UNUSUAL HEADACHES AND

FACIAL PAIN

Dr Joe Guadagno RVI

Primary headaches

• Migraine• Tension type headache• Medication overuse headache

Usually combination of all 3..............

Miscellaneous : Primary thunderclap headaches Primary exertional headacheeg Reversible vasoconstrictor syndromes

Usual headaches……

More Unusual Headaches…..

…….that might need a referral to the Neurology Rapid Access Clinic (NRAC) i.e. not the others just mentioned

Case• 32 year old joiner presented at 6.25 am to A&E with

an unbearable headache. • Had been awoken from sleep with an excruciating

left retro-orbital pain. Headache was associated with photosensitivity on the left side.

• Headache had woken him about 60 mins early. • Described feeling that he wanted to “bash his head”

on the wall. His headache had settled spontaneously by the time you arrived.

Cluster Headache

Trigeminal Autonomic Cephalalgias Cluster Headache Paroxysmal Hemicrania SUNCT

Short-lasting UnilateralNeuralgiform headache with Conjunctival injection and Tearing

or SUNA Short-lastingUnilateralNeuralgiform headache withAutonomic Features

Unilateral head pain, predominantly V1

Excruciating Cranial autonomic

symptoms Parasympathetic hyperactivity Sympathetic deficit

Attack frequency and duration differs

Treatment responses differ Highly disabling disorders

Trigeminal Autonomic Cephalalgias Cluster Headache Paroxysmal Hemicrania SUNCT

Short-lasting UnilateralNeuralgiform headache with Conjunctival injection and Tearing

or SUNA Short-lastingUnilateralNeuralgiform headache withAutonomic Features

Unilateral head pain, predominantly V1

Excruciating Cranial autonomic

symptoms Parasympathetic hyperactivity Sympathetic deficit

Attack frequency and duration differs

Treatment responses differ Highly disabling disorders

Cluster Headache

• Severe • Unilateral • Orbital, supraorbital or

temporal pain• 15-180 minutes

duration• Attack frequency

ranging from 1 every other day to 8 daily

• Associated symptoms:-Conjunctival

injection-Lacrimation-Ptosis-Miosis-Eyelid oedema-Nasal congestion-Rhinorrhea-Forehead and

facial sweating• Sense of restlessness or

agitation during headache

Paroxysmal Hemicrania • Severe

• Unilateral • Orbital, supraorbital

or temporal pain• 2-30 minutes

duration• >5 attacks daily at

least 50% of the time

• Associated symptoms:-Conjunctival

injection-Lacrimation-Ptosis-Miosis-Eyelid oedema-Nasal congestion-Rhinorrhea-Forehead and

facial sweating• Stopped completely

by indometacin

Trigeminal Autonomic Cephalgias Cluster

HeadacheParoxysmal Hemicrania SUNCT

Attack frequency (daily) 1-8 1-40 3-200

Duration of attack 15-180mins 2-30mins 5-240secs

Pain quality Sharp, throbbing

Sharp, throbbing

Neuralgiform

Pain intensity Very severe Very severe Very severe

Circadian periodicity 70% 45% Absent

Cluster Headache - TREATMENT

Medical Treatment

Abortive (acute) Therapy

Preventative Therapy

Transitional Therapy

Acute Treatments for Cluster Headache

Time= 15min 15 min 30 min 30 min

N= 150 134 77 69

Cohen et al, JAMA 2009; van Vliet J et al, Neurology 2003; Cittadini E et al. Arch Neurol 2006; Ekbom K et al. Acta Neurol Scand. 1993

• Randomised, controlled, double blind studies in cluster headache

* *

*

*

*P<0.05

Cluster Headache - ManagementAbortive (acute) Treatment oxygen and/or a subcutaneous or nasal triptan for the acute treatment of

cluster headache. When using oxygen:

use 100% oxygen at a flow rate of at least 12 litres per minute with a non-rebreathing mask and a reservoir bag and

arrange provision of home and ambulatory oxygen.

When using a subcutaneous or nasal triptan, ensure the person is offered an adequate supply two subcutaneous injections daily or three nasal sprays daily

Do not offer paracetamol, NSAIDS, opioids, ergots or oral triptans for the acute treatment of cluster headache.  

http://guidance.nice.org.uk/CG150

Cluster Headache TREATMENT

Medical Treatment

Abortive (acute) Therapy

Preventative TherapyTransitional Therapy

Cluster Headache - PREVENTIVE TREATMENTS

Verapamil

• Usually 240-480mg daily• Up to 960mg daily• 80-120mg increments

every 10-14 days with ECG monitoring

Constipation

Nausea and vomiting

Fatigue

Pedal oedema

Bradycardia

Hypotension

Cardiac arrhythmias

Gabai I & Spierings E, Headache, 1989; Leone M et al., Neurology. 2000

Verapamil - preventive treatment of cluster headache

Leone M et al. Neurology. 2000.

* p < 0.001 vs placeboAtt

acks

per

day

N=30

6/15 0/1512/15 0/15

*

*15 15

Cluster Headache- PREVENTIVE TREATMENTS Doses Comments

Verapamil 240-960mg/d ECG monitoring required

Lithium 400-2000mg/d(0.8-1.0mM)

Regular serum lithium levels, thyroid function and renal function checks

Methysergide 3-12mg/d Monitoring for visceral fibrosis

Topiramate 50-800mg/d

Gabapentin 900-3600mg/d

Melatonin 9-15mg/d

Valproate 600-2000mg/d

Cluster Headache TREATMENT

Medical Treatment

Abortive (acute) Therapy

Preventative Therapy

Transitional Therapy

Cluster Headache - TRANSITIONAL TREATMENTS

Corticosteroids

• Rapid onset of action and highly effective at high doses• Attacks recur once the dose is decreased• Indications:

– Initial add-on until other preventatives effective– Short-term use for multiple daily attacks

• Prednisolone regime – 1mg/kg (up to maximum of 60mg) od for 5 days– Taper thereafter over 2-3 weeks – Simultaneously introduce a suitable prophylactic

Couch J and Ziegler D, Headache 1978

Migraine

• Unilateral throbbing followed by dull ache• Painful• Can have aura phase (visual, sensory

etc..)• Associated nausea photophobia,

phonophobia• Drive to lie down in dark room and sleep• Can wake from sleep• Wiped out for days sometimes “hangover”

phase with general dysfunction• Attack frequency usually no more than 1

per every few days or every day (ie transformed migraine NOT CLUSTER)

Cluster

• Strictly unilateral with stabbing or boring quality

• Excruciatingly severe!• No aura phase usually• Associated trigeminal autonomic

features (eyelid oedema, conjunctival injection, tearing blocked nose etc)

• Pacing behavior around room; agitated ++

• Typically alarm clock headache in early hours of am

• Attack frequency 1-8 per day

• sharp, stabbing pains occurring as a single stab or as a series of stabs, • occurring mostly in the eye and orbit, temple, or parietal regions. • Stabs last a few seconds, and may recur throughout the day, usually at

irregular intervals. • occurs more commonly in migraine sufferers.

• official term is Primary Stabbing Headache. • also been referred to as "jabs and jolts headache”

• NB no autonomic disturbance and no trigger points..

‘Ice Pick Headaches’

Treatment – usually none (re-assurance), sometimes NSAIDs or Indomethacin

• occur exclusively at night, wakes from your sleep at the same time, usually between 1 and 3 am.

• nick named “alarm clock headache”. • can be unilateral or bilateral • Pain is throbbing although not everyone experiences this. • Pain begins abruptly and can last from 15 minutes to 6 hours, although

typically it is about 30-60 minutes. • more common amongst women than men.

• N.B. pain is not associated with autonomic features (such as a blocked

nose or watering eyes). • Similarly, nausea, photophobia and phonophobia are not usually

associated with hypnic headache.

Hypnic Headache

Treatment – NSAIDs or Indomethacin at night. Caffeine?

" I was standing in the shower with the hot water spraying on my face. It was a fast, jarring jolt of lightning pain on the left side of my face. For the next couple of weeks I was immobile. All activities and interest stopped. My time was spent waiting apprehensively for the next jab of staggering pain to hit my face. I dreaded waking up to start another day of electrical-like pains."

Case

Distinguishing features for classical TN:

• Character and location of the pain• Light touch provocation

n.b. On examination - patients will have no sensory deficit.

Trigeminal Neuralgia

Exhibits tactile trigger areas within the trigeminal distribution - which precipitates an attack when stimulated.

- there are rarely autonomic features.

Triggers include:Washing faceShavingEatingBrushing teethApplying make-upTalkingCold wind

To confirm an accurate diagnosis, several provoking factors are usually needed.

• Location: Predominantly affecting V2 and V3 distributions. Unilateral 97%.

• Age: any, most commonly over 50 years• Gender: more in women• Quality: sharp, stabbing or electrical• Temporality: paroxysmal, remissions and recurrences• Trigger Zone: often remote to pain, commonly nasolabial• Trigger stimuli: slight touch, wind, speaking, brushing teeth

• Neurological Examination: NORMAL

Trigeminal Neuralgia

Pharmacotherapy

Microvascular Decompression

Trigeminal Ganglion Block (or radiofreq. ablation)

Distinct group of patients who have a form of facial neuralgia that has all the characteristics of tension-type headache, except that it affects the midface; - called midfacial segment pain.

Pain is described as a ‘feeling of pressure’, although some patients feel that their nose is blocked when they have no nasal airway obstruction.

Mid facial segment pain is symmetric; it might involve areas of the nasion (the root of the nose), under the bridge of the nose, on either side of the nose, the peri- or retro-orbital regions, or across the cheeks.

There might be hyperesthesia of the skin and soft tissues over the affected area.

Nasal endoscopy and CT scans are typically normal.

Most respond to low-dose amitriptyline, but noticeable improvement might require up to 6 weeks.

Mid Facial Segment Pain

Spreading facial parasthesia – MS brainstem relapse?

So Remember…..

TN Cluster

Thank You – any questions?