Le tic douloureux de la pommette
G Soulagne 1804
La migraine et les algies de la face

mise à jour du
19 janvier 2006
1998; 38; 5; 369-371
 SUNCT Syndrome
Two Cases in Argentina
Elsa Raimondi, Lorenzo Gardella


Abstract : Two patients suffering from SUNCT syndrome are presented. Some features are remarkable. The first patient was a 69-year-old man whose first crisis was located in the right supraorbital region. After a 4-month spontaneous remission, the pain returned to the upper part of the cheek, radiating to the supraciliary region on the same side, with lacrimation and conjunctival injection. Rhinorrhea was absent. The painful attacks were triggered by head movements. Clinical improvement occurred with carbamazepine treatment. The second patient was a 48-year-old woman whose painful attacks lasted from 30 to 45 seconds followed by a burning sensation lasting 2 hours. Autonomic signs such as conjunctival injection, lacrimation, and edema and ipsilateral ptosis of the upper lid were rather marked. There was never any rhinorrhea. Her attacks were triggered by head and eye movements. She responded to the administration of corticosteroids and carbamazepine. According to these features, the two patients had SUNCT syndrome, and the positive carbamazepine response suggests a relationship with trigeminal neuralgia.

Since Sjaastad et al described it in 1978, this syndrome acquired greater attention when a detailed description and the term SUNCT appeared in 1989. The clinical manifestations consist of shortlasting crises of a neuralgic-type pain, felt unilaterally, and centered in the orbital/periorbital area; of moderate or severe intensity and accompanied by conjunctival injection, tearing, and rhinorrhea. Twenty-one cases have been reported to date. Since the beginning, reference has been made to the similarities of SUNCT and cluster headache, as both show autonomic signs, and also to first-division trigeminal neuralgia because of the neuralgic characteristics of this pain. However, Sjaastad and Kruszewski have made a particular emphasis on highlighting the differences so as to grant SUNCT its own identity. Worldwide experience of this syndrome is still limited, so new case descriptions are required to obtain better knowledge and understanding of it. For that reason, two patients diagnosed in Argentina are presented here.
Case 1.-A 69-year-old man who, in 1994, had neuralgic pain crises of 10 to 20 seconds duration and moderate intensity. Located in the medial right supraciliary area, such attacks were triggered by a gentle touch of the area and in some cases by lateral head movements. There was no pairs between paroxysms, and he never had a nighttime crisis. Analgesics did not provide significant relief. Towards the second half of that year, there was a spontaneous remission for 4 months. By February 1995, daily painful crises returned. They were episodes of neuralgiform pain similar to a 15- to 20-second electric current, and with the peculiarity of beginning in the upper part of the cheek and radiating to the supraciliary region on the same side. As it went by behind the eye, it caused conjunctival injection and profuse tearing. There was no rhinorrhea. Attacks recurred between three and five times a day, and were especially triggered by touching the right supraciliary and upper palpebral areas, and by mastication, laughter, yawning, and lateral head movements to the right. Neurological examination was always normal, as were a brain CT scan and magnetic resonance imaging (MRl). There were no remarkable antecedents. After unsuccessful treatment with several analgesics, in June of the same year, treatment with daily carbamazepine 600 mg was initiated. In a 2-month period, the interval between crises begun to increase and after 3 months of treatment, medication was gradually discontinued with only one or two attacks per week.
Case 2.-A 48-year-old woman consulted a physician in April of 1997 due to a painful condition in the right orbitofrontal area. She experienced between six and seven episodes daily. Each consisted of an abrupt and intense peak of neuralgic pain like a spark or electric shock of 30 to 45 seconds duration and followed by a painful burning feeling which persisted from 35 minutes to 2 hours.
The neuralgic pain began in the medial canthus of the right eye and then involved a triangular orbitofrontal area and, with less intensity, the upper half of her right cheek. During the height of the pain, there was ocular congestion, tearing, edema, and ipsilateral palpebral ptosia. Crises were spontaneous, and also triggered by touching the periorbital area, brushing her teeth, yawning, masticating, washing her hair, exposure to strong wind, lateral head movements to the right, and rapid eye movements. Most of the attacks occurred between 6 AM and 8 PM, but she also had some nocturnal attacks, apparently triggered by movements. After the painful episodes, she suffered from hyperesthesia in the affected area and the skin in the frontal region ipsilaterally looked rough and irregular like sandpaper.
Neurological examination, except for the above description, was always normal, as were a brain CT scan and MRI. lndomethacin, amitriptyline, ergotamine, and verapamil treatments were not helpful. The later use of prednisolone (60 mg daily for 5 days and then 20 mg daily for 10 days) and carbamazepine (800 mg per day for 11 weeks) brought benefit by reducing the painful crises to one or two a day and then providing complete relief. This cluster of painful attacks was preceded 2 years before by another episode which had a prolonged sawtoothlike temporal pattern. The previous clinical history was not otherwise remarkable.
The patients presented here show some clinical characteristics reminiscent of SUNCT syndrome.
Location of pain : In both cases, the most painful location was in the orbital area, even when in the first patient, the painful discharge started in the upper part of the cheek and radiated rapidly to the eye. In the second patient. there was a painful feeling in the upper half of the ipsilateral cheek. In our interpretation, the main localization of pain was still in the orbital periorbital region.
Intensity and duration of pain : In both cases, the intensity of the crises was between moderate and intense, hang an electric current characteristic. In patient 1, every episode lasted between 15 and 20 seconds and in patient 2, every paroxysm was initiated by an intense peak of pain of about 30 seconds, followed by a "plateau" of lesser intensity which could last between 35 minutes and 2 hours. Attacks with atypical prolonged features, as in this case, were described by Pareja and coworkers in 1996.
Frequency : In one case, there were between three and five exclusively daytime crises and in the other case there were also nocturnal crises, which were the first to disappear when the number of attacks decreased.
Triggering factors : Both patients had similar triggers located in either the first or in the second division of the trigeminal nerve. Additionally, painful attacks were triggered by head rotation, and in the second patient by rapid lateral eye movements. These features have been reported in SUNCT by Sjaastad et al.
Accompanying signs : The first patient had conjunctival injection and profuse tearing when the painful discharge was felt behind the eye. In the second patient, there was edema and palpebraI ptosis and the skin in the ipsilateral frontal region looked rough and irregular like "sandpaper." Neither patient had miosis or rhinorrhea. The prominent autonomic phenomena are consistent with SUNCT syndrome, in contradistinction to trigeminai neuralgia, in which such features are rarely seen.
Medication : Patient 1 had a partial remission with carbamazepine after 3 months of treatment. In patient 2, the crises were reduced to one or two a day after 16 days of combined treatment with carbamazepine and corticosteroids and 9 weeks of carbamazepine alone.
In both patients, the differential diagnoses included cluster headache, trigeminal neuralgia, chronic paroxysmal hemicrania, and Raeder syndrome. Organic pathologies were excluded by imaging studies. The neuralgic characteristics of the pain, location, duration, accompanying autonomic symptoms, and extratrlgeminal triggers, all make the condition described by the two patients similar to SUNCT syndrome. A remarkable characteristic is the fact that in both patients, the location of pain was not confined to the first trigeminal division, but, in part, also involved the second division. Also notable was the prolonged duration of each crisis in patient 2, as well as the skin changes in the frontal area.
We have to mention, especially, the response to treatment. No analgesic was beneficial. The only favorable response occurred with carbamazepine, alone in one case and given with corticosteroids in the other. We believe medication was actually responsible for improvement, since both patients had a long history of pain and with these drugs their crises disappeared or were greatly reduced over a 2- to 3-month period. We do not believe this was just a spontaneous remission. Even though it is mentioned that one of the characteristics of SUNCT is its partial or lack of response to carbamazepine, we, nevertheless, believe that our cases fitted properly into this syndrome and due to all the features described above, we believe it is closer to trigeminal neuralgia than cluster headache and the like.