Cluster Headache

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ANYONE WHO SUFFERS FROM CLUSTER HEADACHES CAN RELATE TO THIS DRAWING; JD Fletcher

Introduction
Most severe of primary headache syndromes Most severe pain syndromes afflict humans Nickname; suicide headache Belongs to a group of TACs

Trigeminal autonomic cephalalgias

Idiopathic headache Short-lasting Severe headache Accompany autonomic symptoms Cluster is the most common

Pathophysiology
Hypothalamic activation with secondary activation of the trigeminal autonomic reflex The intense pain is caused by the dilation of blood vessels which creates pressure on the trigeminal nerve Another theory: Neurogenic inflammation of the walls of the cavernous sinus obliterates venous outflow

Epidemiology
< 1%; Mostly affects men 124 per 100,000 Male : Female = 4.3:1 Male preponderance in cluster headache is decreasing

Genetics
1st degree relatives by 14-39x 2nd degree relatives by 2-8x Supports the hypothesis of genetic disease, at least in some families

Smoking
85% of pts w/ cluster are smokers But quitting smoking has no effect However, smoking may be a risk factor

Trigger
Not associated with triggers, such as foods, hormonal changes or stress But once a cluster period begins, consumption of any alcohol can quickly trigger For this reason, many people with cluster headache avoid alcohol for the duration of a cluster period Other possible triggers include the use of medications such as nitroglycerin

Clinical features
Orbital, supraorbital, temporal pain Accompanied by autonomic phenomena up to 8 times/day Unilateral Remain on the same side during attack Side shift in 15% In constrast to migrain, pts are restless

Autonomic symptoms
Ptosis, Miosis, Lacrimation, Conjunctival injection, Rhinorrhea, Horners syndrome Occur only during the pain attack and are ipsilateral to the pain Parasympathetic overactivity and sympathetic impairment In some, miosis and ptosis persist indefinitely, but intensify during attacks Sweating also increase on the painful side 3% lack autonomic symptoms

Circadian periodicity
Circadian rhythmicity is characteristic short-lived (15-180 min) painful attacks Episodic form is most common (80-90%), attacks occur daily for some weeks followed by a period of remission Chronic form attacks occur w/o significant periods of remission On average, cluster period lasts 6-12 wks while remissions can last up to 12 mo or longer

Diagnosis
Neuroimaging is suggested to exclude a cranial lesion in pts with suspected cluster headache ICHD-2 criteria (international classification of headache disorders)

ICHD-2 criteria

Neuroimaging
CT or MRI for exclude 2nd headache

Laboratory investigations
EEG, CSF not helpful

Differential diagnosis
Paroxysmal hemicrania Short lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT syndrome) Trigeminal neuralgia Primary stabbing headache Secondary cluster headache

Paroxysmal hemicrania
Female predominance; 3-6% of all TACs; 20-40 yrs Similar to cluster But shorter 2-30 min and more frequent (>5 attacks/day) Autonomic symptoms often less severe 80% chronic form 20% episodic form Important criteria is COMPLETE RESPONSE TO INDOMETHACIN

SUNCT syndrome
Short lasting Unilateral Neuralgiform headache attacks with Conjunctival injection and Tearing May be a subset of SUNA

Management
1. Patient education 2. Lifestyle changes
eg. Avoiding alcohol during active cluster period

3. Acute therapy 4. Preventive treatment

Acute therapy
1st Line Rx
Subcutaneous sumatriptan oxygen inhalation Repeated or frequent oxygen intake in a short period of time should be avoided, because there is evidence that the attack frequency may increase in some patients with overuse of oxygen

Side effect of Triptans

Nonischemic chest pain Distal paresthesia Be avoided in patients with
IHD Stroke Prinzmetal's angina uncontrolled HT pregnancy

Preventive treatment
Start as soon as possible Verapamil is the agent of choice May be effective include glucocorticoids, lithium, Topiramate, and methysergide

Verapamil
Usually started 240 mg daily Increasing TDD by 80 mg q 10-14 days EKG should be obtained after each dose increment above TDD of 480 mg

Glucocorticoid
in several open-label studies and case series, approximately 70 to 80 percent of patients with cluster headache responded to glucocorticoid therapy 60 to 100 mg once a day x 5 days, and then tapering by decreasing 10 mg q day

Topiramate
starting 25 mg/day titrated 25 mg weekly S/E
cognitive disturbances paresthesia weight loss

Contraindicated in nephrolithiasis

DBS
Remain investigation

Reference
UpToDate 19.1; Cluster headache: Epidemiology, clinical features, and diagnosis Author Arne May, MD UpToDate 19.1; Cluster headache: Acute and preventive treatment Author Arne May, MD Review Article; Cluster HeadacheAcute and Prophylactic Therapy head Avi Ashkenazi, MD; Todd Schwedt, MD

http://en.wikipedia.org/wiki/Cluster_headache