Trigeminal Autonomic Cephalalgias (TACs): Cluster Headache, Paroxysmal Hemicranias, SUNCT, SUNA
G44 Other headache syndromes
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144
Paul Rizzoli
ICD-10 G44 Other headache syndromes G44.0 Cluster and other TACs G44.00 Cluster, unspecified G44.01 Episodic cluster G44.02 Chronic cluster G44.03 Episodic paroxysmal hemicrania G44.04 Chronic paroxysmal hemicrania G44.05 Short lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) G44.09 Other TACs The trigeminal autonomic cephalalgias, including cluster headache, paroxysmal hemicrania, short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT), and short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA), are grouped together as primary headache disorders charac terized by unilateral trigeminal distribution pain in association with ipsilateral cranial autonomic features. They differ in their attack frequency and duration (Table 144.1).
P. Rizzoli, MD (*) Department of Neurology, Brigham and Women’s/ Faulkner Hospital, 1153 Centre Street, Suite 4H, Boston, MA 02130, USA e-mail: [email protected]
Cluster Headache This disorder in its episodic form is very distinctive; an episodic headache disorder more frequently appearing in men and characterized by attacks of daily severe unilateral orbital pain with ipsilateral autonomic features, episodes of which occur in clusters of up to 1–2 months duration, typically recurring annually at roughly the same time of the year. During a headache, the patient will usually become agitated and pace in distinction to the sensory-avoiding behavior of the migraine patient. Examination may disclose an ipsilateral Horner’s syndrome. Functional imaging studies localize the disorder to the posterior hypothalamic region. An episode of recurrent cluster attacks lasting more than one year may be considered chronic cluster.
Diagnosis and Management Cluster is a rare form of headache with a prevalence under 1 % of the US population. Multiple structural and vascular lesions may mimic cluster and imaging is warranted for a new or changed pattern of headache. Individual attacks may respond to injection of subcutaneous sumatriptan or inhalation of high flow oxygen administered by a non-rebreather mask. Initial and empiric treatment at the onset of a cluster period may include a course of PO corticosteroids and
© Springer International Publishing Switzerland 2017 R.J. Yong et al. (eds.), Pain Medicine, DOI 10.1007/978-3-319-43133-8_144
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P. Rizzoli
538 Table 144.1 Comparison of trigeminal autonomic cephalalgias Name Cluster
Location Unilateral orbital
Duration Attack frequency/day Associated features 15–180 min 1–8 Lacrimation, conjunctival injection, rhinorrhea 2–40 Tearing, conjunctival Paroxysmal V-1, ophthalmic 2–30 min Hemicrania division injection, rhinorrhea SUNCT Unilateral orbital 15 s to 4 min 3–200 Conjunct inject AND to temporal region lacrimation SUNA Unilateral orbital 15 s to 4 min 3–200 Conjunct inject OR to temporal region lacrimation + rhinorrhea/ nasal congestion
anesthetic blockade of the ipsilateral occipital nerve. Verapamil
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