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Dr Joe Guadagno RVI

Atypical Headaches for GP Event March 2015 - JG

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Dr Joe Guadagno RVI

Primary headaches

Migraine

Tension type headache

Medication overuse headache

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

Miscellaneous : Primary thunderclap headaches

Primary exertional headache

Eg Reversible vasoconstrictor syndromes

Usual headaches……

Unusual Headaches…

…….that might need a referral to the Neurology Rapid Access Clinic (NRAC)

i.e. not the others just mentioned

" 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."

The distinguishing features for classical TN are:

Character and location of the pain

Light touch provocation

Examination will reveal patients will have no sensory deficit.

Trigeminal Neuralgia

- Classical Clinical Features

Exhibit tactile trigger areas within the trigeminal distribution

- which will precipitate an attack when stimulated.

There are rarely autonomic features.

Triggers include:

Washing face

Shaving

Eating

Brushing teeth

Applying make-up

Talking

Cold wind

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

• Location: Trigeminal Nerve. 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/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;

- it is 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?

Case

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

with an unbearable headache.

• He had been awoken from sleep with an

excruciating left retro-orbital pain. The headache

was associated with photosensitivity on the left

side.

• His headache had woken him about 60 mins

early.

• He 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

Unilateral

Neuralgiform headache with

Conjunctival injection and

Tearing

orSUNA

Short-lasting

Unilateral

Neuralgiform headache with

Autonomic 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

CephalalgiasCluster Headache Paroxysmal Hemicrania SUNCT

Short-lasting

Unilateral

Neuralgiform headache with

Conjunctival injection and

Tearing

orSUNA

Short-lasting

Unilateral

Neuralgiform headache with

Autonomic 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 Cephalalgias

Cluster

Headache

Paroxysmal

HemicraniaSUNCT

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

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

Pain qualitySharp,

throbbing

Sharp,

throbbingNeuralgiform

Pain intensity Very severe Very severe Very severe

Circadian periodicity 70% 45% Absent

Cluster Headache

TREATMENT

Medical Treatment

Abortive 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

Verapamil in the preventive treatment of cluster

headache

Leone M et al. Neurology. 2000.

* p < 0.001 vs placebo

N=30

6/15 0/15

12/15 0/15

*

*15 15

Cluster Headache

PREVENTIVE TREATMENTSVerapamil

• 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

Management of Cluster Headache

Abortive 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

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 HeadacheTRANSITIONAL 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’

• 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

So Remember…..

TN Cluster

Thank You!