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.     

Two new SUNCT cases responsive to lamotrigine

Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) is a rare and debilitating headache form generally unresponsive to treatment. Following a recent report of a SUNCT patient who responded to lamotrigine, we tried this drug in two new SUNCT patients, reported here. In both cases prophylaxis was successful, suggesting lamotrigine might be the first effective treatment for this rare and debilitating headache 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 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.     

SUNCT syndrome: forehead sweating pattern

The forehead sweating function has been assessed in SUNCT syndrome-a short-lasting, unilateral, neuralgifonn headache syndrome with autonomic phenomena on the symptomatic side (conjunctival injection, lacrimation, etc.). In the three patients (of a total of six) who could be studied during paroxysms, increased evaporation was present on the symptomatic side of the forehead compared to the non-symptomatic side during attacks or to the symptomatic side between attacks. Basal sweating was generally within control limits, so long as the attack frequency was not so high as to influence the interictal level. During attacks precipitated by eating chocolate or sour apple (in the case of one of the patients), forehead sweating was also increased on the symptomatic side. The forehead sweating responses to heating and pilocarpine were without any notable or systematic asymmetries. The forehead sweating pattern in SUNCT syndrome may differ from the patterns in unilateral headaches like cluster headache, on the one hand (in which there is generally an asymmetry during heating and pilocarpine tests), and chronic paroxysmal hemicrania (CPH) and cervicogenic headache, on the other (where there is no systematic increase during attacks).     

SUNCT Syndrome in the Female

SYNOPSIS
Two female patients suffering from SUNCT syndrome are presented. They are the first female SUNCT cases reported (of a total of 16 cases hitherto seen by us, reported to us, or published). Also some minor modifications of the hitherto known clinical symptomatology of SUNCT syndrome have been observed.
Attacks could be triggered in a variety of ways. A short latency between the application of a precipitating stimulus and the onset of pain was noted. An occasional inability of a given precipitating maneuver to activate the pain was also noted. The temporal pattern of pain was partly characterized by the typical, "plateau-like" pattern, but with ultrashort exacerbations. In part, the pain attacks were characterized by steeple or spike-like pain waves, that did not quite subside to the baseline ("repetitive pattern"). Autonomic signs such as lacrimation and conjunctival injection were rather marked, as is generally the case in SUNCT syndrome. Rhinorrhea was present in both patients, and in one patient the rhinorrhea was unusually marked. Carbamazepine treatment seemingly brought about a slight decrease in the frequency of attacks.     

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 intravenous lidocaine

Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) is a primary headache syndrome that has been reported to be resistant to treatment with intravenous lidocaine. We report four cases of SUNCT in whom intravenous lidocaine (1.3-3.3 mg kg(-1) h(-1)) completely suppressed the headaches for the duration of the infusion. The headache returned after cessation of treatment. Two patients went on to have their symptoms controlled on topiramate (50-300 mg daily). One patient had typical migrainous aura in association with some of the attacks of pain but never migrainous headaches. These cases suggest that treatment with lidocaine can be considered when acute intervention is required to suppress a severe exacerbation of SUNCT, and further broaden the therapeutic and clinical background of this syndrome.     

Meningioma causing gabapentin-responsive secondary SUNCT syndrome

Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) is one of the rarest and most serious headache disorders. Cases of symptomatic SUNCT syndromes are reported, which demonstrate that brain imaging is very important for diagnosis. In this study, we describe the first case of secondary SUNCT syndrome caused by a meningioma. So far, a clearly effective therapy for SUNCT syndrome has not been known. In this case, however, SUNCT was completely responsive to gabapentin. This underlines that this drug is worthy of being considered as a potential therapeutic option in the treatment of SUNCT syndrome.     

"SUNCT" syndrome. A case of transformation from trigeminal neuralgia?

A patient with typical trigeminal neuralgia involving the first branch of the nerve developed short-lasting unilateral attacks in the same area which were associated with severe vasomotor phenomena consistent with the recently described SUNCT syndrome. This evolution suggests that SUNCT might correspond, at least in this case, to a "transformed" trigeminal neuralgia and emphasizes the close relationship between these unilateral facial pain syndromes.     

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     

Respiratory Studies in SUNCT Syndrome

Seven SUNCT patients (six men, one woman) took part in this study. In four patients, respiratory variables were compared during and outside attacks. In five patients, peripheral chemosensitivity was tested and compared with a control group matched with respect to age. sex, and smoking habits. The results indicate that SUNCT patients hyperventilate during attacks. Moreover, they appear to hyperventilate slightly under basal conditions. The tests for peripheral chemoreceptor activity indicated no differences between the SUNCT and the control groups except for one variable, namely the mean ventilatory response to a single breath of 13% CO₂. It is possible that this indicates a blunted response of the peripheral chemoreceptors. On the other hand, it may also represent a chance finding, since none of the other results presented suggested such a conclusion, and the size of the test group was very small. The results do not indicate that a reduction in oxygen saturation can trigger SUNCT since low levels of oxygen saturation were only rarely accompanied by SUNCT, whereas many attacks were not associated with any appreciable lowering in arterial oxygen saturation.     

Chronic paroxysmal hemicrania, hemicrania continua and SUNCT syndrome in association with other pathologies: a review

We present a review of 22 cases of headache mimicking chronic paroxysmal hemicrania (CPH) (17 female and five male; F : M ratio 3.4), nine cases mimicking hemicrania continua (HC) (seven female and two male) and seven cases mimicking SUNCT syndrome (five male and two female) found in association with other pathologies published from 1980 up to the present. All case reports were discussed with respect to diagnostic criteria proposed by International Headache Society (IHS) for CPH, by Goadsby and Lipton for HC and SUNCT, and evaluated to identify a possible causal relationship between the pathology and the onset of headache. The aim of the present review was to evaluate if the presence of associated lesions and their location could help elucidate the pathogenesis of trigeminal autonomic cephalalgias (TACs).     

SUNCT Syndrome. A Clinical Review

The clinical features of SUNCT syndrome have been reviewed in 21 patients. There were 17 men and 4 women, rendering a clear male preponderance (ratio of 4.25). The mean age at onset was around 51 years. Attacks were experienced mostly in the orbital/periorbital area and always recurred on the same side, with an erratic temporal pattern and remissions of varying lengths. Most attacks were moderate to severe in intensity and burning, electrical, or stabbing in character. The attacks were regularly accompanied by prominent, ipsilateral, conjunctival injection, tearing; and rhinorrhea or nasal obstruction. There were many precipitating mechanisms. Exclusively spontaneous attacks were described in 3 patients. The usual duration of paroxysms ranged from 10 to 60 seconds, whereas the longest duration varied from 60 to 300 seconds. The frequency of attacks during the symptomatic periods varied from less than I attack daily to more than 30 per hour. hi the majority of patients, supplementary examinations failed to show any notable abnormality. However, 2 patients were documented to have a symptomatic form of SUNCT, with a vascular malformation in the ipsilateral cerebellopontine angle. A variety of drugs and local anesthetic blockades. inclusive of tic douloureux drugs, were tried, but a persistent, convincingly beneficial effect was generally lacking SUNCT syndrome is in the differential diagnosis when encountering unilateral, orbital/periorbital headache syndromes.     

SUNCT Syndrome: Trials of Drugs and Anesthetic Blockades

Nine patients with the SUNCT syndrome (Spanish and Norwegian patients) have, over many years, been given several drugs effective in the cluster headache syndrome, trigeminal neuralgia, and other headaches, as well as drugs not previously used in headache. Various cranial nerves were also anesthetized in an endeavor to ameliorate the suffering of those patients.
Although a partial effect was obtained with carbamazepine and corticosteroids in some patients, none of the drugs or anesthetic blockades had consistent, lasting, complete effect on headache paroxysms in SUNCT. The essentially negative outcome of this study aids in further characterizing SUNCT as a separate disorder, and, above all, in distinguishing it from trigeminal neuralgia and the cluster headache syndrome.     

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.     

Cluster headache and SUNCT: similarities and differences

SUNCT is probably a distinct syndrome, although it shares some common features with cluster headache (CH): male sex preponderance, clustering of attacks, unilaterality of headache without sideshift, pain of non-pulsating type with its maximum in the periocular area, ipsilateral autonomic phenomena (e.g. conjunctival injection, lacrimation, rhinorrhea, increased forehead sweating), systemic blood pressure increment with heart rate decrement, blood flow velocity decrement in the middle cerebral artery, and hyperventilation. In spite of these similarities, SUNCT syndrome differs clearly from CH as regards a number of clinical variables, such as duration, intensity, frequency, and nocturnal preponderance of attacks. The two syndromes also differ markedly as regards precipitation of attacks, the usual age at onset, and efficacy of various treatment alternatives. Laboratory investigations have disclosed differences as regards presence or absence of Horner-like picture and possibily also the respiratory sinus arrhythmia pattern. All in all, these differences seem sufficiently ponderous to make it likely that SUNCT syndrome and CH differ essentially. SUNCT seems to be a “neuralgiform” headache, but different from trigeminal neuralgia. Received: 2 April 2001 / Accepted: 23 July 2001     

Attack‐Related Brainstem Activation in a Patient With SUNCT Syndrome: An Ictal fMRI Study

The authors report functional magnetic resonance imaging (fMRI) study data of a 60‐year‐old patient having short‐lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) syndrome. Three consecutive pain attacks were detected during the imaging session and strong brainstem activation was found. It was concluded that the brainstem can be involved in the pain signal transmission in SUNCT syndrome.     

Abrupt onset and termination of cutaneous allodynia (central sensitization) during attacks of SUNCT

The presence of central sensitization and cutaneous allodynia has not been readily studied in other primary headache syndromes outside of migraine. If central sensitization does occur, is the temporal profile any different in the short-lasting more aggressive syndromes such as SUNCT than in migraine? A patient with SUNCT was examined during and in between attacks looking for the presence and duration of cutaneous allodynia.