SUNCT (Short-lasting Unilateral Neuralgiform headache attacks with Conujuctival injection and Tearing)
By Sylvia Lucas MD, Ph.D.
Director of the Headache Clinic, University of Washington Medical Center
“My husband has the devil eye” is how Jay’s wife described his beet-red eye during his severe episodes of pain. With no prior history of headache, at age 26 Jay had a sudden onset of short bursts of pain around his left eye and temple. These episodes increased in frequency and, within six months, had become constant.
The pain consists of sharp, stabbing episodes that last a few seconds, perhaps longer, and occur up to thirty times a day. After six months, Jay noticed a dull background of pain, which is now always present. During the stabbing episodes, his left eye becomes bright red and teary. He also has tinnitus (ringing in the ear), ear pain and vertigo (dizziness). His CT and MRI scans and neurologic exam were normal.
SUNCT (Short-lasting Unilateral Neuralgiform headache attacks with Conjunctival injection and Tearing) is a member of the trigeminal autonomic cephalalgias (TACs). This group of headaches is characterized by one-sided head pain associated with significant autonomic features (e.g., teary, red and/or droopy eye, sweating and nasal congestion). The TACs include cluster headache, paroxysmal hemicrania, SUNCT and SUNA, which is SUNCT without conjunctival injection (red eye).
The duration of attacks is slightly different with each TAC. Cluster headache episodes last from 15 minutes to three hours; paroxysmal hemicrania attacks last 2 to 30 minutes; SUNCT attacks range from 5-240 seconds. A key difference between paroxysmal hemicrania and SUNCT is that the latter does not respond to indomethacin and, indeed, Jay failed two separate courses of this drug.
The severe, stabbing SUNCT headaches may occur up to 30 times an hour; from 3-200 attacks per day. The tearing and red eye, which may be quite startling as it is in Jay’s case, must be present on the same side as the pain. Other symptoms may include eyelid droop, runny nose, nasal congestion, facial redness and sweating. Typically, nausea, vomiting, light and sound sensitivity, and aura are absent. SUNCT sufferers are predominantly male.
Just over half of SUNCT patients are pain-free between attacks, but a significant number feel a dull background ache or pressure when the stabbing ends. Though many SUNCT patients have triggers—for example, touching certain areas of the face (trigger zones), chewing, brushing teeth or swallowing—Jay did not.
SUNCT appears to be a life-long condition, though it can be episodic, with bouts of attacks that last less than one year separated by attack-free periods of more than one year. However, slightly over half of SUNCT patients have the chronic form with no remissions. Jay has never had a remission.
SUNCT is a rare syndrome. As a result, it is often underdiagnosed. Jay had seen three neurologists before a diagnosis was made. Because of its rarity, placebo-controlled double blind trials have not been done.
Successful treatment of SUNCT is very difficult. In contrast to other short-lasting headache forms, it is not responsive to indomethacin. The triptans are ineffective with SUNCT and attacks are too short and intense for other acute therapies to work. Intravenous lidocaine has been tried on a few patients, but relief is brief.
The key to successful management is preventive treatment, and the best response has been to the anti-epilepsy drug class. Many patients have a partial or complete response with lamotrigine. If this fails, then topiramate, gabapentin, or carbamazepine may be used. Patients who have responded to IV lidocaine have a higher likelihood of responding to the anti-epileptics.
Surgical approaches are also being tried, including local blockade of the greater occipital nerve, which has shown some effectiveness. Hypothalamic stimulation and surgical procedures to the trigeminal nerve or ganglion have been tried on too few patients to evaluate effectiveness.
Jay has tried numerous medications with limited success. Hydrocodone merely dulls the sharpness. The only preventive he responded to is topiramate, which significantly decreased the red eye and moderately decreased the severity of the pain. However, he has had significant cognitive side effects on this medication, which make it difficult to concentrate at work. By decreasing the topiramate and adding lamotrigine, his cognitive symptoms improved somewhat, but, unfortunately, the headache still persists.