Statuslike SUNCT in Two Young Women


Address all correspondence to Dr. Enrique Montes, Servicio de Neurología, HRT Hospital Universitario Virgen del Rocío, Avda Manuel Siurot s/n, 41013 Sevilla, Spain.


Objective.—To describe the statuslike pattern of SUNCT (short-lasting unilateral neuralgiform pain with conjunctival injection and tearing) in two young women.

Background.—SUNCT syndrome is a rare condition characterized by a short-lasting periocular pain associated with marked autonomic symptoms. Twenty-five cases have been reported in the literature with a high preponderance of males and a mean age of 51 years. The frequency of episodes shows a wide variability, not just among individuals but also in the same patient, and a statuslike pattern of almost continuous attacks has been described.

Methods.—We report the cases of two young women (aged 26 and 23 years) with typical SUNCT features who suffered bouts of up to 60 paroxysms of pain per hour. Paraclinical investigations showed no abnormalities.

Conclusions.—Although unusual, paroxysms in SUNCT may overlap into a clinical status. A strong relationship with hormonal changes was noted in one patient. In both cases, the pain was refractory to treatment with indomethacin, carbamazepine, and hypnotics, and only intravenous methylprednisolone with oral carbamazepine may have been partially effective in one case.


short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing

In 1978, Sjaastad et al1 described an entity termed SUNCT (short-lasting unilateral neuralgiform pain with conjunctival injection and tearing) characterized by recurrent, brief (15 to 120 seconds) unilateral pain attacks localized in the periocular area, with a rather abrupt onset and end, associated with marked ipsilateral autonomic features (massive conjunctival injection, watering of the eye, rhinorrhea, subclinical forehead sweating, and changes in cardiovascular variables). SUNCT has been classified among the trigeminal autonomic cephalgias and new diagnostic criteria have recently been proposed.2,3

Generally, SUNCT starts around aged 51 years, with a range between 41 and 70 years for most cases.4 Until 1994 when two women were reported, only men had been described.5 The sex ratio for 25 reported cases is 2.57 men for every woman.4

The frequency of attacks shows great variability not only between individuals but also in the same patient. The number of paroxysms may range from a few attacks per day to nearly continuous attacks, in what has been termed SUNCT status, which is an almost unbearable condition due to the intensity and frequency of the attacks.6-8 We describe the status-like pattern of the SUNCT syndrome in two young women.


The clinical notes of 254 patients with a diagnosis of cluster-type headache were reviewed. SUNCT was diagnosed when the patients had moderate to severe, periocular, neuralgic pains lasting less than 2 minutes accompanied by local ipsilateral autonomic signs and evolving in phases exceeding 1 week; indomethacin, in doses of up to 300 mg per day, had to be ineffective. The features and course of the disorder were recorded in these patients, with a follow-up over several years. Routine laboratory tests, blink reflex studies, cranial computed tomography (CT) scan and/or magnetic resonance imaging (MRI), cerebral angiography (patient 2), and angioMRI (patient 1) were performed. Treatment included indomethacin, carbamazepine, nonsteroidal anti-inflammatory drugs (NSAIDs), hypnotics, and corticosteroids. One patient received short courses of 1 g of intravenous methylprednisolone for 3 days followed by oral prednisone, with gradual tapering over a month.


Patient 1.

A 26-year-old woman started having 5 to 6 daily attacks of pain lasting 5 to 10 minutes for 2 to 3 days. She was asymptomatic for 2 or 3 months and then the episodes recurred again. The pain was felt in the right eye and upper lip and was associated with a tingling sensation on the right temple. The patient felt that the right side of the face was somewhat greasy. Usually she was free from pain at nighttime unless she got up. She was first examined in 1994 at aged 29 years. For 1 week, the attacks were continuous, day and night. The pain was described as an electric shock, which lasted between a few seconds and 2 minutes, and occurred spontaneously or was triggered by chewing, combing, touching, brisk movements of the head, or by eye closure, so she was forced to stay still and avoided any contact on her face. Even a slight breeze could unleash the pain. On two occasions, 60 and 18 attacks per hour were recorded. There was a persistent right palpebral ptosis, ocular redness, lacrimation, and nasal stuffiness. Aside from this, her general and neurological examination, routine laboratory tests, blink reflex, cranial CT, MRI, and angioMRI showed no abnormalities. Indomethacin (300 mg daily), carbamazepine (1200 mg daily), and subcutaneous sumatriptan were tried with no response. She was admitted to hospital and treated with carbamazepine (1200 mg per day) and intravenous methylprednisolone (1 g) for 3 consecutive days, followed by 80 mg of oral prednisone with gradual tapering in a month. There was an early response both in the frequency and intensity of the pain, even when the dose of carbamazepine had to be reduced to 600 mg daily due to dizziness and the presence of nystagmus. She was discharged and remained asymptomatic for nearly 13 months. In November 1995, she returned, complaining of a 3-month period of similar attacks of pain in the same area, between 2 and 20 episodes per day, in spite of treatment with carbamazepine 800 mg daily. She was again admitted and administered intravenous methylprednisolone for 3 days, with a good response, but 2 weeks later, the attacks resumed. Further trials with pimozide, indomethacin, gabapentin, and carbamazepine showed no effect. This active phase continued for 30 days until she was hospitalized and treated with corticosteroids. Finally, in July 1997, she experienced a mild period of 5 to 10 attacks per day during 8 days which was followed by another statuslike phase lasting 12 days. On one occasion, 8 attacks were witnessed in less than 30 minutes. These painful episodes predominated during daytime, but nocturnal attacks also occurred. She was again given intravenous corticosteroids and carbamazepine with success. Since then she has entered a period of remission without treatment.

Patient 2.

This case has been reported previously in part.9 At aged 23 years, this young woman, a migraine sufferer, started to experience brief episodes of pain in her left eye and temple, that occurred spontaneously or on bending. These attacks were in the daytime, lasted for less than 1 minute, recurred approximately seven times a day, always on the same side, and were accompanied by ipsilateral lacrimation, conjunctival injection, and rhinorrea. She was free from pain between the paroxysms. These episodes revealed a close relationship with the menstrual cycle, appearing around ovulation. Treatment with indomethacin, flunarizine, ergots, carbamazepine, and several NSAIDs were ineffective. Nine months after the onset, the pain increased; the attacks recurred every 5 minutes both during the day and at nighttime for more than 2 weeks. When she was evaluated, at aged 24, she was in the second month of gestation of a planned pregnancy. In 1 hour, 15 attacks as described above were witnessed. The left palpebral fissure was constantly narrowed between the attacks. Her general medical and neurological examination were otherwise unremarkable. She underwent termination of the pregnancy, and the pain remitted. Routine laboratory tests, blink reflex study, cranial CT and MRI, and a left carotid angiography showed no abnormalities. The pain reappeared a week later, shortly after the angiogram. The attacks occurred three or four times a day and lasted between 10 and 30 seconds. Treatment with indomethacin was again ineffective. Subsequently, she suffered only one attack per day during the week around ovulation for 7 months. Currently, she has been free from pain without treatment for 9 years, except for occasional migraine, and her palpebral fissures are symmetrical.


These two young women suffered from repetitive unilateral pain attacks, associated with prominent conjunctival injection, lacrimation, ptosis, and nasal stuffiness on the symptomatic side as described in SUNCT syndrome.1 The pain was short-lived (ranging from a few seconds to 2 minutes), neuralgiform in quality, and intense. The affected area was constant for each patient and did not shift sides. As in most of the previously reported cases, the attacks could be precipitated by mechanical factors such as movements of the neck or stimulation of areas innervated by the trigeminal nerve.10 Spontaneous attacks were also noted. The pain episodes occurred mainly in the daytime, but they were also nocturnal during the worst periods. The condition showed an irregular and unpredictable temporal pattern with periods of remission alternating with symptomatic phases. The follow-up period exceeded 5 years in patient 1 and 9 years in patient 2, and the patients have been free from pain for the last 3 and 7 years, respectively. No evidence of structural lesion or other central nervous system dysfunction has been found, ruling out symptomatic SUNCT.

Different variations in the temporal pattern and frequency of SUNCT attacks have been described not only between individuals but even in the same patient. Terms such as plateau-like, saw tooth like, and staccato-like have been used in previous reports to account for the different presentations of this syndrome.5-7 The frequency of attacks may range from a few episodes daily in the mild periods to a nearly constant succession of paroxysms, as reported in the so-called SUNCT status.8 As many as 15 and 60 attacks of pain per hour could be witnessed in these two patients, and in both, typical attacks recurred every few minutes for the better part of the day. Patient 1 experienced two status periods lasting 8 and 12 days, and patient 2 suffered a status period for more than 2 weeks. The cause for this evolution of SUNCT is unknown. We should like to point out that the status was precisely observed in two young women, and in one of them, the pain disappeared after termination of her pregnancy. This fact points towards some possible interaction between SUNCT and hormonal changes.

A strictly unilateral and short-lasting pain, well-defined in the area innervated by the fifth cranial nerve, is a typical feature of trigeminal neuralgia; this is the main differential diagnosis in SUNCT syndrome. As a matter of fact, some authors have considered that SUNCT is really a first branch trigeminal neuralgia.10 It is important to note that our patients were very young when the pain started, autonomic signs were prominent, attacks occurred both in the day and at night, they were not followed by a refractory period, and the course was clusterlike. Moreover, a recent functional MRI study has demonstrated a significant activation in the area of hypothalamic gray matter during pain attacks in SUNCT.11 All these features, as well as the lack of response to carbamazepine, are contrary to the diagnosis of trigeminal neuralgia3,12 and allow its classification as a distinct entity among the trigemino-autonomic cephalgias.2

The response of SUNCT to treatment is usually disappointing.13 Although a recent report addresses the possible usefulness of lamotrigine,14 these results await confirmation. Indomethacin, NSAIDS, and hypnotics were ineffective for us. Response to carbamazepine was absent, and ergotamines, calcium antagonists, and oral corticosteroids were also ineffective. This is consistent with previous reports, showing the lack of effect of several drugs on the pain and may help to define SUNCT as a separate condition from other unilateral headaches. Megadoses of intravenous methylprednisolone have been particularly effective in cases of episodic cluster headache15 and possibly in the cluster-tic syndrome,16 a condition which shares many characteristics with SUNCT. Accordingly, we tried an intravenous bolus of corticosteroids (with oral carbamazepine) in one patient, and this combination seemed to be effective. Therefore, it may be worth trying intravenous corticosteroids with carbamazepine in other patients with SUNCT status.