Cluster Headache.eng

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CLUSTER HEADACHE

Chronobiological and clinical features.

Authors: Mioara Rizescu

Alexandru Diaconescu

Motto: “The unknowns are countless !!”

1



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



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

4



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



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

8



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



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



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



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



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



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



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



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



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



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



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



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



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



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



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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