SUNCT: bilateral hypothalamic activation during headache attacks and resolving of symptoms after trigeminal decompression

Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) is a primary head-pain syndrome, which is often refractory to any medical treatment. Concerning the pathophysiology of SUNCT, hypothalamic involvement ipsilaterally to the pain has been suggested based on the clinical features and one functional imaging case report. Here we now report a new case with SUNCT and the concomitant cerebral activation pattern (fMRI) during the pain attacks. In addition to an activation of several brain structures known to be generally involved in pain processing, bilateral hypothalamic activation occurred during the pain attacks, arguing for a central origin of the headache. Interestingly, this patient became completely pain free after surgical decompression of the ipsilateral trigeminal nerve. We hypothesize that in this case with a central predisposition for trigeminal autonomic cephalgias, a peripheral trigger with ectopic excitation might have contributed to the clinical picture of SUNCT.     

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.     

Bilateral SUNCT-like headache in a patient with prolactinoma responsive to lamotrigine

Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) syndrome is a rare trigeminal autonomic cephalalgia. The cases of SUNCT with attacks that affected both sides simultaneously have only rarely been reported and some of them had underlying pathology. We have reported a case of bilateral SUNCT-like headache secondary to a prolactinoma and responsive to lamotrigine treatment.     

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.     

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 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.     

A case of SUNCT syndrome responsive to zonisamide

Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) is a rare headache syndrome that represents a subtype of trigeminal autonomic cephalalgia thought to be highly refractory to treatment. More recently, numerous anticonvulsant agents including lamotrigine, topiramate, gabapentin, and carbamazepine have been reported to be partially or completely effective for treating SUNCT. We report the case of a patient with SUNCT in whom symptoms were completely relieved with carbamazepine at 600?mg/day. However, carbamazepine had to be discontinued due to severe rash. Zonisamide was selected for continued treatment, as a Na-channel blocker like carbamazepine but with lower risk of producing skin rashes as caused by carbamazepine. Attacks ceased completely with 300?mg/day of zonisamide achieving a blood serum level of 19?μg/ml. This is the first case report to describe zonisamide alone completely eliminating SUNCT symptoms. Zonisamide should be considered a viable candidate drug for the treatment of SUNCT.     

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.     

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.     

CLUSTER, NEURALGIAS, AND TACS

A number of primary headache syndromes are marked by their short duration of pain. Many of these syndromes have their own unique treatment, so they must be recognized by practicing physicians. In this article, a number of the short-lasting headache disorders are reviewed, including chronic paroxysmal hemicrania, SUNCT syndrome, hypnic headache, exploding head syndrome, primary stabbing headache, and cough headache.     

Short-lasting headache syndromes and treatment options

A number of primary headache syndromes are marked by their short duration of pain. Many of these syndromes have their own unique treatment, so they must be recognized by practicing physicians. In this article, a number of the shortlasting headache disorders are reviewed, including chronic paroxysmal hemicrania, SUNCT syndrome, hypnic headache, exploding head syndrome, primary stabbing headache, and cough headache.     

Short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA) secondary to epidermoid cyst in the right cerebellopontine angle successfully treated with surgery

Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) syndrome is a rare headache syndrome classified among the trigeminal autonomic cephalalgias. It is usually idiopathic, although infrequent secondary forms have been described. Recently, the term short-lasting unilateral headache with cranial autonomic symptoms (SUNA) has been defined by the International Headache Society (ICHD-2) as similar to SUNCT with less prominent absent conjunctival injection and lacrimation. We report a patient with paroxysmal orbito-temporal pains, phenotypically suggesting SUNA, secondary to epidermoid cyst in the cerebellopontine angle which disappeared after tumor resection. Neuroimaging should be considered in all patients with SUNA, notably in those with atypical presentation as our patient who presented on examination trigeminal hypoesthesia and tinnitus. Realization of a brain MRI would rule out injuries that causes this type of syndrome.     

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     

SUNCT syndrome in a patient with prolactinoma and cabergoline-induced attacks

Short-lasting unilateral neuralgiform headache with conjuntival injection and tearing (SUNCT) syndrome is a rare trigeminal autonomic cephalalgia. We report a patient with prolactinoma and cabergoline-induced SUNCT attacks and the literature is reviewed for a better understanding of the pathophysiology.     

Post-traumatic short-lasting unilateral headache with cranial autonomic symptoms (SUNA)

Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) syndrome is a rare headache syndrome classified among the trigeminal autonomic cephalalgias. It is usually idiopathic, although infrequent secondary forms have been described. Recently the term short-lasting unilateral headache with cranial autonomic symptoms (SUNA) has been defined by the International Headache Society (ICHD-2) as similar to SUNCT with less prominent or absent conjunctival injection and lacrimation. We report two patients with paroxysmal orbito-fronto-temporal pains, phenotypically suggesting SUNA, occurring after traumatic head injury.     

SUNCT and trigeminal neuralgia attributed to meningoencephalitis

We describe a 46-year-old female with viral meningoencephalitis (likely varicella-zoster virus) who developed a SUNCT syndrome followed a few days later by trigeminal neuralgia. Both disorders resolved in parallel with the resolution of encephalitis, which suggests a causal link. In conclusion, headache attributed to intracranial infection may have the clinical features of SUNCT or TN.     

Headache induced by dopamine agonists prescribed for prolactinoma: think SUNCT!

Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) syndrome may be associated with pituitary prolactinoma and may be induced by treatment of prolactinoma with dopamine agonists. Endocrinologists treating patients with prolactinoma need to be aware of this syndrome as its pathophysiology and treatment differ from that of other headache syndromes.     

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).     

Familial SUNCT

Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) is a rare type of primary headache. In this report we describe the occurrence of SUNCT in a family. Unfortunately, one of the siblings was already dead. However, clear and detailed information from close relatives and her general practitioner confirmed the diagnosis of SUNCT. It is likely that genetic factors contribute to all types of trigeminal autonomic cephalalgias.     

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.