Cluster Headache

Symptoms of cluster headache are distinctive. Attacks, often multiple, frequently occur at the same time each day, often awakening patients from sleep.

When attacks occur, pain is always unilateral and occurs on the same side of the head in an orbitotemporal distribution. It is excruciating, peaking within minutes; it usually subsides spontaneously within 30 minutes to 1 hour. Patients are agitated, restlessly pacing the floor, unlike patients with migraine who prefer to lie quietly in a darkened room. The restlessness can be so severe that it leads to bizarre behavior (eg, banging the head on a wall).

Autonomic features, including nasal congestion, rhinorrhea, lacrimation, facial flushing, and Horner syndrome Horner Syndrome Horner syndrome is ptosis, miosis, and anhidrosis due to dysfunction of cervical sympathetic output. (See also Overview of the Autonomic Nervous System.) Horner syndrome results when the cervical... read more , are prominent and occur on the same side as the headache.

Diagnosis of cluster headache is based on the distinctive symptom pattern and exclusion of intracranial abnormalities.

Other unilateral primary headache syndromes with autonomic symptoms, which are sometimes grouped together with cluster headache as trigeminal autonomic cephalgias, should be excluded:

Chronic paroxysmal hemicrania and hemicrania continua, unlike SUNCT and cluster headache (and migraine Migraine Migraine is an episodic primary headache disorder. Symptoms typically last 4 to 72 hours and may be severe. Pain is often unilateral, throbbing, worse with exertion, and accompanied by symptoms... read more ), respond dramatically to indomethacin, but not to other nonsteroidal anti-inflammatory drugs (NSAIDs).

Acute attacks of cluster headache can be aborted with either a parenteral triptan or dihydroergotamine, zolmitriptan taken by nasal spray, and/or 100% oxygen given by nonrebreathing face mask. Also, evidence suggests that acute attacks of episodic cluster headache can be treated with a handheld device that delivers noninvasive vagus nerve stimulation Vagus nerve stimulation A seizure is an abnormal, unregulated electrical discharge that occurs within the brain’s cortical gray matter and transiently interrupts normal brain function. A seizure typically causes altered... read more (1 Treatment references Cluster headaches cause excruciating, unilateral periorbital or temporal pain, with ipsilateral autonomic symptoms (ptosis, lacrimation, rhinorrhea, nasal congestion). Diagnosis is clinical... read more , 2 Treatment references Cluster headaches cause excruciating, unilateral periorbital or temporal pain, with ipsilateral autonomic symptoms (ptosis, lacrimation, rhinorrhea, nasal congestion). Diagnosis is clinical... read more ).

All patients with cluster headache require preventive drugs because cluster headache is frequent, severe, and incapacitating. Prednisone (eg, 60 mg orally once a 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. Galcanezumab (a monoclonal antibody that binds the calcitonin gene-related peptide [CGRP] ligand) is usually used only when other treatments for episodic cluster headaches are ineffective.

Noninvasive transcutaneous supraorbital nerve stimulation delivered using a device applied to the forehead can reduce the frequency of cluster headaches, as can noninvasive vagus nerve stimulation (3 Treatment references Cluster headaches cause excruciating, unilateral periorbital or temporal pain, with ipsilateral autonomic symptoms (ptosis, lacrimation, rhinorrhea, nasal congestion). Diagnosis is clinical... read more ).