Cluster Headache

Question 1

Define cluster headache.

Answer 1

Cluster headache is an attack of severe pain localised to the unilateral orbital, supra-orbital, and/or temporal areas that lasts from 15 minutes to 3 hours. It occurs from once every other day to 8 times per day.

Question 2

How common is cluster headache?

Answer 2

• One of the few headaches which affect men predominantly (3:1)
• 8-10% of all headaches
• Onset 20-40yrs
• 10% have chronic form, 90% episodic form

Question 3

What is the pathophysiology of cluster headache?

Answer 3

Associated with ANS symptoms secondary to PNS hyperactivity and SNS hypoactivity.

Trigemino-parasympathetic reflex arc → trigeminal pain and cranial autonomic features of cluster headache.

3 cardinal features of cluster headaches:

• trigeminal distribution of pain
• ipsilateral cranial autonomic symptoms
• circadian/circannual pattern of attacks

Question 4

What are the triggers/risk factors for cluster headache?

Answer 4

• Alcohol (50-60%) - within 1hr of ingestion (migraines in contrast are triggered several hours later)
• Sleep e.g. daytime naps
• Volatile smells e.g. perfume and painr
• Warm temperatures
• Male sex
• FH
• Cigarette smoking
• Head injury

Question 5

Where is the pain localised in cluster headache?

Answer 5

Pain is often localised to unilateral orbital, supra-orbital and/or temporal areas or maxillary region.

Question 6

How do patients describe the pain in cluster headache?

Answer 6

• Excruciating - peaks within a few mins
• Worst ever experienced with women comparing it to childbirth
• Pain is boring, sharp, piercing, burning or pulsating
• Many complain of a constant pressing/burining background pain or “shadows” between attacks

Question 7

Does the pain spread anywhere in cluster headache?

Answer 7

Usually strictly unilateral but some patinets report shifting between or during bouts of headaches but not during the attack itself.

Question 8

What associated symptoms occur with cluster headaches? What distinguishes it from migraine?

Answer 8

Autonomic features accompanying pain include:

• ptosis and miosis (partial Horner’s syndrome)
• conjunctival injection,
• lacrimation,
• rhinorrhoea,
• nasal stuffiness,
• eyelid and facial swelling,
• aural fullness,
• facial sweating,
• and redness.

Most patients become very restless or agitated during an acute attack, unlike people with migraine who often report motion sensitivity during attacks.

Question 9

How often do cluster headaches occur in patients who experience them?

Answer 9

• Can occur from once every other day to 8 times per day.
• Can show cyclical periodicity occurring at the same time of year or the same time of day.
• 90% have episodic cluster headaches e.g. attacks last 7 days to 1 year separated by remission periods lasting at least 1 month.

Question 10

What is partial Horner’s syndrome?

Answer 10

Ptosis and miosis

Question 11

What are the risk factors for cluster headache?

Answer 11

Male - 2-3:1 male to female ratio

FH - x14 risk

Head injury - no causative links established

Smoking - 85% are smokers but stopping doesn’t reduce frequency of cluster headache

Drinking alcohol

Question 12

What symptoms need to be present to fulfil International Headache Society criteria for cluster headache?

Answer 12

At least one autonomic feature for diagnosis (although autonomic features are absent in 3% of patients)

Lacrimation is the most frequent symptom, followed by conjunctival injection, nasal congestion, rhinorrhoea, and partial Horner’s syndrome (ptosis and miosis).

Question 13

What investigations should you do for cluster headaches?

Answer 13

• Examine for partial Horner’s syndrome/ipsilateral ptosis

Other:

• ESR - to exclude GCA in >50yr olds
• Pituitary function tests - if abnormal may suggest pituitary adenoma
• Brain CT/MRI - to eliminate secondary causes. Consider MRI pituitary

Should all be normal in cluster headache.

Question 14

What are the differences between trigeminal neuralgia and cluster headache?

Answer 14

Question 15

How do you diagnose cluster headache?

Answer 15

Purely clinical diagnosis - it is a primary headache syndrome

Question 16

What is the acute management of cluster headache?

Answer 16

• SC sumatriptan - 6mg, can repeat after an hour but max is 12mg/day; 75% response rate within 15mins
  
  • (2nd line: zolmitriptan then intranasal sumatriptan/intranasal lidocaine)
• 100% oxygen - 80% response rate within 15mins

Question 17

When should triptans not be used for acute attacks?

Answer 17

• Coronary artery disease
• Peripheral vascular disease
• Cerebrovascular disease
• Uncontrolled hypertension
• Severe hepatic impairment

Instead, oxygen and lidocaine are used for acute attacks instead.

Question 18

What is the prophylactic management of cluster headache?

Answer 18

Verapamil - gradually increase then taper off if headache free for 2 weeks

2nd line: Lithium OR topiramate OR gabapentin OR melatonin

3rd line: valproate semisodium

4th line: surgery - occipital nerve stimulation or deep brain stimulation in the posterior hypothalamic region

Question 19

What are the complications of cluster headache?

Answer 19

Depression - usually improves with treatment

Question 20

What is the prognosis with cluster headache?

Answer 20

Symptoms tend to improve with increasing age

No long-term complications despite severity of symptoms