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5th August 2024

Primary headache syndromes

Overview

Headache is a common symptom which usually has a benign cause. Those which are not a symptom of underlying disease are known as primary headaches. In this section, we will cover tension headaches, migraines, cluster headaches and trigeminal neuralgia. The full diagnostic criteria for all headache disorders can be found in the International Classification of Headache Disorders (ICHD) Criteria, which is in the references section.

Tension-type headaches

Tension headaches are commonly triggered by stress. The patient usually reports a mild, bilateral, constricting pain as if a tight band is being stretched around their head. The course can be episodic or chronic. They usually respond well to simple analgesia and non-pharmacological treatments such as relaxation techniques and cognitive behavioural therapy. In cases of chronic tension-type headaches, where headaches are occurring on more than 15 days per month, prophylaxis with a low-dose tricyclic antidepressant can be initiated.

Migraine

Migraine is a common neurovascular disorder affecting around 1 in 6 people. It commonly occurs in young to middle-aged females. There is a strong genetic component to the disorder with around half of migraine sufferers reporting a family history. Migraines are thought to be caused by an inflammatory process in the brain resulting in non-painful stimuli being perceived as painful, for example sound and light.

  • The headache follows a chronic, episodic course with each episode lasting approximately 4-72 hours.
  • It is described as a severe throbbing pain which is made worse by normal daily activities and associated with photophobia, phonophobia and nausea.
  • Migraine can be split into two types: migraine with aura and migraine without aura. Auras typically consist of temporary unilateral sensory or visual disturbances, however they can also include motor symptoms in cases of hemiplegic migraine.
  • Acute symptomatic treatment options include combination therapy with an oral triptan and NSAID or oral triptan and paracetamol. Alternatively, monotherapy with paracetamol, aspirin, NSAIDs or a triptan can be offered. Antiemetics should also be offered if needed. Triptans should be avoided in migraine with hemiplegic aura as they may increase the chance of stroke.
  • Preventative measures include trigger avoidance and pharmacological therapies. In those patients who wish to start preventative medication, topiramate or propranolol should be offered. Topiramate is teratogenic and, therefore, should be avoided in females of childbearing age.  Other preventative options include amitriptyline, venlafaxine and verapamil.
  • It is also important to ensure that those who experience migraine with aura are not taking a combined oral contraceptive pill as this will increase their risk of stroke.
  • Migraines can be disabling, however the majority of patients respond well to treatment and the frequency of episodes generally decreases with age.

Cluster headaches

Cluster headaches are characterised by severe unilateral pain around the eye and side of the face associated with autonomic features including nasal congestion, ptosis and lacrimation. Patients are generally agitated during an attack and move around a lot. Males are more likely to be affected than females.

Episodes last 15-180 minutes and occur in clusters lasting from a few weeks to 3 months with an attack-free period of at least 3 months. There is also a chronic form of the disorder where the clusters last for 1 year or more or the remission period is less than 3 months.

Cluster headaches are classified as a subtype of trigeminal autonomic cephalalgia in the ICHD-3 criteria. The other subtypes are listed in the table below.
Paroxysmal hemicrania Signs and symptoms are very similar to cluster headaches however the acute attacks are shorter (2-30 mins) and more frequent (occurring up to 40 times per day). Attacks respond completely to indomethacin.
Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) Moderate to severe unilateral stabbing/saw-tooth pain lasting from seconds to minutes. Associated with ipsilateral redness of the eye and lacrimation.
Short-lasting unilateral neuralgiform headache attacks with autonomic features (SUNA) Similar to SUNCT, however these patients experience only one or neither of conjunctival injection and lacrimation.
Hemicrania continua Unilateral headache associated with autonomic features lasting for at least 3 months. This headache also responds to indomethacin.
  • Acute attacks are treated with high flow oxygen and/or subcutaneous or nasal triptans. Intranasal lidocaine can be used instead of triptans, for example in those with coronary artery disease where triptans are contraindicated.
  • First line prophylactic treatment is verapamil.
  • As preventative treatments can take a few weeks to work, corticosteroids can be started at the same time as verapamil as a bridging therapy. High-dose prednisolone has been shown to induce remission quickly, however it should be weaned after 5 days due to the risks of long-term steroid use.

Trigeminal neuralgia

Trigeminal neuralgia describes neuropathic facial pain along at least one division of the trigeminal nerve. The pain usually lasts a few seconds and the attacks can be triggered by stimuli such as touch and the cold. The majority of cases are caused by compression of the nerve root, however it is also seen in multiple sclerosis, head injury, cerebellopontine angle tumour, arteriovenous malformations or herpes zoster infection. The presence of sensory loss, bilateral symptoms, optic neuritis or presentation under the age of 40 are concerning for a secondary cause of trigeminal neuralgia such as multiple sclerosis. These cases would need further investigation with MRI.

The first line treatment is with carbamazepine which is started at a dose of 100-200 mg twice a day and gradually uptitrated. If medical treatment fails, then surgical options such as microvascular decompression or ablative procedures can be considered.

Other primary headache disorders

There are a number of other primary headache disorders described in ICHD. These headaches are generally poorly understood and can mimic secondary headache disorders. The table below briefly outlines some of these conditions.

Primary headache disorder Clinical features Differentials to rule out
Primary cough headache Precipitated by coughing and other valsalva manoeuvres Structural intracranial lesions
Primary exercise headache Precipitated by exercise Structural intracranial abnormalities, ICH, migraine
Primary headache associated with sexual activity Precipitated by sexual activity with increased intensity at orgasm ICH (particularly SAH), arterial dissection, reversible cerebral vasoconstriction syndrome, CVST
Primary thunderclap headache Sudden onset, high intensity headache ICH (particularly SAH), arterial dissection, reversible cerebral vasoconstriction syndrome, CVST, unruptured aneurysmal malformation
Nummular headache Pain in a small circumscribed area of the scalp Cranial bone lesions, dermatological scalp lesions

References

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