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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
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
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
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 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 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
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 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’
• 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!

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Atypical Headaches for GP Event March 2015 - JG

  • 2. 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……
  • 3. Unusual Headaches… …….that might need a referral to the Neurology Rapid Access Clinic (NRAC) i.e. not the others just mentioned
  • 4. " 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."
  • 5. 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
  • 6.
  • 7.
  • 8. 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.
  • 9. • 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
  • 10.
  • 12.
  • 15. 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
  • 16. Spreading facial parasthesia – MS brainstem relapse?
  • 17. 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.
  • 19. 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
  • 20. 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
  • 21. 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
  • 22. 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
  • 23. Trigeminal Autonomic Cephalalgias Cluster Headache Paroxysmal 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
  • 24. Cluster Headache TREATMENT Medical Treatment Abortive Therapy Preventative Therapy Transitional Therapy
  • 25. 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
  • 26. 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
  • 27. 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
  • 28. 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
  • 29. 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
  • 30. 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
  • 31. 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
  • 32. • 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’
  • 33. • 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