	Cluster	Paroxysmal hemicrania	SUNCT/SUNA	Migraine	Trigeminal neuralgia
Sex	3M: 1F	M=F	1.5M :1F
Pain
Quality	Sharp/stab/throb	Sharp/stab/throb	Sharp/stab/throb
Severity	Very severe	Very severe	Severe
Distribution	V1>C2>V2>V3	V1>C2>V2>V3	V1>C2>V2>V3		V1(10%) V2(35%) V3(30%)
Attacks
Freq (/day)	1-8	20	100	<1
Length	30-180	2-30	1-5	Hours
Triggers
Alcohol	+++	+	-
Cutaneous	-	-	+++
Agitation /restlessness	90%	80%	65%
Episodic vs chronic	90:10	35:65	10:90
Circadian periodicity	Present	Absent	Absent
Treatment effects
Oxygen	70%	No effect	No effect
Sumatriptan	90%	20%	<10%
Indomethacin	No effect	100%	No effect
Migraine features with attacks
Nausea	50%	40%	25%
Photophonia/ phonophobia	65%	65%	25%

Cluster Headache

Epidemiology

~0.1%
M:F ~5:1 (85% male)
Onset in teens – 20’s
RF

Family history confers significant risk

Clinical manifestations

Up to 8 attacks/day
Strictly unilateral during an attack
Last 15min to 3hours
Occur daily for up to several weeks
Rarely chronic – over a year without remission
Can shift sides between different attacks in 15%
Attacks often nocturnal
Alcohol can be a trigger during a bout
Patients often restless, move about (c.f. migraine)
Pain

Severe
Orbital, supraorbital or temporal

Autonomic

Ptosis
Miosis
Lacrimation
Conjunctival injection
Rhinorrhoea
Nasal congestion
3% of patients lack autonomic symptoms

Chronic course in 10%

Diagnosis

• Clinical

• Imaging

• Should be done for all new patients given the risk of secondary (i.e. structural) causes

Criteria ICHD-3

• Severe or very severe unilateral orbital, supraorbital and/or temporal pain lasting 15-180 min (when untreated)1

• Either or both of the following:

o At least one of the following symptoms or signs, ipsilateral to the headache:

- a) conjunctival injection and/or lacrimation

- b) nasal congestion and/or rhinorrhoea

- c) eyelid oedema

- d) forehead and facial sweating

- e) forehead and facial flushing

- f) sensation of fullness in the ear

- g) miosis and/or ptosis

o A sense of restlessness or agitation

• Attacks have a frequency between one every other day and 8 per day for more than half of the time when the disorder is active

Treatment

Avoid precipitants

• Alcohol

• Vasodilating drugs

• High altitude

Acute

• Oxygen

o High flow oxygen for at least 15min – proven effective – relieves 60-80%

o 7-12L/min – stop if no response in 15-20min, use for ~30min if response obtained.

o Continued use for ongoing attacks within a short period of time may be couter-productive

• Triptans

o Sumatriptan

- >75% response rate to 6mg s/c sumatriptan

- Can give up to two injections/day

- Some patients may respond to a smaller 3mg dose

- Intranasal (20mg) may have some benefit but much less effective than SC

o Zolmitriptan

- Intranasal 5-10mg – only 28% response rate

- Oral 5-10mg also a low response rate

o Other triptans – no evidence

• Lignocaine

o 1ml of 4-10% solution – dripped or sprayed into the nostril ~ 30% response rate

o Often not effective on its own, can be added as adjunct to other agents

o Applied with patient lying down, head back and rotated to affected side, remain there for 5min

• Ergots

o Intranasal not effective

o Oral may be of some benefit - 1-2mg

o IV DHE may be helpful in severe cases

• Octreotide

o Probably not effecitve

o Single dose of 100mcg s/c was effective in one trial (less than above Rx)

Short term prophylaxis

• Prednisolone

o PredCH Trial protocol – 100mg for 5 days, tapered by 20mg every 3 days (total 17 days)

• Greater occipital nerve block

o Depromedrol 40mg + 1-2ml of 2% lignocaine

o Trial evidence for betamethasone and triamcinolone (10mg)

Long term prophylaxis

• Verapamil

o Is the agent of choice

o 240-480mg/day, short acting preparation preferred (Max 960mg/day)

o 80mg tds increasing by 80mg every 2 weeks with ECG monitoring at each step.

• Lithium

o 600-1600mg/day

o Monitor blood levels, keep b/n 0.4-0.8nmol/L (lower than used for bipolar)

• Topiramate

o Some limited evidence

o Dose up to 100-200mg/day

• Galcanezumab

o Clinically small (but statistically significant effect).

• Valproate and Gabapentin

o Some anecdotal effectiveness however trials generally negative

• Melatonin

o 10mg/day (immediate release)

o Small trials with some positive effect

• Methylsergide – (Not available in Aust.)

o 1mg/day, increased to 12mg/day as tolerated

o Only to be used for max 3-4 months given potential for systemic fibrosis

o Methylergonovine is alternative

• Other agents that have been used and are probably not effective:

o Pizotifen, baclofen, clonidine, botox

Nerve Stimulation

· Invasive and non-invasive VNS have been used with some effect

· DBS used in some cases

· Occipital nerve stimulator effective in some case reports/small series

Prognosis

80% of patients continue to have attacks after 15years

References

Wei DY, Goadsby PJ. Cluster headache pathophysiology - insights from current and emerging treatments. Nat Rev Neurol. 2021 May;17(5):308-324. doi: 10.1038/s41582-021-00477-w. Epub 2021 Mar 29.

Paroxysmal hemicrania

Rare (3-6% of trigeminal autonomic cephalgias)
Female>male
Onset 20-40
Similar to cluster but shorter attacks (2-30min)
More frequent attacks
Less severe autonomic features
More often chronic with out periods of significant remission
Complete response to indomethacin within one week

SUNCT/SUNA

Short-lasting Unilateral Neuralgiform headache with Conjunctival injection and Tearing) (SUNCT)
Short-lasting Unilateral Neuralgiform headache with Autonomic symptoms (SUNA)
Rare
Short lasting, but frequent, severe, unilateral attacks associated with autonomic features and which are often triggered.
Similar to Trigeminal neuralgia in many ways
Most data from:

Lambru G, Stubberud A, Rantell K, et al. J Neurol Neurosurg Psychiatry 2021;92:233–241.

Onset age (mean) 43

Female 63%

Onset age (range) 13-76yrs

Site of pain (dec frequency)

Periorbital

Cheek

Temporal

Retroorbital

Forehead

Occiput

Jaw

Laterality

Right ~50%

Left ~40%

Side variable (10%

Mean daily frequency (range)

44 (1-250)

Mean attack severity (out of 10)

Autonomic features:

Conjunctival injection and lacrimation (distinguish SUNCT from SUNA)

Rhinorrhoea (30-50%)

Ptosis (23-50%)

Facial flushing (~40%)

Aural fullness (20%)

Mean attack duration (sec)

170

(range: 1-1200)

Course

Episodic 10%

Chronic 90%

Triggerability

Triggered 5%

Spontaneous 36%

Both 57%

Trigger factors:

Chewing/eating

Cold wind

Light touch

Brushing teeth

Treatment

Lamotrigine, Topiramate and gabapentin are moderately effective in prevention
Lacodamide has been tried with some effect
IV lidocaine infusion – 90% respond, lasts mean 22 days
GON block

38% respond, lasts mean 41days