SUNCT syndrome, or "transient unilateral neuropathic pain with conjunctival injection and tearing", is a rare headache disorder that belongs to the category of trigeminal autonomic headache. This type of headache is often described as a stabbing or burning sensation, located primarily around the eyes, and usually affects only one side of the body. These episodes of intense pain are brief, lasting anywhere from five seconds to six minutes, and may occur up to 200 times a day.
"The pain level of each attack can seriously affect daily life, but most patients do not need hospitalization."
People affected by SUNCT syndrome often describe headache attacks as severe pain, with the average number of such attacks being about 60 per day, ranging from 3 to 200. Based on the duration of the attack, these attacks can be divided into single stings, groups of stings, and zigzag attack patterns, ranging from a shortest of five seconds to a long of 240 seconds. The nature of the pain is usually tingling, pulsating or electric, and the common sites of occurrence include the eye sockets, eyelids and temples.
SUNCT is often associated with autonomic symptoms, including tearing, ipsilateral eyelid ptosis, eyelid swelling, nasal obstruction, and conjunctival congestion. The appearance of these autonomic nervous system symptoms may vary depending on the different branches of the trigeminal nerve. Rarely, such attacks may constitute a subtype called transient unilateral neuralgic headache with autonomic features (SUNA), in which autonomic symptoms are less prominent than in SUNCT.
SUNCT syndrome occurs spontaneously most of the time, but can sometimes be triggered by everyday stimuli such as touching the face, eating, talking, coughing, and bathing. Additionally, light and certain environmental stimuli may trigger attacks. The onset of this condition may vary with the seasons and is related to the function of the hypothalamus.
“Knowing what triggers may be can help patients better manage their condition and avoid common irritants.”
The key to diagnosing SUNCT syndrome is to exclude other similar headaches, such as cluster headaches. According to the criteria of the International Headache Society, the diagnosis of SUNCT is mainly assessed based on the location, frequency and duration of pain. Many patients are misdiagnosed with other types of headaches because of the difficulty in identification, and proper diagnosis can help with more effective treatment.
PathophysiologyThe pathophysiology of SUNCT is not fully understood, but some studies suggest a possible link between the hypothalamus and the condition. Functional magnetic resonance imaging showed activation in the patients' posterior hypothalamus during seizures. In addition, activation of the trigeminal nerve and associated structures of the upper cervical spinal cord also appears to occur during the attack. This knowledge is still under further exploration and the boundaries have not been clearly determined.
TreatmentSince the therapeutic effect of SUNCT has not yet been confirmed and its cause is still unclear, patients usually use anti-epileptic drugs such as lamotrigine to improve symptoms. Certain types of treatment, such as intravenous lidocaine, may be effective in reducing the frequency and duration of attacks. However, treatment may need to be adjusted depending on individual patient responses.
"Different treatments may have different effects on different patients, so individualized treatment plans should be the core of treatment."
The first SUNCT patient was diagnosed in 1977, in a 62-year-old man who developed the condition after being struck by a fishing rod. As the understanding of SUNCT continues to deepen and the number of cases gradually increases, more and more patients and medical workers are beginning to understand the characteristics and management of this disease.
Although our understanding of SUNCT syndrome is still developing, early knowledge and subsequent research are reminding us how we should strive to find more effective treatments for such a rare disease.