Abstract
Headache is one of the most common presentations in primary care and accounts for 4.4% of all consultations. Most headaches are due to primary headache disorders such as migraine and tension-type headache. However, headaches can be associated with serious underlying conditions such as intracranial tumours, and it is often concerns about this that will trigger a patient to consult their GP. Since headache is predominantly managed in primary care (with only 2–3% referred to specialists), GPs must have a clear and focused strategy for diagnosis and management. This article aims to provide an overview of the assessment of patients presenting with headache to enable GPs confidently to manage this common symptom.
The GP curriculum and headache
Be able to manage the primary contact with patients who have a neurological problem
Have knowledge of common causes of headache—including tension headache, migraine, cluster headache, cervical neuralgia, sinusitis, dental pain and drug rebound headache
Have knowledge of important causes of headache—including raised intracranial pressure, thunderclap headache (subarachnoid haemorrhage, enlarging aneurysm or migraine), temporal arteritis, trigeminal neuralgia, herpes zoster, cancers and brain infections
Understand the indications for emergency referral of people with intracranial haemorrhage, raised intracranial pressure and temporal arteritis
Understand avoidance of triggers and prophylaxis for migraine
Demonstrate a structured logical approach to the diagnosis of ‘difficult’ symptoms with multiple causes, e.g. headache, dizziness.
History
The correct diagnosis of a headache disorder relies on good history taking since there are no diagnostic tests for the most common underlying causes such as migraine, tension-type, cluster and medication overuse headache (MOH). Diagnosis may take time and it can be useful to see the patient on more than one occasion and to ask them to keep a headache diary to bring to the next appointment. However, it is important to rule out serious underlying conditions such as those requiring acute treatment or urgent referral at the first visit (Box 1). The British Association for the Study of Headache (BASH) recommends a structured approach to history taking including the points summarized in Box 2. It is important to explore patients’ fears about their symptoms and be aware that the presentation may be linked with psychosocial worries, depression or anxiety.
Red flags
New-onset headache in a patient older than 50 years or younger than 10 years
Progressively worsening headache
Thunderclap headache
Headache associated with postural change
Persistent morning headache associated with nausea
Precipitated by physical exertion or Valsalva manoeuvres
Atypical aura (duration more than 1 hour or including motor weakness)
Abnormal neurological examination (or papilloedema)
Headache with new-onset seizures
Aura for the first time in a combined oral contraceptive user
Associated with fever, neck stiffness and photophobia
Jaw claudication or visual disturbance
New-onset headache in a patient with a history of human immunodeficiency virus (HIV) infection
New-onset headache in a patient with a history of cancer
Points to cover when taking a history from a patient presenting with a history of headache
Why is the patient consulting now?
How recent is the onset?
How frequently occurring?
Temporal pattern (i.e. episodic/daily/unremitting) and duration
Intensity of pain
Site and spread
Nature and quality of pain
Associated symptoms
Predisposing and/or trigger factors
Aggravating and/or relieving factors
Family history of similar headache
What does the patient do during the headache?
How much is activity/function limited?
What medication has he or she used and how often?
Completely well? Or residual persisting symptoms?
Patient ideas, concerns and expectations
Examination
The physical examination can demonstrate to patients that their symptoms are being taken seriously. The guidelines from the Scottish Intercollegiate Guidelines Network (SIGN) and BASH advise that in all patients, the optic fundi should be examined and blood pressure taken.
Raised blood pressure is an uncommon cause of headache but patients often perceive this to be the cause and will expect their blood pressure to be measured. In addition, drugs used in headache treatment can affect blood pressure so a baseline is important.
Further examination such as a full neurological examination is likely to reassure both patient and practitioner that a serious underlying cause has not been missed. However, in a time-limited primary care consultation, a comprehensive neurological examination may not be feasible. It is worth developing an examination framework for e.g. the cranial nerves, which is quick to use in routine clinical practice.
Although the examination should be focused, it can be used as a therapeutic tool in reassurance of the patient and the value of this should not be underestimated. The full range of relevant examinations that may be helpful are listed in Box 3. It is not suggested that all these be employed but that the patient's symptoms lead the GP to perform the most appropriate examination.
Examination
Blood pressure measurement
Fundoscopy
Cranial nerve examination
Tone, power, reflexes, co-ordination and sensation in all four limbs
Assessment of gait
Palpation of temporal arteries (in patient over 50 years)
Head and neck examination for muscle tenderness/stiffness
Ear, nose and throat examination
Measurement of head circumference with plotting on centile chart (in patients under 10 years)
Differential diagnosis
The International Classification of Headache Disorders (2nd edition) is a hierarchical system which classifies all causes of headaches into three major groups—the primary headache disorders of migraine, tension-type and cluster headaches; the secondary causes (such as trauma, vascular causes, infection, tumour and medication overuse) and the cranial neuralgias. A simplified version of this classification is summarized in Table 1.
The International Classification of Headache Disorders (2nd edition—simplified)
Reproduced with permission of International Headache Society.
A thorough history and examination of the patient presenting with headache will often be enough to confidently reach one of these diagnoses. Starting with the most common, the causes of headache with their classic presenting features will now be considered.
Migraine
Migraine is the most common severe primary headache disorder with a prevalence of 15% in the general population. Women are affected more commonly than men (ratio 3:1). It is subdivided into migraine with and migraine without aura. An aura occurs in up to a third of migraine sufferers and precedes the headache, consisting of progressive visual and/or sensory and/or speech symptoms with a duration of between 5 and 60 minutes. Symptoms may be positive (zig zag lines, flickering lights, tingling, etc) or negative (visual loss and numbness).
Occasionally, aura may occur without the development of headache. If this occurs in a known migraine sufferer and is similar to previous attacks, it is unlikely to be concerning. However, a transient ischaemic attack remains a differential diagnosis for aura, particularly if it is of rapid onset or differs from the usual pattern. It is important to take the age of the patient into account. Prolonged aura (i.e. duration greater than 1 hour) or aura with motor symptoms should prompt referral (BASH, 2010).
The classic history of migraine headache includes:
Unilateral location (although, in children headache is more frequently bilateral and gastrointestinal disturbance more prominent)
Pulsating quality
Attack lasts 4–72 hours
Builds up over minutes to hours
Moderate to severe intensity
Limits usual activities
Aggravated by mild exertion, e.g. climbing stairs
Associated with nausea and/or vomiting
Associated with photophobia and phonophobia
There is often a positive family history of migraine. There are also recognized trigger factors in some patients which may include:
Relaxation after stress (particularly weekends and holidays)
Changes in routine—too much or too little sleep, missing meals and long distance travel
Menstruation
Dietary triggers (e.g. cheese, chocolate, alcohol)
Chronic tension-type headache
Chronic tension-type headache (CTTH) affects 3% of people and is defined as occurring on more than 15 days/month (and may occur daily). CTTH is much more disabling than the more common episodic type which causes up to 50% of all headaches but does not usually prompt presentation to the GP. Classically, the pain is bilateral, described as a pressure or tight band around the head. There is often associated neck pain or neck muscle tightness. When compared with migraine, pain is usually less severe, not associated with nausea and not worsened by activity. Stress is often an aetiological factor (particularly in pain that worsens in severity throughout the day) but is not always implicated. Depression and/or anxiety may coexist and these symptoms should be explored.
Medication overuse headache
MOH is usually described as a chronic daily headache, causing a dull generalized pain worse on awakening in the morning and after physical exertion. The headache has developed or worsened while taking regular symptomatic analgesics for the relief of headaches for 3 months or more. A particular problem is pre-emptive use of medication to try to prevent headache occurring.
Any acute headache treatments can cause the phenomenon but combination analgesics containing caffeine, barbiturates or codeine are particularly implicated. The overuse of triptans (on more than 10 days/month) is increasingly recognized as a cause. Even simple paracetamol or non-steroidal anti-inflammatories (NSAIDs) taken on more than 15 days/month can be associated with MOH. Frequency of administration appears to be important—low doses on a daily basis are more of a problem than larger doses less often. Painkillers taken on a regular basis for conditions other than headache are much less likely to cause this phenomenon.
If the condition is suspected, the painkiller or triptan should be stopped completely (not through a gradual tapering course). Improvement is normally seen after 2 weeks for triptans but can take up to 2 months for other agents, especially opioids. Patients should be warned about withdrawal symptoms in the early stages and supported during this time.
Trigeminal autonomic cephalalgias
Trigeminal autonomic cephalalgias (TACs) are relatively rare with an estimated prevalence of 0.05%. The main types of TACs are cluster headache and paroxysmal hemicrania. Cluster headache occurs more frequently in men (male to female ratio 6:1) and in smokers. Usual onset of the condition is in middle age. The pain is described as excruciating and is unilateral and centred around the orbital area (although it can switch sides between attacks). It is associated with one or more autonomic features occurring on the ipsilateral side, which include conjunctival injection and lacrimation, rhinorrhoea or nasal blockage, ptosis, facial flushing and sweating. The sufferer is restless during the attack and finds it difficult to keep still—sometimes beating his head against the wall with the pain. Attacks last between 15 minutes and 3 hours and have a rapid onset. Attack frequency varies between several times in a day to once every other day and usually occurs at the same time of day (often during the night). A bout of attacks can last between 1 and 3 months at a time.
Paroxysmal hemicrania has similar characteristics to cluster headache being unilateral in nature and associated with autonomic symptoms. However, the attacks are normally shorter in duration (2–45 minutes) and more frequent (occurring up to 40 times/day). The diagnosis is important to make as the condition will show a complete response to treatment with indomethacin.
Serious causes of headache
Intracranial tumours
The overall risk of brain tumour when a patient presents with headache in primary care is 0.09%. This reduces to 0.045% if a diagnosis of primary headache (e.g. migraine, tension-type) can be made (Kernick, 2011). It is rarely the sole presenting feature and is usually accompanied by a focal neurological deficit or new-onset seizures (both red flags for urgent referral). The likelihood of diagnosis increases with age, being rare under the age of 50 years. Nonetheless, GPs should be alert to the features of headache secondary to intracranial tumour. The pain is classically worse on lying down and may wake the patient from sleep. It may be precipitated by valsalva manoeuvres, sexual intercourse or physical exertion. However, intracranial tumour presenting with headache may have no particular clinical pattern and, in the absence of an alternative diagnosis, a low index of suspicion is advised.
Subarachnoid haemorrhage
Typically described as a ‘thunderclap headache’, the pain associated with subarachnoid haemorrhage is severe and sudden—likened to being hit on the back of the head. Headache may be associated with neck stiffness, nausea and vomiting, seizures and/or loss of consciousness. Focal cranial nerve signs may also be present. If subarachnoid haemorrhage is suspected, immediate specialist medical review via the acute medical services or emergency department is required.
Giant cell arteritis
Also known as temporal arteritis; giant cell arteritis (GCA) classically presents with headache in the temporal region associated with scalp tenderness, jaw claudication and/or visual disturbance. Signs can include a thickened tender temporal artery with absent pulsation. However, the headache may be atypical and diffuse in nature, and the condition is sometimes insidious in onset with a prodrome of systemic symptoms (e.g. fever, anorexia, fatigue) for many weeks prior to the onset of headache.
In all patients over the age of 50 years presenting with new headache, GCA should be considered in view of the potential for serious complications such as permanent visual loss. In patients with visual symptoms, give prednisolone 60 mg and refer for same day assessment by an ophthalmologist. If visual symptoms are not present, check erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP), prescribe 60 mg prednisolone daily and refer urgently to rheumatology, ophthalmology or neurology (depending on local referral pathways) for consideration of temporal artery biopsy (Clinical Knowledge Summary, 2010).
Meningitis
The headache in meningitis is usually accompanied by other clinical signs such as pyrexia, neck stiffness, photophobia, nausea and vomiting, lethargy and altered consciousness. Skin rash is a late sign. The headache is frontal or generalized and progressive over a short period (usually hours). If suspected, patients should be referred as an emergency to the on-call specialist medical team.
Angle-closure glaucoma
Consider a diagnosis of angle-closure glaucoma in any patient presenting with headache associated with a red eye. It rarely presents before middle age and is more common in women with a positive family history and hypermetropia. The pain is classically severe and associated with nausea and vomiting, a fixed mid-dilated pupil and a hazy cornea. Angle-closure glaucoma is an ophthalmological emergency and must be referred immediately.
Idiopathic intracranial hypertension
Initially episodic, idiopathic intracranial hypertension progresses to cause a chronic daily headache with features of raised intracranial pressure (i.e. pain worse on lying down and on coughing or straining). Other features include nausea and vomiting, pulsatile tinnitus, visual disturbances and papilloedema. It mainly affects women of childbearing age and is strongly associated with obesity. Urgent referral is necessary to confirm the diagnosis and prevent complications such as visual loss.
Carbon monoxide poisoning
Subacute carbon monoxide (CO) poisoning can produce symptoms of headache, nausea, vomiting, lethargy, dizziness, muscular weakness and blurred vision. It is an uncommon but important cause of headache to be considered. Measurement of blood carboxyhaemoglobin levels can confirm the diagnosis.
Investigation
Good history taking is the cornerstone of the diagnosis of headache and investigations are rarely indicated. The main investigation requested in the assessment of headache is some form of neuroimaging [i.e. computerized tomography (CT) or magnetic resonance imaging (MRI) scan]. Referral for neuroimaging is indicated in any patient presenting with red flags (see Box 1). However, neuroimaging may also be considered where there is diagnostic uncertainty which exists after 8 weeks.
It is important to discuss with the patient both the potential benefits and the risks of performing imaging. Although many patients may benefit from the reassurance of a normal scan, there is evidence that this reassurance does not persist beyond 12 months (Howard et al., 2005). There is also up to a 10% chance of incidental findings reported from neuroimaging. This causes further anxiety and uncertainty for the patient and may have unintended consequences such as an effect on life insurance applications.
Blood tests for ESR and/or CRP are indicated if GCA is suspected. Cervical spine X-rays are usually unhelpful even if the headache is thought to originate in the neck as they do not alter management. Eye testing by an optician is unlikely to aid diagnosis.
Management
Migraine
Management of migraine is divided into acute and prophylactic treatments. Prophylaxis should be considered in patients who are suffering two or more migraines per month. The aim of prophylaxis is to reduce the number of attacks but acute treatments will still be required. Acute treatments include analgesics with or without the addition of an anti-emetic:
First line: aspirin 600–900 mg or ibuprofen 400 mg with or without paracetamol 1000 mg (dispersible forms may aid absorption)
Anti-emetic: prokinetic agents are preferred in view of gastric stasis, which occurs in migraine, e.g. metoclopramide 10 mg or domperidone 10–20 mg (combination analgesic and anti-emetic preparations include MigraMax and Paramax sachets)
Second line: 5-HT1 agonists (triptans), e.g. sumatriptan 50 mg orally with a further 50 mg after 2 hours if required (also available as a 6 mg subcutaneous injection if vomiting is a predominant symptom)
Patients may have contraindications to aspirin or NSAIDs such as asthma or a history of peptic ulceration. Contraindications to the triptans include ischaemic heart disease, coronary vasospasm and uncontrolled hypertension.
The first-line analgesics should be taken as soon as possible in the migraine attack, including the aura phase. Whereas triptans should be taken at the onset of headache and not in the aura period. Consideration should be given to other medications which may need to be discontinued, e.g. the combined oral contraceptive (contraindicated in migraine with aura). Opioids should not be used in the treatment of migraine.
BASH guidelines (2010) recommend that prophylaxis should be considered when attacks occur frequently or when acute treatments are ineffective or contraindicated. A prophylactic medication is taken on a daily basis and should be trialled for 3 months before determining efficacy. If a prophylactic agent is effective, it should be continued for 4–6 months before considering withdrawal by tapering the dose. The options include:
First line: beta-blocker—i.e. atenolol 24–100 mg twice daily first line; metoprolol 50–100 mg twice daily second line; propranolol MR 80–160 mg daily third line. Contraindicated in asthma.
Alternative first line: amitriptyline 10–150 mg 1–2 hours before bedtime (starting at 10 mg and increased by 10 mg/week; usual maintenance dose is 50–75 mg). Particularly useful if migraine coexists with tension-type headache, disturbed sleep, depression or other chronic pain. Other less sedating tricyclics may be used as alternatives to amitriptyline, but there is no evidence base for their effectiveness in migraine.
Second line: antiepileptics—e.g. topiramate 25 mg daily (increased by 25 mg every 2 weeks, aiming for maintenance dose of 50 mg twice daily) or sodium valproate 300 mg twice daily (increased to 600 mg twice daily if necessary). Avoid in pregnancy and breastfeeding.
Third line: gabapentin 300 mg daily (increased gradually to 800 mg three times daily as needed) but evidence for efficacy is not robust; combination of amitriptyline and a beta-clocker methysergide 1–2 mg three times daily is an effective alternative but should only be initiated by a specialist and can cause retroperitoneal fibrosis.
Other agents: pizotifen and clonidine are widely used for prophylaxis of migraine, but there is little evidence of efficacy. Verapamil and selective serotonin reuptake inhibitors (e.g. fluoxetine) are of uncertain benefit. There is emerging evidence that lisinopril, montelukast, candesartan, riboflavin and coenzyme Q10 may all have some prophylactic effects on migraine but there is currently insufficient evidence to support their use.
Tension-type headache
The management of tension-type headache begins with a full explanation and discussion with the patient about the diagnosis. It is important to listen to the patient's concerns as reassurance that the condition is self-limiting and not a result of any serious underlying pathology may be therapeutic in itself. If the patient is suffering from episodic tension-type headache (i.e. fewer than 15 episodes/month), acute treatment may be recommended (e.g. paracetamol or NSAIDs). The patient should be advised not to take acute treatments on more than 2 days/week and be alert to the development of MOH. Opioids such as codeine should be avoided.
In CTTH, relaxation training, cognitive–behavioural stress management (CBT) and electromyographic biofeedback training have all been shown to be effective. Prophylaxis with amitriptyline 10 mg at night (increasing by 10 mg weekly to a maintenance dose of 50–75 mg daily) is the preferred pharmacological option (SIGN, 2008).
Cluster headache
Patients with suspected cluster headache should be referred to neurology for confirmation of the diagnosis, exclusion of secondary causes and initiation of preventative therapies (Clinical Knowledge Summaries, 2009d). While awaiting the referral, acute treatment should be prescribed.
First line for treatment of the acute attack is sumatriptan 6 mg administered subcutaneously (can be used up to twice daily). Oral preparations are not effective. High-flow oxygen should also be considered and will require referral via the Home Oxygen Order Form to obtain an oxygen cylinder for the patient's home (15 minutes of 100% oxygen—i.e. 12 l via a non-rebreathe mask can abort an attack). Patients should be advised to avoid alcohol, smoking and inhalation of any volatile fumes (e.g. solvents) as these may all trigger an attack. A trial of indomethacin may be considered if the alternative diagnosis of paroxysmal hemicrania is a possibility—a complete resolution of symptoms should be seen in this case.
Short-term prevention with prednisolone (60 mg daily reduced after 5 days on a gradual tapering regime) may be initiated. Long-term prophylaxis is usually with verapamil (240–960 mg/day) and should be initiated by a specialist. Alternative prophylactics include lithium and antiepileptic medications.
Referral
The General Practice Research Database shows that only 2–3% of patients presenting with headache in primary care are referred on to neurology. The majority of patients referred are aged 55–64 years. This supports the advice from a recent ‘Guidelines in Practice Update’ (Kernick, 2011) that in patients over the age of 50 years where a diagnosis remains uncertain after 8 weeks, referral should be considered. Figure 1 is a flowchart summarizing the common management and referral pathways for headache.
Management of headache flowchart.
Referral may also be considered to allay the concerns of an anxious patient but can also be a reflection of a GP's approach to managing uncertainty in a condition where diagnostic tests are not readily available. Ridsdale and colleagues (BJGP, 2007) looked at the differences between patients referred to neurologists and those managed in primary care. The only significant differences between the two groups were in their consultation rates and in the degree of anxiety the patients expressed. Referred patients presented to the GP more frequently in the 3 months before being referred than those patients with headache who were not referred. Referred patients were also more likely to report significant anxiety surrounding the cause of their headache than patients managed in primary care. However, there was no difference in overall satisfaction with the care provided by the GP between the referred and non-referred groups. This suggests that in patients consulting frequently with a lot of anxiety about their headaches in whom there is no clinical indication for referral, it may be more important for the GP to address these anxieties than to refer to secondary care.
Summary
GPs should adopt a structured approach to the diagnosis and management of patients with headache, excluding important causes first and going on to look for more common diagnoses such as migraine and tension-type headache.
Key points
Headache is a common presentation in primary care and the most likely underlying causes are migraine or tension-type headache
The overall risk of brain tumour when presenting with headache alone to primary care is low
Consider serious causes of headache (in particular GCA or intracranial tumour) in patients over the age of 50 years presenting with new-onset headache
Avoid opioid medications in headache as they are most likely to predispose to MOH