2013 The American Academy of Neurology Institute.

HEADACHE IN THE EMERGENCY DEPARTMENT

Stephanie J. Nahas, MD, MSEd
Thomas Jefferson University
Philadelphia, PA

Introduction

Headache is the fourth most common symptom for which patients present to the emergency department.
1

Management of these patients requires an accurate diagnosis. This can be challenging, but an orderly approach,
beginning with a thorough history and focused general medical and neurologic examinations, will lead to
appropriate management decisions. The first step is to answer the question: is this a secondary headache
disorder? In other words, is there a potentially serious underlying cause to the headache? If the answer is
maybe or most likely, the clinician must order the appropriate diagnostic tests quickly in order to confirm or
exclude the top items in the differential diagnosis to determine the best course of care. If a primary headache is
most likely, accurate diagnosis remains important as this dictates proper treatment. Primary headache syndromes
are diagnosed according to criteria based on clinical features.
2


Although the probability of secondary headaches increases in the urgent setting, most patients will still have a
primary problem.
1
Does the patient have migraine? A simple three-question inventory called ID Migraine serves
as a moderately sensitive (81%) and specific (75%) screening tool with excellent positive predictive value (93%)
for the presence of migraine in clinic patients (Table 1). While not validated in the acute care setting, it remains a
useful starting point. It is important not to forget that secondary headaches can mimic migraine, so before jumping
to this diagnosis, assess for red flags which suggest the possibility of secondary headache. If one remembers
2SNOOP4 red flags (Table 2), this will reduce the chances of missing them. Comfort signs, such as a long-
standing, stable, or slowly progressive disorder; normal neurologic exam; typical triggers, clinical features, and
history; and response to appropriate medication, also are important to recognize as potential indicators of a
primary disorder, and may offset certain lesser red flags, thus avoiding unnecessary diagnostic testing, which can
be costly and waste time. If secondary headache is excluded, diagnostic criteria dictate the diagnosis of primary
headache.

Table 1. ID Migraine
3


1. Nausea: Are you nauseated or sick to your stomach when you have a headache?

2. Disability: Has a headache limited your activities for a day or more in the last 3 months?

3. Photophobia: Does light bother you when you have a headache?

2 out of 3 symptoms: 93% migraine
3 out of 3 symptoms: 98% migraine

Lipton, et al., 2003


2013 The American Academy of Neurology Institute.

Table 2. Remember 2SNOOP4 Red Flags
4


stands for for example think of
2S Systemic symptoms
Secondary risk factors
Fever, weight loss
HIV, cancer, immune
suppression
Infections, metastatic cancer,
carcinomatous meningitis
N Neurologic
symptoms/signs
Altered consciousness, focal
deficits
Encephalitis, mass lesion,
stroke
O Onset Split-second, thunderclap Subarachnoid hemorrhage,
and others (see Table 3)
O Older New headache after age 50 Giant cell arteritis
P4 Prior history


Positional


Precipitants



Papilledema
First, newly progressive, or
different from usual

Dramatic change upright vs.
recumbent

Cough, Valsalva, or coitus
consistently triggers intense
headache

Visual obscurations

Any secondary cause can
lead to change in pattern

Intracranial hypotension,
orthostatic dysautonomia

Posterior fossa pathology,
vasospasm, increased
intracranial pressure

Increased intracranial
pressure

Modified from Dodick, D.W., 2010

The initial assessment should be swift but thorough. Focus questioning to the history of the evolution of the
current headache, its clinical characteristics, and any associated features or phenomena. Pay particular attention
to the following in the physical examination:
Vital signs: fever, hypertension, hypotension
Carotid, vertebral, and intracranial arteries: palpate cervical arteries for tenderness, auscultate all forbruits
Temporal arteries: palpate for tenderness and presence of pulses
Neurologic examination
o Mental status: level of consciousness, presence of encephalopathy, delusions, or hallucinations
o Pupils and fundi: pupillary shape, symmetry, and reactivity; papilledema; retinal hemorrhage
o Vision: diplopia, dysconjugate gaze, conjunctival injection, visual field deficit
o Neck: discern between stiffness from muscle spasm and rigidity from meningeal irritation
o Extremities: focal weakness or sensory loss, limb ataxia, asymmetric reflexes, Babinski sign
o Gait and station: ataxia, hemiparesis, Romberg sign

Identifying red flags

(1) Sudden onset of severe headache (thunderclap headache). We all learn that the top three items in the
differential diagnosis of thunderclap headache (defined as a very severe headache which reaches maximal
intensity within one minute) are subarachnoid hemorrhage, subarachnoid hemorrhage, and subarachnoid
hemorrhage, but other etiologies can cause it (Table 3). First, check brain computed tomography (CT) to look for
obvious bleed, then lumbar puncture if the CT is negative, to look for evidence of occult bleeding and to check
cerebrospinal fluid (CSF) pressure. Even with bloodless CT and CSF, vascular imaging with magnetic resonance
angiography (MRA) or computed tomographic angiography (CTA) (since noninvasive and obtainable more swiftly,
these are preferable to formal angiogram in this circumstance) is suggested to exclude dissection, unruptured
aneurysm, and reversible cerebral vasoconstriction syndrome (RCVS), the latter of which is becoming
increasingly recognized as a cause of thunderclap headache, especially recurrent thunderclap headache.
5

Magnetic resonance venography (MRV) or computed tomographic venography (CTV) will identify cerebral venous
thrombosis, which should be suspected if there is papilledema or a prothrombotic state (e.g., hereditary,
autoimmune, hormonal, pharmacologic). While spontaneous intracranial hypotension is usually not of thunderclap
onset, some cases have been described, and this should be suspected especially if the headache is positional
(better with recumbency) or associated with magnetic resonance imaging (MRI) findings of pachymeningeal

2013 The American Academy of Neurology Institute.

enhancement, pituitary and venous engorgement, and hindbrain descent. The diagnosis can be confirmed with
lumbar puncture (opening pressure will be less than 10 cm CSF, sometimes unmeasurable or even negative) or
radionucleotide myelogram/cisternogram (rapid CSF turnover is evidenced by early appearance of the tracer in
the kidneys/bladder and failure of tracer to appear in the cerebral conxevities after 16-24 hours, and the site of
leak might also be seen).
6
MRI with gadolinium and general medical evaluation will assess for most other causes
of thunderclap headache.
7
A diagnosis of primary thunderclap headache may be assigned when all appropriate
investigation is negative.

Table 3. Differential Diagnosis of Thunderclap Headache
8


Vasular Etiologies Non-Vascular Etiologies
Subarachnoid hemorrhage
Cervical arterial dissection
Aneurysmal thrombosis or
expansion
Reversible cerebral
vasoconstriction syndrome
Cerebral venous thrombosis
Hypertensive crisis
Pituitary apoplexy
Spontaneous intracranial
hypotension or hypovolemia
Colloid cyst of the third ventricle
Sinusitis (especially sphenoid)
Meningitis (rare but reported)
Primary cough, sexual, and
exertional headache
Primary thunderclap headache
(idiopathic)


Modified from Nahas, S.J., 2011.

(2) Worsening headache pattern. Patients, and sometimes clinicians, worry that headache is a sign of a brain
tumor or other mass. Such headaches often present with focal neurologic deficits or papilledema, but if the mass
is slow-growing or in a silent area, progressive headache could be the only symptom. Historical clues which may
suggest the possibility of a mass manifesting only as headache include known cancer (metastatic disease), HIV
(toxoplasmosis), immune suppression (empyema), and recent fall in an elderly patient (subdural hematoma). If a
mass lesion is suspected, MRI with gadolinium is the best test to detect it. CSF hypovolemia may also present as
a progressively worsening headache, and is not always prominently orthostatic. Sometimes these headaches are
only worse in the latter part of the day, or are paradoxically worse when lying down.

Two possibilities exist when headache progression occurs in the context of a well-established primary disorder: 1)
a superimposed new primary or secondary headache disorder and 2) chronification of primary headache. Some
patients will land in the emergency department with nothing more than chronic migraine simply from last straw
syndrome.

Migraine and tension-type headache can become chronic over months to years in susceptible patients. Chronic
daily headache, defined as headache on more days than not, is a primary disorder (except when a definite
contributor is identified, such as excessive use of pain medication). Risk factors for chronification include high
frequency of headaches, frequent use of acute medications, obesity, stressful life events, alcohol overuse,
hypothyroidism, viral infections, head trauma, snoring, and sleep disturbances.
9
When atypical clinical features
are present, or exacerbating factors cannot be identified easily, further investigation should be considered.

(3) Headache with systemic illness or systemic symptoms. Intracranial and extracranial infections, hypoxia and
hypercapnia, dialysis, uncontrolled hypertension, hypotension, hypothyroidism, fasting, collagen vascular
disorder, and arteritis are among the many systemic disorders can cause acute headache.
10
Fever, neck stiffness,
rash, fatigue, malaise, and arthralgias are particularly suggestive of a systemic problem. Remember that an acute
infection isolated to the sphenoid sinus usually presents without nasal drainage; suspect it when a febrile
headache involves facial paresthesiae or cranial nerve palsies. Giant cell arteritis is a preventable but
underdiagnosed cause of blindness in the elderly. This should be suspected when an older person with
headaches also notes fatigue and constitutional symptoms, attests to jaw claudication, or has temporal artery
hardness and tenderness with a weak or absent temporal artery pulse. When suspected, treatment with steroids
should be initiated, even before confirmation that the erythrocyte sedimentation rate is elevated or that
inflammation is present on temporal artery biopsy. In patients with cancer, brain and leptomeningeal metastasis
should be considered.
11


2013 The American Academy of Neurology Institute.

(4) Headache with focal signs other than typical visual or sensory aura. Table 4 outlines clinical clues that are
useful in differentiating neurologic transients of epileptic or vascular etiology from migraine aura. Toxic metabolic
disorders may also cause headache with transient neurologic symptoms, but more often than not, there is only
prominent encephalopathy. More persistent neurologic deficits associated with headache suggest stroke,
encephalitis, and mass lesions. Occasionally, CSF hypovolemia can manifest with signs referable to hindbrain
dysfunction (visual disturbances, diplopia, tinnitus or other hearing changes, facial weakness or numbness, other
bulbar signs, or altered consciousness), particularly when bran sag is severe. Transient visual obscurations,
pulsatile tinnitus, and papilledema in an obese patient with normal imaging suggests intracranial hypertension,
and lumbar puncture must be performed expeditiously, both for diagnostic and therapeutic purposes.

Table 4. Transient Neurological Symptoms in Migraine, Epilepsy, and TIA
8,12


Feature Migraine Epilepsy TIA
Duration and spread 5 to 60 minutes,
gradual onset with
slow spread
Brief, often shorter
than 1 min, sudden
onset with rapid
spread
Hours, sudden onset
with rapid spread
Automatisms Unusual Common with
complex partial
seizures
Very unusual
Gastrointestinal
symptoms
Abdominal pain (rare),
nausea (common)
Epigastric rise
("butterflies")
Unusual

Visual disturbances Simple positive or
negative phenomena
More complex visual
phenomena
Usually negative
phenomena
Paresthesiae Common (5 to 60
minutes)
Common (seconds to
minutes)
Common (hours)
Level of
consciousness
Usually responsive Often unresponsive May be altered
Olfactory
hallucinations
Uncommon More common Very uncommon
Aphasia/dysphasia Uncommon,
dysphasia more
common
Somewhat common Very common
Dj vu Rare Common Very Rare

Modified from Nahas, S.J., 2011 and Bigal, et al., 2003.

(5) Headache triggered by cough, exertion, or orgasm. These headaches often are of primary origin, but suspect
pathology if the headache is severe and unequivocally associated with the trigger. Valsalva-induced headache
may indicate increased intracranial pressure, occipitocervical junction disorder, or a hindbrain malformation.
Sometimes orgasmic headaches are due to subarachnoid hemorrhage, reversible cerebral vasoconstriction
syndrome, or mass lesions.
13
Exertional headache is often migraine, but sometimes represents a rare disorder
termed cardiac cephalgia, an anginal equivalent.
14


(6) Headache during pregnancy or postpartum. A pregnant or postpartum woman with a history of migraine or
tension-type headache, who has a normal examination and a headache without atypical features, usually needs
no investigation. In any case, however, consider cerebral venous thrombosis, stroke, pre-eclampsia, and pituitary
apoplexy, especially in the third trimester or the early post-partum period.
15,16


Diagnosing a primary headache disorder

It is beyond the scope of this session to detail the diagnostic criteria
2
of primary headaches, but a thorough history
aids in reaching the correct diagnosis. Criteria for some primary headache disorders which may present to the
emergency department are detailed in the following figures. If the headache is difficult to classify, the possibility of
secondary headache should be reconsidered.


2013 The American Academy of Neurology Institute.

Figure 1: Diagnostic Criteria for Migraine and Aura
2


Migraine without Aura
Description: Recurrent headache disorder manifesting in attacks lasting 472 hours. Typical characteristics of the
headache are unilateral location, pulsating quality, moderate or severe intensity, aggravation by routine physical
activity and association with nausea and/or photophobia and phonophobia.
Diagnostic criteria:
A. At least 5 attacks1 fulfilling criteria BD
B. Headache attacks lasting 472 hours (untreated or unsuccessfully treated)
C. Headache has at least two of the following characteristics:
1. unilateral location
2. pulsating quality
3. moderate or severe pain intensity
4. aggravation by or causing avoidance of routine physical activity (eg, walking or climbing stairs)
D. During headache at least one of the following:
1. nausea and/or vomiting
2. photophobia and phonophobia
E. Not attributed to another disorder

Typical Migraine Aura
Description:Typical aura consisting of visual and/or sensory and/or speech symptoms. Gradual development,
duration no longer than one hour, a mix of positive and negative features and complete reversibility characterise
the aura which is associated with a headache fulfilling criteria for 1.1 Migraine without aura.
Diagnostic criteria:
A. At least 2 attacks fulfilling criteria BD
B. Aura consisting of at least one of the following, but no motor weakness:
1. fully reversible visual symptoms including positive features (eg, flickering lights, spots or lines)
and/or negative features (ie, loss of vision)
2. fully reversible sensory symptoms including positive features (ie, pins and needles) and/or
negative features (ie, numbness)
3. fully reversible dysphasic speech disturbance
C. At least two of the following:
1. homonymous visual symptoms1 and/or unilateral sensory symptoms
2. at least one aura symptom develops gradually over 5 minutes and/or different aura symptoms
occur in succession over 5 minutes
3. each symptom lasts 5 and < 60 minutes
D. Headache fulfilling criteria BD for 1.1 Migraine without aura begins during the aura or follows aura
within 60 minutes
E. Not attributed to another disorder


Figure 2: Diagnostic Criteria for Trigeminal Autonomic Cephalgias -- Cluster Headache and Paroxysmal
Hemicrania
2


Cluster Headache
Description:Attacks of severe, strictly unilateral pain which is orbital, supraorbital, temporal or in any combination
of these sites, lasting 15180 minutes and occurring from once every other day to 8 times a day. The attacks are
associated with one or more of the following, all of which are ipsilateral: conjunctival injection, lacrimation, nasal
congestion, rhinorrhoea, forehead and facial sweating, miosis, ptosis, eyelid oedema. Most patients are restless
or agitated during an attack.
Diagnostic criteria:
A. At least 5 attacks fulfilling criteria BD
B. Severe or very severe unilateral orbital, supraorbital and/or temporal pain lasting 15180 minutes if
untreated
C. Headache is accompanied by at least one of the following:
1. ipsilateral conjunctival injection and/or lacrimation
2. ipsilateral nasal congestion and/or rhinorrhoea
3. ipsilateral eyelid oedema

2013 The American Academy of Neurology Institute.

4. ipsilateral forehead and facial sweating
5. ipsilateral miosis and/or ptosis
6. a sense of restlessness or agitation
D. Attacks have a frequency from one every other day to 8 per day
E. Not attributed to another disorder

Paroxysmal Hemicrania
Description: Attacks with similar characteristics of pain and associated symptoms and signs to those of cluster
headache, but they are shorter-lasting, more frequent, occur more commonly in females and respond absolutely
to indomethacin.
Diagnostic criteria:
A. At least 20 attacks fulfilling criteria BD
B. Severe or severe unilateral orbital, supraorbital or temporal pain lasting 2-30 minutes
C. Headache is accompanied by at least one of the following:
1. ipsilateral conjunctival injection and/or lacrimation
2. ipsilateral nasal congestion and/or rhinorrhea
3. ipsilateral eyelid edema
4. ipsilateral forehead and facial sweating
5. ipsilateral miosis and/or ptosis
D. Attacks have a frequency above 5 per day, although periods with lower frequency may occur
E. Attacks are prevented completely by therapeutic doses of imdomethacin
F. Not attributed to another disorder


Figure 3: Diagnostic Criteria for Hemicrania Continua
2


Hemicrania Continua
Description: Persistent strictly unilateral headache responsive to indomethacin.
Diagnostic criteria:
A. Headache for >3 months fulfilling criteria BD
B. All of the following characteristics:
1. unilateral pain without side-shift
2. daily and continuous, without pain-free periods
3. moderate intensity, but with exacerbations of severe pain
C. At least one of the following autonomic features occurs during exacerbations and ipsilateral to the side
of pain:
1. conjunctival injection and/or lacrimation
2. nasal congestion and/or rhinorrhoea
3. ptosis and/or miosis
D. Complete response to therapeutic doses of indomethacin
E. Not attributed to another disorder

It is worth noting that even headaches that fit diagnostic criteria for primary disorders, mimics do exist. For
migraine, keep in consideration hemorrhage, TIA, meningitis, sinusitis, glaucoma, optic neuritis, and idiopathic
intracranial hypertension. Table 5 lists common masqueraders of trigeminal autonomic cephalgias (TACs). In
particular for TACs, consider evaluating for pathology involving the carotid artery, the cavernous sinus, and the
hypothalamus.

Table 5: Secondary Causes of TACs
Vasular Etiologies Non-Vascular Etiologies
Cervical arterial dissection
Intra-cavernous carotid artery thrombosis
Cerebral venous or cavernous sinus
thrombosis
Subclavian steal
Lateral medullary infarction
Glaucoma
Sinusitis (especially sphenoid)
Trigeminal nerve root compression
Cavernous sinus metastasis
Giant meningioma
Pituitary tumors
Clival epidermoid
Idiopathic intracranial hypertension

2013 The American Academy of Neurology Institute.

Management

Once a diagnosis is realized, a management plan may be crafted. Management of secondary headaches aims to
correct the underlying cause, and symptomatic medications may be used based on the clinical features of the
headache itself. Often the phenomenology of the headache will resemble migraine, and as long as there are no
contraindications, acute migraine medications may be used concomitantly.

Acute migraine treatment may be achieved with nonspecific therapies (e.g., NSAIDs, neuroleptics,
anticonvulsants, and steroids) and with specific therapies (e.g., ergots or triptans).
17
Table 5 details some
common choices. Remember to assess for contraindications to these options, such as peptic ulcers for NSAIDs
and steroids, QTc prolongation for neuroleptics other than metoclopramide and promethazine, and liver disease
for valproic acid. It is advisable to give diphenhydramine or a short-acting benzodiazepine before a neuroleptic to
prevent akathesia, and to give dihydroergotamine after the antiemetic. Cluster headache is treated acutely with
100% oxygen (10 liters via nonrebreather mask) or a 5-HT
1B/1D
agonist (ergot or triptan).
18
Hemicrania continua,
paroxysmal hemicrania, and exertional headaches are treated with indomethacin 25 to 75 mg twice to three times
daily.
2
For patients presenting with an exacerbation of a chronic problem, begin to outline a longer term
preventive regimen based on established guidelines.
19


Table 5: Some Acute Treatment Options for Migraine in the Emergency Department

Drug Typical Dose (IV) Notes
Anti-inflammatory
Ketorolac 15-30 mg Ensure low bleeding risk
Ibuprofen 200-800 mg Recently formulated for IV administration
Methylprednisolone 100-200 mg Caution with unstable bipolar, diabetes mellitus
Dexamethasone 10-20 mg Caution with unstable bipolar, diabetes mellitus
Neuroleptic
Metoclopramide 10 mg No issue with QTc
Prochlorperazine 10 mg Dystonic reaction could be more likely
Promethazine 12.5-25 mg No issue with QTc, anticholinergic properties
Droperidol 1.25-2.5 mg Akathesia could be more likely, black box for QTc
Chlorpromazine 12.5-50 mg Watch for hypotension, especially orthostatic
Haloperidol 1-5 mg Watch for delayed dystonic reaction
Anticonvulsant
Valproic acid 500-1000 mg Should be infused rapidly over 10 minutes
Levetiracetam 1000 mg Limited evidence but well-tolerated
5-HT
1B/1D
agonist
Dihydroergotamine 0.5-1 mg Monitor blood pressure, watch for nausea and cramps
Sumatriptan 4-6 mg (SC) Alternative to dihydroergotamine, fewer side effects
Other
Magnesium sulfate 1-2 g Can be caustic to veins
Acetaminophen 325-500 mg Recently formulated for IV administration

Summary

Management of headache in the emergency department requires a thorough systematic evaluation. The most
important first step is to decide how likely a secondary cause to the headache exists, and then to proceed to
investigate this possibility expeditiously to guide diagnosis and treatment. If secondary causes are ruled out, a
primary headache diagnosis is assigned based on clinical features, and appropriate treatment can then be
initiated.


2013 The American Academy of Neurology Institute.

References

1. Friedman BW, Lipton RB. Headache in the emergency department. Curr Pain Headache Rep. 2011;15(4):302-
7.

2. Headache Classification Committee of the International Headache Society. The International Classification of
Headache Disorders. Cephalalgia 2004;24:1-160.

3. Lipton RB, Dodick D, Sadovsky R, Kolodner K, Endicott J, Hettiarachchi J, et al. A self-administered screener
for migraine in primary care: The ID migraine validation study. Neurology 2003;61(3):375-382.

4. Dodick DW. Pearls: Headache. Semin Neurol. 2010;30(1):74-81.

5. Calabrese LH, Dodick DW, Schwedt TJ, Singhal AB. Narrative review: reversible cerebral vasoconstriction
syndromes. Ann Intern Med 2007;146(1):34-44.

6. Mokri B. (2005). Spontaneous intracranial hypotension spontaneous CSF leaks. Headache Currents
2005;2(1):11-22.

7. Yu YE, Schwedt TJ. Abrupt-onset severe headaches. Semin Neurol 2010;30(2):192-200.

8. Nahas SJ. Diagnosis of acute headache. Curr Pain Headache Rep. 2011;15(2):94-7.

9. Dodick DW. Review of comorbidities and risk factors for the development of migraine complications (infarct and
chronic migraine). Cephalalgia. 2009;29(SUPPL. 3):7-14.

10. Gladstone JP and Bigal ME. Infectious, toxic, and metabolic headaches. In: Silberstein SD, Lipton RB, Dodick
DW, editors. Wolffs Headache and Other Head Pain. New York: Oxford University Press 2008:247-82.

11. Ward TN, Levin M. Headache in giant cell arteritis and other arteritides. Neurological Sciences.
2005;26(SUPPL. 2):S134-7.

12. Bigal ME, Lipton RB, Cohen J, Silberstein SD. Epilepsy and migraine. Epilepsy Behav 2003;4(Suppl 2):13-24.

13. Wang S-, Fuh J-. The "other" headaches: Primary cough, exertion, sex, and primary stabbing headaches.
Curr Pain Headache Rep. 2010;14(1):41-6.

14. Bini A, Evangelista A, Castellini P, Lambru G, Ferrante T, Manzoni GC, et al. Cardiac cephalgia. Journal of
Headache and Pain. 2009;10(1):3-9.

15. Contag SA, Mertz HL, Bushnell CD. Migraine during pregnancy: Is it more than a headache? Nature
reviews.Neurology. 2009;5(8):449-56.

16. Loder E, Golub J, Rizzoli P, Anger J. Postpartum headache: Avoiding diagnostic and therapeutic pitfalls.
Headache and Pain: Diagnostic Challenges, Current Therapy. 2006;17(4):155-65.

17. Rizzoli PB. Acute and preventive treatment of migraine. CONTINUUM Lifelong Learning in Neurology.
2012;18(4):764-82.

18. Halker R, Vargas B, Dodick DW. Cluster headache: Diagnosis and treatment. Semin Neurol. 2010;30(2):175-
85.

19. Silberstein SD, Holland S, Freitag F, Dodick DW, Argoff C, Ashman E. Evidence-based guideline update:
Pharmacologic treatment for episodic migraine prevention in adults report of the quality standards
subcommittee of the American Academy of Neurology and the American Headache Society. Neurology.
2012;78(17):1337-45.