evaluation of headache in adults

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Official reprint from UpToDate www.uptodate.com ©2015 UpToDate Authors Zahid H Bajwa, MD R Joshua Wootton, MDiv, PhD Section Editor Jerry W Swanson, MD Deputy Editor John F Dashe, MD, PhD Evaluation of headache in adults All topics are updated as new evidence becomes available and our peer review process is complete. Literature review current through: Jul 2015. | This topic last updated: Jun 02, 2015. INTRODUCTION — Headache is among the most common medical complaints. An overview of the approach to the patient with headache is presented here. The approach to adults presenting with headache in the emergency department is reviewed elsewhere. (See "Evaluation of the adult with 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 benign headaches fall under a few categories, including migraine, tension-type, cluster, and chronic daily headache. While episodic tension-type headache 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 tension-type headache (TTH) is approximately 65 percent (see "Tension-type headache in adults: Pathophysiology, clinical features, and diagnosis", section on 'Epidemiology'), but most people with tension-type headache do not present to physicians 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]. 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. (See "Pathophysiology, clinical manifestations, and diagnosis of migraine in adults".) Migraine trigger factors ( table 2) 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 3), 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".) ® ® Evaluation of headache in adults http://www.uptodate.com/contents/evaluation-of-headache-in-adults?to... 1 de 21 14/08/2015 12:35

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Page 1: Evaluation of Headache in Adults

Official reprint from UpToDate

www.uptodate.com ©2015 UpToDate

AuthorsZahid H Bajwa, MDR Joshua Wootton, MDiv, PhD

Section EditorJerry W Swanson, MD

Deputy EditorJohn F Dashe, MD, PhD

Evaluation of headache in adults

All topics are updated as new evidence becomes available and our peer review process is complete.Literature review current through: Jul 2015. | This topic last updated: Jun 02, 2015.

INTRODUCTION — Headache is among the most common medical complaints. An overview of the approach tothe patient with headache is presented here. The approach to adults presenting with headache in the emergencydepartment is reviewed elsewhere. (See "Evaluation of the adult with 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 inadults: Pathophysiology, clinical features, and diagnosis" and "Cluster headache: Epidemiology, clinical features,and diagnosis".)

EPIDEMIOLOGY AND CLASSIFICATION — As many as 90 percent of all benign headaches fall under a fewcategories, including migraine, tension-type, cluster, and chronic daily headache. While episodic tension-typeheadache is the most frequent headache type in population-based studies, migraine is the most commondiagnosis in patients presenting to primary care physicians with headache. The one-year prevalence of episodictension-type headache (TTH) is approximately 65 percent (see "Tension-type headache in adults:Pathophysiology, clinical features, and diagnosis", section on 'Epidemiology'), but most people with tension-typeheadache do not present to physicians for care. As an example, a study of two primary care units in Brazil foundthat migraine was the most prevalent primary headache disorder, accounting for 45 percent of patients reportingheadache as a single symptom [1].

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 lowprevalence in the general population (<1 percent). (See "Cluster headache: Epidemiology, clinical features, anddiagnosis", section on 'Epidemiology'.)

Clinicians can easily become familiar with the most common primary headache disorders and how to distinguishthem (table 1).

Migraine — Migraine is a disorder of recurrent attacks. The headache of migraine is often but not alwaysunilateral and tends to have a throbbing or pulsatile quality. Accompanying features may include nausea,vomiting, photophobia, or phonophobia during attacks. (See "Pathophysiology, clinical manifestations, anddiagnosis of migraine in adults".)

Migraine trigger factors (table 2) 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 trigeminalautonomic cephalalgias (table 3), all of which involve unilateral, often severe headache attacks and typicalaccompanying autonomic symptoms. Cluster headache is characterized by attacks of severe unilateral orbital,supraorbital, or temporal pain accompanied by autonomic phenomena. Unilateral autonomic symptoms areipsilateral to the pain and may include ptosis, miosis, lacrimation, conjunctival injection, rhinorrhea, and nasalcongestion. Attacks usually last 15 to 180 minutes. (See "Cluster headache: Epidemiology, clinical features, anddiagnosis".)

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Cluster headache may sometimes be confused with a life-threatening headache, since the pain from a clusterheadache can reach full intensity within minutes. However, cluster headache is transient, usually lasting lessthan one to two hours.

Secondary headache — Physicians who evaluate patients with headache should be alert to signs that suggesta serious underlying disorder. (See 'Danger signs' below and 'Patient settings' below.)

In the Brazilian primary care study, 39 percent of patients presenting with headache had a headache that wasdue to a systemic disorder (most commonly fever, acute hypertension, and sinusitis), and 5 percent had aheadache that was due to a neurologic disorder (most commonly post-traumatic headache, headachessecondary to cervical spine disease, and expansive intracranial processes) [1].

Misconceptions — A number of misconceptions may hinder headache evaluation and diagnosis.

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

A systematic case history is the single most important factor in establishing a headache diagnosis anddetermining the future work-up and treatment plan. An imaging study is not necessary in the vast majority ofpatients presenting with headache. Nevertheless, brain imaging is warranted in the patients with danger signssuggesting a secondary cause of headache. (See 'Indications for imaging studies' below.)

History and examination — A thorough history can focus the physical examination and determine the need forfurther investigations and neuroimaging studies. A systematic history should include the following:

Although sinus headache is commonly diagnosed by physicians and self-diagnosed by patients, acute orchronic sinusitis appears to be an uncommon cause of recurrent headaches, and many patients presentingwith sinus headache turn out to have migraine [2-4]. (See 'Sinus symptoms' below.)

Patients frequently attribute headaches to eye strain. However, an observational study suggested thatheadaches are only rarely due to refractive error alone [5]. Nevertheless, correcting vision may improveheadache symptoms in some of these patients.

There is a common belief, particularly among patients, that hypertension can cause headaches. While thisis true in the case of hypertensive emergencies, it is probably not true for typical migraine or tensionheadaches. As an example, a report from the Physicians' Health Study of 22,701 American malephysicians ages 40 to 84 years analyzed various risk factors for cerebrovascular disease and found nodifference in the percentage of men with a history of hypertension in the migraine and nonmigraine groups[6]. Furthermore, a prospective study of 22,685 adults in Norway found that high systolic and diastolicpressures were actually associated with a reduced risk of nonmigrainous headache [7].

Rule out serious underlying pathology and look for other secondary causes of headache.●

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 and betweenmigraine and some secondary causes of headache such as sinus disease.

Age at onset●

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●

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The examination of an adult with headache complaints should cover the following areas:

The neurologic examination should cover mental status testing, cranial nerve examination, funduscopy andotoscopy, and symmetry on motor, reflex, cerebellar (coordination), and sensory tests. Gait examination shouldinclude getting up from a seated position without any support and walking on tiptoes and heels, tandem gait, andRomberg test.

The majority of patients with headache complaints have a completely normal physical and neurologicexamination. However, some types of primary headache may be associated with specific abnormalities:

Other abnormalities on examination should raise suspicion for a secondary headache disorder. Likewise, dangersigns (ie, red flags) should prompt further evaluation, as discussed in the sections below.

Danger signs — Paying attention to danger signs is important since headaches may be the presenting symptomof a space-occupying mass or vascular lesion, infection, metabolic disturbance, or a systemic problem. Thefollowing features in the history can serve as warning signs of possible serious underlying disease [8-10]. (See"Evaluation of the adult with headache in the emergency department".)

The mnemonic SNOOP is a reminder of the danger signs ("red flags") for the presence of serious underlyingdisorders that can cause acute or subacute headache [11,12]:

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●

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●

With tension-type headache, there may be pericranial muscle tenderness.●

With migraine, there may be manifestations related to sensitization of primary nociceptors and centraltrigeminovascular 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 evidenceof autonomic activation.

Systemic symptoms, illness, or condition (eg, fever, weight loss, cancer, pregnancy, immunocompromisedstate including 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 >40 years) or sudden (eg, "thunderclap")●

Other associated conditions or features (eg, head trauma, illicit drug use, or toxic exposure; headacheawakens from sleep, is worse with Valsalva maneuvers, or is precipitated by cough, exertion, or sexualactivity)

Previous headache history with headache progression or change in attack frequency, severity, or clinicalfeatures

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Any of these findings should prompt further investigation, including brain imaging with MRI or CT. (See'Indications for imaging studies' below.)

Other features suggesting a secondary headache source — Other features that suggest a specific source ofheadache pain include the following:

Diagnostic instruments — As mentioned above, the most common headache syndromes frequently presentwith characteristic symptoms (table 1). However, there may be considerable symptom overlap; onepopulation-based survey found that less than one-half of patients who complained of headaches that met criteriafor migraine were properly diagnosed [14]. 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 sinusdisease [15]; in addition, a study of primary care patients with recurrent sinus headache found that 90 percentexperienced attacks that met the International Headache Society (IHS) criteria for migraine [16]. (See 'Sinussymptoms' below.)

Given these pitfalls, a number of diagnostic instruments have been proposed, mainly to assist with the diagnosisof 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 theprevious three months and indicated either that they might want to speak with a healthcare professional abouttheir headaches or that they experienced a headache that limited their ability to work, study, or enjoy life [17].The screen employs three questions:

During the last three months, did you have the following with your headaches?

The ID migraine screen is positive if the patient answers yes to two of the three items. In a systematic review of13 studies that involved over 5800 patients, the pooled sensitivity and specificity of ID migraine was 0.84 and0.76, respectively [18]. 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.

Impaired vision or seeing halos around light suggests the presence of glaucoma. Suspicion for subacuteangle closure glaucoma should be raised by relatively short duration (often less than one hour) unilateralheadaches that do not meet criteria for migraine arising after age 50 [13].

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.●

Blurring of vision on forward bending of the head, headaches upon waking early in the morning thatimprove with sitting up, and double vision or loss of coordination and balance should raise the suspicion ofraised intracranial pressure; this should also be considered in patients with chronic, daily, progressivelyworsening headaches associated with chronic nausea.

In patients who present with headache that is relieved with recumbency and exacerbated with uprightposture, the diagnosis of headache attributed to spontaneous intracranial hypotension should beconsidered. An additional major feature of this headache syndrome is diffuse meningeal enhancement onbrain MRI. The accepted etiology is cerebrospinal fluid (CSF) leakage, which may occur in the context ofrupture of an arachnoid membrane. (See "Headache attributed to spontaneous intracranial hypotension:Pathophysiology, clinical features, and diagnosis".)

The presence of nausea, vomiting, worsening of headache with changes in body position (particularlybending over), an abnormal neurologic examination, and/or a significant change in prior headache patternsuggests the headache was caused by a tumor. (See "Clinical presentation and diagnosis of brain tumors".)

Intermittent headaches with high blood pressure are suggestive of pheochromocytoma. (See "Clinicalpresentation and diagnosis of pheochromocytoma".)

You felt nauseated or sick to your stomach●

Light bothered you (a lot more than when you don't have headaches)●

Your headaches limited your ability to work, study, or do what you needed to do for at least one day●

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Another simple and validated instrument, the brief headache screen, consists of three to six questions [19]. Oneversion includes the following four questions:

In one study, the presence of episodic disabling headache correctly identified migraine in 136 of 146 patients (93percent) with episodic migraine, and 154 of 197 patients (78 percent) with chronic headache with migraine, witha specificity of 63 percent [19]. Only 6 of 343 patients (2 percent) with migraine were not identified by disablingheadache. Thus, virtually any patient with severe episodic headaches can be considered to have migraine.

Questions 2 and 3 can be helpful for identifying patients with medication overuse (eg, patients who usesymptomatic medications more than three days per week and/or who have daily headaches). Question 4 isparticularly helpful for identifying patients who may have an important secondary cause of headache; a patientwith a stable pattern of headache for six months is unlikely to have a serious underlying cause.

Indications for imaging studies — Patients with any of the danger signs noted above need urgent brainimaging (see 'Danger signs' above). Our approach is to perform neuroimaging in the following situations [20]:

MRI is the preferred brain imaging modality for most patients because it is more sensitive than CT scan fordetecting edema, vascular lesions, and other types of intracranial pathology, particularly in the posterior fossa.However, CT is more widely available and is therefore more useful in urgent or emergency care situations whenthere is concern for subarachnoid hemorrhage as the cause of thunderclap headache.

It may also be reasonable to image a patient presenting with nonmigrainous featureless headache, ie, bilateralnonthrobbing headache without nausea and without sensitivity to light, sound, or smell [21]. Such an approachwould have an estimated yield of 2 percent for detecting a treatable cause.

In the remaining patients, there are no randomized, controlled trials that help delineate when imaging isnecessary, and no such trials are likely to be forthcoming as blinding and randomization would present ethicalproblems. As a result, the decision to scan or not to scan in headache is likely to remain one of clinical judgment[21].

The vast majority of patients without danger signs do not have a secondary cause of headache [22,23]. As anexample, in a study of 373 patients with chronic headache at a tertiary referral center, all had one or more of thefollowing characteristics that prompted referral for head CT scanning: increased severity of symptoms orresistance to appropriate drug therapy, change in characteristics or pattern of headache, or family history of anintracranial structural lesion [24]. Only four scans (1 percent) showed significant lesions (two osteomas, one lowgrade glioma, and one aneurysm); only the aneurysm was treated.

Neuroimaging is usually not warranted for patients with migraine and a normal neurologic examination, althougha lower threshold for imaging is reasonable for patients with atypical migraine features or in patients who do notfulfill the strict definition of migraine [25]. However, brain imaging for no other reason than reassurance issometimes performed in clinical practice. In the end, patients are seeking a reason for the problem. It isimportant that the clinician provide the patient with a clear explanation of both the diagnosis and the reason forthe brain scan, especially if the decision is made to obtain imaging in someone suspected of having primaryheadache [21].

Indications for lumbar puncture — Lumbar puncture (LP) for cerebrospinal fluid analysis is urgently indicated

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?●

Focal neurologic signs or symptoms●

Onset of headache with exertion, cough, or sexual activity●

Orbital bruit●

Onset of headache after age 40 years●

Recent significant change in the pattern, frequency, or severity of headaches●

Progressive worsening of headache despite appropriate therapy●

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in patients with headache when there is clinical suspicion of subarachnoid hemorrhage in the setting of anegative or normal head CT scan. In addition, LP is indicated when there is clinical suspicion of an infectious orinflammatory etiology of headache. These issues are discussed elsewhere. (See "Clinical manifestations anddiagnosis of aneurysmal subarachnoid hemorrhage", section on 'Diagnosis of subarachnoid hemorrhage' and"Lumbar puncture: Technique, indications, contraindications, and complications in adults", section on'Indications'.)

PATIENT SETTINGS — Differences in patient demographics, comorbidities, and headache features can guidethe evaluation to help ensure appropriate diagnosis and management.

Emergency — The evaluation of the adult presenting to the emergency department with headache is reviewedin detail elsewhere (see "Evaluation of the adult with headache in the emergency department"). The main goal ofthe evaluation is to differentiate the relatively small number of patients with serious or life-threatening headachesfrom the majority with benign primary headaches (algorithm 1 and algorithm 2).

Sudden onset — Severe headache of sudden onset (ie, that reaches maximal intensity within a few seconds orless than one minute after the onset of pain) is known as thunderclap headache because its explosive andunexpected nature is likened to a "clap of thunder." Thunderclap headache requires urgent evaluation as suchheadaches may be harbingers of subarachnoid hemorrhage and other potentially ominous etiologies (table 4).These include cerebral venous thrombosis, cervical artery dissection, spontaneous intracranial hypotension,pituitary apoplexy, retroclival hematoma, ischemic stroke, acute hypertensive crisis with reversible posteriorleukoencephalopathy syndrome, "orgasmic" headache associated with sexual activity, third ventricular colloidcysts, bacterial and viral meningitis, complicated sinusitis, and reversible cerebral vasoconstriction syndromes.(See "Thunderclap headache".)

For all patients with thunderclap headache, we recommend head CT and, if head CT is normal, lumbar puncturewith measurement of opening pressure and cerebrospinal fluid analysis to exclude subarachnoid hemorrhage.For patients with thunderclap headache who have nondiagnostic head CT and lumbar puncture, imaging of thecerebral circulation is necessary. We suggest obtaining brain MRI and noninvasive neurovascular imaging suchas MR or CT angiography/venography. (See "Thunderclap headache", section on 'Diagnostic evaluation'.)

New or recent onset headache — The absence of similar headaches in the past is another finding thatsuggests a possible serious disorder.

In contrast, patients with migraine usually have had similar types of headaches in the past.

Brain tumor is a rare cause of headache but should be considered in patients presenting with focal neurologicsigns. It should also be considered when new-onset headaches occur in adults older than 50 years. A priorhistory of headache does not rule out the possibility of brain tumor, and a change in headache pattern is adiagnostic "red flag." The features of brain tumor headache are generally nonspecific and vary widely with tumorlocation, size, and rate of growth. The headache is usually bilateral, but can be on the side of the tumor. Braintumor headache often resembles tension-type headache, but may resemble migraine or a variety of otherheadache types. (See "Brain tumor headache".)

Chronic headache — Chronic daily headache is not a specific headache type, but a syndrome thatencompasses a number of primary and secondary headaches. The term "chronic" refers either to the frequencyof headaches or to the duration of the disease, depending upon the specific headache type. (See "Overview ofchronic daily headache".)

With headache subtypes of long duration (ie, four hours or more), "chronic" indicates a headache frequency of15 or more days a month for longer than three months in the absence of organic pathology. These headachesubtypes are:

New headache in patients older than 40 years may suggest underlying pathology●

New headache type in a patient with cancer suggests metastasis●

New headache type in a patient with Lyme disease suggests meningoencephalitis●

New headache type in a patient with HIV suggests an opportunistic infection or tumor●

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With headache subtypes of shorter duration (ie, less than four hours), "chronic" refers to a prolonged duration ofthe condition itself without remission. The headache subtypes in this category are the following:

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) andsome types of primary headache (hypnic headache, cough headache, and migraine accompaniments) [26].

Chronic migraine headache (see "Chronic migraine")●

Chronic tension-type headache (see "Tension-type headache in adults: Pathophysiology, clinical features,and diagnosis")

Medication overuse headache, which is typically preceded by an episodic headache disorder (usuallymigraine or tension-type headache) that has been treated with frequent and excessive amounts of acutesymptomatic medications (see "Medication overuse headache: Etiology, clinical features, and diagnosis")

Hemicrania continua, a strictly unilateral, continuous headache with superimposed exacerbations ofmoderate 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 andunremitting from onset or within three days of onset at most, typically in individuals without a priorheadache history (see "New daily persistent headache")

Chronic cluster headache (see "Cluster headache: Epidemiology, clinical features, and diagnosis")●

Chronic paroxysmal hemicrania, characterized by unilateral, brief, severe attacks of pain associated withcranial autonomic features that recur several times per day with individual headache attacks that usuallylast 2 to 30 minutes (see "Paroxysmal hemicrania: Clinical features and diagnosis")

Short-lasting unilateral neuralgiform headache attacks, characterized by sudden brief attacks of severeunilateral head pain in orbital, peri-orbital, or temporal regions, accompanied by ipsilateral cranialautonomic symptoms (see "Short-lasting unilateral neuralgiform headache attacks: Clinical features anddiagnosis")

Hypnic headache, also known as "alarm clock headache," which occurs almost exclusively after the age of50 years and is characterized by episodes of dull head pain, often bilateral, that awaken the sufferer fromsleep (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")

Giant cell (temporal) arteritis is a chronic vasculitis of large and medium sized vessels. The greatest riskfactor for developing giant cell arteritis is aging. The disease seldom occurs before age 50 years, and itsincidence rises steadily thereafter. A new type of headache occurs in two-thirds of affected individuals. Thehead pain tends to be located over the temporal areas but can be frontal or occipital in location. Theheadaches may be mild or severe. Other common symptoms include fever, fatigue, weight loss, jawclaudication, visual symptoms, particularly transient monocular visual loss and diplopia, and symptoms ofpolymyalgia rheumatica. (See "Clinical manifestations of giant cell (temporal) arteritis".)

Trigeminal neuralgia is defined clinically 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. (See"Trigeminal neuralgia".)

Chronic subdural hematoma may present with the insidious onset of headaches, light-headedness,cognitive impairment, apathy, somnolence, and occasionally seizures. (See "Subdural hematoma in adults:Etiology, clinical features, and diagnosis".)

Acute herpes zoster and postherpetic neuralgia often involve cervical and trigeminal nerves. Pain is themost common symptom of zoster and approximately 75 percent of patients have prodromal pain in the

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Pregnancy — New headache or change in headache during pregnancy may be due to migraine or tension-typeheadaches, but many other conditions can present with headache at this time, particularly pre-eclampsia,post-dural puncture headache, and cerebral venous thrombosis. Among pregnant women with the onset of newor atypical headache, approximately one-third have migraine, one-third have pre-eclampsia/eclampsia-relatedheadache, and the remaining one-third have a variety of other causes of headache.

Pre-eclampsia must be ruled in or out in every pregnant woman over 20 weeks of gestation with headache. (See"Headache in pregnant and postpartum women".)

Fever — Fever associated with headache may be caused by intracranial, systemic, or local infection, as well asother etiologies (table 5).

Immunocompromised — New headache type in a patient with HIV or other immunocompromised statesuggests an opportunistic infection or neoplasm as the cause.

Traumatic brain injury — Headache is variably estimated as occurring in 25 to 78 percent of persons followingmild traumatic brain injury. Paradoxically, headache prevalence, duration, and severity is greater in those withmild head injury compared with those with more severe trauma. Most often, headache following head trauma canbe classified similarly to nontraumatic headaches; migraine and tension-type headache predominate. (See"Postconcussion syndrome", section on 'Headaches'.)

Sinus symptoms — Although sinus headache is commonly diagnosed by physicians and self-diagnosed bypatients, acute or chronic sinusitis appears to be an uncommon cause of recurrent headaches [2-4].

Autonomic features characteristically occur in trigeminal autonomic cephalgias such as cluster headaches andare 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, anddiagnosis of migraine in adults".)

The prominence of sinus symptoms often leads to the misdiagnosis of "sinus headache" in patients who meetdiagnostic criteria for migraine or, less often, tension-type headache. This point is illustrated by an observationalstudy that enrolled 2991 patients with a history of physician- or self-diagnosed sinus headache and no previoushistory of migraine; 88 percent of these patients fulfilled criteria for migraine or migrainous headache, and 8percent fulfilled criteria for tension-type headache [28]. In the patients with migraine or migrainous headache,sinus pain, pressure, and congestion commonly occurred in association with typical migraine features such aspulsing 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 [29,30].Sinus-related pain or headache is typically described as a pressure-like or dull sensation that is usually bilateraland periorbital. However, it can be unilateral with deviated septum, middle or inferior turbinate hypertrophy, or

dermatome where the rash subsequently appears. The major risk factors for postherpetic neuralgia areolder age, greater acute pain, and greater rash severity. (See "Clinical manifestations of varicella-zostervirus infection: Herpes zoster" and "Postherpetic neuralgia".)

Brain tumor should be considered as a possible cause of new-onset headaches in adults over age 50years, 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 50years and is characterized by episodes of dull head pain, often bilateral, that awaken the sufferer fromsleep. (See "Hypnic headache".)

Primary cough headache most often affects people older than age 40 years and is provoked by coughing orstraining in the absence of any intracranial disorder. (See "Primary cough headache".)

Late-life migraine accompaniments are symptoms related to the onset after the age of 40 years of migraineaura without headache [27]. The most common symptoms are visual auras, followed by sensory auras(paresthesia), speech disturbances, and motor auras (weakness or paralysis). The most commonpresentation is gradual evolution of aura symptoms with spread of transient neurologic deficits over severalminutes and serial progression from one symptom to another.

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unilateral sinus disease. In addition, sinus-related pain is typically associated with nasal obstruction orcongestion, lasts for days at a time, and is usually not associated with nausea, vomiting, photophobia, orsonophobia. (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 mucosaldisease as revealed by imaging [30].

In general, the following principles apply to the relationship of rhinosinusitis and headache [29,31,32]:

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 5 to 6 gradereading level, and they answer the four or five key questions a patient might have about a given condition. Thesearticles are best for patients who want a general overview and who prefer short, easy-to-read materials. Beyondthe Basics patient education pieces are longer, more sophisticated, and more detailed. These articles are writtenat the 10 to 12 grade reading level and are best for patients who want in-depth information and arecomfortable with some medical jargon.

Here are the patient education articles that are relevant to this topic. We encourage you to print or e-mail thesetopics 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.)

SUMMARY AND RECOMMENDATIONS

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 carefullyfor otolaryngologic conditions.

Headache associated with fever and purulent nasal discharge is likely rhinogenic in origin.●

th th

th th

Basics topics (see "Patient information: Headache (The Basics)")●

Beyond the Basics topics (see "Patient information: Headache causes and diagnosis in adults (Beyond theBasics)" and "Patient information: Headache treatment in adults (Beyond the Basics)")

While episodic tension-type headache 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.Clinicians can easily become familiar with the most common primary headache disorders and how todistinguish them (table 1). (See 'Epidemiology and classification' above.)

Using the patient history as the primary diagnostic tool, the initial headache evaluation should determinewhether there is a potentially dangerous secondary cause of headache or whether the headache is due toone of the common types of primary headache. (See 'Evaluation' above.)

The mnemonic SNOOP is a reminder of the danger signs ("red flags") for the presence of seriousunderlying disorders that can cause acute or subacute headache:

Systemic symptoms, illness, or condition (eg, fever, weight loss, cancer, pregnancy,immunocompromised state including 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 >40 years) or sudden (eg, "thunderclap")•

Other associated conditions or features (eg, head trauma, illicit drug use, or toxic exposure; headacheawakens from sleep, is worse with Valsalva maneuvers, or is precipitated by cough, exertion, orsexual activity)

Previous headache history with headache progression or change in attack frequency, severity, orclinical features

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REFERENCES

Bigal ME, Bordini CA, Speciali JG. Etiology and distribution of headaches in two Brazilian primary careunits. Headache 2000; 40:241.

1.

Cady RK, Schreiber CP. Sinus headache or migraine? Considerations in making a differential diagnosis.Neurology 2002; 58:S10.

2.

Mehle ME. What do we know about rhinogenic headache? The otolaryngologist’s challenge. OtolaryngolClin North Am 2014; 47:255.

3.

Eross E, Dodick D, Eross M. The Sinus, Allergy and Migraine Study (SAMS). Headache 2007; 47:213.4.

Gil-Gouveia R, Martins IP. Headaches associated with refractive errors: myth or reality? Headache 2002;42:256.

5.

Buring JE, Hebert P, Romero J, et al. Migraine and subsequent risk of stroke in the Physicians' HealthStudy. Arch Neurol 1995; 52:129.

6.

Hagen K, Stovner LJ, Vatten L, et al. Blood pressure and risk of headache: a prospective study of 22 685adults in Norway. J Neurol Neurosurg Psychiatry 2002; 72:463.

7.

Edmeads J. Emergency management of headache. Headache 1988; 28:675.8.

Lipton RB, Bigal ME, Steiner TJ, et al. Classification of primary headaches. Neurology 2004; 63:427.9.

Lynch KM, Brett F. Headaches that kill: a retrospective study of incidence, etiology and clinical features incases of sudden death. Cephalalgia 2012; 32:972.

10.

Dodick D. Headache as a symptom of ominous disease. What are the warning signals? Postgrad Med1997; 101:46.

11.

Venkatesan A. Case 13: a man with progressive headache and confusion. MedGenMed 2006; 8:19.12.

Shindler KS, Sankar PS, Volpe NJ, Piltz-Seymour JR. Intermittent headaches as the presenting sign ofsubacute angle-closure glaucoma. Neurology 2005; 65:757.

13.

Lipton RB, Diamond S, Reed M, et al. Migraine diagnosis and treatment: results from the AmericanMigraine Study II. Headache 2001; 41:638.

14.

Any of these findings should prompt further investigation, including brain imaging with MRI or CT. (See'Danger signs' above and 'Indications for imaging studies' above.)

Differences in patient demographics, comorbidities, and headache features can guide the evaluation tohelp ensure appropriate diagnosis and management. (See 'Patient settings' above.)

Thunderclap headache may be the harbinger of subarachnoid hemorrhage and other potentiallyominous etiologies (table 4) (see 'Sudden onset' above)

The absence of similar headaches in the past is another finding that suggests a possible seriousdisorder (see 'New or recent onset headache' above)

Chronic daily headache is a syndrome that encompasses a number of primary and secondaryheadaches (see 'Chronic headache' above)

Older patients are at increased risk for secondary types of headache (eg, giant cell arteritis, trigeminalneuralgia, subdural hematoma, acute herpes zoster and postherpetic neuralgia, and brain tumors)and some types of primary headache (hypnic headache, cough headache, and migraineaccompaniments) (see 'Older patients' above)

Pre-eclampsia must be ruled in or out in every pregnant woman over 20 weeks of gestation withheadache (see 'Pregnancy' above)

Fever associated with headache may be caused by intracranial, systemic, or local infection, as well asother etiologies (table 5) (see 'Fever' above)

Headache is a frequent sequelae of mild traumatic brain injury (see 'Traumatic brain injury' above)•

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Barbanti P, Fabbrini G, Pesare M, et al. Unilateral cranial autonomic symptoms in migraine. Cephalalgia2002; 22:256.

15.

Cady RK, Schreiber CP. Sinus headache: a clinical conundrum. Otolaryngol Clin North Am 2004; 37:267.16.

Rapoport AM, Bigal ME. ID-migraine. Neurol Sci 2004; 25 Suppl 3:S258.17.

Cousins G, Hijazze S, Van de Laar FA, Fahey T. Diagnostic accuracy of the ID Migraine: a systematicreview and meta-analysis. Headache 2011; 51:1140.

18.

Maizels M, Burchette R. Rapid and sensitive paradigm for screening patients with headache in primarycare settings. Headache 2003; 43:441.

19.

Kumar KL, Cooney TG. Headaches. Med Clin North Am 1995; 79:261.20.

Goadsby PJ. To scan or not to scan in headache. BMJ 2004; 329:469.21.

Tsushima Y, Endo K. MR imaging in the evaluation of chronic or recurrent headache. Radiology 2005;235:575.

22.

You JJ, Gladstone J, Symons S, et al. Patterns of care and outcomes after computed tomography scansfor headache. Am J Med 2011; 124:58.

23.

Dumas MD, Pexman JH, Kreeft JH. Computed tomography evaluation of patients with chronic headache.CMAJ 1994; 151:1447.

24.

Silberstein SD, Rosenberg J. Multispecialty consensus on diagnosis and treatment of headache.Neurology 2000; 54:1553.

25.

Hale N, Paauw DS. Diagnosis and treatment of headache in the ambulatory care setting: a review ofclassic presentations and new considerations in diagnosis and management. Med Clin North Am 2014;98:505.

26.

Vongvaivanich K, Lertakyamanee P, Silberstein SD, Dodick DW. Late-life migraine accompaniments: Anarrative review. Cephalalgia 2014.

27.

Schreiber CP, Hutchinson S, Webster CJ, et al. Prevalence of migraine in patients with a history ofself-reported or physician-diagnosed "sinus" headache. Arch Intern Med 2004; 164:1769.

28.

Cady RK, Dodick DW, Levine HL, et al. Sinus headache: a neurology, otolaryngology, allergy, and primarycare consensus on diagnosis and treatment. Mayo Clin Proc 2005; 80:908.

29.

Tarabichi M. Characteristics of sinus-related pain. Otolaryngol Head Neck Surg 2000; 122:842.30.

Levine HL, Setzen M, Cady RK, et al. An otolaryngology, neurology, allergy, and primary care consensuson diagnosis and treatment of sinus headache. Otolaryngol Head Neck Surg 2006; 134:516.

31.

Marmura MJ, Silberstein SD. Headaches caused by nasal and paranasal sinus disease. Neurol Clin 2014;32:507.

32.

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GRAPHICS

Characteristics of migraine, tension-type, and cluster headache

syndromes

Symptom Migraine Tension-type Cluster

Location Unilateral in 60 to 70

percent; bifrontal or global

in 30 percent

Bilateral Always unilateral, usually

begins around the eye or

temple

Characteristics Gradual in onset,

crescendo pattern;

pulsating; moderate or

severe intensity;

aggravated by routine

physical activity

Pressure or

tightness which

waxes and wanes

Pain begins quickly, reaches

a crescendo within minutes;

pain is deep, continuous,

excruciating, and explosive

in quality

Patient

appearance

Patient prefers to rest in a

dark, quiet room

Patient may remain

active or may need

to rest

Patient remains active

Duration 4 to 72 hours Variable 30 minutes to 3 hours

Associated

symptoms

Nausea, vomiting,

photophobia,

phonophobia; may have

aura (usually visual, but

can involve other senses

or cause speech or motor

deficits)

None Ipsilateral lacrimation and

redness of the eye; stuffy

nose; rhinorrhea; pallor;

sweating; Horner's

syndrome; focal neurologic

symptoms rare; sensitivity

to alcohol

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Headache triggers

Diet

Alcohol

Chocolate

Aged cheeses

Monosodium glutamate

Aspartame

Caffeine

Nuts

Nitrites, nitrates

Hormones

Menses

Ovulation

Hormone replacement (progesterone)

Sensory stimuli

Strong light

Flickering lights

Odors

Sounds, noise

Stress

Let-down periods

Times of intense activity

Loss or change (death, separation, divorce,

job change)

Moving

Crisis

Changes of environment or habits

Weather

Travel (crossing time zones)

Seasons

Altitude

Schedule changes

Sleeping patterns

Dieting

Skipping meals

Irregular physical activity

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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 than half

the time)

1 to 200 per day Continuous pain

with

exacerbations

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

injection and

lacrimation with

SUNCT)

Yes

Restlessness

and/or

agitation

Yes Yes Frequent Yes

Migrainous

features

(nausea,

photophobia, or

phonophobia)

Yes Yes Rare Frequent

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

Oxygen

Nil Lidocaine

intravenous

infusion

Nil

Prophylactic

treatment

Verapamil

Methysergide

Lithium

Indomethacin Lamotrigine

Topiramate

Gabapentin

Indomethacin

SUNCT: short-lasting unilateral neuralgiform pain with conjunctival injection and tearing; SUNA: short-

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lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms.

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Management of headache in the emergency department-I

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Management of headache in the emergency

department-II

CNS: central nervous system; CSF: cerebrospinal fluid; CT: computed tomography

scan; LP: lumbar puncture; RBC: red blood cell; WBC: white blood cell.

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Etiologies of thunderclap headache

Subarachnoid hemorrhage

Sentinel headache

Reversible cerebral vasoconstriction syndromes

Cerebral venous thrombosis

Cervical artery dissection

Spontaneous intracranial hypotension

Pituitary apoplexy

Orgasmic headache associated with sexual activity

Retroclival hematoma

Ischemic stroke

Acute hypertensive crisis

Colloid cyst of the third ventricle

Infections (eg, acute complicated sinusitis)

Primary thunderclap headache

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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

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Sinus symptoms are common in patients with

migraine

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.

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Disclosures: Zahid H Bajwa, MD Consultant/Advisory Boards: Allergan (migraine [onabotulinumtoxinA]); Depomed [Migraine, pain(Diclofenac potassium)]; Kaleo [Opioid toxicity (Naloxone)]. Speaker's Bureau: Depomed [Migraine, pain (Diclofenac potassium)]. RJoshua Wootton, MDiv, PhD Nothing to disclose. Jerry W Swanson, MD Nothing to disclose. John F Dashe, MD, PhD Nothing todisclose.Contributor disclosures are reviewed for conflicts of interest by the editorial group. When found, these are addressed by vetting through amulti-level review process, and through requirements for references to be provided to support the content. Appropriately referencedcontent is required of all authors and must conform to UpToDate standards of evidence.

Conflict of interest policy

Disclosures

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