Headache

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6 Headache

Introduction

Headache is the most common neurological condition for which consultation is sought.¹ The approach adopted in this review offers a clinical strategy to guide the family doctor without need to resort to the International Headache Society (IHS) classification.²

This should not be interpreted as undervaluing that classification,² which is pivotal in allowing international consensus necessary for clinical research and trials, but clinical trials do not necessarily reflect everyday practice. The IHS criteria² allow researchers around the world to recruit patients with rare headache diagnoses to trial novel treatments with the ability to recruit sufficient patients to achieve statistical power. While this is fundamental for scientific progress, it does little to assist the general practitioner who sees very few cases of such conditions; for example, cluster headache. Cluster headache was not randomly chosen for this example, but rather because it is diagnosed more often by family doctors than neurologists, and affects less than 1% of those with headaches.³

What follows is an idiosyncratic approach to headache management developed over years of clinical experience, rather than based purely on evidence-based medicine.

Broad-Brushed Clinical Approach

A practical, if unscientific, approach to headache is to consider that primary headaches lie on a continuum (see Fig 6.1). At one end of this continuum lies tension-type headaches, while at the other is migraine (with or without aura). In the middle is what might be called tension-vascular headache with features of both, a term excluded from the IHS classification.²

FIGURE 6.1 Headache continuum.

Differentiating tension-type headache from migraine

Most patients who present to the neurologist with ‘bad headaches’ automatically assume these are migraines. Migraine and ‘bad headache’ have become synonymous for patients, but doctors need to determine the type of headache to select the appropriate treatment.

This starts with a concise history dating from the onset of the headaches. The doctor needs to know: how long the patient has had headaches; where they occur in the head (unilateral, bilateral, frontal, occipital, vertex or completely generalised); the nature of the pain (be it constant and gripping, throbbing and pulsating, or stabbing and lancinating); associated features (visual symptoms, gastrointestinal (GI) symptoms of nausea and/or vomiting, photophobia, phonophobia or osmophobia); the frequency and duration; and precipitating and relieving factors. This will assist in differentiating migraine from tension-type headache (see Table 6.1).

TABLE 6.1 Differentiation between migraine and tension-type headaches

Migraine	Tension headache
Unilateral—often in the temple	Bilateral—like a band or in both temples
Throbbing pain—pulsating	Tight, gripping pain—constant
May be provoked by: • often occurs once stress has passed (let-down phase) • smells • foods (e.g. chocolate) • hormonal changes

May be provoked by times of maximal stress

May have:

• teichopsia (zigzag bright shimmering colours)

• fortification spectra (like seeing the top of a cowboy fortress)

• see rainbow effects

• see photons of light (bright flashes)

Either no visual symptoms or simply some blurring of vision Nausea and vomiting is common Nausea may be present but rarely vomiting

• positive family history

• positive family history

Acute intervention:

• triptans

• ergots

• occasionally early use of analgesics

• pizotifen offers effective prophylaxis

Prophylaxis:

• tricyclic antidepressants (amitriptyline and imipramine offer effective prophylaxis)

May be provoked by alcohol May be eased by alcohol

Detailed physical examination is warranted for all headache patients, particularly fundoscopy and searching for focal neurological signs, which should alert the doctor to more serious illness and the need for investigation. It is most important to look for venous pulsations on fundoscopy, which, if present, exclude raised intra-cranial pressure (see Fig 6.2).

FIGURE 6.2 Ophthalmoscopic disc examination.

While migraine (with and without aura) and tension-type headaches make up the vast bulk of primary headaches encountered, the doctor should not ignore the possibility of other headache types. For the majority of patients, the above approach to history and examination will be sufficient to provide a firm diagnosis, thereby obviating the need to order expensive tests.

Red Flags for the Need for Further Investigation

Some symptoms should alert the doctor to the need for further testing (see Box 6.1).

These ‘red flags’ include marked exacerbation of headaches with coughing, straining or sneezing.⁴ If the headaches are provoked by stooping or postural changes, the doctor should order cerebral imaging. Associated neurological symptoms, such as sensory changes or weakness, demand further testing. Headache associated with eye movement and impaired vision suggests retrobulbar neuritis. Headache with enlarged blind spot may suggest papilloedema or raised intracranial pressure. Headache with stiff neck, nausea and vomiting should be sent to the hospital for consideration for lumbar puncture.

When in doubt, the general practitioner faced with a ‘red flag’ should involve a consultant early in the clinical picture. If the provisional diagnosis was that of a simple primary headache for which response to treatment was unexpectedly poor, there is need to revisit that diagnosis and reconsider investigations.

A change in the nature, quality or site of the headache should also raise suspicion. It is easy to become complacent when treating a patient with longstanding headaches and thus an index of suspicion is vital for good patient care.

Diagnostic Criteria

a Tension-type headache

This is the most common type of headache in which attacks are usually mild to moderate but can be quite intrusive and severe.⁵ They are classified within the IHS classification² as episodic or chronic, for which the underlying cause is often presumed to be emotional but is uncertain with a variety of aetiologies considered.⁵ It is said to be most common in the fifth decade with females affected somewhat more than males.⁵

Infrequent headaches do not usually cause medical presentation unless the pain is significant. Those who present may complain of frontal, occipital or band-like pain that usually has tight and gripping quality (see Table 6.1). It may involve the neck, causing concern of neck pathology that may or may not be found. The finding of neck pathology, such as osteoarthritis, does not necessarily have any relevance to the headache as both conditions are very common and may co-exist.

The physical examination in these patients should be normal, both generally and neurologically. It cannot be overemphasised that the examination of the patient with headache necessitates a fundoscopic examination with ophthalmoscope, as this remains the closest the doctor will get to actually look at the brain to exclude raised intracranial pressure.

b Migraine

While migraine is often perceived as the most common headache type, the relative prevalence is approximately 5% for migraine as compared to approximately 27% for tension-type headache in an adult population.⁶ This is important because the correct diagnosis dictates the appropriate treatment.

Migraine is classified into those with and without aura,⁷ which replaces the older terms of ‘common’ and ‘classical’ migraine. Unlike tension-type headaches, migraines are usually unilateral, throbbing in quality and often associated with nausea, maybe vomiting, and visual symptoms such as teichopsia (zigzag shimmering lights), fortification spectra (like the pointed top of a cowboy fortress), photons of light or a rainbow effect (Table 6.1). Patients may describe coloured lights (as if looking through a prism). The patient may experience photophobia, phonophobia and/or osmophobia.⁷

The relationship between headaches and alcohol is helpful as alcohol relaxes muscles and hence can relieve tension-type headaches, while it exacerbates migraines (see Table 6.1). Other provocateurs for migraines include: low blood sugar; hormonal changes (such as catamenial); odours (such as perfumes); allergies; excess or insufficient sleep; and transformation from other headache types.

People who have migraines often report a positive family history of headaches, suggesting a genetic propensity. It must be remembered that tension-type headaches are also very common, thereby possibly giving a false impression regarding the relevance of family history, which may be coincidental.

c Symptomatic headaches

The community cannot afford to investigate every headache when tension-type headaches are so common. Conversely, one cannot ignore headaches that are symptomatic of underlying illness. Some of the ‘red flags’ have already been discussed (Box 6.1). Headaches exacerbated by coughing, sneezing or stooping justify urgent cerebral imaging. Absence of venous pulsations on fundoscopy or evidence of raised intracranial pressure with stiff neck call for more detailed assessment and referral. It must be remembered that 10% of the normal population do not have venous pulsations.

Sudden onset of severe headaches with nausea, vomiting, stiff neck and visual obscuration may suggest subarachnoid haemorrhage. Skin rash, symptoms of upper respiratory tract infection, generalised aches and pains, photophobia and possible exposure to infected individuals may suggest meningoencephalitis. Lumbar puncture is warranted in these patients, usually preceded by cerebral imaging and definitely by fundoscopy, to exclude raised intracranial pressure (see Fig 6.2).

Headaches associated with neurological signs, such as weakness, sensory changes and/or a history of seizures, may suggest a space-occupying lesion. Cerebral imaging, such as magnetic resonance imaging, is mandatory in such cases.

It must be remembered that weakness and sensory changes follow set patterns within neurological evaluation (see Chs 4 and 5). Similarly, headaches associated with such symptoms and signs that do not conform to anatomically defined patterns, suggest psychological illness and may justify an alternative approach to management.

d Other headache types

While there are many headache types included within the IHS classification ² only the most common forms, referable to general practice, will be discussed in this section.

i Cluster headache has already been identified as over-diagnosed by family doctors. These headaches often occur on ‘the-morning-after-the-night-before’, are generally unilateral with suffused red eye, lacrimation and stuffy nose. The headaches usually occur at the same time each day, frequently in the early hours of the morning (often waking the patient), and are more common in males. The IHS classification requires patients to have at least five attacks occurring from one every other day to eight per day without other cause. They are associated with unilateral, orbital, supraorbital or temporal pain lasting 15–180 minutes (if untreated) and are associated with one or more of the following: ipsilateral conjunctival injection or lacrimation; ipsilateral nasal congestion or rhinorrhea; ipsilateral eyelid oedema; ipsilateral forehead and facial swelling; ipsilateral miosis or ptosis; or a sense of restlessness or agitation.⁸

ii Cervicogenic headache is another common family practice diagnosis characterised by chronic hemicranial pain said to be referred from bony or soft tissues in the neck. This is thought to be generated from the spinal trigeminal nucleus ⁹ but is often indistinguishable from other headache types, such as tension-type headache,⁹ and thus is treated within the spectrum of those headache types.

iii Thunderclap headache ¹⁰ presents with a sudden onset of frightening head pain that may occur during sexual intercourse, often at the climax. It is thought to relate to a sudden rise in blood pressure that may accompany coitus, and requires sensitive management and reassurance to avoid emotional consequences. It is usually self-limiting. Thunderclap headache may be the first sign of a variety of other conditions, such as subarachnoid haemorrhage, unruptured intracranial aneurysm, cerebral venous sinus thrombosis, cervical artery dissection, acute hypertensive crisis, spontaneous intracranial hypotension, ischaemic stroke, pituitary apoplexy or intracranial infection. It follows that these need to be excluded before reassuring the patient.

iv Chronic daily headache (CDH) is a term frequently used but excluded from the IHS classification.^11,¹² Rather than being a specific headache type, it defines severity. The IHS prefers terms such as chronic migraine or chronic tension-type headache. Headaches may change from one type of chronic headache to another.¹² Headaches in CDH should occur at least every other day (15 days per month) lasting a minimum of four hours per day for a period of at least six months. CDH is the most refractory form of headache and is the final common pathway for a variety of headache types.¹³

Management

Management encompasses both diagnosis and treatment, and the above diagnostic criteria offer better selection of treatment options. Treatment seeks immediate headache cessation and prophylaxis against recurrence.

Tension-type headaches respond to oral aspirin 500–1000 mg or non-steroidal anti-inflammatory agents.⁵ Many of these are available without prescription, thereby allowing excessive use, which may itself cause headaches. Analgesia-overuse headaches always must be considered and addressed when necessary.

Prophylaxis for tension-type headaches includes tricyclic antidepressants, such as amitriptyline or imipramine, starting at 25 mg nocte and building up to a maximum of 200 mg nocte. Tricyclics offer effective intervention, with amitriptyline used if there is a poor sleep pattern and imipramine where sleep is adequate. Recently, a patient on maximal dose of tricyclic antidepressants developed sufficient bradycardia to experience congestive cardiac failure. Patients should be warned that if breathing problems or palpitations occur an urgent consultation with the doctor is required.

Side-effects include fatigue, anti-cholinergic properties with thirst (which some interpret as hunger causing excessive eating and weight gain), palpitations and GI disturbance. The medication is taken half-an-hour before retiring but if GI disturbance ensues it should be taken with the evening meal.

Headache treatment is not restricted to pharmacological intervention and should include: review of lifestyle issues; counselling; exploration of diet and alcohol consumption; work-related habits; and an holistic approach to patient care, that transcends headache boundaries and is relevant to all headache types.

Immediate migraine intervention includes the triptans or ergot-containing compounds taken at the very onset of symptoms (either the aura or headache). The triptans include sumatriptan, zolmitriptan and naratriptan, which may cause a sensation of chest pressure, flushing, paraesthesia and drowsiness.⁶ Some patients may respond to one type of triptan and not another. Many ergot-containing compounds are no longer commercially available and may require a compounding pharmacist, but should not be lightly discarded from the therapeutic options.

Pizotifen offers an effective prophylaxis against migraines but little benefit in tension-type headaches. Thus it is mandatory to differentiate between them (see Table 6.1). The dosage starts at 0.5 mg b.d. and can be increased up to 1.5 mg t.d.s. (IX per day). Side-effects may include increased appetite and fatigue.

Methysergide is still an effective prophylaxis for migraine (4–8 mg/day) but it carries too many risks to be first-line therapy. Immediate side-effects include leg swelling, muscle pain, chest pain and nausea but the long-term risks, which are attached to ergotainine-based products such as pleural and retroperitoneal fibrosis, are worrying and limit its use.

For those headaches that fall somewhere between tension-type headaches and migraines, so-called tension-vascular headaches (see Fig 6.1), propranolol offers prophylaxis. Immediate headache cessation may rely on either of the above approaches though more commonly the tension-type headache approach is favoured.

The dosage of propranolol starts with 10–20 mg b.d. and can increase up to a maximum of 160 mg q.i.d. Care must be taken to avoid exacerbating cardiac disease or failure, asthma or provoking excessive hypotension. A history to identify these conditions is mandatory before prescribing medication and the patient must be warned to seek immediate medical attention if breathing problems or lightheadedness occurs.

Recently use of antiepileptic medications, such as valproate, topiramate, gabapentin,¹² pregabalin and even levetiracetam,¹³ have been used for headache therapy but it is wise to involve a consultant before embarking on their use. Similar caution should apply to the use of botulinum toxin that also has received recent acclaim following clinical trial.¹⁴

While cluster headache is rare, its features are classical, thereby making diagnosis easier. Immediate headache treatment may be offered by oxygen inhalation by a non-rebreathing mask at 6.10 L/min for 15 minutes. Subacute triptan administration (for example, 6 mg sumatriptan) or intranasal triptan (such as sumatriptan or 10 mg zolmitriptan) is efficacious. Prophylaxis is given early in an attack and maintained for a couple of weeks after it. Virapamil, a calcium channel blocker given 80–160 mg t.d.s. or 240 mg sustained release daily, is currently accepted treatment. Lithium is also used, as are steroids.⁸