004; 164:1769. Copyright © 2004
American Medical Association. All rights reserved.
Contributor Disclosures
Zahid H Bajwa, MD Grant/Research Support: Amgen [Chronic migraine (Observational study)]; Nevro [Painful diabetic neuropathy (High-frequency spinal cord stimulation)].
Speaker's Bureau: AstraZeneca [Chronic pain (Naloxegol)]; DSI [OIC (Naloxegol)]. Consultant/Advisory Boards: DSI [Chronic pain (Morphine)]. R Joshua Wootton, MDiv, PhD Other
Financial Interest: McGraw-Hill [Textbook royalties]. Franz J Wippold II, MD, FACR Nothing to disclose Jerry W Swanson, MD, MHPE Nothing to disclose Jeﬀrey G Jarvik, MD,
MPH Equity Ownership/Stock Options: Physiosonics [HIFU (ICP monitors)]. John F Dashe, MD, PhD Nothing to disclose Susanna I Lee, MD, PhD Nothing to disclose
Contributor disclosures are reviewed for conflicts of interest by the editorial group. When found, these are addressed by vetting through a multi-level review process, and through
requirements for references to be provided to support the content. Appropriately referenced content is required of all authors and must conform to UpToDate standards of evidence.
Conflict of interest policy

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EVALUATION — The appropriate evaluation of headache complaints includes the following:

● Presence or absence of aura and prodrome

● Frequency, intensity, and duration of attack

● Number of headache days per month

● Time and mode of onset

● Quality, site, and radiation of pain

● Associated symptoms and abnormalities

● Family history of migraine

● Precipitating and relieving factors

● Effect of activity on pain

● Relationship with food/alcohol

● Response to any previous treatment

● Review of current medications

● Any recent change in vision

● Association with recent trauma

● Any recent changes in sleep, exercise, weight, or diet

● State of general health

● Change in work or lifestyle (disability)

● Change in method of birth control (women)

● Possible association with environmental factors

● Effects of menstrual cycle and exogenous hormones (women)

● Obtain blood pressure and pulse

● Palpate the head, neck, and shoulder regions

● Check temporal and neck arteries

● Examine the spine and neck muscles

● Age ≤50 years

● Features typical of primary headaches (table 1)

● History of similar headache

● No abnormal neurologic findings

● No concerning change in usual headache pattern

● No high-risk comorbid conditions

● No new or concerning findings on history or examination

● With TTH, there may be pericranial muscle tenderness.

the sections below. (See 'Danger signs' below.)

● You felt nauseated or sick to your stomach.

● How often do you get other (milder) headaches?

● How often do you take headache relievers or pain pills?

● Has there been any recent change in your headaches?

● Headache and possible carbon monoxide exposure.

In patients with a new or recent onset of headache, high-risk features include:

● Chronic migraine headache (see "Chronic migraine")

Misconceptions — Several misconceptions may hinder headache evaluation and diagnosis.

● Basics topic (see "Patient education: Headaches in adults (The Basics)")

SUMMARY AND RECOMMENDATIONS

• Age ≤50 years

• Features typical of primary headaches

• History of similar headache

• No abnormal neurologic findings

• No concerning change in usual headache pattern

• No high-risk comorbid conditions

• No new or concerning findings on history or examination

Use of UpToDate is subject to the Subscription and License Agreement.

Topic 3349 Version 21.0

Characteristics of migraine, tension-type, and cluster headache syndromes

Symptom Migraine Tension-type Cluster

Children and adolescents: Bilateral in majority