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[A rare headache syndrome. SUNCT syndrome, hemicrania continua and red ear syndrome].

Rare headache syndromes that are so fare not yet admitted to the classification system of the International Headache Society are the SUNCT syndrome (short-lasting, unilateral, neuralgiform headache attacks with conjunctival injection and tearing), the hemicrania continua and the red ear syndrome. The clinical characteristics, differential diagnoses and therapeutic strategies of these pathophysiologically unclarified diseases are presented with three casuistic reports.

Aged↗

SUNCT syndrome associated with temporomandibular disorders: a case report.

This case report relates the association between a rare neuralgiform syndrome (SUNCT) and masticatory myofascial pain (TMD); two different diseases with different diagnosis criteria and treatments. SUNCT syndrome was treated with a balloon microcompression procedure of the trigeminal ganglion, and the myofascial pain with injections and physical therapy. The patient was without pain at a twelve-month follow-up evaluation.

Bruxism↗

SUNCT syndrome versus idiopathic stabbing headache (jabs and jolts syndrome).

Both SUNCT syndrome and idiopathic stabbing headache (ISH) (jabs and jolts syndrome) have to be considered when encountering shortlasting headaches. Since there are no specific tests for these headaches, the differential diagnosis depends entirely upon assessment of the clinical features. These headaches are generally easily distinguishable clinically. There seem to be symptomatic forms of SUNCT.

Age of Onset↗

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.

Aged↗

[A specific form of headache with neuralgia-like manifestations (SUNCT-syndrome)].

A recently described SUNCT is a rare head ache syndrome characterized by shortlasting, unilateral, neuralgia-like headache attack with conjunctival injection and tearing; 26 cases of SUNCT-syndrome have been reported in medical literature. The authors observed the described symptoms in 3 cases (among 86 patients with trigeminal paroxysmal manifestations). Clinical features of SUNCT-syndrome are presented. In addition, the authors discuss possible etiologic factors and treatment as well as a differential diagnosis of SUNCT-syndrome, from trigeminal neuralgia, cluster headache, chronic paroxysmal hemicrania and combined syndromes.

Adult↗

SUNCT syndrome in the female.

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.

Aged↗

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.

Aged↗

SUNCT syndrome secondary to prolactinoma.

Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) syndrome is a rare form of primary headache disorder, although secondary causes, particularly posterior fossa abnormalities, are well known. We report a case of SUNCT syndrome secondary to a prolactinoma. Administration of dopamine agonists led to complete resolution of the SUNCT attacks. This case, together with other similar case reports in the literature, highlight the importance of excluding a diagnosis of pituitary adenoma in all suspected cases of SUNCT syndrome, especially as the headache can precede more classical pituitary symptoms by a considerable period of time. Clinicians managing patients with suspected SUNCT syndrome should elicit a history of symptoms associated with pituitary neoplasms, perform a magnetic resonance imaging scan of the brain and pituitary, and screen for serum hormonal abnormalities.

Adenoma↗

SUNCT syndrome: an update.

SUNCT (Shortlasting Unilateral Neuralgiform Headache attacks with Conjunctival injection and Tearing) is a syndrome characterised by shortlived (5-240 s), strictly unilateral, orbital/periorbital, moderate-to-severe pain attacks, accompanied by rapidly developing conjunctival injection and lacrimation. Most attacks are triggered by mechanical stimuli, but there are also spontaneous attacks. Symptomatic periods alternate with remissions in an unpredictable fashion. In active periods, the attacks predominate during daytime, with a frequency that ranges from < 1 attack/day to > 30 attacks/h SUNCT is mainly a primary disorder, but is sometimes associated with intracranial structural lesions (symptomatic SUNCT). SUNCT has been included in the group of trigeminal autonomic cephalalgias, which are thought to depend on the activation of the trigeminal system together with the disinhibition of a trigeminofacial autonomic reflex. According to a few reports, SUNCT patients may benefit from carbamazepine, lamotrigine, gabapentin, topiramate or various surgical procedures. However, well-designed clinical trials are required before these therapeutic options can be sufficiently validated.

Carbamazepine↗

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 1 attack daily to more than 30 per hour. In 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.

Adult↗

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.

Adult↗

[SUNCT syndrome--case report and review of the literature].

The SUNCT syndrome is a recently described headache disorder characterised by shortlasting, unilateral headache attacks with conjunctival injection and tearing. Our report presents a further case and compares the findings in our patient with those in the cases described earlier. In addition, we review the literature and discuss possible aetiological and pathogenetic factors as well as the differential diagnosis of SUNCT syndrome, trigeminal neuralgia and cluster headache syndromes.

Adult↗

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.

Aged↗

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.

Aged↗

Bilateral SUNCT syndrome associated to chronic maxillary sinus disease.

SUNCT syndrome (short lasting unilateral neuralgiform headache with conjunctival injection and tearing) is defined as short attacks of periorbital unilateral pain and accompanied by ipsilateral lacrimation and redness of the same eye. We present an unusual SUNCT case with bilateral pain that started five years ago after an acute maxillary sinus infection that evolved to chronic sinusitis. This association has been described in few SUNCT cases, but its causal role remains uncertain. The patient was a 58 year old man that fulfilled a headache diary that showed the usual circadian pattern, worsening in the morning and afternoon, and responded to treatment with gabapentine. He was submitted to a functional endoscopic sinus surgery and evolved with milder pain. In a review of 21 patients, 5 had a past medical history of sinusitis, but the causal role of this association remained uncertain.

Amines↗

Influence of lamotrigine over the SUNCT syndrome: one patient follow-up for two years.

The SUNCT syndrome is characterized by a short-lasting headache in the first division of the trigeminal nerve, associated with ipsilateral autonomic symptoms. It is highly refractory to prophylactic medication. We describe a case where lamotrigine reduced the intensity, duration, and frequency of attacks and increased the remission period of this disorder. Over a two-year period, the attacks came back immediately whenever the patient reduced the dose or neglected treatment. We concluded that lamotrigine is effective in treating SUNCT syndrome when used in high doses for a prolonged period of time.

Analgesics↗

SUNCT syndrome: forehead sweating pattern.

The forehead sweating function has been assessed in SUNCT syndrome--a short-lasting, unilateral, neuralgiform 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.

Aged↗

[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.

Acetates↗