Cluster headache (CH) is an idiopathic

syndrome consisting of recurrent attacks

of sudden, severe, short-lasting, unilateral
periorbital pain
Sex
More common in men (5:1).
Cases of CH affecting multiple members
within a single family have been reported,
thus a genetic predisposition in some
individuals may exist.
Age
Affects the middle-aged
Attacks of CH are typically short in
duration (5-180 minutes) and occur
from a frequency of once every other
day up to 8 times a day, particularly
during sleep.
As opposed to migraine, CH is not
preceded by aura, affording patients
little or no warning.

Pain is generally described as excruciating,
penetrating, and non-throbbing.
It may radiate to other areas of the face and
neck, but is typically periorbital.
It may be triggered by stress, relaxation, extreme
temperatures, glare, allergic rhinitis, and sexual
activity.
CH is rarely triggered by ingestion of specific
foods, although tobacco or alcohol products
may precipitate an attack.
An attack of CH is a dramatic event during
which the patient may be extremely restless. In
desperation, CH patients may rock, sit, pace, or
bang themselves against a hard surface.
According to its duration, the International Headache
Society (IHS) classifies CH into episodic and chronic.
Episodic CH occurs in periods (clusters) lasting in
duration from 7 days to 1 year, but separated by
pain-free intervals lasting at least 2 weeks in
duration. Typically, a cluster lasts 2 weeks to 3
months.
Chronic CH is defined as that occurring for more
than 1 year without remission or without remissions
lasting less than 2 weeks. It is subdivided into
chronic CH from onset and chronic CH evolving
from episodic.
Chronic CH is notoriously difficult to treat and
resistant to standard prophylactic agents.
The association of prominent autonomic
phenomena is a hallmark of CH.
Such signs include ipsilateral nasal
congestion and rhinorrhea, lacrimation,
conjunctival hyperemia, facial
diaphoresis, palpebral edema, and a
complete or partial Horner's syndrome
(which may persist between attacks).
Tachycardia is a frequent finding.
CH is strictly a clinical diagnosis. On rare
occasions, structural lesions may mimic its
presentation, prompting the need for
neuroimaging study (CT or MRI).
The following can present with findings
suggestive of CH:
Meningiomas of the cavernous sinus
Arteriovenous malformations
Pituitary adenomas
Nasopharyngeal carcinoma
Vertebral artery aneurysms
Metastatic carcinoma of the lung

Pharmacologic management of CH may be divided into
abortive/symptomatic and preventive/prophylactic.
Prophylactic Agents:
Start at onset of a CH cycle and continue until the
patient is headache-free for at least 2 weeks.
The agent may be then tapered slowly to prevent
recurrences.
Abortive therapy is directed at stopping or reducing
the severity of an acute attack, while prophylactic
agents are used to reduce the frequency and intensity
of individual headache exacerbations.
Due to the fleeting, short-lived nature of the attacks,
effective prophylactic therapy should be considered
the cornerstone in treatment.
Verapamil:
Perhaps the most effective calcium
channel blocker for prophylaxis of CH.
The recommended dose is 80-120 mg
(immediate release), 3-4 times a day.
Patients intolerant to verapamil should be
tried on nimodipine, diltiazem or
nifedipine.
Lithium Carbonate
Methysergide

Drug Category: Ergot alkaloids - Are highly
effective in relieving acute CH pain.

Drug Name: Ergotamine (Cafatine, Cafergot,
Cafetrate, Ercaf)
A vasoconstrictor of smooth muscle in cranial
blood vessels, an alpha-adrenergic blocker,
and a nonselective 5-HT agonist.
Adult Dose
po: Administer 2 tabs at the first sign of onset
and 1 tab q 30 min prn thereafter. Do not
exceed 6 tabs/attack or 10 tabs/wk
Drug Name: Sumatriptan
As selective agonists for serotonin 5HT1 receptors in
cranial arteries, they cause vasoconstriction and
reduce inflammation associated with the antidromic
neuronal transmission in cluster headaches.
A reduction in the severity of headache can occur
within 15 minutes of a sc injection.
An intranasal dosage form was recently introduced in
the U.S. market, offering an attractive alternative to
self-injections.
Adult Dose po: Administer 25-100 mg and a second
dose 2 h later if a satisfactory response is not
obtained. Do not exceed 300 mg/d
Drug Category: Oral opioids and other
analgesics - The short-lived and
unpredictable character of CH
precludes the effective use of oral
narcotics or analgesics. Despite their
lack of efficacy, it is not unusual for CH
sufferers to abuse these substances.
Drug Category: Calcium channel
blockers - Inhibit the initial
vasoconstrictive phase
Drug Category: Corticosteroids - Are
extremely effective in terminating a CH
cycle and in preventing immediate
headache recurrence. High-dose
prednisone is prescribed for the first few
days followed by a gradual taper.
Drug Name: Prednisone
Adult Dose: wAdminister 40-60 mg/d in
divided doses for 5 d and taper slowly
over a 2-wk to 1-mo period
The International Headache Society (IHS)
began developing a classification system for
headaches in 1985. Finalized in 1988, this
system includes a tension-type headache
category, further defined as either episodic or
chronic. Headache categories also are
defined by whether they are associated with
pericranial muscle disorders.
Episodic tension headache usually is
associated with a stressful event. This
headache type is of moderate intensity, self-
limited, and usually responsive to
nonprescription drugs.

Chronic tension headache often recurs daily
and is associated with contracted muscles of
the neck and scalp. This type of headache is
bilateral and usually occipitofrontal.
Tension-type headache is the most common
type of chronic recurring head pain. In the
past, pain etiology was presumed to be the
muscular contraction of pain-sensitive
structures of the cranium, but the IHS
intentionally abandoned the terms muscular
contraction headache and tension headache
because no research supports muscular
contraction as the sole pain etiology.

Both muscular and psychogenic factors
are believed to be associated with
tension-type headache.
[1]
A study by
Kiran et al indicated that patients with
chronic tension headaches for longer
than 5 years tend to have lower cortisol
levels.
[2]
This was postulated to be due to
hippocampus atrophy resulting from
chronic stress, a cause of chronic tension
headaches.

Frequency
United States
Headaches account for 1-4% of all emergency
department (ED) visits and is the ninth most common
reason for a patient to consult a physician.
Physicians classify 90% of headaches reported to
them as muscle contraction or migraine headaches.
International
According to one report, the cumulative 30-year
prevalence of headache subtypes in Zurich,
Switzerland is reported to be 3.0% for migraine with
aura, 36% for migraine without aura, and 29.3% for
tension-type headache.
[3]

No literature suggests that headache frequency is
different in other regions of the world
Sex
A female preponderance of migraine exists, 14-17%,
compared with 5-6% in males.
Age
All ages are susceptible, but most patients are young
adults.
Approximately 60% of headache onset occurs in
those older than 20 years.
Headache onset is unusual in those older than 50
years.
In elderly patients, the practicing physician should
never assume that headache onset is due to benign
causes, such as tension-type headaches, until
pathologic etiologies are explored.


Pain onset in tension-type headache can have
a throbbing quality and is usually more gradual
than onset in migraines. Compared with
migraines, tension-type headaches are more
variable in duration, more constant in quality,
and less severe.
IHS diagnostic criteria for tension-type
headaches states that 2 of the following
characteristics must be present
[4]
:
Pressing or tightening (nonpulsatile quality)
Frontal-occipital location
Bilateral - Mild/moderate intensity
Not aggravated by physical activity
Tension-type headache history is as follows:
Duration of 30 minutes to 7 days
No nausea or vomiting (anorexia may occur)
[5]

Photophobia and/or phonophobia
[5]

Minimum of 10 previous headache
episodes;
[5]
fewer than 180 days per year with
headache to be considered "infrequent"
Bilateral and occipitonuchal or bifrontal pain
Pain described as "fullness, tightness/squeezing,
pressure," or "bandlike/viselike"


Tension-type headache history is as follows:
May occur acutely under emotional distress or
intense worry
Insomnia
Often present upon rising or shortly thereafter
Muscular tightness or stiffness in neck, occipital, and
frontal regions
Duration of more than 5 years in 75% of patients with
chronic headaches
Difficulty concentrating
No prodrome
New headache onset in elderly patients should suggest
etiologies other than tension headache.
The physical examination serves mainly to exclude the
possibility of other headache causes.
Vital signs should be normal.
Normal neurologic examination
Tenderness may be elicited in the scalp or neck, but no
other positive physical exam findings should be noted.
Pain should not be elicited over temporal arteries or
positive trigger zones.
Some patients with occipital tension headaches may
be very tender when upper cervical muscles are
palpated.
Pain associated with neck flexion and stretching of
paracervical muscles must be distinguished from
nuchal rigidity associated with meningeal irritation
Stress may cause contraction of neck and
scalp muscles, although no evidence
confirms that the origin of pain is sustained
muscle contraction.
Stress and/or anxiety
Poor posture
Depression
Brain Abscess
Depression and
Suicide
Encephalitis
Glaucoma, Acute
Angle-Closure
Headache, Cluster
Headache, Migraine
Meningitis
Otitis Media
Sinusitis
Stroke, Hemorrhagic
Stroke, Ischemic
Subarachnoid
Hemorrhage
Subdural Hematoma
Temporal Arteritis
Temporomandibular
Joint Syndrome
Trigeminal Neuralgia
Laboratory work should be unremarkable in
cases of tension-type headache.
Specific tests should be obtained if the history
or physical examination suggests another
diagnostic possibility.
Head CT scan or MRI is necessary only when
the headache pattern has changed
recently, the headache cannot be clearly
defined by the clinician as a common
primary headache disorder (that is not a
cluster, migraine, or tension-type of
headache), or neurologic examination
reveals abnormal findings.
[5]

Such history or physical examination
evidence would suggest an alternate
cause of headache.
Caution should be used in patients with
aura in headache that is sensory or motor,
or if the aura has changed in character
and is not described as typical of their
migraine aura.
These patients may warrant neuroimaging.
Prehospital Care
Most patients with severe headache should not
receive opiate analgesics until the responsible
physician can complete an appropriate history
and neurologic examination.
Emergency Department Care
Ascertain that the patient is not overusing
medication, shows no evidence of drug
dependency, and is not depressed.
If headache cause includes dental pathology,
sinus disease, trigger points, or CNS pathology,
initiate care to treat the specific cause.

While the emergency physician must be able
to identify patients with serious headache
etiology, more than 90% of patients in the ED
have migraine, tension, or mixed-type benign
headache. Therefore, providing symptomatic
relief should be a priority.
Various modalities are used in the treatment of
tension headaches. These include hot or cold
packs, ultrasound, electrical stimulation,
improvement of posture, trigger point
injections, occipital nerve blocks, stretching,
and relaxation techniques.
Regular exercise, stretching, balanced
meals, and adequate sleep may be part
of a headache treatment program.
[6]

Relaxation techniques such as
meditation are effective for chronic
headaches as measured by headache
parameters. Patients with chronic
headaches have been showed to have
low levels of cortisol that normalized with
the practice of meditation over time.
Class Summary
These agents may alleviate headache
pain by inhibiting prostaglandin synthesis,
reducing serotonin release, and blocking
platelet aggregation. Although the
effects of NSAIDs in the treatment of
headache pain tend to be patient
specific, ibuprofen is usually the DOC for
initial therapy. Other options include
naproxen, ketoprofen, and ketorolac.

Ibuprofen (Ibuprin, Advil, Motrin)
Usually DOC for treatment of mild to
moderately severe headache, if no
contraindications.
Naproxen (Naprosyn, Naprelan)
For relief of mild to moderately severe pain.
Inhibits inflammatory reactions and pain by
decreasing enzyme cyclooxygenase
activity, thus inhibiting prostaglandin
synthesis.
Ketoprofen (Oruvail, Orudis, Actron)
For relief of mild to moderately severe
pain and inflammation. Small dosages
initially indicated in small and elderly
patients and in those with renal or liver
disease. Doses over 75 mg do not
increase therapeutic effects. Administer
high doses with caution and closely
observe patient for response.

Ketorolac (Toradol)
Inhibits prostaglandin synthesis by decreasing
activity of enzyme cyclooxygenase, which
results in decreased formation of prostaglandin
precursors. PO form offers no advantage over
other less expensive PO NSAIDs.
Indomethacin (Indocin, Indochron E-R)
Absorbed rapidly; metabolism occurs in liver by
demethylation, deacetylation, and
glucuronide conjugation. Useful in diagnosis as
it helps other headache syndromes (eg,
chronic paroxysmal hemicrania).

Class Summary
These agents alleviate headache, possibly
by inhibiting prostaglandin synthesis.

Aspirin (Anacin, Ascriptin, Bayer Aspirin,
Bufferin)
Treats mild to moderately severe pain.
Inhibits prostaglandin synthesis, which
prevents formation of platelet-aggregating
thromboxane A2.

Class Summary
These agents are used in combination
with aspirin and acetaminophen for pain
relief and to induce sleep. Caffeine is
used to increase its GI absorption.
However, butalbital is associated with
rebound headaches. Increasing use of
these combination preparations may fail
to provide pain relief and worsen
headache symptoms.
Butalbital, aspirin, caffeine (Fiorinal)
Drug combination used to relieve tension
headaches. Barbiturate component has
generalized depressant effect on CNS.

Acetaminophen, butalbital, and caffeine
(Fioricet)
Drug combination used to relieve tension
headaches. Barbiturate component has
generalized depressant effect on CNS.
Class Summary
Patients with infrequent headaches can be
treated with simple analgesics initially.

Acetaminophen (Tylenol, Panadol, Aspirin
Free Anacin)
DOC for pain in patients with documented
hypersensitivity to aspirin or NSAIDs or upper
GI disease or taking oral anticoagulants
Acetaminophen with codeine (Tylenol #3)
Indicated for treatment of mild to
moderately severe headache.

Acetaminophen and oxycodone (Percocet)
Indicated for relief of moderately severe to
severe pain. DOC for aspirin-hypersensitive
patients.
Class Summary
These agents are useful in aborting
headache and treating emesis that results
from acute pain.

Promethazine (Phenergan)
Antidopaminergic agent effective in
treating emesis. Blocks postsynaptic
mesolimbic dopaminergic receptors in
brain and reduces stimuli to brainstem
reticular system.

Prochlorperazine (Compazine)
May relieve nausea and vomiting by blocking
postsynaptic mesolimbic dopamine-receptors,
through anticholinergic effects, and depressing
reticular activating system. In addition to
antiemetic effects, has advantage of
augmenting hypoxic ventilatory response,
acting as respiratory stimulant at high altitude.
Metoclopramide (Reglan) can be used as an
alternative to prochlorperazine. Studies show
prochlorperazine is better.
Metoclopramide (Reglan)
Dopamine antagonist that stimulates
acetylcholine release in the myenteric
plexus. Acts centrally on chemoreceptor
triggers in the floor of the fourth ventricle,
which provides important antiemetic
activity.
Class Summary
These are direct vasoconstrictors of smooth
muscle in cranial blood vessels. Their activity
depends on the CNS vascular tone at the
time of administration.

Ergotamine tartrate (Cafergot, Cafatine,
Cafetrate)
Alpha-adrenergic and serotonin antagonist.
Causes constriction of peripheral and
cranial blood vessels.
Dihydroergotamine (D.H.E. 45, Migranal Nasal Spray)
Alpha-adrenergic blocking agent with direct stimulating
effect on smooth muscle of peripheral and cranial blood
vessels; depresses central vasomotor centers.
Mechanism of action is similar to ergotamine;
nonselective 5HT1 agonist with wide spectrum of receptor
affinities outside 5HT1 system; also binds to dopamine.
Thus, has alpha-adrenergic antagonist and serotonin
antagonist effect. Indicated to abort or prevent vascular
headache when rapid control needed or when other
routes of administration not feasible.
Available in IV or intranasal preparations, tends to cause
less arterial vasoconstriction than ergotamine tartrate.
Physical therapy for patients with
headache includes warm and cold
packs, ultrasound, and electrical
stimulation.
Regular exercise, stretching, balanced
meals, and adequate sleep are part of a
headache prevention program.
Trigger point injections, occipital nerve
blocks, or changes that improve posture
may be used.

Deterrence and prevention of
headache may include the following:
Physical therapy
Biofeedback and relaxation therapy
Cervical traction
Injection of trigger points

Complications of headache may
include the following:
Undue reliance on nonprescription caffeine-
containing analgesics
Dependence on/addiction to narcotic
analgesics
GI bleed from use of NSAIDs
Risk of epilepsy 4 times greater than that of
the general population

Headache may become chronic if life
stressors are not changed.
Most cases are intermittent and do not
interfere with work or normal life span.

For excellent patient education
resources, see eMedicine's Headache
Center. Also, visit eMedicine's patient
education articles, Causes and
Treatments of Migraine and Related
Headaches and Tension Headache.