DESCRIPTION 
- Excruciatingly painful primary headache disorder
- Infrequent cause of ED visits and affects only 0.1% of the population
- Often has abated by time of presentation
- Attacks last between 15 and 180 min (75% last < 60 min)
- More common in men (~3:1)
- Onset usually between 30 and 50 yr of age
- Headaches occur in clusters lasting weeks to months followed by remission > 1 mo
- Commonly occur 1–3 times per day during cluster period that lasts 2–3 mo
- Often occur during the same time of day
- Often occur during the same time of the year
- Highest incidence in spring and fall
- Chronic cluster headache:
- Remission < 1 mo
- Do not experience remission
- 10% of patients
- May have many clinical and pathophysiologic similarities with migraine and variants
- Often follows a trigeminal nerve dermatome
ETIOLOGY
A well-described physiologic reflex arc, the trigeminovascular reflex, potentiates the trigeminal pain and cranial autonomic features of cluster headache by positive feedback mechanisms.
[Outline]
SIGNS AND SYMPTOMS
History
- Unilateral pain (usually does not change sides between headaches)
- Sharp, stabbing, boring
- Acute onset and builds in intensity quickly with climax at 5–15 min
- Pain stops abruptly
- Often exhausted after episode
- Location:
- Radiation to:
- Ear
- Cheek
- Jaw
- Teeth (often have had extensive dental workup for pain in the past
- Nose
- Ipsilateral neck
- Episodes are often nocturnal
- Attacks are more likely after ingestion of alcohol, nitroglycerine, or histamine-containing compounds
- More likely in times of stress, prolonged strain, overwork, and upsetting emotional experiences
- No prodrome or aura
Physical Exam
- Agitated, restless
- Prefer to stand and move around as opposed to migraine patients who prefer to lie quietly in a dark room
- Accompanying autonomic symptoms:
- Ipsilateral to headache
- Nasal congestion or rhinorrhea (or both)
- Conjunctival injection or lacrimation (or both)
- Facial flushing
- Eyelid edema
- Ptosis, miosis, or both (partial Horner's syndrome)
- Sweating of face/forehead
ESSENTIAL WORKUP
- An accurate history and physical exam should confirm the diagnosis
- Life-threatening alternatives should be ruled out
DIAGNOSIS TESTS & INTERPRETATION
Lab
Imaging
CT scan/MRI if suspect hemorrhage, tumor, etc.
DIFFERENTIAL DIAGNOSIS
[Outline]
PRE-HOSPITAL
- Recognize more severe life-threatening causes of headache
- Administration of oxygen by face mask may alleviate symptoms
INITIAL STABILIZATION/THERAPY
- Rule out life-threatening causes of headache
- Administration of supplemental oxygen
MEDICATION
- Ergots: DHE 0.5–1 mg IV; repeat in 1 hr if necessary
- Fentanyl: 2–3 µg/kg IV
- Morphine: 2–4 mg IV or IM, may repeat q10min
- NSAIDs: Ketorolac 15–30 mg IM or IV
- Oxygen: 100% via face mask
- Prochlorperazine: 10 mg IM or IV
- Somatostatin: 100 µg SQ
- Sumatriptan: 6 mg SC, may repeat in 1 hr (max. of 2 doses in 24 hr)
- Verapamil immediate release: Preventive drug of choice. Start at 80mg TID
First Line
- Oxygen: 12 L/min via nonrebreather mask for 15 min:
- May increase to 15 L/min if refractory headache
- Sumatriptan
- DHE
Second Line
[Outline]
DISPOSITION
Admission Criteria
- Persistent headache unresponsive to usual measures
- Unclear headache diagnosis
Discharge Criteria
- Patients with moderate to complete pain relief, a normal neurologic exam, and with a confident diagnosis of cluster headache
- Consider prescribing oxygen and/or SC sumatriptan for management at home
Issues for Referral
Follow-up with a neurologist should be arranged
- Cohen AS, Burns B, Goadsby PJ. High-flow oxygen for treatment of cluster headache: A randomized trial. JAMA. 2009;302:2451–2457.
- Friedman BW, Grosberg BM. Diagnosis and management of the primary headache disorders in the emergency department setting. Emerg Med Clin North Am. 2009;27:71–87.
- McGeeney BE. Cluster Headache Pharmacotherapy. Am J Ther. 2005;12:351–358.
- Nesbitt AD, Goadsby PJ. Cluster headache. BJM. 2012;344:e2407.
See Also (Topic, Algorithm, Electronic Media Element)