Classifying headache and facial pain

migraine

Recurrent attacks that last 4 to 72 hours. Typically one-sided (not side-locked [NB2], can be bilateral), pulsating, moderate to severe intensity, aggravated by routine physical activity, associated with nausea and/or photophobia, phonophobia or osmophobia.

Can occur with or without aura.

Migraine can present primarily as neck pain or mid-facial pain rather than headache. The associated features listed above are important in making the diagnosis.

See treatment.

migraine with aura

Migraine that is preceded by aura (ie reversible focal neurological symptoms that usually develop over 5 to 20 minutes and last for less than 60 minutes).

Aura symptoms can affect vision, senses, speech and/or language, motor function, brainstem and retina.

Exclude transient ischaemic attack.

aura without headache (acephalgic migraine)

Typical aura of migraine that is not accompanied, or followed, by a headache of any sort. Most common form is scintillating scotoma.

Can occur at times in a patient who usually has a headache after aura. Is the predominant form of migraine in a few patients.

Differential diagnosis must exclude aura mimics, especially transient ischaemic attack. See advice on differential diagnosis and treatment.

tension-type headache

Lasts from 30 minutes to 7 days. Usually bilateral, feels like pressure or tightness in head. Mild to moderate intensity (rarely severe enough to prevent walking or climbing stairs).

Not associated with nausea, may be associated with photophobia or phonophobia.

Does not fit diagnostic criteria for other headache types better than criteria for tension-type headache.

See treatment.

trigeminal autonomic cephalgias (see subtypes) (trigeminal neuralgia is a different condition)

Unilateral and side-locked [NB2] (usually follow distribution of first division of trigeminal nerve) with unilateral autonomic features (eg tearing, conjunctival injection/irritation, ptosis, nasal stuffiness/rhinorrhoea, fullness of the ear, tinnitus, facial flushing or sweating). Possible photophobia or phonophobia (usually unilateral).

Patient often agitated and restless.

See treatment for cluster headache, short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing, and hemicrania continua and paroxysmal hemicrania.

reversible cerebral vasoconstriction syndrome (RCVS)

Thunderclap, recurring over 1 to 2 weeks. May be triggered by exertion, Valsalva manoeuvre, sexual activity or strong emotion. Can also be triggered post partum or by serotonergic and sympathomimetic drugs.

Can be associated with fluctuating neurological deficits and seizures.

Angiography shows 'string and bead’ appearance, but may be normal in first week. Changes on MRI are mainly posterior and may include oedema, infarction, subarachnoid haemorrhage or intracranial haemorrhage.

new daily persistent headache

Persistent and daily since onset (usually patient remembers starting date), present for more than 3 months. No other characteristic features (may be like a migraine or like a tension-type headache). Can resolve spontaneously over several months or become chronic. Treat as for main phenotype.

primary headache associated with sexual activity (benign sex headache)

More frequent in males than females. Usually benign, although thunderclap headache at orgasm can (rarely) be associated with subarachnoid haemorrhage or infarction. May occur before (usually milder) or at (usually more abrupt and severe) orgasm. Often resolves spontaneously over a few months. Consider reversible cerebral vasoconstriction syndrome. Exclude space-occupying lesion or aneurysm.

See treatment.

primary exercise headache (benign exertional headache)

Only occurs after strenuous physical activity, especially in hot weather or at high altitude. Lasts less than 48 hours. Exclude space-occupying lesion, aneurysm, carotid stenosis, posterior fossa mass lesion or Chiari malformation. May be an unusual presentation of angina.

See treatment.

primary stabbing headache (also known as ‘ice-pick headache’ or ‘jabs and jolts’)

Transient and localised stabs of pain in the head. Occur spontaneously in the absence of organic disease in underlying structures or cranial nerves. Each stab lasts a few seconds. Stabs recur irregularly. Mainly extratrigeminal, but can change site. No associated autonomic features. Can occur with a migraine and often ease when migraine is treated. Isolated cases are so brief and infrequent that treatment is not warranted. Persistent cases may respond to indometacin.

primary cough headache (benign cough headache)

Provoked by cough or Valsalva manoeuvre, can last seconds to 2 hours. Exclude space-occupying lesion or aneurysm, posterior fossa pathology, Chiari malformation and cerebrospinal fluid obstruction.

hypnic headache

Typical patient is older (more than 50 years) and woken early (1 to 3 am) by bilateral headache, often with nausea. Headache usually lasts 30 to 60 minutes (up to 4 hours). Can often be prevented by 1 to 2 cups of coffee before bed. Lithium, melatonin and indometacin may also be used. [NB3]

low cerebrospinal fluid (CSF) pressure headache

Generally worse in evening and improved by lying flat. May be associated with ‘coat-hanger’ pain across the shoulders or pulsatile tinnitus. Intracranial pressure less than 6 cmH20. May be spontaneous or follow trauma or dural puncture. See treatment.

increased cerebrospinal fluid (CSF) pressure headache

Associated with raised intracranial pressure (more than 25 cmH20, measured by lumbar puncture manometry in the lateral decubitus position). Typically worse in the morning and when lying down, improved by upright posture. Aggravated by cough, straining and Valsalva manoeuvre. May be associated with transient visual obscuration, pulsatile tinnitus and papilloedema. Exclude a space-occupying lesion, venous sinus thrombosis or obstruction, and use of drugs such as tetracyclines and vitamin A analogues (eg isotretinoin, acitretin). Consider idiopathic intracranial hypertension (see treatment), especially if recent weight gain. Visual field loss and permanent damage can follow initial transient visual problems.

cervicogenic headache

Usually accompanied by neck pain and is unilateral (side-locked [NB2]) with radiations from posterior to anterior.

Due to disorder of cervical spine (eg bone, disc, soft tissue). Neck has reduced range of movement. Headache is provoked by neck manoeuvres or digital pressure on affected structures.

High cervical or greater occipital nerve block may relieve symptoms.

drug-induced headache

Follows intake of drugs (eg alcohol, marijuana, cocaine, monosodium glutamate, nitrates, ciclosporin, phosphodiesterase inhibitors, carbon monoxide, exogenous hormones).

headache induced by metabolic/other medical condition

Patient has a medical condition associated with the headache (eg obstructive sleep apnoea, hypoxia, arterial hypertension, phaeochromocytoma, epilepsy, hypoglycaemia, hypercapnia, haemodialysis).

rhinosinusitis (acute or chronic)

Frontal, maxillary or periorbital pressure or throbbing pain. Sinuses tender to palpate. Patient may have a sense of blockage and a nasal or postnasal discharge.

Pain worse when bending forward.

trigeminal neuralgia

Recurrent, unilateral, shock-like pain in one or more divisions of the trigeminal nerve (especially V2 and V3). Can be triggered by simple stimuli (eg touch [eating, brushing teeth, speaking, shaving] or exposure to cold winds). Attacks last seconds to minutes, and are typically followed by a brief refractory period. May be associated with moderate persistent background pain. Often affects people (mainly women) aged 40 to 70 years. See advice on imaging and treatment.

May be associated with neurovascular compression or multiple sclerosis plaque.

glossopharyngeal neuralgia

Severe, transient, unilateral stabbing pain in the distribution of the ninth cranial nerve (eg ear, base of tongue, tonsillar fossa, below the angle of the jaw). Provoked by swallowing (particularly cold liquids), talking, coughing, chewing or yawning. Onset usually after 60 years of age. Two percent of patients lose consciousness in association with pain paroxysm, probably due to cardiac arrhythmia. See advice on treatment.

greater occipital neuralgia

Shooting or stabbing pain over the occipital region (over the territory of the greater and lesser occipital nerve), typically unilateral. Pain may radiate to the ipsilateral fronto-orbital region. Pressure over the site of the nerve often evokes the pain. May be allodynia or dysaesthesia over the posterior scalp. See advice on treatment.

postherpetic trigeminal neuropathy

Unilateral head or facial pain that persists or recurs for at least 3 months in the distribution of one or more branches of the trigeminal nerve. Variable sensory changes and a history of acute varicella zoster virus reactivation in the affected region.

facial pain:

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eye and facial pain/headache

Localised to the orbit or associated with eye signs (see causes).

dental pain

Usually localised to the dental area or face, can rarely cause more generalised headache. May be due to infection or traumatic irritation around a tooth. See advice on treatment.

temporomandibular joint (TMJ) dysfunction

Pain associated with pathology of TMJ or chewing muscles. Aggravated by active and passive jaw movements or pressure over affected structures. See advice on treatment.

atypical (persistent idiopathic) facial pain

Persistent poorly localised facial and/or oral pain that recurs daily for more than 2 hours over more than 3 months. Pain is dull, aching or nagging (can have sharp exacerbations), and does not follow the distribution of a peripheral nerve. May start after insignificant trauma or minor surgery, but persists after healing. No clinical neurological deficit or dental pathology. Distribution can increase over time. See advice on treatment.