19th June 2023

Primary Headache Syndromes

Migraine

Diagnosis

Migraine is a common primary headache disorder characterised by intermittent moderate to severe headaches and associated symptoms such as photophobia, phonophobia, visual disturbance, nausea, and vomiting.

There are several different types of migraine, the clinical features and diagnostic criteria of which are detailed below.

Migraine without aura

At least 5 attacks fulfilling the following:

• Headache lasting 4-72 hrs in adults
• Headache with at least 2 of the following characteristics:
  • Unilateral
  • Pulsating quality
  • Moderate to severe intensity
  • Aggravation by, or causing avoidance of, routine activities of daily life
• Headache with associated symptoms, including at least one of the following:
  • Nausea and /or vomiting
  • Photophobia and phonophobia

• Headache not accounted for by any other diagnosis

Migraine with aura

At least 2 attacks fulfilling the following:

• One or more typical fully reversible aura symptoms, including
  • Visual symptoms – e.g. zigzag lines, scotomas
  • Sensory symptoms – e.g. unilateral paraesthesia or numbness
  • Speech and/or language symptoms – e.g. dysphasia

• At least 3 of the following:
  • ≥ 1 aura symptom spreads gradually over at least 5 mins
  • 2 or more aura symptoms occur in succession
  • Each individual aura symptom last 5-60 mins
  • ≥ 1 aura symptom is unilateral
  • ≥ 1 aura symptom is positive (e.g. zigzag lines, scintillating scotoma, as opposed to negative symptoms such as temporary visual loss and dark spots.)
  • The aura is accompanied or is followed within 60 mins by headache.

• Headache not accounted for by any other diagnosis

It should be noted that occasionally, aura can occur without headache or can be atypical in nature, e.g. causing motor weakness, diplopia, or decreased consciousness. These presentations often prompt further urgent investigation to rule out a more serious cause, such as a cerebrovascular event.

Episodic and chronic migraines

Differentiation depends on the frequency of headaches:

• Episodic – migraine on < 15 days per month
• Chronic – headache ≥ 15 days per month (with migraine features ≥ 8 days per month)

Menstrual-related migraine

Suspected when migraine occurs in the days immediately before and after the start of menstruation for consecutive menstrual cycles.

Management

• Avoidance of triggers where identifiable. Common triggers include disturbed sleep, irregular meals, caffeine excess, and stress.

• Avoidance of medication overuse headaches by restricting acute analgesic medication to a maximum of 2 days a week.

• Use of combined hormonal contraception is contraindicated in women who experience migraine with aura due to the risk of vascular events; alternative contraceptive options should be explored with the patient if required.

• Options for acute pharmacological management include:
  • Simple analgesia, e.g. ibuprofen, high dose aspirin (900mg) or paracetamol.
  • Triptan, e.g. sumatriptan (first choice), available in intranasal or subcutaneous formulations if unable to tolerate orally.
  • Consider antiemetics, e.g. metoclopramide or prochlorperazine, even in the absence of nausea and vomiting.
  • Avoid opioids

• Preventative treatment can be considered if migraines are frequent or severe enough to significantly impact the person’s quality of life, or if acute treatments are ineffective. Options for pharmacological prevention of migraine include:
  • Propranolol
  • Topiramate (contraindicated in pregnancy)
  • Amitriptyline
  • Frovatriptan (unlicensed), Zolmitriptan (unlicensed) for Menstrual related Migraine

NICE recommends referral to neurology if trial of 3 or more drugs is unsuccessful or if there is a development of atypical or red flag features.

Management of migraine in pregnancy and breastfeeding

• 1st line acute treatment – paracetamol
• 2nd line treatment – Ibuprofen (1st and 2nd trimester only) or triptan if paracetamol is ineffective.

NSAIDs are contraindicated in the 3rd trimester of pregnancy due to the risk of premature closure of ductus arteriosus.

Recommendation is to avoid triptan unless the potential benefit outweighs the risk and to minimise exposure of the drug to the infant by avoiding breastfeeding for 12 hours post treatment.

Cluster Headache

Diagnosis

Cluster headache is a primary headache disorder characterised by:

• Severe unilateral periorbital pain
• Ipsilateral autonomic symptoms such as conjunctival injection, lacrimation, eyelid oedema, nasal congestion, rhinorrhoea, forehead and facial flushing or sweating
• Typically symptoms last < 3 hours

As the name suggests, these headaches present as clusters, occurring in series lasting for a period (weeks or months) separated by intervals of remission. The different classifications of cluster headaches are based on their frequency:

• Episodic – occur in periods lasting 7 days to 1 year with pain-free intervals of at least 1 month.

• Chronic – occurs in periods for more than 1 year without being pain free or with periods of pain free intervals of less than 1 month.

Clinical features

• Unilateral periorbital pain with autonomic symptoms as described above
• Attacks last 15 min – 3 hours
• Attacks of headache may show circadian periodicity
• Males are affected four times more than females. Age group 20-40 years.

Management

• Subcutaneous or nasal triptan
• Zolmitriptan nasal spray (unlicensed)
• Offer short burst oxygen therapy – 100% oxygen at 12-15 litres/min via a non-rebreather face mask for 15-20 mins. Provision of home oxygen.

NICE does not recommend offering paracetamol, NSAIDs, opioids, ergots or oral triptans for acute treatment.

Pharmacological Prevention

• Verapamil
• Lithium (unlicensed)

Paroxysmal hemicrania

Paroxysmal hemicrania, like cluster headache, is a trigeminal autonomic cephalgia. The presentation is similar and can include headache and autonomic symptoms like those of cluster headaches. However, chronic paroxysmal hemicrania have an increased frequency of attacks (up to 15 in 24 hrs) and tend to have a shorter duration of attacks (5-30 mins) compared to cluster headaches. Paroxysmal hemicrania often responds well to indomethacin therapy.

Tension-type Headache

Diagnosis

Tension headache is the most common primary headache disorder.

Clinical features include:

• Generalised (or more rarely unilateral) headache, often described as a ‘band-like’ tightness around the head. It can seem to originate from, or spread to, the neck.
• Mild to moderate intensity headache lasting minutes to days. It does not tend to be exacerbated by routine physical activities.
• Common triggers or exacerbating factors include stress, caffeine intake, and disturbed sleep.
• On examination, pericranial tenderness to touch may be present.
• Tension headaches can be classified into different types depending on the frequency of episodes:
  • Infrequent (<1 episode/month)
  • Frequent (episodes 1-14 days/month for >3 months)
  • Chronic (15 days or more/month for >3 months)
• Tension headaches may be associated with photophobia or phonophobia. Nausea and vomiting are not common features.

Management

Treatment of tension-type headaches includes:

• Avoidance of triggers or exacerbating factors, e.g. caffeine excess
• Simple analgesia e.g. paracetamol, aspirin, NSAIDs. However, caution not to overuse as it could lead to medication overuse headache.
• Avoidance of opioids
• Management of associated comorbidities such as mood disorders, chronic pain, and sleep disorders.

Prophylaxis of tension-type headache

Prophylaxis is indicated if chronic features and includes:

• Non-pharmacological, e.g. acupuncture
• Pharmacological options include a trial of low dose amitriptyline (off label use)

Idiopathic Intracranial Hypertension

Diagnosis

• Idiopathic intracranial hypertension (IIH) is a rare condition resulting in increased intracranial pressure without underlying cause, such as hydrocephalus or a mass lesion. CSF composition is also normal. It is also termed Benign Intracranial Hypertension. It mostly affects young women and has a strong association with obesity.
• Clinical features include:
  • Headache with increasing severity and frequency. Headache typically worse on waking and on performing manoeuvres which increase intracranial pressure (ICP), such as bending over.
  • Visual disturbance or loss, horizontal diplopia due to abducens nerve (CNVI) palsy, reduced peripheral vision, blurred vision with loss of acuity and colour sensitivity, momentary darkening of vision on standing or bending (visual obscuration).
  • Pulsatile tinnitus, dizziness, mild cognitive disturbance, and upper cervical or radicular back pain
  • On examination, there may be oculomotor nerve palsies, papilloedema or optic atrophy.

Idiopathic Intracranial Hypertension diagnostic criteria:

1. Papilloedema
2. Normal neurological examination (except 6th CN palsy)
3. Normal brain parenchyma on imaging with no evidence of hydrocephalus, mass lesion or venous thrombosis.
4. Normal CSF composition
5. Raised opening pressure on lumbar puncture ≥ 25cm

IIH without papilledema (IIHWOP) diagnostic criteria:

1. Normal neurological examination
2. Normal neuroimaging
3. Normal CSF composition
4. Raised opening pressure on lumbar puncture ≥ 25cm
5. Unilateral or bilateral 6th cranial nerve palsy

Investigations

Investigations for IIH involve assessing complications of raised ICP and ruling out other causes. Investigations include:

• Ophthalmology examination to assess visual acuity, visual fields and fundoscopy
• Blood pressure measurement to rule out malignant hypertension
• Brain imaging within 24hrs of identification of papilloedema with CT/MRI and CT venography to rule out mass lesion, hydrocephalus, or venous thrombosis.
• Lumbar puncture in lateral decubitus position to measure CSF opening pressure and composition.

Management

There are 3 principles when it comes to the management of IIH:

1. Treat the underlying disease
2. Protect the vision
3. Manage headache itself

To treat the underlying disease – Weight loss is the only disease modifying therapy for typical IIH known to induce disease remission. Patients with IIH with a BMI >30 kg/m2 should be counselled about weight management, whilst also considering the biopsychosocial complexities around weight and weight loss.

To protect the vision – in the presence of declining visual function, surgical options are the most appropriate management options:

• CSF diversion procedures, e.g. ventriculoperitoneal shunt
• Optic nerve fenestration.

Lumbar drain can be a useful temporary option to protect vision whilst preparing for urgent surgical intervention.

To manage headache 

• Acetazolamide – carbonic anhydrase inhibitor. Reduces CSF production and can improve quality of life.

• Topiramate – if intolerant to acetazolamide. It has some carbonic anhydrase activity and can cause appetite suppression. Patients should be counselled about possible side effects, including teratogenicity, before prescribing topiramate.
• Acute headaches can be managed with NSAIDs and paracetamol, however, opioids are not recommended.
• Serial LP procedures (also known as therapeutic taps) can provide temporary relief of headaches; however, the CSF is rapidly replaced. It is not recommended due to potential associated risks, anxiety, and potential for acute and chronic back pain as a complication.

Follow up is advised with a multidisciplinary team, including ophthalmologist and neurologist. Frequently co-existing conditions like obstructive sleep apnoea, low mood, and polycystic ovary syndrome should be managed under the relevant specialist team.