SYMPTOMS AND SIGNS
Headache and facial pain
Mark W Weatherall
Abstract
Headache and facial pain are very common. Headache accounts for
4.4% of all consultations in general practice, approximately 5% of
all medical admissions to hospital and over 25% of neurology outpa-
tient consultations. Tension-type headache is a near-universal part of
the human condition, more than 95% of us experiencing it at some
point in our lives; at the more severe end of the spectrum, migraine af-
fects 10e20% of the population worldwide, and 1e2% of the popula-
tion in developed countries have chronic daily headache. Headache is
so common that, even though for many people it is no more than an
inconvenience, the cumulative burden of migraine alone causes it to
rank high in the World Health Organization’s league tables of
disease-related disability, above all other neurological disorders
other than stroke and dementia. As all doctors will encounter patients
with headaches and facial pain, they must have a basic working
knowledge of the common primary headaches and the important sec-
ondary causes, as well as a rational manner of approaching the patient
with these conditions that allows a diagnosis to be made quickly and
safely. This article provides those resources.
Keywords Cluster headache; facial pain; headache; migraine;
paroxysmal hemicrania; SUNCT syndrome; tension-type headache;
trigeminal neuralgia; triptans
History and examination
In no part of neurology is accurate history-taking more important
than in the diagnosis of headache. It is important not only to give
patients time to tell their story fully (it will often be the first time
that anyone has listened to them talking about their pain), but
also to clarify the history with specific questions aimed at filling
in the gaps in what patients disclose spontaneously.
It is important to ask questions about the pattern of the pain,
its character and severity, other symptoms that accompany the
pain, and treatments, both current and previous. It is also
important to ask questions about the patient’s previous medical
history, current non-headache medications, allergies, family
history and social history (including caffeine consumption). It is
helpful to ask about markers of migraine, such as recurrent
abdominal pain, motion sickness and a tendency to hangovers.
Finally, it is useful to know if the patient has seen other
Mark Weatherall PhD FRCP FRCP Edin is Consultant Neurologist to the
Imperial College Healthcare and London North West NHS Trusts, UK.
He trained in Neurology at Preston, Manchester, and in the Headache
Group at the National Hospital for Neurology and Neurosurgery. He
runs the Princess Margaret Migraine Clinic at Charing Cross Hospital.
His interests include the history of headache, chronic headaches, and
visual snow. Competing interests: I have received honoraria for
speaking and attendance at advisory boards from Allergan Inc,
Janssen Cilag plc, and Eisai Pharmaceuticals.
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Key points
C Headache diagnosis rests primarily on accurate history-taking
C Most serious secondary headache disorders present with daily
persistent headaches
C Neuroimaging is not indicated if a confident primary headache
diagnosis can be made
C Opiates are not appropriate acute treatments for headache
disorders
C Effective acute and preventive treatment options exist for
migraine and cluster headache
practitioners about their headaches, what conclusions were
reached and what investigations (if any) were done.
This may sound ambitious, but patients volunteer much of
this information without being specifically asked, and it does not
take too much time to fill out the gaps. Time can be saved in
most cases by limiting clinical examination to a few relevant
specifics: blood pressure and pulse rate; fundoscopy in all cases;
inspection and palpation of the head and neck structures in most
cases; and a brief screening cardiovascular and neurological ex-
amination in all cases except those where, on the basis of the
history, serious intracranial or systemic pathology is suspected.
Investigation
How far to investigate patients with headache and facial pain is
controversial; the decision is made more complicated by the
prevalent cultural myth that headaches are commonly caused by
brain tumours. On the contrary, where an uncomplicated pri-
mary headache diagnosis can be made, the chances of the patient
having a brain tumour are 0.045%;1 no investigation is indicated,
not least because there is a 1e2% chance of picking up an
incidental intracranial abnormality that can cause anxiety or
even have an adverse influence on life insurance applications.
Imaging should be reserved for situations where clinical
assessment suggests the possibility or probability of an under-
lying tumour; examples include the finding of papilloedema on
fundoscopy, fixed abnormal neurological signs, headaches
associated with new-onset seizures or significant alterations in
consciousness, memory or coordination, and headaches in pa-
tients with a history of cancer elsewhere in the body. In such
cases, magnetic resonance imaging is the modality of choice;
computed tomography, with its associated radiation exposure,
should be reserved for the detection of acute intracranial
bleeding. Where an underlying systemic cause is suspected,
blood tests may be indicated; these should be done in patients
over 60 years with new-onset headache (including full blood
count, erythrocyte sedimentation rate, C-reactive protein con-
centration). Where patients have daily headaches, lumbar
puncture can be required to ensure that the pressure and con-
stituents of the cerebrospinal fluid (CSF) are normal.
1 Ó 2016 Published by Elsevier Ltd.
 SYMPTOMS AND SIGNS

Diagnosis paroxysmal hemicrania and SUNCT syndrome (Short-lasting

It is important to try to make a diagnosis. Sometimes this is not
possible at the first attempt e very rarely, it remains impossible,
and even the International Classification of Headache Disorders
(ICHD) recognizes this by including a category of ‘unclassifiable’
headaches.2 However, a diagnosis e or diagnoses e can usually
be made, and the importance of explaining this to the patient
cannot be overestimated. In most cases, this can be accompanied
by reassurance that there is no serious underlying cause. The
pattern of headaches and facial pain is the best guide to diag-
nosis, remembering that primary headache disorders (migraine,
tension-type headache, cluster headache, etc.) present more
commonly to doctors than do secondary headaches, and that it is
unusual for patients to seek medical opinions about mild head-
aches, such as tension-type headache.
Episodic headaches
Most primary headache disorders are episodic. Asking about the
duration of attacks and the symptoms associated with them al-
lows episodic headaches to be subdivided along useful diagnostic
lines (Table 1). It is important to remember, however, that not
everybody’s headaches have all the features that can potentially
be seen in any given disorder.
Chronic headaches
Chronic headaches develop in two ways. In one set of cases,
patients with a pre-existing primary headache disorder (usually,
but not exclusively, migraine) have ever-increasing attacks until
they reach a stage where they do not recover headache freedom
in between, a pattern originally called ‘transformed migraine’. In
many cases, overuse of acute headache medications contributes
to this process, patients fulfilling the ICHD criteria for
medication-overuse headache. Many patients revert to having
episodic headaches simply by stopping painkillers; those who do
not, and those in whom medication overuse was not an issue in
the first place, have chronic migraine. There are chronic varieties
of other, rarer primary headaches, such as cluster headache,
Unilateral Neuralgiform headache attacks with Conjunctival in-
jection and Tearing) as well as a rare but underdiagnosed pri-
mary headache disorder called hemicrania continua.
In the other set of cases, patients start to have a headache one
day and it simply never goes away. This is the ‘new daily
persistent headache’ syndrome. This is important to recognize,
because many of the serious causes lie within this set of head-
aches (Table 2); an example is the ‘thunderclap headache’ typical
of subarachnoid haemorrhage, which is a medical emergency.
After investigation, however, many cases of new daily persistent
headache do not have an underlying cause and are simply
chronic versions of the familiar episodic headache disorders.
Facial pain
Facial pain can arise from the skull, neck, ears, eyes, nose, si-
nuses, teeth or mouth. In most cases, the presence of pathology
affecting one or other of these structures is fairly obvious, but in
some cases a proper assessment requires specialist input.
Ophthalmological review is mandatory in all cases where facial
pain is accompanied by disturbances of vision, to rule out
important and treatable conditions such as scleritis, optic
neuritis and intermittent angle-closure glaucoma. Diseases of
the cranial bones, such as osteomyelitis, Paget’s disease and
myeloma, are very rare. Sinus disease is common, and acute
sinusitis excruciatingly painful, but the relevance of sinus
thickening or opacification on imaging in patients with chronic
facial pain is unclear. Disorders of the teeth and the temporo-
mandibular joints can require detailed imaging and a specialist
dental or maxillofacial opinion. Temporal arteritis should al-
ways be considered as a potential diagnosis in elderly patients
with facial pain.
Neuralgic pain affecting the face can arise from a number of
the cranial nerves and their branches. Trigeminal neuralgia is the
archetype e consisting of lancinating neuralgic pains most
commonly affecting the V2 and V3 branches, and triggered by
touch or motion of the affected area. It occurs almost exclusively
in elderly patients and is amenable to medical or surgical

Episodic primary headache disorders

Duration Severity Other features Likely diagnosis Prevalence

Hours to days Mildemoderate None Tension-type headache Near universal

Hours to days
30e180 minutes
(1e4/day, often in
bouts lasting weeks)
2e45 minutes (1e10/day,
often in bouts lasting weeks)
Seconds (1e300/day)
Moderateesevere Nausea, sensitivity to lights, noises, smells, Migraine without aura Very common
touch, movement
As above, but preceded by visual
sensory Migraine with aura Common
disturbance lasting 5e60 minutes
Severe Strictly unilateral, eye-watering, conjunctival Cluster headache Unusual
injection, nasal congestion, ptosis, eyelid
oedema, agitation
Severe Strictly unilateral, eye-watering, conjunctival Paroxysmal hemicrania Rare
injection, nasal congestion, ptosis, eyelid
oedema, agitation, absolute response to
indometacin
Severe Strictly unilateral, eye watering, conjunctival SUNCT syndrome Extremely rare
injection

Table 1

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 SYMPTOMS AND SIGNS
Serious secondary headache disorders
Thunderclap headache: differential diagnosis
C Subarachnoid haemorrhage
C Cerebral venous sinus thrombosis
C Reversible cerebral vasoconstriction syndrome
C Carotid/vertebral artery dissection
C Pituitary apoplexy
C Intracerebral haemorrhage/haematoma
C Hypertensive encephalopathy
C Idiopathic thunderclap haemorrhage (CalleFleming syndrome)
New daily persistent headache phenotype
C Raised cerebrospinal fluid (CSF) pressure, including:
 Space-occupying lesions
 Cerebral venous sinus thrombosis
 Idiopathic intracranial hypertension
C Low CSF volume (after lumbar puncture, spontaneous CSF leak)
C Meningitis (acute/chronic)
C Hypoxia/hypercapnia
C Substance abuse/withdrawal
C Systemic inflammatory conditions, including temporal arteritis
Table 2
intervention.3 Many cases are thought to result from the close
proximity of a blood vessel to the trigeminal nerve as it exits the
brainstem, the pulsation of the vessel causing intermittent stim-
ulation of the nerve and consequent pain. Neuralgic or neuro-
pathic trigeminal pain occurring in people under 50 years old
should be thoroughly investigated, looking for evidence of un-
derlying systemic or central nervous system inflammation.
Glossopharyngeal neuralgia presents with pain in the throat,
brought on by swallowing; nervus intermedius neuralgia causes
pain deep in the auditory canal. Herpes zoster can be painful and
may lead to disabling post-herpetic neuralgia.
Much facial pain is caused by primary headache disorders,
particularly migraine and cluster headache. Both conditions are
commonly misdiagnosed as ‘sinus headaches’. Patients with
facial pain should be asked the same questions as patients with
headache, and a headache diagnosis considered if their attacks
are accompanied by features otherwise typical of migraine or
cluster headache.
If the possibilities above are considered fully, there is no
reason to make a diagnosis of ‘atypical facial pain’.
Treatment of primary headache disorders
There are three things to consider when treating headaches: life-
style adjustments, acute treatments and preventive treatments.
Not all patients need to take medication; many headaches settle
with rest or sleep, and many patients find that lifestyle adjust-
ments, such as regularizing meals and sleep, significantly reduce
the frequency of their attacks. Medications are of two types: acute
treatments taken when headaches occur, and preventive treat-
ments taken regularly to try to reduce the number of headaches.
Consensus guidelines for the treatment of migraine and tension-
type headache are available on the website of the British Associ-
ation for the Study of Headache (www.bash.org.uk).
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Acute headache treatments
Many headache sufferers seek out a quiet, cool, dark environ-
ment that reduces external stimuli. In such an environment, they
may find that they can sleep, and this may be their most effective
therapy. Children in particular often find that a short period of
sleep is sufficient to abolish migraines altogether. Physical
treatments such as massage, heat packs, icepacks, menthol
forehead strips, etc., can help in some cases. Relaxation exercises
and other behavioural interventions such as biofeedback have
been shown to be helpful, particularly in children and
adolescents.
When acute medications are taken, certain principles apply.
Attacks should be treated early, when the pain is still mild.
Effective doses should be used, treatments being titrated steadily
up to the maximum tolerated dosage before being abandoned as
ineffective. Associated symptoms such as nausea should also be
treated. Finally, an appropriate route of delivery should be cho-
sen: some medications can be given by nasal spray or via a
suppository. Suggested acute treatments are listed in Table 3.
Simple analgesics are often effective, but some people cannot
tolerate them because of adverse effects or other problems such
as asthma, stomach ulcers or kidney impairment. Some patients
simply do not respond to them. At this stage, avoid prescribing
codeine-containing medications, which carry a high risk of
medication-overuse headache and addiction. Cluster headache
patients may respond to high-flow oxygen; details of how to
Acute headache treatments
Tension-type headache
C Paracetamol 0.5e1 g
C Aspirin 300e1200 mg
C Ibuprofen 200e600 mg
Migraine
C Paracetamol 1 g
C Aspirin 900e1200 mg
C Ibuprofen 400e800 mg
C Naproxen 250e500 mg
C Triptans
 Sumatriptan 50e100 mg oral, 10e20 mg nasal, 6 mg
subcutaneous
 Almotriptan 12.5 mg
 Eletriptan 40e80 mg
 Frovatriptan 2.5 mg
 Naratriptan 2.5e5 mg
 Rizatriptan 5e10 mg, sublingual melt
 Zolmitriptan 5e10 mg oral, sublingual melt, 5 mg nasal
C Combinations
 Sumatriptan 50 mg þ naproxen 250e500 mg
C All of the above taken alone or with domperidone 10 mg oral or
Buccastem 3 mg sublingual
Cluster headache
C Inhaled oxygen, 12e15 litres/minute
C Sumatriptan 20 mg nasal, 6 mg subcutaneous
C Zolmitriptan 5 mg nasala
a
Unlicensed use in the UK.
Table 3
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 SYMPTOMS AND SIGNS

Preventive headache treatments

Starting dose Target dose

Tension-type headache
Amitriptyline 10 mg at night 50e75 mg at night
Migraine
b-Blockers
Propranolol 10 mg 8-hourly 40e80 mg 8-hourly
Metoprolol 25 mg 12-hourly 100 mg 12-hourly
Atenolol 25 mg daily 100 mg daily
Tricyclicsa
Amitriptyline 10 mg at night 75e100 mg at night
Nortriptyline 10 mg at night 75e100 mg at night
Dosulepin 25 mg at night 75e100 mg at night
Pizotifen 0.5 mg at night 2e3 mg at night
Anticonvulsants
Topiramate 12.5 mg at night 50e100 mg 12-hourly
Sodium valproatea 200 mg at night 400e800 mg 12-hourly
Supplementsa
Riboflavin (vitamin B2) 400 mg daily
Magnesium (di)citrate 600 mg daily
Co-enzyme Q10 300 mg daily
Specialist options
Flunarizinea 5 mg daily 5e10 mg daily
Onabotulinum toxin A 155e195 U (PREEMPT protocol)
Greater occipital nerve blockade with methylprednisolone þ lidocaine
Cluster headache
Prednisolonea 40e60 mg for 1 week (to abort bout)
Verapamila 40 mg 8-hourly 240e320 mg 8-hourly
Topiramatea 12.5 mg at night 50e100 mg 12-hourly
Melatonina 2e3 mg at night 6e9 mg at night
Lithium carbonatea Guided by serum lithium concentrations
Occipital nerve stimulation
Trigeminal neuralgia
Carbamazepine
Lamotriginea
Pregabalina
Baclofena
Phenytoina
Trigeminal rhizotomy (destructive nerve root procedures employing glycerol, balloon compression, radiofrequency or stereotactic radiosurgery)
Microvascular decompression of compressing blood vessel
a
Unlicensed use in the UK.

Table 4

provide this can be found on the Organisation for the Under- Preventive treatments for headache
standing of Cluster Headache website (www.ouchuk.org).
There are no firm rules about when preventive medications
Migraine and cluster headache both respond to triptans (5-
should be introduced. Generally speaking, preventive treatment
HT1B/1D receptor agonists). Most triptans given in tablet form
should be considered when headache frequency or severity in-
or via nasal spray reduce migraine significantly after 2 hours in
creases to a point where it is significantly interfering with work,
about two-thirds of patients; about one-third are rendered
school or social life. For patients with tension-type headache or
headache-free. Nausea and chest tightness are the most common
migraine, this is usually when they are experiencing one or two
adverse effects, although these are rarely dose-limiting. There are
attacks each week, but if the attacks are prolonged or the
few conditions in which the use of triptans is inadvisable or
response to acute treatment is poor, preventive treatment can be
contraindicated: severe ischaemic heart disease, peripheral
introduced at a lower frequency. Patients with severe variants of
arterial disease, uncontrolled hypertension and severe Raynaud’s
migraine, such as basilar-type or hemiplegic migraine, may
phenomenon.

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 SYMPTOMS AND SIGNS

warrant preventive treatment even if their attacks are infrequent.
Patients with cluster headache, paroxysmal hemicrania and
SUNCT syndrome almost invariably require some form of pre-
ventive therapy and should be referred for specialist advice.
Numerous medications have been shown to be effective in the
preventive treatment of primary headache disorders. Not all of
these are licensed for this indication in the UK. Details of some of
the more commonly used preventive treatments are shown in
Table 4. Choice of treatment is influenced by the pattern of
headaches, patient co-morbidity, tolerability, teratogenicity, po-
tential adverse effects, ease of use and patient choice. Preventive
treatments should be commenced at low dosage to minimize the
likelihood of adverse effects. The dosage should be steadily and
regularly increased until the medication proves effective, intol-
erable adverse effects prevent further increase or a maximum
recommended dosage is reached, at which point it can be
concluded that the medication will not be effective for that in-
dividual patient. At this point, another preventive treatment can
be tried.
If preventive treatment proves effective, it should be
continued for a few months before tapering the dosage. If there is
little or no effect, patients should be referred to a specialist
headache clinic for consideration of non-pharmacological in-
terventions, such as greater occipital nerve blocks (useful in
reducing headache frequency and/or severity in 30% patients,
although the duration of effect can be limited), onabotulinum
toxin A injections for chronic migraine (two successive sets of
injections of 155e195 U in seven areas of the head and neck have
been shown to reduce headache days by 50% over 6 months in
such patients),4 non-invasive neurostimulation (of the vagus or
supraorbital nerve), or occipital nerve stimulation for refractory
migraine or chronic cluster headache (which has the potential to
render patients with previously refractory migraine free from
attacks in up to one-third of cases). New options for migraine
treatment, in particular calcitonin gene-related peptide anti-
bodies, are in late-stage clinical trials.5 A
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