Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing syndrome: a review

The clinical features of short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) syndrome have been reviewed in 50 patients reported in the English language literature. SUNCT syndrome is a rare condition that predominates slightly in men. The mean age at onset is 50 years. It is characterized by strictly unilateral attacks centered on the orbital or periorbital regions, forehead, and temple. Generally, the pain is severe and neuralgic in character. The usual duration ranges from 5 to 250 seconds, although the reported range of duration is 2 seconds to 20 minutes. Ipsilateral conjunctival injection and lacrimation are present in most, but not all patients. Most patients are thought to have no refractory periods and this has probably been unreported in the past. Episodic and chronic forms of SUNCT exist. The attack frequency varies from less than one attack daily to more than 60 attacks per hour. The attacks are predominantly diurnal, although frequent nocturnal attacks can occur in some patients. A functional magnetic resonance imaging study in SUNCT syndrome has demonstrated ipsilateral hypothalamic activation. SUNCT was thought to be highly refractory to treatment. However, recent open-label trials of lamotrigine, gabapentin, topiramate, and intravenous lidocaine have produced beneficial therapeutic responses. These results offer the promise of better treatments for this syndrome, but require validation in controlled trials.     

SUNCT Syndrome: Repetitive and Overlapping Attacks

SYNOPSIS
A 79-year-old patient had been suffering from right orbital pain attacks since he was 77. The pain, of an "electrical" or burning character, was moderate to severe in intensity. Single attacks lasted for 30–60 seconds each and were accompanied by prominent ipsilateral conjunctival injection and tearing as well as slight rhinorrhoea. The temporal pattern of attacks was irregular with spontaneous remissions alternating with unpredictable symptomatic phases. During active periods, attacks could be triggered by several precipitants acting on trigeminal and extra-trigeminal innervated areas. On most occasions, the patient had 2–3 attacks daily but, at worst, he might have almost continuous short-lasting attacks in periods of 1 to 3 hours length. When attacks had such frequency they presented either as repetitive or overlapping single episodes. According to the clinical features it is concluded that the case reported is a new case of SUNCT syndrome.     

SUNCT Syndrome. Atypical Temporal Patterns

New clinical features of the SUNCT syndrome are described in a series of 3 men (mean age of 65 years, range 56 to 80). The mean age at the onset of symptoms was 55 years (range 39 to 77). Although in all patients the great majority of attacks were typical, on a few occasions unusual features of the painful attacks were either reported or witnessed by the investigators. We have classified these clinical phenomena as (1) low-grade background pain or discomfort, and (2) relatively Long-lasting attacks.
Neither neurological examination nor neuroimaging studies revealed structural lesions as responsible for the atypical features. The fact that these atypical attacks occurred in the usual symptomatic area and were accompanied by the usual ipsilateral autonomic signs, suggests that they are an integral part of the clinical picture of SUNCT. The possibility that another, concurrent headache was responsible for the unusual attacks is considered unlikely. These clinical phenomena should, accordingly, provisionally be considered as additional but rare clinical features of the SUNCT syndrome.     

SUNCT Syndrome. Statuslike Pattern

Four SUNCT patients with a clinical "SUNCT status" witnessed by the investigators are reported. Such an extreme aggregation of typical attacks lasting for the better part of the day, 1 to 3 days in a row has not previously been observed. Appropriate supplementary examinations ruled out an intracranial lesion in all four patients. Indeed an observation period of more than 5 years speaks against a symptomatic etiology of SUNCT in these cases. Although rarely seen, clinical status may be a feature of the SUNCT syndrome.     

SUNCT syndrome responsive to lamotrigine

BACKGROUND: Short-lasting, unilateral, neuralgiform headache attacks with conjunctival injection, tearing, rhinorrhea (SUNCT syndrome) is a headache form generally refractory to drug therapy. Occasional patients with SUNCT have been reported with a successful response to lamotrigine. OBJECTIVE: To report two patients with SUNCT treated with lamotrigine. METHODS: Clinical history, neurologic examination, and brain magnetic resonance imaging. RESULTS: Both patients with SUNCT syndrome were successfully treated with lamotrigine. In both cases, when lamotrigine was tapered off, the attacks reappeared, only to disappear when the dose was again increased. In addition, lamotrigine was well tolerated and no undesired side-effects were reported. CONCLUSION: If the positive effect of lamotrigine in patients with SUNCT is confirmed in other cases, lamotrigine could become the first specific treatment for SUNCT syndrome.     

Case report on a patient with SUNCT-syndrome

Case report on a patient with SUNCT-syndrome (short lasting, unilateral neuralgiform headache attacks with conjunctival injection, sweating, and rhinorrhoea) who was successfully treated with gabapentin. SUNCT, a still relatively unknown headache syndrome, is characterized by attacks of periorbital pain with accompanying ipsilateral autonomic symptoms. Along with this case report the differences of SUNCT to similar headaches are emphasized. Due to clear diagnostic criteria the inclusion of SUNCT in the IHS classification (International Headache Society) as a separate clinical entity should be favoured.     

SUNCT Syndrome. Two Cases in Argentina

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.     

SUNCT and high nocturnal prolactin levels: some new unusual characteristics

Abstract SUNCT is a rare condition characterised by a short-lasting periorbital pain associated with autonomic symptoms and is usually unresponsive to pharmacological treatment. We report a case of SUNCT syndrome linked to a pituitary micro-adenoma, with only nocturnal attacks. The nocturnal levels of prolactin (PRL) were increased, while other hormonal, haematological, serological and biochemical investigations and levels of PRL did not reveal abnormal findings during the day-time. PRL serum secretion after thyrotropin-releasing hormone test was lower than nocturnal secretion, but not enough to induce severe attacks. We suggest that in our patient the rise of nocturnal levels of PRL could have a direct role in the worsening of this headache, perhaps secondarily to an altered regulation of the hypothalamic–hypophysial axis, however the actual influence of sleep and the interaction between all neurotransmitters and hormones needs to be clarified further.     

Shortlasting, unilateral, neuralgiform headache attacks with conjunctival injection and tearing (SUNCT syndrome): V. Orbital phlebography

We studied six patients with SUNCT, a unilateral headache syndrome with shortlasting attacks and ipsilateral autonomic phenomena with orbital phlebography and MRI. All but one orbital phlebogram showed abnormalities on the headache side (in one patient bilaterally), involving the superior ophthalmic vein or/and the cavernous sinus. No systematic changes were demonstrated on MRI. The phlebography findings were similar to those observed in the Tolosa-Hunt syndrome and in cluster headache.     

SUNCT Syndrome: Duration, Frequency, and Temporal Distribution of Attacks

Duration, frequency, and temporal distribution of attacks have been objectively estimated in 11 SUNCT patients (3 women and 8 men). The mean age at the time of the study was 69 years (range 52 to 81). The duration of a total of 348 attacks was measured from videotape records, polygraphic tracings, or by stopwatch.The duration of attacks ranged from 5 to 250 seconds, with an unweighted mean of 61 seconds. Both frequency and exact timing of attacks were assessed in four patients who filled in a time chart with the exact onset of 585 consecutive attacks. The majority of attacks occurred during daytime, with a bimodal distribution; ie, morning and afternoon/evening peaks, and only a few attacks were noted at night (ie, 1.2% of the attacks). The unweighted mean frequency of attacks was 28 per day (range 6 to 77).
Duration and timing of attacks in SUNCT syndrome may be of help in the differential diagnosis versus other disorders with the same localization, especially first division trigeminal neuralgia.     

Symptomatic SUNCT Syndrome Associated With Ipsilateral Paranasal Sinusitis

Sinusitis has rarely been associated with short‐lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) syndrome. We describe a case of symptomatic SUNCT syndrome caused by sinusitis, which showed typical features of SUNCT syndrome. The patient's symptoms completely resolved without recurrence after endoscopic sinus surgery followed by antibiotic treatment. We suggest that ipsilateral paranasal sinusitis accompanied by the spreading of inflammation through a dehiscence in the lamina papyracea could lead to the development of SUNCT syndrome.     

A case of early-onset and monophasic trigeminal autonomic cephalalgia: could it be a SUNCT?

A 2-year-old female came to the Neurological Emergency Room of “Giovanni XXIII” Hospital in Bari, 6 h after the onset of severe facial pain, which occurred soon after awakening. Stabbing pain affected the right frontal and periorbital area, with ipsilateral conjunctival injection, swelling of the eyelids and tearing. Except the duration, from 5 to 30 s., the attacks were stereotyped including the occurrence and features of autonomic signs. Based on the typical clinical findings and the normal magnetic resonance imaging (MRI), we diagnosed short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing syndrome (SUNCT). The spontaneous remission within a few hours made prophylactic therapy unnecessary. At the last follow-up, after 3 months, the patient was still symptom free. In our case, after an active period lasting 2 days the disease disappeared completely. However the typical features of the disease (unilateral pain, short duration and high frequency of the attacks, autonomic signs ipsilateral to pain, numbers of attacks) were all present. While the diagnostic criteria of the International Headache Society classification for SUNCT did not include the duration of disease, it is likely that the active period lasting 2 days could be an expression of the clinical variability of the disease.     

SUNCT syndrome in association with persistent Horner syndrome in a Chinese patient

This is the first case report of a chinese patient with SUNCT (shortlasting, unilateral, neuralgiform headache attacks with conjunctival injection and tearing) presenting with persistent Horner's syndrome. She had episodic, brief, right periorbital pain in association with ipsilateral eye injection, lacrimation and rhinorrhea as well as persistent ipsilateral miosis and ptosis. She had partial response to a combination of indomethacin and carbamazepine therapy.     

Conjunctival congestion in SUNCT syndrome

The purpose of this observational-retrospective study was to assess which type of conjunctival injection and related phenomena are present during SUNCT attacks. We studied the videorecords of 23 SUNCT attacks in three patients. Conjunctival injection was found to mostly involve vessels of the palpebral territory stemming from both superior and inferior palpebral vessels that supply the tarsal conjunctiva and most of the ocular (bulbar) conjunctiva. Episcleral injection was also observed. During attacks, the appearance of the conjunctiva suggested conjunctival edema or chemosis. Furthermore, the presence of edema below the capsule of Tenon was strongly suspected. During SUNCT attacks, there was a dramatic conjunctival congestion of the eye on the symptomatic side that tended to extend to the rest of the external tunica of the eye. Received: 24 January 2000 / Accepted in revised form: 19 May 2000     

Trigeminal Neuralgia. Clinical Manifestations of First Division Involvement

A series of 19 patients with what originally had been diagnosed as a first division (V₁) trigeminal neuralgia was collected. The inclusion criteria were severe, rather short-lasting pain attacks within the V₁ area, combined with trigger mechanisms. There were 10 women and 9 men, and the mean age of onset was 57.8 years. Fifteen of 16 with adequate information on attack duration had paroxysms of a "few seconds'" duration or less, whereas 10 patients had paroxysms lasting ≥2 seconds. In an exceptional case, only "more long-lasting" attacks (greater than 30 seconds' duration) were experienced.
In regard to autonomic phenomena, lacrimation was most frequently present (in a total of 8 patients; 3 rather regularly, 5 more irregularly). The combination of lacrimation, conjunctival injection, and rhinorrhea was present in only 2 (of 19), and in neither of them in a major way. Typically, autonomic phenomena occurred during the later stages of disease and during particularly severe and long-lasting attacks. Seven of 14 with adequate information also had nocturnal attacks. Initially, a more or less complete carbamazepine effect was reported by 10 of 13 patients. Precipitation mechanisms were the same as with second and third division tic, but were mainly located within the V₁ area, particularly initially.
A comparison with SUNCT syndrome has been made. SUNCT is a predominantly male disorder, with only exceptional attacks of ≥10 seconds' duration, and generally with attacks of 15 seconds or longer. Autonomic symptoms and signs are more pronounced than in V₁ tic. Carbamazepine generally provides minor, if any, benefit in SUNCT. The present work strongly indicates that the two disorders are essentially different.     

SUNCT may be Another Manifestation of Orbital Venous Vasculitis

A patient with more than 20 years of SUNCT, i.e., long lasting periods with frequent attacks of intense orbital pain with a duration of about one minute, associated with ipsilateral conjunctival injection, lacrimation, rhinorrhea and facial sweating is described. Some attacks were possibly related to increased cerebral blood flow but could also be triggered from the oral area. Orbital phlebography showed pathologic changes on the side of the pain, changes which were normalized when these attacks ceased to appear. Due to these findings in conjunction with serum evidence of inflammation, associated systemic symptoms and susceptibility to steroids and azathioprine, venous vasculitis is suggested to be the cause of SUNCT in this patient. Carbamazepine and sumatriptan decreased the frequency, intensity and duration of attacks, although not completely.     

SUNCT syndrome associated with cavernous angioma of the brain stem

A 62-year-old male patient with 2 years f SUNCT syndrome is described. The patient presented with long-lasting periods of frequent attacks of intense orbital pain with a duration of about 1 min, associated with ipsilateral conjunctival injection, lacrimation and rhinorrhea. Cranial MRI and cerebral angiography demonstrated an ipsilateral cavernous angioma of the pons, involving the trigeminal roots. As the pain was refractory to most treatments, including carbamazepine, the patient asked for, and eventually underwent, direct surgical excision of the malformation. Following the operation, his neurological conditions rapidly deteriorated and he died of postoperative complications (hemorrhage).     

Duration of attacks of first division trigeminal neuralgia

Objective measurements of duration of attacks have been performed in 8 (5 female and 3 male) patients suffering from primary first division (V-1) trigeminal neuralgia. The mean age of the patients was 67.5 +/- 11.4 years, and the mean age at onset 64.0 +/- 9.7 years. During the study the patients were off treatment. A total of 192 attacks were witnessed by the authors and exactly timed by a stop-watch. The duration of attacks ranged from 2 to 32 s, with a mean of 6.5 +/- 6.1 s. The unweighted mean was 8.8 +/- 5.7 s, with a range of 2.4-17.5 s. With the present data the duration of attacks of V-1 neuralgia has been exactly determined, and the clinical distinction of V-1 neuralgia from other shortlasting headaches, particularly from SUNCT, has been substantially clarified.     

Objective assessment of autonomic signs during triggered first division trigeminal neuralgia

A total of 26 episodes of V-1 trigeminal neuralgia attacks have been recorded in two female patients. Autonomic phenomena were assessed according to a semiquantitative scale. Attacks lasted 17 +/- 5 s. Mild lacrimation without conjunctival hyperaemia, rhinorrhea or ptosis was observed, even in relatively long lasting episodes. This is in clear contradiction with SUNCT (shortlasting, unilateral, neuralgiform headache with conjunctival injection, tearing and rhinorrhea) attacks that are always dramatically accompanied by both lacrimation and conjunctival injection of the symptomatic side from the very onset of symptoms. Carbamazepine provided complete and sustained relief of symptoms in both patients. Herein we will show differential autonomic features of V-1 trigeminal neuralgia vs. SUNCT that will both aid the clinician to distinguish both syndromes and stress that both entities are nosologicaly different.     

SUNCT Syndrome: A Hungarian Case

A Hungarian patient with short-lasting, unilateral, neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) is presented in this paper. This male patient was first diagnosed as having first division trigeminal neuralgia. The location and duration of the attacks and the prominent accompanying autonomic feature on the symptomatic aide, such as conjunctival injection, lacrimation, nasal stuffiness, and the inefficacy of drugs, led to a reconsideration of the diagnosis. The pain paroxysms occurred frequently during a 3-to 4-month period, followed by a longer remission phase. Mechanical precipitating maneuvers were observed during bouts of pain. The clinical picture is reminiscent of the SUNCT syndrome, first described by Sjaastad et al in 1978.
SUNCT and trigeminal neuralgia are in many ways similar, although, some decisive differences have also been noted. Further observations are needed to distinguish the two disorders and to clarify this syndrome as a new headache type or as a trigeminal neuralgia variant.