Headache: A Merck Manual of Patient Symptoms podcast
Cluster headaches cause excruciating, unilateral periorbital or temporal pain, with ipsilateral autonomic symptoms (ptosis, lacrimation, rhinorrhea, nasal congestion). Diagnosis is clinical. Acute treatment is with parenteral triptans, dihydroergotamine, or O2. Prevention is with verapamil, lithium, topiramate, divalproex, or a combination.
Cluster headache affects primarily men, typically beginning at age 20 to 40; prevalence in the US is 0.4%. Usually, cluster headache is episodic; for 1 to 3 mo, patients experience ≥ 1 attack/day, followed by remission for months to years. Some patients have cluster headaches without remission.
Pathophysiology is unknown, but the periodicity suggests hypothalamic dysfunction. Alcohol intake triggers cluster headache during the attack period but not during remission.
Symptoms and Signs
Symptoms are distinctive. Attacks usually occur at the same time each day, often awakening patients from sleep. Pain is always unilateral in an orbitotemporal distribution. It is excruciating, peaking within minutes; it usually subsides spontaneously within 30 min to 1 h. Patients are agitated, restlessly pacing the floor, unlike migraine patients who prefer to lie quietly in a darkened room.
Autonomic features, including nasal congestion, rhinorrhea, lacrimation, facial flushing, and Horner syndrome, are prominent and usually occur on the same side as the headache.
Diagnosis is based on the distinctive symptom pattern and exclusion of intracranial abnormalities.
Other unilateral primary headache syndromes with autonomic symptoms should be excluded:
Chronic paroxysmal hemicrania and hemicrania continua, unlike SUNCT and cluster headache (and migraine), respond dramatically to indomethacin, but not to other NSAIDs.
Acute attacks of cluster headache can be aborted with either a parenteral triptan or dihydroergotamine (see see Headache: Drugs for Migraine and Cluster Headaches*) and/or 100% O2 inhalation given by nonrebreathing face mask.
All patients require preventive drugs because cluster headache is frequent, severe, and incapacitating. Prednisone (eg, 60 mg po once/day) or a greater occipital nerve block (with a local anesthetic and a corticosteroid) can provide prompt temporary prevention while preventive drugs with slower onset of action (eg, verapamil, lithium, topiramate, divalproex) are initiated.
short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT)
Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) is a rare headache disorder characterized by extremely frequent attacks of unilateral head pain and autonomic activation.
SUNCT, like cluster headache, is a primary headache disorder characterized by unilateral pain in the trigeminal nerve distribution and by autonomic manifestations. As such, SUNCT and cluster headaches are sometimes grouped together as trigeminal autonomic cephalgias.
In SUNCT, pain paroxysms are typically periorbital, are extremely frequent (up to 200/day), and last from 5 to 250 sec. Conjunctival injection is often the most prominent autonomic feature; tearing may also be obvious.
Diagnosis is clinical. SUNCT should be distinguished from trigeminal neuralgia, which causes similar symptoms; SUNCT differs in that
Treatment can include IV lidocaine for acute attacks and, for prevention, anticonvulsants (eg, lamotrigine, topiramate, gabapentin) and occipital nerve stimulation or blockade.
Last full review/revision November 2012 by Stephen D. Silberstein, MD
Content last modified December 2012