Some individuals suffer more than one of these types of attacks, and there is heterogeneity among patients in the duration of the pain and the number of episodes per day. SUNCT and SUNA attacks can be episodic with spontaneous remissions lasting weeks or longer, or these can be chronic with long symptomatic periods without spontaneous remission. Although attacks are often spontaneous, a wide array of attack triggers occur, including washing or brushing hair, shaving, touching the face or scalp, chewing or eating, brushing teeth, talking, shaving, bathing or showering, coughing, blowing the nose, exercise, and exposure to light.
Prophylactic medication is the mainstay of treatment. Lamotrigine, topiramate, and gabapentin are probably the most helpful, although a variety of other agents are useful in a few patients. Rapid treatment with lidocaine may be helpful with severe acute episodes of pain. Occipital nerve blockade with a local anesthetic and a corticosteroid are helpful in some individuals.
PAROXYSMAL HEMICRANIA
Paroxysmal hemicrania (PH) is a rare trigeminal autonomic cephalgia with an estimated prevalence of 1 in 25,000. It is distinguished by unilateral, short-lived attacks of intense pain associated with cranial autonomic features that repeat many times daily, with an average of approximately 10 to 12 per day. This is most commonly localized to the trigeminal nerve’s first division and usually lasts 15 to 20 minutes. Usually the pain is described as “torturous” and is often characterized as boring, burning, sharp, stabbing, throbbing, or shooting. As with CH, there may be one or more migrainous-associated features, including photophobia, phonophobia, and nausea or vomiting. Also similar to CH, PH is frequently associated with restlessness or agitation. Approximately 20% of patients have episodic PH, diagnosed when remissions last 1 month or longer; the remaining patients have chronic paroxysmal hemicrania, in which a remission does not occur within 1 year.
Although the preponderance of PH attacks occur spontaneously, approximately 10% may be triggered mechanically, typically by flexing or by rotating the head. Attacks are sometimes elicited by external pressure over the greater occipital nerve, C2 root, or the transverse processes of C4-5. Alcohol ingestion provokes attacks in approximately 20% of patients.
An absolute unequivocal response to a therapeutic dose of indomethacin is the primary diagnostic criterion for PH. This remains the gold standard for PH treatment. Both cyclo-oxygenase-II (COX-II) selective inhibitors, for instance, celecoxib, and topiramate are effective in some patients. Greater occipital nerve block with local anesthetic and a corticosteroid are beneficial in some patients. Finally, there may be a role for neuromodulation, such as occipital nerve stimulation in some patients.
HEMICRANIA CONTINUA
Hemicrania continua (HC) is a relatively uncommon, yet likely underdiagnosed form of chronic daily headache. It is typified by a continuous, one-sided headache that changes in severity, waxing and waning, yet not resolving entirely. Episodes of worsening are typically associated with ipsilateral cranial autonomic symptoms (conjunctival injection, lacrimation, nasal rhinorrhea) but milder in severity than that seen with the trigeminal autonomic cephalalgias (TACs). The exacerbations may also be accompanied by migrainous symptoms, such as nausea, photophobia, and phonophobia. Because of the overlapping features, HC should be considered when evaluating a patient for chronic cluster headache or chronic migraine (especially if the headache is always on the same side). Similar to the TACs, it is important to rule out secondary causes of HC, such as pituitary, pineal, or posterior fossa lesions, internal carotid artery dissection, and unruptured aneurysms.
A diagnosis of this disorder also requires an absolute and marked response to indomethacin. HC sometimes responds well to other nonsteroidal anti-inflammatory drugs (NSAIDs), including COX-II inhibitors. Some patients are reported to have a favorable result with topiramate and occipital nerve blocks. There may also be a role for neuromodulation, such as occipital nerve stimulation in some patients.
Related posts:
Stay updated, free articles. Join our Telegram channel
Full access? Get Clinical Tree
