Purpose: Secondary trigeminal autonomic cephalalgias (TACs) are typically associated with posterior fossa abnormalities, such as tumors and vascular malformations. However, TACs following brainstem infarctions are rarely reported. This study aimed to characterize the clinical and anatomical features of TACs after unilateral dorsolateral medullary infarction.
Methods We analyzed four patients with dorsolateral medullary infarction who developed secondary TACs, diagnosed using the International Classification of Headache Disorders, third edition criteria. All patients underwent detailed neurological examinations and neuroimaging, including diffusion-weighted magnetic resonance imaging and magnetic resonance angiography. Additionally, five published cases were identified through a literature review and analyzed in conjunction with our cohort.
Results All patients exhibited stabbing or electric shock-like pain in the ipsilateral periorbital, hemifacial, and temporal regions. Headaches developed weeks to months post-stroke with brief attacks (1–2 minutes) occurring 1–5 times daily. Lacrimation and conjunctival injection were common. Three patients were diagnosed with short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT), while a fourth had short-lasting unilateral neuralgiform with cranial autonomic symptoms (SUNA). Each patient, as well as four of the five from the literature, exhibited ipsilateral facial sensory loss, suggesting involvement of the trigeminal spinal tract and nucleus. Delayed headache onset was more frequent in persistent cases.
Conclusion Headache characteristics were more consistent with SUNCT/SUNA than with typical cluster headaches. Careful neurological examination is essential to detect focal signs and guide neuroimaging for identifying secondary causes. Clinicians should consider secondary TACs in patients with new-onset SUNCT/SUNA and focal brainstem signs.
Chronic paroxysmal hemicrania, short-lasting unilateral neuralgiform headache attacks, and hemicrania continua are rare headache disorders characterized by severe unilateral headache accompanied by ipsilateral autonomic symptoms. Accurate diagnosis and specific treatment approaches for these conditions are crucial for appropriate management. This article covers the clinical features, diagnostic criteria, and treatment strategies of each disorder, aiming to differentiate them from other major headaches and provide targeted treatment strategies to improve patient outcomes.
Trigeminal Autonomic Cephalalgia (TAC) encompasses cluster headache, paroxysmal hemicrania, short-lasting unilateral neuralgiform headache attacks (SUNHA), and hemicrania continua. The treatment of cluster headache consists of acute therapy and preventive treatment. The available options for acute treatment in South Korea include high-flow O2 inhalation, zolmitriptan oral medication, and intranasal lidocaine spray. In the transitional phase of cluster headache, oral steroids and suboccipital steroid injections are commonly used. Verapamil and lithium have been widely used as preventive medications, but recently, galcanezumab, a monoclonal antibody targeting calcitonin gene-related peptide, has emerged as a recognized preventive treatment for cluster headache. In addition, neuromodulation techniques, such as noninvasive vagus nerve stimulation, sphenopalatine ganglion stimulation, occipital nerve stimulation, and deep brain stimulation, are also available for the treatment of cluster headache. Paroxysmal hemicrania and hemicrania continua are absolutely responsive to indomethacin; however, when indo methacin is contraindicated, alternative treatments are necessary. Reported alternatives include topiramate, vagus nerve stimulation, verapamil, and carbamazepine for paroxysmal hemicrania, and topiramate, gabapentin, and neurostimulation for hemicrania continua. Treatment options for SUNHA are limited, but short-term preventive approaches such as intravenous lidocaine, as well as the use of lamotrigine for preventive treatment, can be considered.