Cluster and Facial Headache

Abstract

Trigeminal autonomic cephalalgias (TACs) are primary headache disorders characterized by unilateral head pain of varying duration associated with ipsilateral cranial autonomic features. TACs differ primarily in episode duration, frequency, and periodicity. Cluster headache and short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) are more common among men, while chronic paroxysmal hemicrania (CPH) and hemicrania continua (HC) among women. The duration of headache attacks (15–180 min) helps differentiate cluster headache from the other TACs and also from migraine headache (4–72 h). SUNCT is best differentiated from trigeminal neuralgia (TN) by location of pain (V1 versus V2–V3 for TN) and the prominent ipsilateral autonomic features (rare in TN). Cerebral blood flow studies reveal hyperactivation of the hypothalamus ipsilateral to the pain in cluster headache, contralateral to the pain in CPH and HC, and bilateral in SUNCT. Oxygen therapy and subcutaneous sumatriptan are the agents of choice for the treatment of acute attacks of cluster headache. Prevention of cluster headache is most frequently accomplished with short-term corticosteroids and long-term verapamil. Electrocardiographic monitoring is necessary for those patients with cluster headache requiring verapamil doses greater than 240 mg. Paroxysmal hemicrania and hemicrania continua are prevented absolutely by therapeutic doses of indomethacin. Co-administration of a proton pump inhibitor may improve the tolerability of long-term indomethacin dosing. SUNCT is the most brief, least common, and most refractory TAC. Lamotrigine is the treatment of choice.