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CLUSTER HEADACHE
Chronobiological and clinical features.
Authors: Mioara Rizescu
Alexandru Diaconescu
Motto: “The unknowns are countless !!”
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Motto:
“ Doctors pour drugs of which theyknow little, into patients of whom
they know less, for diseases of
which they know nothing” !!! -
Voltaire
“ Headache is a common disorderand yet knowledge of its
epidemiology is limited” - M. Bond3
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Cluster headache (CH)
• Underdiagnosed
• Is the most severe form of headache known in
humans, sometimes rendered as “suicide
headache”.
• Known for over a century
• Frequent confused with classic trigeminal
neuralgia
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Cluster headache (CH)
• belongs to the group of primary headaches
• In IHA - classified in the "Autonomous
Trigeminal Cefalgias" with:
-SUNCT
-Chronic paroxysmal hemicrania
-Episodic paroxysmal hemicrania
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Trigeminal Autonomous Cefalgias
(TACs)A – common characters: – Shortness
– Unilateral orbito-temporal pain (periocular)
– Very intense
– Vegetative symptoms: lacrimation, rhinorea, nasal
obstruction ...
B – differences: – Duration and frequency of individual attacks
– The appearance and intensity of pain
– Response to various therapeutic measures 6
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CH Epidemiology (1)
• 0.1-0.9% prevalence in the general population(migraine 6%)
• Predominantly in Caucasians, may occur in other
racial categories.• Predominantly male ("headache of men") 6:1
ratio, 2:1 since 1960
• Typical onset in 3rd-4th decade but can occur atany age, extremely rare in children and
adolescents; never over 70 years
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CH Epidemiology (2)
• The incidence decreases after age 50
• It can appear in women after 50 years
(unknown cause), but never in men !
• Not related to the menses
• Tends to disappear during pregnancy
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Predisposing factors for CH
• Leonine facies (typical!?)
• Heigh (the tallest members of the family)
• Possibly an autosomal dominant gene (4% ofmen and 10% of women with CH)
– Frequency 14 times higher in first-degree relatives;
2 times higher in the second degree relatives – Recorded in some sets of monozygotic twins
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Risk factors for CH(without direct cause-effect relationship)
• Smoking: 85% of those with CH are smokers, but
quitting is not beneficial
• Chronic alcohol consumption• Post-Traumatic (brain injury)
• 1st degree relatives with CH
• Family history of migraine; 51% of those withCH have also personal history of migraine
• Male gender10
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Clinical forms of CH (1)
Episodic cluster headache (ECH) - 90% of CH
– Attack of 15 min 3 hrs
– 1 8 attacks daily
– Repeated daily for weeks months; typical: 6 12
weeks
– Spontaneous remission occurs, for months or evenseveral years
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Clinical forms of CH (2)
Chronic cluster headache (CCH) - 10% of CH – 90% of CCH subjects with ECH that in time
have no longer remissions: "secondary
CCH”; 10% had primary chronic form – Remission of less than 28 days or,
– Absence of remission in one year
– Severe consequences on quality of life
– 20% practically do not respond to
medication12
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Risk factors for CHC
• Late onset in lifetime
• Frequent episodes of CH with very short
remissions• Heavy smoking
• Regular alcohol consumption and / or high
volumes• Appearance of resistance to a particular
drug that was effective in the past
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Pain in CH• Unilateral, typically always on the same side at the
same subject.
• At 14-18% can move contralaterally in a cycle or at
subsequent cycles.
• Always severe and with constant intensity during the
attack.• Periocular and temporal localization; irradiation
possible to forehead, nose, cheeks and the neck on the
same side.
• Occurs at about 90 min after being asleep in the first
REM, and cause the awakening.
• Associate agitation, restlessness +/- aggression
• Aggravated by clinostatism and motionless. 14
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Autonomic manifestations in CH
(Exclusive during the attack, ipsilateral)
• Lacrimation, conjunctival hyperemia ("red eye"),
miosis, palpebral ptosis
• Swelling of the forehead, face, eyelids
• Nasal obstruction, watery rhinorrhea
• +/- Symptoms "migraine-like”: sensitivity to
light, noise, associated with vomiting, aura (no
typical)
• Tachycardia quickly installed into the attack
followed by bradycardia15
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Pre-attack symptoms (inconstantly)
• Sweating• General discomfort
• Hemifacial erythema – homolateral
• Paresthesia as “burning” – homolateral
Gestures for pain relief :• Pressing or striking the head against a hard surface
• Applying cold objects on the painful area
• Breathing in cold air
• Jogging “on place”
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CH Episode triggers
• Stress• High altitude
• Air traffic
• Meteorologic fronts - warming weather
• Alcohol (sometimes triggers immediate the
attack; during periods of remission alcohol
does not trigger an attack)• Administration of nitroglycerin and other
vasodilators
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CH pathogenesis (1)
VASCULAR THEORY
• Swelling of the cavernous sinus walls during the attack
• Neighbouring of the trigeminal nerve (V) and common
oculomotor (III) with cavernous sinus.
• Dilation of the internal carotid artery and of theophthalmic artery by the loss of vascular tone during the
attack (angiographic evidence)
• CH episodes have marked sensitivity to vasodilators
(alcohol, histamine, nitroglycerin)
But CH is incorrectly defined as vascular headache!
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CH pathogenesis (2)
NEUROLOGIC THEORY
• Activation of the trigeminal nerve causes pain in and
around the eye.
• Enabling VNS causes associated symptoms (lacrimation,
rhinorrhea etc.)• The hypothalamus (as part of the brain that regulates the
rhythm of sleep / awake / watch) is considered the trigger
that activates trigeminal nerve and VNS
• Obs.: Hypothalamus became a target for new therapies
in CH
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CH pathogenesis (3)
NEUROVASCULAR THEORY - I
• It is clear the involvement of both vascular and nervous
system
• At the onset of the attack activate the nerve-path of the
brain-base: "trigeminal autonomic reflex path”
• Hypothalamus, for reasons not yet identified, cause the
stimulation and activation of the trigeminal nerve.
• PET images, in the attack shows the activation of thehomolateral (to the pain) hypothalamus and increasing
blood flow in this area, changes that not exist outside the
attack time.20
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NEUROVASCULAR THEORY - II• The defect of the hypothalamic center regulating
biological rhythms of the secretion of some hormones.• Melatonin - biological marker significantly correlated
with hypothalamic function and integrity of the circadian
system.
• Melatonin levels are lower than normal in CH patients.
• Melatonin levels are lower in those with CH both in
active phase but also in remission periods.
• Nocturnal production of melatonin in patients with CH inthe active phase is lower than in periods of remission at
the same patients.
• Note: CH is regarded as a thalamic syndrome with
secondary neurovascular involvement. 21
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CH Chronology • The attacks have certain circadian rhythm:
appearance at night in the first REM sleep cycle;at the same person at the same time.
• Cycles were given annual; appearance for several
weeks, starting in the summer and wintersolstices.
• Cycles takes (sometimes) all the spring or
autumn long.• Each subject has its own rhythm.
• Note: cyclicity led to suspect the involvement of
the hypothalamus in the pathogenesis 22
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Positive diagnosis of CH (1)• In practice is exclusively made on chronobiological characters
of pain:
• Extremely intense pain, unilateral ocular-temporal, with
appearance in crisis "cluster attack”
• Trend in daily attacks for periods of weeks: “period / cycle /cluster episode “
• Complete remission (without CH between cycles)
• Circadian periodicity and circannual attacks "alarm clock
headache"
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Positive diagnosis of CH (2)
• The pain characters and autonomic symptoms are presentexclusively in attack (persistence outside the attack exclude the
diagnosis of CH)
• IHS criteria: the parameters of severity, duration, frequency of
symptoms• Trigger-Testing with nitroglycerin, alcohol
• Polysomnography (some CH subjects have obstructive sleep
apnea)
• Exclusion of other causes of secondary cluster: tumors, vascularmalformations, stroke (angio-MRI)
• Note: If a headache attack is longer than 4 hours the diagnosis of
CH should be reconsidered
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Differential diagnosis of CH• Migraine (aura, photophobia, response to propranolol).
• Episodic paroxysmal hemicrania both respond
• Chronic paroxysmal hemicrania to NSAIDs
• SUNCT - Pain 30 sec - 4min, with frequent repetition until
30/hour.
• Continous hemicrania - mild, periodic exacerbations,
response to NSAIDs.
• Trigeminal neuralgia - duration: seconds 2 minutes,
localized to face and cheeks, non-periodic, response tocarbamazepine / gabapentin.
• Symptomatic CH (caused by an identifiable anatomic
lesion).
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CH Treatment• Objectives
– controlling pain attacks – preventing recurrence
– improving QOL
• Options – Medications
– Surgical procedures (for cases unresponsives to drugs)
• Forms of treatment:
Curative: to suppress the attack
Prevention: - "transient prevention "
- Long-term prevention.
• Based on chronological characters of CH 26
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Treatment of acute attacks
• OXYGEN - 100% on face mask, 20min, 7-10 L
/ min, the subject standing, not lying, positive
answer in about 80%, under 50 years.
• TRIPTANS - Selective serotonin receptor
antagonists:
– Sumatriptan, injected sc (suppress access in 15
minutes) or nasal spray (suppress access in 30
minutes); inactive in the oral form/tablets, no preventive effect and can develop resistance
– Zolmitriptan, po: slow effect, used when O2 is
ineffective and the subject does not tolerate
injections 27
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CH Treatment (1)CORTICOSTEROIDS
– Prophylactic transient medication in CH, improvement after 1-2 days, reduce or eliminate attacks, short courses of 7-14 days,
rarely as single medication, recurrence occurs to dose
reduction or interruption.
ERGOT ALKALOIDS – Reduces inflammation and suppresses dilation of cerebral
vessels.
– Dihydroergotamine mesylate (DHE-45) iv in attack (active in
15 minutes) im, sc, nasal spray (effective in longer time); doesnot affect the episode duration and frequency of attacks
– Ergotamine po 1-2 hours before bedtime prevents the attack
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CH Treatment(2)
LIDOCAINE
– Nasal drops / spray
– Active in 10 minutes
– Reduces pain intensity
– Rarely suppress the attack – Useful as adjuvant therapy.
OCTREOTIDE
– Good results in the treatment of attacks
– No sufficient studies till now
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CCH Treatment
• Sumatriptan + corticosteroids (CS)• In severe cases: hospitalization; iv ergotamine + CS
• Pizotifen - used in Europe, not in the U.S., useful
also in ECH, long term effects unknown yet.
Treatment in refractory CH (10-20%)
• Pizotifen small studies
• Valproic acid limited effect
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Prophylactic treatment of CH (1)• Objectives:
– Suppression of pain – Keeping suppression during the probable time of episode
– Reduce the frequency / duration / intensity of pain when the
attacks can not be suppressed.
• Medications :
1. Verapamil - effective in ECH and CCH
2. Corticosteroids for short periods
3. Lithium – effective in both ECH and CHC, long lastingimprovements, develop tolerance, attention in combination
with NSAIDs and thiazide.
4. Valproic acid - suppress attacks in 1-4 days, active in forms
with "migraine-like"symptoms 31
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Prophylactic treatment of CH(2)
• Medications :
5. Topiramate - prescribing in low-dose, improvement after 1-2
weeks, promising in CH, still under investigation. Beware of
side effects!: Kidney stones, impaired cognitive status, weight
loss.
6. Methysergide - 20-70% effective in CCH, not to prescribe
more than 3-4 months, attention to side effects!: Pulmonary and /
or retroperitoneal fibrosis.7. Additional medication: no sufficient scientific data:
lamotrigine, gabapentin, tizanadine, baclofen, lamictal,
clonidine.
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Alternative medications -non- approved by the FDA for CH
• Melatonine – in high doses can stop the attack,
induce remission in 3-5 days in 50% of cases,
minimal side effects, is used in doses ranging from 3
mg - 6g, is debating the efficiency in refractory CH• Capsaicin - Intranasal homolateral, suppresses pain
in 60% of cases
• Civamide - synthetic form of capsaicin, intranasal,reduce attacks number .
• Botox - botulinum toxin type A - Effective and Safe
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The surgical procedures in CH
• Indications: – The lack of response to drug therapy
– Only subjects with constant symptoms on the same
side
• Procedures:
– Involving trigeminal nerve
– Involving autonomic pathways
– New / Experimental
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Procedures involving V nerve
• Alcohol or lidocaine injection in supra- and
infraorbital nerves
• Gasser-ganglion alcoholization
• Radiofrequency gangliorhyzolysis
• Rhyzotomy with glycerol
• Sectioning the root• Gamma-knife radiosurgery
• Microvascular decompression +/- nervus
intermedius sectioning 35
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Procedures involving vegetative paths
• Petros superficial nerve section• Intermedius nerve section
• Radio-frequency lock of spheno-palatine ganglion
New & experimental procedures• Greater occipital nerve block
• Deep hypothalamic stimulation (Italy)
• Surprisingly CH passes if extracted or implanted
teeth (Franco Mangini)36
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Complementary therapies
• Magnesium (sulfate) - iv• Acupuncture
• Chiropractic therapy
• Bio-feedback• Progressive muscle relaxation
• Massage, reflexology, aromatherapy
• ! Caffeine increases the effectiveness of drugs
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Th G id CH
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Therapy Guide CH• In acces:
– Oxigen
– Sumatriptan SC, nasal
spray
– Zolmitriptan PO
– Dihidroergotamine
– Lidocaine intranasal
• Rapid suppression of attacks :
– Corticosteroids – Ergot derivatives
– Occipital nerve
blockade
• Therapy to maintain remission
– Verapamil
– Methysergide maleate(unavailable in USA)
– Lithium
– Sodic divalproat / valproic acid
– Topiramat
– MELATONIN
• Refractory CH Therapy
– IV desensitization with
histamine phosphate
– V nerve neurolysis with thermalradiofrequency, glycerol
injection
– Gamma-surgery
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INSTEAD OF CONCLUSION
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INSTEAD OF CONCLUSION
• Detailed knowledge of chronobiology allow clinicaldiagnosis of CH and use of preventive therapy in CH
attacks and relapses
• It is a very frustrating condition underdiagnosed ormisdiagnosed.
• Requires patient self-education on the pathological
condition, symptom control and reduce the impact on dailywork and life.
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