Author:
and rew K.Chang
Kevin P.Collins
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 15-180 min (75% last <60 min)
- More common in men (∼3:1)
- Onset usually between 30-50 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
- 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
- Often evolves from episodic cluster headaches
- 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 and subsequent hypothalamic dysfunction
- Release of CGRP from trigeminal perivascular afferents causes vasodilation/modulation of nociceptive activity of trigeminal neurons leading to severe pain
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 syndrome)
- Sweating of face/forehead
Essential Workup
- An accurate history and physical exam should confirm the diagnosis
- Life-threatening alternatives should be ruled out
Diagnostic Tests & Interpretation
Imaging
CT scan/MRI if suspect hemorrhage, tumor, etc.
Differential Diagnosis
- Migraine headache
- Trigeminal neuralgia
- Meningitis
- Temporal arteritis
- Intracerebral mass lesion
- Herpes zoster
- Intracerebral bleed
- Dental causes
- Orbital/ocular disease (acute glaucoma)
- Temporal mand ibular joint syndrome
Prehospital
- 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 mcg/kg IV
- Lidocaine: 4-10% spray 0.2 mL intranasal ipsilateral nostril; sphenopalatine block may be beneficial in refractory cases
- Morphine: 2-4 mg IV/IM, may repeat q10min
- NSAIDs: Ketorolac 15-30 mg IM/IV
- Oxygen: 100% via face mask
- Prochlorperazine: 10 mg IM/IV
- Somatostatin: 100 mcg SQ
- Sumatriptan: 6 mg SC, may repeat in 1 hr (max of 2 doses in 24 hr); 20 mg intranasal spray; single dose in one nostril only
- Verapamil: Immediate release: Preventive drug of choice. Start at 80 mg t.i.d
- Zolmitriptan: 5/10 mg intranasal spray, single dose in one nostril only
First Line
- Oxygen: 12 L/min via nonrebreather mask for 15 min:
- May increase to 15 L/min if refractory headache
- Sumatriptan/Zolmitriptan
- DHE
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/intranasal sumatriptan/zolmitriptan for management at home
Issues for Referral
Follow-up with a neurologist should be arranged
- CohenAS, BurnsB, GoadsbyPJ. High-flow oxygen for treatment of cluster headache: A rand omized trial . JAMA. 2009;302(22):2451-2457.
- FriedmanBW, GrosbergBM. Diagnosis and management of the primary headache disorders in the emergency department setting . Emerg Med Clin North Am. 2009;27(1):71-87.
- HoffmannJ, MayA. Diagnosis, pathophysiology, and management of cluster headache . Lancet Neurol. 2018;17(1):75-83.
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