The diagnosis and investigation of adult headache
presentations in primary care
David Kernick
General Practitioner
St Thomas Health Centre
Cowick Street
Exeter
Address for correspondence:
St Thomas Health Centre
Cowick Street
Exeter
su1838@eclipse.co.uk
Case
A twenty-one year old female presents with a recent history of a severe headache that
lasted four hours. She had to take to her bed and felt nauseated. Her mother had
migraine at this age. Her father had died suddenly in his forties but she was unaware
of the cause of death. Over the next six months she had three similar but less severe
attacks that responded to simple analgesia and domperidone. The GP diagnosed
migraine and shortly afterwards she moved away from the practice.
1
Background
In a three-month period, 70% of the adult population will have headache.1
3% of
GPs consultations are for headache and 4% of headache consultations will result in a
referral to secondary care.2 Invariably there will be an underlying concern for patient
and practitioner that there is a serious underlying cause and in particular a brain
tumour.
The two major classifications of headache are primary where there is no identifiable
underlying disorder and secondary, where an underlying cause can be identified.
(Table 1 shows the main sub headings of these groups.) Some will present as medical
emergencies. Others will not be life threatening but have a major impact on quality of
life. In many cases a diagnostic threshold will not be reached and careful observation
is required until a diagnosis can be made. Table 2 shows an estimate of the incidence
of important presentations in primary care.3 4 5
Table 1 in here
Table 2 in here
This article addresses two questions: firstly, what clinical features increase diagnostic
accuracy; secondly, who needs further investigation? Unfortunately, the evidence
base on which to base these answers is limited. Studies are characterised by small
sample sizes, a wide range of estimates, retrospective recall bias and specialist centre
focus.
Diagnosing headache in primary care
The primary headaches
Migraine
Migraine is the most likely cause for headache presentations in primary care3 but one
third will be incorrectly diagnosed6. A number of simple screening questions have
been developed that have high sensitivity and specificity when compared with the
2
formal International Headache Society criterion. For example, recurrent headaches
that interfere with function, lasting at least 4 hours with no new headache in past 6
months7. A pooled analysis identified five relevant features that are shown in table 3
8
.
Table 3 in here
Cluster headache
Cluster headache affects 0.1% of the population and is invariably misdiagnosed or
inappropriately treated. The cluster attack is predominately unilateral, peri-orbital,
excruciatingly painful, occurs in short bursts over a period of time (the cluster period)
and is associated with peri-orbital or nasal autonomic features. 90% of attacks occur
daily for 6-8 weeks with spontaneous resolution once or twice a year. In view of the
paucity of data in cluster headache, it is recommended that all new cases of cluster
headache are imaged. This can be relaxed for presentations of established disease.
Tension type headache
Tension type headache is poorly understood. The headache is usually dull, occipital
and bilateral. It has been suggested that in many cases tension type headache is part of
a migraine spectrum and based on similar neural mechanisms.
Some other less common primary headaches are shown in table 4
Table 4 in here
Subarachnoid haemorrhage
The incidence of subarachnoid haemorrhage (SAH) is 10 per 100,000 persons per
year. 50% will not survive and one third of survivors need life long care9
10 11
. A
missed diagnosis is the most common cause of neurological litigation12. Risk factors
include smoking, excess alcohol consumption, hypertension, a positive family history,
polycystic kidney disease and connective tissue disorders. The following features are
more frequent in SAH: occipital location, nausea, neck stiffness, impaired
consciousness. The most common presentation is a “thunderclap headache” that
reaches maximum intensity within 10 seconds and lasts for a few hours. 12% of such
patients have a SAH rising to 25% if examination is abnormal. The term “sentinel”
headache or “warning bleed” is misleading as it simply represents a small
subarachnoid haemorrhage that was not identified at the time. The “worst headache
3
ever” which may not be a thunderclap presentation is always cause for concern. 12%
of these patients with a normal neurological examination will have a subarachnoid
haemorrhage10.
Meningitis
In developed countries, the annual incidence of bacterial meningitis is 3 per 100,000
and viral meningitis 11 per 100,000. Risk factors include diabetes, otitis media,
alcohol abuse, pneumonia and sinusitis13. Physical examination has sensitivities that
are clinically useful14 15:

Fever - 85% (CI 78%-91%)

Neck stiffness - 70% (CI 58%-82%).

Alteration in mental status - 67% (52%-82%).

Jolt accentuation of headache - sensitivity of 97%, specificity 60%, positive
likelihood ratio 2.4.
The evidence for other signs of meningeal irritation is conflicting. Kernig’s sign
(extension of the knee with a hip flex at 90° elicits resistance or pain in the lower
back or posterior thigh) and Brudzinski sign (passive neck flexion in a supine patient
results in flexion of the knees and hips) have low sensitivity but high specificity.
In summary, the absence of all three signs of fever, neck stiffness and altered mental
state virtually eliminate the diagnosis of meningitis. Jolt accentuation is a useful
manoeuvre to confirm a positive diagnosis.
Temporal arteritis
Temporal arteritis is a generalised vasculopathy affecting medium and large arteries
with an incidence of 10 per 100,000 per year. It should be considered in any patient
older than 50 years with headache. Visual impairment due to involvement of the
ophthalmic artery is the main concern and may present as the first symptom. The
headache can mimic the features of other primary headaches although the headache
gradually worsens with time.
Other features include jaw claudication and constitutional symptoms of anorexia,
weight loss, fever, malaise and depression. A raised erythrocyte sedimentation rate
(ESR) can be a useful laboratory indicator. However a raised ESR is a non-specific
finding, while studies of biopsy proven arteritis demonstrate normal ESRs in between
4
5% and 30% of cases16. A review of all clinical studies17 found positive likelihood
ratios of:

Jaw claudication 4.2 (CI 2.8-6.2).

Dyploplia 3.4 (CI 1.3-8.6).

Temporal artery beading 4.6 (CI 1.1 -18.4), prominence 4.3 (CI 2.1 – 8.9) and
tenderness 2.6 (CI 1.9 – 3.7).

The absence of any temporal artery abnormality gave a negative likelihood
ratio of 0.53 (CI 0.38-0.75).
Primary Brain Tumour
The incidence of primary brain tumours is 6-10/100000 population/year of which
72% will present above the age of 5018. 2% to 16% of tumours will present with
isolated headache and 0.09%(CI 0.08-0.10) of headache presentations in primary care
will be due to a primary tumour19.
A review of the literature found the following features associated with headache that
gave important likelihood ratios8:

Abnormal findings on neurological examination - 5.3 (CI 2.4-12)

Headache aggravated by exertion or Valsalva like manoeuvre - 2.3 (CI 1.43.8)
 Headache with vomiting - 1.8 (CI 1.2-2.6)
Other important secondary headaches are shown in table 5.
Table 5 in here
Practical guidance for the practitioner on who to investigate
Due to limited data in the area, guidance is based largely on expert opinion combined
with available evidence. For example, referral guidelines have been issued by The
British Association for the Study of Headache and NICE (see article web site – web
tables 1 and 2). For the practitioner, other inputs on the decision to investigate include
medico-legal case law, organisational factors, service availability and the expectations
of practitioner and patient and the way they handle risk.20
5
The following features of the headache or features associated with it should alert the
physician to a possible serious underlying cause and the need for further investigation.

Thunderclap headache.

Neck stiffness and fever.

Alteration in mental status.

Papilloedema.

Abnormal findings on neurological examination.

Vomiting.

Aggravation with exertion or Valsalva manoeuvre.

Temporal artery abnormalities.

Jolt accentuation.

New isolated headache where a diagnosis has not emerged after eight weeks.

Headaches that have been present for some time but have changed
significantly.

New headache in a patient over 50.
Conclusion to the case.
A year later the GP received a letter from a solicitor specialising in medical
negligence. The patient had been found dead in bed and post-mortem demonstrated a
ruptured cerebral aneurysm.
GPs are sometimes in a dilemma. Not all first presentations of headache can or
should be investigated. However, presentations of “first ever headache” should be
managed cautiously particularly when severe.
TABLES
Main primary headaches
- Migraine
- Tension
- Cluster
type headache
headache and other trigeminal autonomic cephalalgias
Main secondary headaches
- Headache
attributed to head or neck trauma.
- Headache
attributed to cranial or cervical vascular disorder.
6
- Headache
attributed to non-vascular intracranial disorder.
- Headache
attributed to a substance or its withdrawal.
- Headache
attributed to infection
- Headache
attributed to disorder homeostasis
- Headache
or facial pain attributed to disorder of cranium, neck, eyes, nose, sinus,
teeth, mouth or other facial or cranial structures.
- Headache
- Cranial
attributed to psychiatric disorder.
neuralgias and central causes of facial pain.
Table 1. Main categories of primary and secondary headache.21 (A full classification
can be found at www.i-h-s.org ).
Diagnosis
Incidence
Migraine
73%
Other primary headache
23%
(predominantly tension type)
Sub-arachnoid haemorrhage
0.05%
Meningitis
0.02%
Temporal arteritis
0.02%
Primary tumour
0.09%
Table 2. Estimates of the incidence of some important headache presentations in
primary care.

Pulsatile quality of headache.

Duration of 4-72 hours,

Unilateral location nausea or vomiting.

Disabling intensity.

Positive likelihood ratios were:
24 (CI 1.5-388) for four or more features.
3.5 (CI 1.3-9.2) for three features.
0.41 (CI 0.32-0.52) for one or two features.
7
Table 3. Features that indicate migraine and their likelihood ratios
Headache type
Features
Paroxysmal hemicrania
Similar to cluster headache but pain is
shorter lasting and more frequent
characterised by an absolute response to
adequate doses of Indomethacin.
Primary stabbing headache
Short stabs or jabs lasting up to a few
seconds and recurring frequently.
Predominantly felt in distribution of the
first division of the trigeminal nerve.
Hypnic headache
Dull headache that always awakens
patient from sleep often at a similar time,
and occurs at no other time
Hemicrania continua
Persistent unilateral pain of moderate
intensity but with severe exacerbations.
Autonomic features during an
exacerbation. Characterised by an
absolute response to Indomethacin
Table 4. Some less common primary headaches
Comments
Diagnosis
Secondary brain tumours
Brain metastases occur in 20-40% of
patients with cancer. Lung (43% of
secondary tumours) and breast (8% of
8
secondary tumours) are the predominant
primary locations.
Non-progressive space-occupying
Occur with similar frequency to primary
lesions. E.g. cysts, arterio-venous
tumours but their contribution to
malformations and aneurysms.
headache is less certain
Cerebral venous sinus thrombosis.
Risk factor for venous thrombosis often
present.
Idiopathic Intracranial Hypertension
Daily headache with papilloedema in
90% and features of raised intracranial
pressure (morning worsening, Valsalva
worsening) and otological symptoms
(pulsatile tinnitus). Patients usually
obese. The headache is often throbbing
and can resemble daily migrainous
symptoms.
Spontaneous Intracranial Hypotension
Daily headache worsened within 15
minutes of upright posture and resolved
by recumbent posture. Often follows
minor head trauma. Headache is often
absent on wakening and worsens though
the day whilst erect.
Dissection of carotid or
Vertebrobasilar artery
Pain can be head, neck or face.
Neurological features may be minimal,
absent or fluctuating. Always examine
for a horners syndrome in patients with
neck pain ( horners syndrome may
indicate a dissection of carotid artery
Hypertensive encephalopathy
Blood pressure mandatory in all
headache presentations
Carbon monoxide poisoning
Ask about headache in other family
members and type of heating.
Acute angle closure glaucoma
Periorbital pain, visual disturbance
9
(particularly halo), can be nausea or
vomiting.
Table 5. Other causes of secondary headache needing urgent investigation
FOR WEBSITE
Search Strategy
Articles in MedLine were searched using the following MESH terms:
Subarachnoid tumour AND review (literature)
Temporal arteritis AND review (literature)
Meningitis AND headache
Brain tumour AND review (literature)
Web Table 1 - Headache presentations suggesting urgent assessment as suggested by
The British Association for the Study of Headache (www.bash.org.uk)
- Thunderclap
- Associated
- Headache
headache (intense headache with abrupt or “explosive” onset)
with other signs or symptoms
with atypical aura (duration >1 hour, or including motor weakness)
- New
onset headache in a patient older than 50 years
- New
onset headache in a patient younger than 10 years
- Progressive
headache, worsening over weeks
- Headache
associated with postural change
- Headache
markedly worse with wakening, straining or coughing
- New
onset headache in a patient with a history of cancer or HIV infection
Web Table 2 - Headache presentations suggesting urgent assessment as suggested by
NICE (www.nice.org.uk)_
10
 Headaches of recent onset accompanied by features suggestive of raised
intracranial pressure (for example, vomiting, drowsiness, postural related
headache with pulse synchronous tinnitus) or other focal or non-focal neurological
symptoms (for example, blackout, change in personality or memory) - an urgent
referral should be made.
 Unexplained headaches of recent onset present for at least one month but not
accompanied by features suggestive of raised intracranial pressure - discussion
with a local specialist or referral (usually non-urgent) should be considered.
 A new qualitatively different unexplained headache that becomes progressively
severe an urgent referral should be made.
 Reassessment and re-examination is required if the patient does not progress
according to expectations.
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