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Cluster Headache. 3 rd BIENNIAL HULL-BASH NATIONAL MEETING ON HEADACHE Anish Bahra The National Hospital for Neurology and Neurosurgery Whipps Cross University Hospital. Lifetime prevalence Tension-type headache~ 60-80% Migraine headache 15% Cluster Headache 0.1%. - PowerPoint PPT Presentation
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Cluster HeadacheCluster Headache
3rd BIENNIAL HULL-BASH NATIONAL MEETING ON HEADACHE
Anish BahraAnish Bahra
The National Hospital for Neurology and Neurosurgery
Whipps Cross University Hospital
Lifetime prevalence
Tension-type headache ~ 60-80%
Migraine headache 15%
Cluster Headache 0.1%
IHS Classification for Cluster Headache (2004)IHS Classification for Cluster Headache (2004)
• Severe unilateral orbital, supraorbital and/or temporal painSevere unilateral orbital, supraorbital and/or temporal pain
• Associated symptoms:Associated symptoms:
– Conjunctival injection / LacrimationConjunctival injection / Lacrimation
– Nasal congestion / RhinorrheaNasal congestion / Rhinorrhea
– Eyelid oedemaEyelid oedema
– Forehead and facial sweatingForehead and facial sweating
– Ptosis and MiosisPtosis and Miosis
– A sense of restlessness / agitationA sense of restlessness / agitation
• 15-180 minutes duration15-180 minutes duration
• 1 / alternate days – 8 /day1 / alternate days – 8 /day
Cluster Headache Cluster Headache – Defining features– Defining features
Strictly unilateral V1 pain + Autonomic featuresStrictly unilateral V1 pain + Autonomic features
Restlessness / agitationRestlessness / agitation
Short-duration / daily / boutsShort-duration / daily / bouts
0
10
20
30
40
50
60
70
Right Left Side-shiftbetween
bouts
Side-shiftwithin bout
Bahra, 2002 N=230
Manzoni, 1983 N=180
Kudrow, 1980 N=423
Cluster Headache – Laterality%
0
10
20
30
40
50
60
70
Right Left Side-shiftbetween
bouts
Side-shiftwithin bout
Bahra, 2002 N=230
Manzoni, 1983 N=180
Kudrow, 1980 N=423
Cluster Headache – Laterality%
Site of pain Site of pain →V→V11
%%• Retro-orbitalRetro-orbital 9292• TemporalTemporal 7070• Upper teethUpper teeth 5050• ForeheadForehead 4646• JawJaw 4545• CheekCheek 4545
%%• Lower teethLower teeth 3232• NeckNeck 3131• NoseNose 2020• EarEar 1717• ShoulderShoulder 1313• VertexVertex 77• OcciputOcciput 66• ParietalParietal 11
Bahra A et al. Neurology 2002; 58: 354-361
• JawJaw VV33
• CheekCheek VV22
• Lower teethLower teeth V V33
• NeckNeck CC2/32/3
• NoseNose VV22
• EarEar CC22
• ShoulderShoulder CC3/43/4
• VertexVertex CC22
• OcciputOcciput CC22
• ParietalParietal CC22
• Retro-orbitalRetro-orbital VV11
• TemporalTemporal VV11
• Upper teethUpper teeth VV22
• ForeheadForehead VV11
0
10
20
30
40
50
60
70
80
90
100
Lacrim
ation
Conju
nctiva
l Injec
tion
Nasal
Cong
estio
n
Rhinor
rhoe
a
Ptosis
/lid sw
ellin
gM
iosis
Bahra, 2002 N=230
Manzoni, 1983 N=180
Ekbom, 1970 N=105
Autonomic Features%
0
10
20
30
40
50
60
70
80
90
100
Lacrim
ation
Conju
nctiva
l Injec
tion
Nasal
Cong
estio
n
Rhinor
rhoe
a
Ptosis
/lid sw
ellin
gM
iosis
Bahra, 2002 N=230
Manzoni, 1983 N=180
Ekbom, 1970 N=105
Autonomic Features%
CH – 98%1
Migraine – 25%2
1. Torelli, 2001 n=553 2. Obermann, 2007 n=841
Attack Duration and FrequencyAttack Duration and Frequency
NN CH CH Migraine Migraine
DurationDuration 15-180 mins15-180 mins 4-72hrs 4-72hrs
FrequencyFrequency 1 / day1 / day 1-2 / month 1-2 / month
0
10
20
30
40
50
60
70
80
90
100
Nause
a
Vomiti
ng
Photop
hobia
Phonop
hobia
Aura
Cluster HeadacheBahra, 2002,Torelli, 2001,Ekbom, 1970,Kudrow, 1980MigraineRasmussen, 1991
Additional Features%
CH
Migraine
IHS Classification Criteria for Cluster HeadacheIHS Classification Criteria for Cluster Headache
Episodic Cluster Headache → 90%
7 days - One year
Pain-free interval ≥ one month
Chronic Cluster Headache → 10%
≥ one year without remission
≥ one year with remissions one month
0
5
10
15
20
25
30
35
40
45
50
> 2years
Every 2years
Every18
months
1/year 2/year 3/year 4/year > 4/year
Bout Frequency
% o
f To
tal
50
55
60
65
70
75
80
85
90
95
100
F M A M J J A S O N D J F
• 1mg s/l nitroglycerine provocation
• During (n=28) and out (n=15) of active bout
• Attack precipitated in ALL during active bout
• No attack precipitated out of the bout
Ekbom, K. Arch Neurol 1968; 19: 487
Cluster Headache Attack ProvocationCluster Headache Attack Provocation
Cluster
Headache
Paroxysmal
hemicrania
SUNCT
M:F 3:1 1:1 1.5:1
Episodic:Chronic 90:10 35:65 10:90
Attack Duration 15-180 mins 2-30 mins 5-250s
Attack Frequency 1-8/day > 5 /day 1/day to 30/hr
Restlessness 90% 80% 65%
Periodicity Present Rare Absent
Alcohol Trigger ++ + –
• All patients ?
Cluster Headache & ImagingCluster Headache & Imaging
• Aneurysm of the ACA
• Pituitary tumour
• AVM of the occipital lobe
• Vertebral artery aneurysm
• Meningioma of the cervical canal (C2)
Symptomatic Cluster HeadacheSymptomatic Cluster Headache
• Aneurysm of the ACA
• Pituitary tumour
• AVM of the occipital lobe
• Vertebral artery aneurysm
• Meningioma of the cervical canal (C2)
Symptomatic Cluster HeadacheSymptomatic Cluster Headache
• Typical or atypical
• Response to treatment ≡ Primary CH
• ± Resolution with Rx of precipitating
pathology
Symptomatic Cluster HeadacheSymptomatic Cluster Headache
• Phenotype
• Rx Response
Symptomatic Cluster HeadacheSymptomatic Cluster Headache
Cannot differentiate b/uPrimary & Secondary CH
Locker at al. Headache. 2006 ( n = 558) / Ramirez-Lassepas. Arch Neurol. 1997
Predictors of Secondary Headache Predictors of Secondary Headache
Any one → Sensitivity 98.6%
Likelihood Ratio
Age > 50 years*Age > 50 years* 2.34
Sudden Onset*Sudden Onset* 1.74
Abnormal neurological examination*Abnormal neurological examination* 3.56
Additional Features Additional Features 2.27
• All patients ? → Need Data
• New onset
• Primary CCH
• Atypical history
• + Systemic / neurological features
Cluster Headache & ImagingCluster Headache & Imaging
TherapeuticsTherapeutics
The Sumatriptan Cluster Headache Study Group -1991
Success: 74% Sumatriptan 26% Placebo
Modest further benefit from 12mg 2 & 3mg are effective
No prophylactic benefit
Long term - Well tolerated.
No tachyphylaxis / MOH
ABORTIVE THERAPY : SUMATRIPTAN S/CABORTIVE THERAPY : SUMATRIPTAN S/C
Double-blind cross-over comparison of oxygen 100%
inhalation with air 12l / min for 15 minutes ( n=76) *
Success: 78% Oxygen 20% Placebo
Recommendation : 7 – 12 l/min for 15 minutes
CONCLUSION : Safest treatment but impractical
ABORTIVE THERAPY : OXYGENABORTIVE THERAPY : OXYGEN
Fogan, 1985. / Cohen, 2007*
Sumatriptan 20mg IN
Zolmitriptan 5 and 10mg IN
Zolmitriptan 5 and 10mg po
Lidocaine IN
ABORTIVE THERAPY ABORTIVE THERAPY
Sumatriptan 20mg IN
Zolmitriptan 5 and 10mg IN
Zolmitriptan 5 and 10mg po
Lidocaine IN
ABORTIVE THERAPY ABORTIVE THERAPY
Response at
30 minutes
Double-blind placebo controlled cross-over study
Lignocaine 10% Intranasal ( n = 9)
37 ± 7.8 mins Lignocaine } p < 0.01
59.3 ± 12.3 mins Placebo }
A/Es – Unpleasant taste. Adequate self- administration
4% Lignocaine solution 3-4 drops intranasally
ABORTIVE THERAPY : LIGNOCAINE INABORTIVE THERAPY : LIGNOCAINE IN
Costa et. Al (2000) Cephalalgia ; 20 : 85
PREVENTATIVE THERAPYPREVENTATIVE THERAPY
Multiple daily attacks → Prophylaxis
PREVENTATIVE THERAPY : VERAPAMILPREVENTATIVE THERAPY : VERAPAMIL
• 240-480mg daily
• Up to 1200mg daily
• Start at 80mg tds
• 40-80mg increments
every 10-14 days
• ECG monitoring every
two weeks
– Lethargy
– Constipation
– Pedal oedema
– Bradycardia
Leone et al. (2000) Neurology ; 54 : 1382
Verapamil in Cluster HeadacheVerapamil in Cluster Headache
Arrhythmias No Patients Mean VPM(mg) Dose ±SD
Patients on VPM 217 512 ± 279
ECGs 108 587 ± 264
1° Heart Block 13 578 ± 264
Other HB 9 604 ± 260
Total arrhythmias 21 567 ± 290
PR ≤ 0.2s 9 653 ± 275
Cohen, 2007
Bradycardia No Patients
Mean VPM(mg) Dose ±SD
Heart Rate
(mean)
Total 108 591 ± 264 66
Bradycardia 39 659 ± 253 53
Bradycardia → Stop VPM
4 495 ± 172 49
Arrhythmias in patients on VPM + Other Drug
Rhythm
Lithium 5(1) Junctional (after Lithium stopped)
Methysergide 1(1) 1° Heart Block (after Methysergide stopped)
Triptans 49(8) 1° HB (6), 2° HB (1), Junctional (1)
PREVENTATIVE THERAPY : METHYSERGIDEPREVENTATIVE THERAPY : METHYSERGIDE
• 3-6mg : Increase in one
week
• Then 1mg / week
• Up to 12mg daily
• BNF – 6 months then
drug holiday
– Nausea and vomiting
– Abdominal discomfort
– Vasoconstrictive effects
– Fibrosis
Weight gain
Muscle cramps
Mood changes
PREVENTATIVE THERAPY : LITHIUMPREVENTATIVE THERAPY : LITHIUM
• 300-1500mg
– Level at 4/7 after dose change
– Weekly until dose constant for 4 weeks
– Then 3 monthly
• 0.7-1.2 mmol/l
• Normal renal function and Na+ (Li toxicity)
Tremor
GI side effects
↓ thyroid, euthyroid goitre
Ataxia, nystagmus, dysarthria
Diabetes Insipidus
Drowsiness, confusion, seizures
Short-term use for multiple daily attacks
Attacks recur once the dose is decreased
• 40-80mg for 5 – 7 days
• Taper thereafter over 2 weeks
• Simultaneously introduce a suitable prophylactic
ABORTIVE THERAPYABORTIVE THERAPY : : CORTICOSTEROIDSCORTICOSTEROIDS
Jammes (1975) Dis. Nerv. Syst. ; 36 : 375
• Verapamil – 1200mg daily. ECG monitoring
• Methysergide – 12mg daily. Avoid > 6/12 use
• Lithium – 300-1500mg ( Level 0.7-1.2mmol/l)
• Steroids – 40-80mg. Max. 2 /52. Interim measure
Other considerations → Topiramate / Melatonin /
Sodium Valproate / Gabapentin / Ergotamine
Preventative TherapyPreventative Therapy
• Trigeminal ganglion and nerve
• Sphenopalatine ganglion
• Greater superficial petrosal nerve
• Nervus intermedius
• Greater Occipital Nerve
• Hypothalamus
SURGICAL THERAPYSURGICAL THERAPY
Greater Occipital Nerve Block
Greater Occipital Nerve BlockGreater Occipital Nerve Block
Patients (N) No. Injections Complete response (N)
Partial Response (N)
19 22 10 3
AUDIT. Afridi et al. Pain 2006
Patients N=23 Treatment Group Placebo
Complete response at 1/52 11 None
Complete response at 4/52 8 None
Ambrosini et al. Pain (2005)
Occipital Nerve Stimulation
Duration Duration (yrs)(yrs)
Mo since Mo since ONS ONS
Subjective Subjective outcomeoutcome
Estimated Estimated Change %Change %
Patient Patient RecommendRecommend
Triptan UseTriptan Use
1 7 23 Same - No Same
2 6 27 Same - Yes Same
3 6 11 Improved 95 Yes Less
4 17 17 Improved 60 Yes Less
5 13 27 Improved 20-80 Yes Same
6 24 8 Improved 90 Yes Less
7 13 11 Improved 25 Yes Same
8 54 32(23)* Improved 40 Yes Less
All 13 (6-54) ⌘ 20 (9-27) ⌘
75% Improved
60 (25-95) ⌘
88% Yes 38% Less Use
Burns, The Lancet (2007)
CLUSTER HEADACHE
PET VBM
May et al. (1998) Lancet ; 352 : 275 May et al. (1999) Nat. Med; 5:836
Leone et al. (2001) NEJM ; 345 : 1428
Cluster Headache - Stereotactic Stimulation of the Posterior Hypothalamic Gray Matter
May et al. (1998) Lancet ; 352 : 275
Sumatriptan 6mg sc
High flow oxygen
IN Sumatriptan / IN or Po
Zolmitriptan
IN Lidocaine
Verapamil
Methysergide
Lithium
Topiramate
Corticosteroids
Local V / Upper Cervical nerve block
Local V / Upper Cervical
neurostimulation
Central neurostimulation
Cluster Headache
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