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Last updated: 31 May 2024

Trigeminal autonomic cephalalgias

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Cluster HeadacheParoxysmal HemicraniaSUNCT syndromeTrigeminal autonomic cephalgias

Trigeminal autonomic cephalalgias (TACs) are a group of headache disorders characterized by repetitive, brief episodes of severe unilateral pain accompanied by ipsilateral autonomic symptoms such as rhinorrhea, nasal congestion, lacrimation, and conjunctival injection. The primary conditions within this group include cluster headache, paroxysmal hemicrania, and short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) syndrome. These conditions vary in episode duration, frequency, and rhythmicity. TACs are more prevalent in adults and are rarely observed in children.

Cluster Headache

Cluster headache is the most prevalent form of trigeminal autonomic cephalalgia, with an adult prevalence of less than 1%, showing a male predominance. In children and adolescents, the prevalence is estimated at 0.1%, with onset typically occurring after the age of five. The pathophysiology is not fully understood but is believed to involve hypothalamic activation and neurogenic inflammation. Symptoms include multiple bouts per day, often with circadian rhythmicity, lasting weeks. These severe unilateral orbital, supraorbital, or temporal headaches last between 15 minutes to 3 hours and are associated with unilateral autonomic symptoms and restlessness. Children may exhibit thrashing or emotional outbursts due to the intense pain. There are episodic and chronic forms, and a familial predisposition is noted. Diagnosis is clinical but should be confirmed with a head CT or MRI to exclude underlying brain lesions. Differential diagnosis includes other TACs and brain lesions. Treatment involves preventive medications such as verapamil and acute treatments like oxygen, triptans, or steroids. Patients should avoid triggers, including smoke and alcohol.

Paroxysmal Hemicrania

Paroxysmal hemicrania is rare in children. Episodes are shorter (minutes), more frequent, and less severe than cluster headaches. These headaches respond exceptionally well to indomethacin treatment.

SUNCT syndrome

Short-lasting Unilateral Neuralgiform Headache with Conjunctival Injection and Tearing (SUNCT) syndrome is also rare in children. Episodes are very brief (seconds to minutes), triggered by facial touch or chewing, with limited associated autonomic features, and can occur up to hundreds of times per day.

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