Cluster Headache and Other Trigeminal Autonomic Cephalalgias

Abstract

PURPOSE OF REVIEWThe trigeminal autonomic cephalalgias (TACs) are relatively rare, but they represent a distinct set of syndromes that are important to recognize. Despite their unique features, TACs often go undiagnosed or misdiagnosed for several years, leading to unnecessary pain and suffering. A significant proportion of TAC presentations may have secondary causes.RECENT FINDINGSThe underlying pathophysiology of TACs is likely rooted in hypothalamic dysfunction and derangements in the interplay of circuitry involving trigeminovascular, trigeminocervical, trigeminoautonomic, circadian, and nociceptive systems. Recent therapeutic advancements include a better understanding of how to use older therapies more effectively and the identification of new approaches.SUMMARYTAC syndromes are rare but important to recognize because of their debilitating nature and greater likelihood for having potentially serious underlying causes. Although treatment options have remained somewhat limited, scientific inquiry is continually advancing our understanding of these syndromes and how best to manage them.

Refractory Short-Lasting Unilateral Neuralgiform Headache Attacks With Conjunctival Injection and Tearing (SUNCT) Responding to Erenumab Adjuvant Therapy: A Case Report

“…SUNCT can also be confused with trigeminal neuralgia as they share a similar duration, location, and cutaneous triggers over the area of pain. However, unlike trigeminal neuralgia, SUNCT has strong ipsilateral cranial autonomic features and no refractory period after cutaneous triggering [ 2 ]. For secondary headaches, several systematic reviews of case series have identified causes of SUNCT-like headaches including pituitary tumors (the most common cause), other neoplasms (carcinoid, epidermoid, meningioma, pilocytic astrocytoma, lung adenocarcinoma), vascular (aneurysm, cavernous sinus dural fistula, compression of the trigeminal nerve by the superior or anterior inferior cerebellar artery), inflammatory/infectious (multiple sclerosis, viral meningitis), or an orbital cyst [ 7 , 10 ].…”

“…The TACs consist of five headache disorders: cluster headache, paroxysmal hemicrania, short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT), short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA), and hemicrania continua. These conditions differ in their attack duration, attack frequency, and treatments [ 2 ]. SUNCT, the disease discussed in this case, is officially characterized by 1) at least 20 attacks, 2) moderate or severe pain in the temple and/or one or more distributions of the trigeminal nerve lasting between 1 second and 10 minutes, 3) ipsilateral autonomic features which must include both conjunctival injection and lacrimation, 4) at least one attack most days unless in remission, and 5) not better accounted for by another diagnosis [ 1 ].…”

“…Based on open-label studies and expert opinion, the first-line treatment is lamotrigine [ 4 ]. Intravenous lidocaine may be the most effective treatment [ 2 , 5 ], however, administration requires experienced providers and specialized monitoring given IV lidocaine’s risk of cardiac arrhythmias. Other treatments include carbamazepine, oxcarbazepine, topiramate, gabapentin, pregabalin, duloxetine, and mexiletine [ 2 , 6 ], and case reports have suggested the effectiveness of microvascular decompression [ 7 ].…”

Central Nervous System Involvement of Multiple Myeloma Presenting as Short-lasting Unilateral Neuralgiform Headache with Conjunctival Injection and Tearing: A Case Report

“…Patients may experience a single attack, recurrent attacks with pain-free interictal periods, or a sawtooth pattern background pain with superimposed stabs. 1,2 13 (1) Conjunctival injection and/or lacrimation, (2) Nasal congestion and/or rhinorrhea, (3) Eyelid edema, (4) Forehead and facial sweating, (5) Forehead and facial flushing, (6) Sensation of fullness in the ear, (7) Miosis and/or ptosis both intra and extra-axial (vascular lesion, tumor, or bony abnormalities). Multiple Myeloma (MM) with central nervous system involvement (CNS MM) most commonly presents with visual changes (36%), radiculopathy (27%), headache (25%), confusion (21%), dizziness (7%), and seizures (6%).…”

“…Adapted from 13 (1) Conjunctival injection and/or lacrimation, (2) Nasal congestion and/or rhinorrhea, (3) Eyelid edema, (4) Forehead and facial sweating, (5) Forehead and facial flushing, (6) Sensation of fullness in the ear, (7) Miosis and/or ptosis…”