A detailed systematic history is the key to diagnosing and effectively managing patients with this common and disabling condition
Headache affects most people, at least occasionally. It is high among the reasons why people consult general practitioners and neurologists.1,2 It may signal serious underlying illness, but, more importantly, it is associated with personal and social burdens of pain, disability, damaged quality of life, and financial cost.3,4 It ought to be a huge public health issue, but headache receives little priority in the queue for healthcare resources.5
The diagnosis and management of most headaches require neither advanced neurological skills nor investigations.
Sources and selection criteria
We reviewed published and other accessible information sources, including the World Health Organization's world health report,6 major epidemiological surveys, the classification and diagnostic criteria of the International Headache Society,7 reports of clinical trials, and selected national management guidelines. We used the definitive textbook on headache disorders—The headaches—as a reference source.8 Treatment recommendations in the present paper were based on evidence but, other than for the newer drugs, this was often limited to expert opinion and practice.
Diagnosing headache
Sufficient time committed to a systematic headache history is the key to effective diagnosis (see box B1). The correct diagnosis is not always evident initially, especially when the patient has more than one type of headache. A diary kept for a few weeks can establish the pattern of attacks, symptoms, and medication use. A change in pattern signals something new—aggravating circumstances, or the onset of a new headache disorder. New headache, in young and old patients, needs especially careful inquiry.
Summary box
Headache disorders are common in general populations everywhere
They are potentially disabling, and impose heavy individual and societal burdens; appropriate effective management should have higher priority than it does at present
Most patients with headache have one of three medically non-serious conditions and are best managed in primary care
Recognition of serious causes of headache requires a standardised diagnostic approach to history and examination coupled with an awareness of a relatively small number of important secondary headache disorders
If the history is adequate, physical examination rarely reveals unexpected signs—rather, it reassures patient and physician. Measurement of blood pressure and a brief but comprehensive neurological examination, including that of the optic fundi, are recommended. Examination of the head and neck may reveal muscle tenderness, limited range of movement, or crepitation (which suggest a need for physical forms of treatment but not necessarily the cause of the headache). Investigations, including neuroimaging,9 rarely contribute to the diagnosis of headache when the history and examination suggest no underlying cause.
Important headaches
The International Headache Society classified 13 types and many more subtypes of headache disorder.7 The box on bmj.com gives a simplification of this classification. However, three primary headaches—migraine, tension-type headache, and cluster headache—together with one secondary headache disorder (medication overuse headache) account for the type of headache of most consulting patients.
Migraine
Migraine is a common disorder affecting 2-15% of the world's populations, causing more disability than does epilepsy.6 It is more prevalent in the productive years (late teens to 50s) and in women than in men (3:1). Every day, a million people in European Union countries have a migraine attack (TJ Steiner, unpublished), and an estimated 100 million workdays or schooldays are lost annually because of migraine.
Migraine is a primary headache disorder: evidence is growing of a genetic basis.10 It is no longer seen as a primary vascular disorder. Activation of the trigeminovascular system by a mechanism that may originate in the brainstem causes the release of algesic inflammatory substances, vasodilatation, and plasma extravasation. Why this happens periodically, and what brings the process to an end in spontaneous resolution of attacks, are unknown.
Adults with migraine describe episodic attacks with specific features (box B2), of which nausea has the greatest diagnostic value.11
A third of people with migraine sometimes or always have aura before headache—hemianopic disturbance or a spreading scintillating scotoma (figure) or, less commonly, other reversible focal neurological disturbances such as unilateral paraesthesiae of hand, arm, or face lasting 10-60 minutes. Long duration aura is rare and warrants investigation. Visual auras can occur without headache, especially in older patients (over 40).
Tension-type headache
Episodic tension-type headache—“normal” or “ordinary” headache—is less disabling but more prevalent (up to 80%) than migraine.12 It also occurs in attack-like episodes, mostly lasting a few hours, with variable frequency. Headache is often described as pressure or tightness, like a band around the head, sometimes spreading into or from the neck. It lacks the specific features and associated symptom complex of migraine.
Chronic tension-type headache occurs, by definition, on >15 days a month and can be daily and unremitting.7 It affects 2-3% of adults, who may be substantially disabled and chronically off work as a result.13
Tension-type headache may be stress related or associated with functional or structural cervical or cranial musculoskeletal abnormality. These aetiological factors are not mutually exclusive.
Cluster headache
Cluster headache is an extremely unpleasant condition that affects 1 in 1000 men and 1 in 6000 women; most are in their 20s or older and many are smokers. It is one of a group of conditions (trigeminal autonomic cephalalgias) of uncertain pathophysiology characterised by frequently recurrent, short lasting headache and autonomic symptoms.
Cluster headache is highly recognisable. The episodic form occurs in bouts (clusters), typically of 6-12 weeks' duration once a year or every two years and at the same time of year. Strictly unilateral intense pain around the eye develops once or more daily, commonly at night. The patient, unable to stay in bed, agitatedly paces the room, even going outdoors, sometimes beating his or her head on the wall or floor until the pain diminishes after 30-60 minutes. The eye is red and waters, the nose runs or is blocked on that side, and ptosis may occur. Atypical presentations are more common in women. In the chronic form, which is less common, no remissions occur between clusters, and a continuous milder background headache may additionally develop. The episodic form can become chronic, and the chronic form episodic, but once present, cluster headache can persist for 30 years or more.
Medication overuse headache
Daily or near-daily headache is at epidemic levels, affecting up to 5% of some populations,14 and chronic overuse of headache drugs may account for half of this phenomenon.15 All simple analgesics, and probably non-steroidal anti-inflammatory drugs, ergotamine, and triptans,16 are implicated.17 Medication overuse headache affects more women than men (5:1) and some children.
What constitutes medication overuse in individual cases is not clear. The regular intake of three or more analgesic tablets daily or narcotics or ergotamine on more than two days a week are suggested arbitrary limits.18 Low doses daily carry greater risk than larger doses weekly. A common and probably key factor in medication overuse headache is pre-emptive use of drugs, in anticipation of—rather than for—headache. Medication overuse headache does not develop when analgesics are regularly taken for another indication, such as chronic backache or rheumatic disease.19 Headache must be there to begin with.
A presumptive diagnosis of medication overuse headache is based on symptoms and a detailed history of drug use, including over the counter drugs. A prospective diary record over two weeks may help the drug history. Many patients with medication overuse headache use large quantities of drug: 35 doses a week on average in one study, and six different agents.20 Sooner or later, they seek prescriptions for “something stronger,” bringing them to the general practitioner's attention. However, medication overuse headache is confirmed only when symptoms improve after drugs are withdrawn. The headache is oppressive, present, and often at its worst on awakening in the morning. It is increased after physical exertion. Associated nausea and vomiting are rarely pronounced. A typical history begins with episodic headache up to years earlier (more commonly migraine than tension-type headache), treated with an analgesic or other acute medication. Over time, headache episodes become more frequent, as does drug intake, until both are daily. In the end stage, which not all patients reach, headache persists all day, fluctuating with medication use repeated every few hours. This evolution occurs over a few weeks or much longer,20 depending largely—but not solely—on the medication taken.
Headaches that cause concern
Some causes of headache must not be missed (box B3). In these cases, the patient's history awakens dormant suspicion, and physical examination provides support for a diagnosis that requires immediate action.
Overdiagnosed headaches
Headache should not be attributed to sinus disease in the absence of other symptoms.7 Errors of refraction are overestimated as a cause of headache.
Managing headache
Good headache management is not always easy but it is often rewarding, and it requires the clinician to have an understanding partnership with the patient. Most headache is best managed in primary care; specialist referral is appropriate when the diagnosis remains (or becomes) unclear or the measures below fail.
Migraine
Migraine is sometimes adequately self managed with over the counter remedies. Some specialists emphasise disability assessment to establish priority for medical treatment. There are few patients, however, whose lives cannot be improved by an agreed and supervised management plan, the right medical intervention in which drugs play only a part (box B4), and the objective of minimising detriment to life and lifestyle. Although cure is not a realistic aim, management failure is often due simply to sights being set too low.
Predisposing and trigger factors
A few predisposing factors (stress, depression, anxiety, menstruation, menopause, head or neck trauma) are well recognised; they are not always avoidable but sometimes treatable. Many migraine attacks have no obvious triggers. Diaries are useful in trigger detection. An enforced change in lifestyle to avoid triggers can itself adversely affect quality of life.
Acute drug therapy
Published trials and clinical practice show that drugs are effective, but no single drug works consistently in everyone. Without established predictors of efficacy, patients should work through the options in a rational order to find what is best for them. “Stepped care” is one way of achieving this.21 The goal—resolution of symptoms within two hours—is not attainable in all cases.
Simple oral analgesia—aspirin 900 mg, paracetamol 1000 mg, or ibuprofen 400 mg taken in soluble form and early before gastric stasis develops—works for many patients. A prokinetic anti-emetic (metoclopramide 10 mg or domperidone 20 mg) enhances the analgesic effect by promoting gastric emptying and is most suitable for managing nausea and vomiting. When these teatments fail, expert opinion suggests diclofenac 100 mg and domperidone 30 mg taken rectally.21
Triptans should not be withheld from patients who need them. One triptan may work but not another, so patients may reasonably try each in turn. Because efficacy is generally coupled with side effects, a logical order is proposed (table).
Prophylaxis
When symptom control with best acute treatment is inadequate (the judge of this being the patient), a prophylactic agent (box B5) is added, usually for four to six months, to reduce the number of attacks.22 Because no one treatment is reliably effective, comorbidities and contraindications guide choice. Poor compliance is a major factor impairing effectiveness, so dosing once a day is preferable.23 In some women, hormonal influences are important determinants of the frequency of attacks, and a special approach may be taken to menstrually related migraine.24
Tension-type headache
Most people with tension-type headache manage themselves. Episodic tension-type headache is self limiting and rarely raises anxiety levels, but people consult doctors when it is becoming frequent and may no longer be responding to painkillers.
Predisposing factors
Tension-type headache is more common in sedentary people, and regular exercise may help. Stress may be obvious and likely to be aetiologically implicated. Musculoskeletal involvement may be evident in the history or on examination. Sometimes, neither of these factors is apparent. In the background of chronic tension- type headache, clinical depression will defeat management if not diagnosed and treated appropriately.
Intervention
Reassurance and over the counter analgesics (aspirin 600-900 mg, paracetamol 1000 mg, ibuprofen 400 mg) are sufficient for infrequent episodic tension-type headache (fewer than two days per week) without risk of escalating consumption.
Long term remission is the objective of management in frequent episodic or chronic tension-type headache. Physiotherapy is the treatment of choice for musculoskeletal symptoms. In stress related illness, lifestyle changes to reduce stress, and relaxation or cognitive therapy to develop strategies for coping with stress, are the treatment mainstays. Amitriptyline (10 mg at night, increasing in increments to 50-150 mg as side effects permit) is the drug treatment of choice; it should be withdrawn after improvement has been maintained for four to six months. Sodium valproate (400-1500 mg/day) is also sometimes effective, and its use is increasing. Remission is not always achievable: a pain management clinic may be the final option in long standing chronic tension-type headache.
Cluster headache
Because cluster headache is uncommon in primary care, it may go misdiagnosed for years. This is tragic because although it is horribly painful, it is treatable. The general practitioner has an important role in discouraging inappropriate “treatments” (tooth extraction is not uncommon). Otherwise, urgent referral for specialist management (box B6) is recommended at each onset.
Alcohol potently triggers cluster headache. Most patients avoid it during clusters.
Medication overuse headache
Prevention is ideal, with education being the key factor: in a US study, 95% of patients with medication overuse headache were unaware that it was a medical condition.25
Early intervention is important because the long term prognosis depends on the duration of medication overuse.26 Treatment is by withdrawal of the suspected drug(s). Although this will lead initially to worsening headache and sometimes nausea, vomiting, and sleep disturbances, with forewarning and explanation withdrawal is probably most successfully done abruptly.27 Within two weeks, usually, the headache shows signs of improvement, which continues for weeks to months; 50-75% of patients revert to their original headache type. Most patients require extended support: the relapse rate is around 40% within five years.26
Headaches that cause concern
All of the headaches shown in box B3, except carbon monoxide poisoning, require immediate specialist referral. When carbon monoxide poisoning is suspected, the primary care team is best placed to arrange inspection of domestic gas appliances (gas flames should burn blue, not yellow or orange).
Additional educational resources
International Headache Society (www.i-h-s.org): includes all published guidelines and recommendations of the IHS, and professional educational pages are planned
American Academy of Neurology (www.aan.com): includes a “migraine headache module” with materials for physicians to identify potential areas for change, develop and implement a plan for change, and measure the changes made (with AMA CME credits)
PRODIGY (Practical Support for Clinical Governance) (www.prodigy.nhs.uk): includes guidance on headache management (shortly to be updated) for primary care in the United Kingdom
Information for patients
World Headache Alliance (www.w-h-a.org): a constantly updated source of detailed and quality controlled information on headache for the general public, with many useful links
American Medical Association (www.ama-assn.org/special/migraine/migraine.htm): a “migraine information center” that includes information on other headache disorders for physicians and the general public
Organisation for the Understanding of Cluster Headaches (OUCH UK) (www.clusterheadaches.org.uk): a source of information and self help support for sufferers and carers; the organisation can loan high-flow oxygen regulators
Supplementary Material
Table.
Use
|
Dose regimen
|
---|---|
Appropriate for first use of a triptan | Sumatriptan 50 mg, zolmitriptan 2.5 mg, or almotriptan 12.5 mg orally |
When greater efficacy is needed | Rizatriptan 10 mg, sumatriptan 100 mg, or zolmitriptan 5 mg orally, or sumatriptan 20 mg nasal spray (which some patients prefer) |
In reserve (pending routine clinical experience) | Eletriptan 40 mg or (if needed for efficacy) 80 mg orally |
When a rapid response is important above all | Sumatriptan 6 mg subcutaneously |
When vomiting precludes oral therapy | Sumatriptan 6 mg subcutaneously |
When side effects are troublesome with other triptans | Naratriptan 2.5 mg or almotriptan 12.5 mg orally |
When relapse is a particular problem | Ergotamine tartrate 1-2 mg rectally may be useful |
It is assumed that each patient will try them in order until satisfied with the outcome.
Footnotes
Competing interests: TS has been reimbursed for speaking, attending symposiums, or consulting by many of the companies manufacturing or co-marketing antimigraine drugs, including all marketed triptans. He has also received educational or research grants from them. He is director and trustee of the International Headache Society, World Headache Alliance, European Headache Federation and British Association for the Study of Headache; these charities have accepted financial support from many of these companies. MF has been reimbursed for speaking and for attending regional, national, and international symposiums by various companies marketing triptans. She has also received unrestricted educational grants from them. She is a director and trustee of the British Association for the Study of Headache, which has accepted financial support from several of these companies.
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