Headaches - Adult Leeds

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1 Background Information

This pathway was developed in August 2016 by the Leeds CCGs, and clinicians from A&E and Neurology at LTHT.

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2 Information Resources for Patients and Carers

The Migraine Trust website
Ouch(UK) - Organisation for the understanding of Cluster Headaches website

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3 Additional Resources

British Association for the Study of Headaches - BASH Guidelines

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7 Same Day 'Thunderclap'

Same day / thunderclap onset

  • Worst ever severe headache
  • Exertional
  • VP shunt
  • Focal neurology/decreased GCS

If any of these features are present, refer as an emergency to LGI ED.

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10 Red Flag / 2 Week Wait

Urgent Referral

  • Subacute, progressive, focal neurological deficit developing over days to weeks
  • Adult patients with new onset seizures characterised by one or more of:
  • Focal seizures
  • Prolonged post-ictal focal deficit
  • Status epilepticus
  • Symptoms or signs of raised intracranial pressure
  • Patients with non-migranous headaches of recent onset ONLY IF accompanied by features suggestive of raised intercranial pressure.

Urgent inpatient referral should be considered in patients with any suicidal ideation triggered by the intensity of the attacks.

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11 Known Migraine

Quick info:
Patients may suffer from both migraine and migraine with aura. An individual patient may experience changes in their classical migraine presentation as they age and in women with changes in hormone status such as during pregnancy and peri-menopausal.

The headache is often unilateral, and has a throbbing character. It can last from a few hours to 72 hours in most cases.

In addition patients may experience visual or sensory aura, nausea, or focal neurological deficits such as paraesthesia of hand, arm or face for up to 60 minutes. Auras almost never last longer than 90 minutes.

When to start migraine prophylaxis?
There are no hard and fast rules, but generally preventative pharmacological treatment for migraine should be considered when migraine significantly interferes with a patient’s daily routine, and the use of appropriate acute therapy is not providing adequate symptom control or being used to often that it may trigger medication overuse headache.

Prophylactic treatments should be used in addition to the use of acute therapy. Clinical trials have shown that preventatives reduce the frequency and intensity of attacks but will not terminate migraine attacks completely.

Appropriate review of lifestyle trigger should also be undertaken before and during the prescription of prophylactic medication.

Patients should be encouraged to:

  • Reduce caffeine intake
  • Ensure adequate hydration
  • Avoid skipping meals leading to fluctuations in energy levels
  • Control stress
  • Look at sleep hygiene practices to improve quality and amount of sleep

What are the aims of migraine prophylaxis?
The primary aim is to reduce the frequency of migraine attacks. A goal of achieving a 50% reduction should be considered a
success. Additionally, migraine prophylaxis may help to reduce attack severity and duration and improve response to acute therapy.
Migraine prophylaxis is significantly less effective in the presence of medication over use and this should be addressed first in all patients suspected of overuse.

What types of medication are available?
There are a number of different medication types that can be tried as migraine prophylaxis. Traditionally, the more commonly used medications are beta-blockers, tricyclic antidepressants and anti-epileptic medications. Each of these medications has its benefits in different groups of patients.

What type of medication should be used?
The choice of medication to use for migraine prophylaxis needs to be tailored to each individual patient.

Evidence from clinical trials supports the use of some beta blockers (e.g. propranolol and metoprolol) and topiramate and to a lesser extent gabapentin, sodium valproate (see MHRA guidance - https://www.gov.uk/drug-safety-update/medicines-related-to-valproate-risk-of-abnormal-pregnancy-outcomes) and amitriptyline. Other factors to consider include medication side-effects, plans for pregnancy and factors shown to improve compliance - such as less complex medication regimes (once daily rather than multiple doses per day).

NICE guidelines (‘Diagnosis and management of headaches in young people and adults 2012’) recommend topiramate or
propranolol as first line treatments for migraine prophylaxis and acupuncture or gabapentin as second line treatments.

The guidelines concluded there was insufficient evidence to recommend amitriptyline or pizotifen and these should be reserved as third line treatments.

The most recent British Association for the Study of Headache (BASH) guidelines (‘Guidelines for all healthcare professionals in the diagnosis and management of migraine, tension-type headache, cluster headache and medication-overuse headache 3rd edition (1st revision) 2010’) recommend beta blockers and amitriptyline as first line treatment options and topiramate or sodium valproate (MHRA Guidance - https://www.gov.uk/drug-safety-update/medicines-related-to-valproate-risk-of-abnormal-pregnancy-outcomes) as second line options for migraine prophylaxis.

How long should migraine prophylaxis be used for?
The need for migraine prophylaxis should be reviewed on a six monthly basis. Prophylaxis should always be titrated up and down with monitoring of frequency and intensity in an appropriate headache diary.

A tailored reduction in medication over a 2 to 3 week time period should be considered rather than abrupt withdrawal.

Medication Types:

  • Beta blockers (options to choose from)
    • Atenolol 25mg OD – 50mg OD
    • Propanalol 40mg TDS – 80mg TDS or LA preparation 80mg OD
  • Tricyclic Antidepressants
    • Amitriptyline (10mg - 100mg ON dose adjusted as required and tolerated)
    • Nortriptylline (10mg - 100mg ON dose adjusted as required and tolerated)
  • Pizotifen
    • Pizotifen (0.5mg OD titrate as needed to 1.5mg OD)

Drugs that may be used as a treatment option, neurology can advise (see Leeds Health Pathways guidance):

  • Anti-epileptics
    • Topiramate - Initiate at 25mg OD titrate to 75mg BD as required. Titrate in 25mg steps every 4 weeks to achieve reduction in headache frequency. (NB better tolerability occasionally seen starting with 15mg OD dose then titrating to 15mg BD before increase to 25mg BD)
    • Cautions – Avoid in pregnancy and use with caution in women of child bearing age, beware of risks of weight loss, mood change and personality change, avoid with history of renal stones or renal problems. Check renal and liver function after starting and at dose change
    • Sodium Valproate (300mg - 1000mg BD) - extreme caution in women of childbearing age – (MHRA Guidance) https://www.gov.uk/drug-safety-update/medicines-related-to-valproate-risk-of-abnormal-pregnancy-outcomes
    • Gabapentin (300mg - 800mg TDS) - limited evidence of benefit

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12 Peri-Orbital / Cluster

Quick info:
Cluster headaches are severe unilateral attacks of unbearable pain usually focussed around the eye. They often have features of a red watering eye, rhinitis, or blocked nose.

Typically the features of a cluster headache are listed as pain which is:

  • Unilateral (around the eye, above the eye and along the side of the head/face)
  • Severe or very severe intensity
  • Variable quality (can be sharp, boring, burning, throbbing or tightening)
  • Rapid onset and short-lasting, for 15-180 minutes (typically 30-60 minutes)
  • Patient is restless during an attack
  • Headache commonly wakes the person from sleep within 2 hours of going to sleep, and may also occur at other times.

Often associated with autonomic nervous system activation which can present with any combination of the following features:

  • Red and/or watery eye
  • Nasal congestion and/or runny nose
  • Swollen eyelid
  • Forehead and facial sweating
  • Constricted pupil and/or drooping eyelid
  • May also be a continuous background headache
  • Often a striking circadian rhythm
  • Attacks may be associated with migrainous features such as photophobia, phonophobia, nausea, and vomiting

Management options
Urgent inpatient referral should be considered in patients with any suicidal ideation triggered by the intensity of the attacks.
Consider referral for specialist opinion of cluster headache or alternative trigeminal autonomic cephalgia diagnosis when:

  • At least five episodes of pain have occurred
  • Serious secondary causes for headache and eye pain, such as acute glaucoma have been excluded
  • The likely diagnostic criteria for cluster headache as detailed have been met.
  • Severe or very severe unilateral orbital/supraorbital and/or temporal pain
  • Attacks of pain that last between 15 minutes and three hours if untreated
  • Attacks can commonly be associated with intense restlessness and agitation

All patients should have base line clinical assessment to exclude secondary causes of the headache syndrome including but not limited to:

  • Measurement of blood pressure (BP)
  • Neurological examination
  • Fundoscopy for papilloedema
  • Visual field examination for evidence of a pituitary adenoma (rare association)

Of note attacks can also be associated with features attributable to migraine such as photophobia, phonophobia, nausea, and do not negate the diagnosis of cluster headache

Treatments used
On confirmation of the diagnosis acute and preventative treatment options are available as in migraine patients. There is occasionally overlap between cluster headache and migraine presentations requiring combinations of therapy.

Acute therapies:

  • Triptans - Injectable sumitriptan 6mg SC or nasal zolmitriptan spray. There is no evidence for the use of tablet preparations of triptans
  • Oxygen - High flow oxygen 100% 10 - 15L / min for 10 minutes

Prophylaxis:

  • Verapamil (80mg TDS increasing in 80 mg steps with ECG monitoring to maximum doses of 960mg per day may be required. (Caution in known cardiac patients and look for possible drug interactions.)
  • Lithium (In acute cluster treatment short treatments with 800 mg - 1200 mg to achieve plasma levels 1.0 - 1.4 mmol/l. may be needed. Chronic cluster prophylaxis dose 300mg – 600mg to achieve plasma levels of 0.3 – 0.8 mmol/l/ Needs regular monitoring under advice of neurology.
  • Topirimate (Limited evidence but in doses titrated towards 100mg / day has some response if Lithium and verapamil not options. Use under advice of neurology (see cautions with Topiramate under migraine prophylaxis.)

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13 Tension

Quick info:
Tension-type headache (TTH) is the most common primary headache disorder. Diagnosis of TTH should be considered in a patient presenting with bilateral headache that is non-disabling where there is normal neurological examination.

In general it has the following characteristics:

  • Bilateral location
  • Pressing/tightening (non-pulsating) band like quality
  • Mild-to-moderate intensity
  • Usually not aggravated by routine physical activity, but can be made worse with exertion
  • No nausea or vomiting – anorexia may occur
  • Photophobia and phonophobia are usually absent (unless patient has combination headache with migraine)
  • Stress-related or associated with functional or structural cervical or cranial musculoskeletal abnormality
  • May have musculoskeletal involvement
  • No other pathology attributable

It can be either episodic or become chronic if there are more than 15 per month.

Patients presenting to general practice usually do so because medication is no longer working.

Before diagnosing chronic tension type headache ensure that medication overuse has been properly addressed and re review for combined migraine and tension headache.

Treatment recommendations
Look for and address any stressors
Reassure patient not serious pathology
Exclude any underlying depressive symptomatology
Consider regular exercise and physiotherapy.
Only consider drug therapy after the above treated

Preventatives:

  • Amitriptyline 10mg – 100mg (titrated with response and side effects)

Symptomatic treatments (for episodic occasional use):

  • NSAID’s (note co-morbidities and contra indications)
    • Aspirin sol 600mg (up to 600mg TDS)
    • Ibuprofen 400mg (up to 400mg TDS)

Chronic treatments to try to break cycle:

  • Naproxen 250mg – 500mg BD (short course 3 weeks max)

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14 Medication Overuse

Quick info:
All patients with chronic headache should be assessed for medication overuse if a serious cause has been excluded.

Patients not responding to preventative medication and complaining of worsening headache with time may have medication overuse.

Consider when:

  • episodic tension headaches or migraines that have gradually worsened over time
  • Increasing frequency of both headache and medication use
  • Relapse of headache at shortening periods following medication administration
  • Increasing strengths of analgesia required to bring relief
  • Variable location and character of headache
  • Reduction in effectiveness of preventative medications

Features:

  • Headache which is present for more than 15 days per month
  • Which has developed or worsened while taking regular symptomatic medication
  • Evidence of frequent use of analgesics on 15 or more days a month or triptans 10 days a month. All analgesics can contribute
    • Combination analgesics containing barbiturates, caffeine, and codeine
    • Paracetamol
    • Aspirin or other non-steroidal anti-inflammatory drugs (NSAIDs)
    • Codeine phosphate or dihydrocodeine

Treatment
The only treatment is withdrawal of all implicated medication for 4 weeks at least.

  • Prescribed medication (beware opiate and barbiturate withdrawal may need to be slow)
  • Over the counter medication
  • Caffeine

Patients need to be warned that they may experience:

  • Worsening headache
  • Nausea
  • Vomiting
  • Light headedness (arterial hypotension)
  • Palpitations (tachycardia)
  • Sleep disturbances
  • Restlessness
  • Anxiety

Following detox patients will usually revert to their previous headache type. Failure to revert should trigger review of headache diagnosis and referral to specialist clinic.

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15 Other - Primary Stabbing, Cough, Exertional, Headaches Associated with Sexual Activity, Hypnic

Quick info:

Primary stabbing headache
Primary stabbing headache has previously had multiple names associated with it which include

  • Lidiopathic stabbing headache
  • Ice-pick headache
  • Jabs and jolts
  • Ophthalmodynia periodica

These are all basically the same process and have the following features:

  • Very brief (usually seconds up to a maximum of 1 minute)
  • Sharp / jabbing pains
  • In the head (almost never the face)
  • Either as a single stab or a series of brief repeated volleys of pain.
  • It may move from one area to another in either the same or opposite side of the head.
  • Cranial autonomic symptoms associated with cluster headache and other trigeminal autonomic cephalgias are normally absent in primary stabbing headache.

Treatment - As with other shorter lasting primary headaches, primary stabbing headache tends to respond well to indomethacin

Caution in cardiovascular disease, renal disease and asthma. Use under neurology advice only.

 Primary cough headache
The diagnosis of primary cough headache can only be made when headache is brought on and occurs only in association with coughing, straining or a valsalva manoeuvre and in the absence of any abnormalities on neuro-imaging. Neuro imaging is required with any form of exertional headache to exclude a chiari malformation.

Features:

  • Headache
  • Sudden onset
  • Lasting from one second to 30 minutes
  • Brought on by and occurring only in association with coughing, straining and/or valsalva manoeuvre
  • Not attributed to another disorder (i.e normal imaging excluding structural pathology)

Treatments:

Use alternative agents only under neurology advice.

 Primary exertional headache
After exclusion of primary pathology including vascular pathology primary exertional headaches are estimated to be the cause of up to 2.8% of all referrals to headache clinics

Features:

  • Bilateral in onset
  • Throbbing or pulsatile in quality
  • Last five minutes to 48 hours.
  • Arise during or after physical exercise. Exertion may trigger both benign primary
  • At first presentation all exertional headaches should trigger investigation and exclusion of a primary cause

Treatments - The short duration nature of these headaches makes prevention the better option.

Caution in cardiovascular disease, renal disease and asthma. Use under neurology guidance only.

Primary headache associated with sexual activity
The lifetime prevalence of sexual headache in the only general population based study is estimated at about 1%. It appears to have a male predominance, and some patients will also have a history of migraine, benign exertional headache or tension type headache.
In general it is a benign but self-limiting condition after the exclusion of serious pathology.

3 sub types of sexual headache have been recognized and described.

Type 1

  • Bilateral
  • Usually occipital
  • Pressure-like headache
  • Increasing pain with mounting sexual excitement.

Type 2

  • Explosive
  • Throbbing quality
  • Appear just prior to or at the moment of orgasm.
  • Often arise in the occipital area but may rapidly generalise. (note very similar to SAH headache)

Type 3

  • Monocephalic
  • Triggered by upright position
  • Mimics clinical features and presentation of a low CSF pressure (post lumbar puncture type) headache

Treatments

Hypnic headache
Hypnic headache is relatively rare and has a tendency to affect elderly females

Features:

  • Dull headache
  • Awakens sufferers from sleep.
  • No associated autonomic symptoms
  • Normally classified as mild to moderate
  • Bi-lateral (both sides) in up to two thirds of cases.
  • Brief attacks- lasting between 5 minutes and 3 hours
  • Can occur in clusters several times in a night

Treatments:

  • Start indometacin 25mg TDS (http://www.ihs-classification.org/_downloads/mixed/International-Headache-Classification-III-ICHD-III-2013-Beta.pdf)
  • Continue for 3 days if there are no side effects, if the headache stops remain at this dose for the next 10 days. If headache still present increase to 50mg TDS and if necessary to 75mg TDS
  • Consider need for PPI stomach protection and contra indications to the use of NSAIDS.
    Caution in cardiovascular disease, renal disease and asthma. Use under Neurology guidance only.
  • Alternative are nocturnal caffeine or lithium under specialist guidance

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16 Other - Paroxysmal Hemicrania, Hemicrania Continua, Daily Persistent

Quick info:

Paroxysmal Hemicrania
In common with migraine and cluster headache hemicrania headaches have an episodic and chronic form. They have similar features to cluster type headache with autonomic activation but are much shorter.

Features:

  • Unilateral
  • Last 10 - 30 minutes
  • Multiple episodes 5 - 40 attacks per day
  • More common in females
  • Almost absolutely responsive to Indometacin

Treatment:

Caution in cardiovascular disease, renal disease and asthma. Use under Neurology guidance only.

Hemicrania Continua Headache
This is very similar to paroxysmal hemicranias but the episode does not relapse and remit

Treatment:

Consider need for PPI stomach protection and contra indications to the use of NSAIDS.
Caution in cardiovascular disease, renal disease and asthma. Use under Neurology guidance only.

New Daily Persistent Headache
New daily persistent headache is not a diagnosis. The true causation of new daily persistent headache is one of the other headache syndromes that have turned chronic.

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18 Refer to Neurology

Quick info:
Refer to neurology if:

  • Failure to respond to two 1st line prophylactic agents
  • Change in features

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19 Rapid Transfer SJUH

Quick info:
Please send the following patients to SJUH.

  • Severe headache and temporal region tenderness
  • pyrexial
  • Concerns regarding malignant hypertension or known CA/HIV/immunocompromise
  • Ophthalmology

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20 Rapid Transfer LGI

Quick info:
If concerns regarding SAH, consider Perry's rules:

Rule 1

  • Age > 40
  • c/o neck pain/stiffness
  • Witnessed LOC
  • Onset with exertion

Rule 2

  • Arrival by ambulance
  • Age > 45
  • Vomiting at least once
  • Diastolic BP > 100mmHg

Rule 3

  • Arrival by ambulance
  • Systolic BP > 160mmHg
  • c/o neck pain or stiffness
  • Age 45-55

A study on 1999 patients demonstrated these rules to have 100% sensitivity1 and the rules have been verified within the local
population with the same finding2

References:
1. Perry et al. BMJ 2011;18:343
2. Binbay A, Chadwick A, Hassan TB. Tolerable risk in patients with Lone Acute Sudden Headache (LASH) presenting to the
Emergency Department. How far have we progressed?

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21 Red Flags

Quick info:
Red flags for headache 24 hours and no focal neurology

  • Known CA
  • New or increased frequency seizures
  • Worst in morning or on coughing/straining/bending over

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25 Request Urgent MRI

Quick info:
If scan normal and significant concerns, refer to on-call registrar via PCAL