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.     

Pathophysiology and treatment of trigeminal autonomic cephalalgias

Trigeminal autonomic cephalalgias (TACs) are a group of primary headache disorders, which are characterized by strictly unilateral pain, together with ipsilateral cranial autonomic symptoms. TACs include cluster headache (CH), paroxysmal hemicrania (PH) and short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT syndrome). These diseases all have one thing in common: an activation of trigeminal nociceptive afferentia with a reflex-like activation of cranial autonomic efferentia via the facial nerve. TACs show differences not only in the length and frequency of attacks but also in the response to drug treatment. It is important to recognize and differentiate between these syndromes because they react very well, but very selectively to therapy.     

Neues zur Pathophysiologie und Therapie der trigeminoautonomen Kopfschmerzsyndrome

Trigeminal autonomic cephalalgias (TACs) are a group of primary headache disorders, which are characterized by strictly unilateral pain, together with ipsilateral cranial autonomic symptoms. TACs include cluster headache (CH), paroxysmal hemicrania (PH) and short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT syndrome). These diseases all have one thing in common: an activation of trigeminal nociceptive afferentia with a reflex-like activation of cranial autonomic efferentia via the facial nerve. TACs show differences not only in the length and frequency of attacks but also in the response to drug treatment. It is important to recognize and differentiate between these syndromes because they react very well, but very selectively to 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).     

A patient with SUNCT syndrome responsive to sodium valproate

SUNCT syndrome (shortlasting unilateral neuralgiform headache attacks with conjunctival injection and tearing) is a rare, debilitating headache that is difficult to be treated. We describe a SUNCT patient, initially treated with lamotrigine, with a positive response but with an unbearable side-effect (somnolence) that lead to withdrawal. The drug was replaced with sodium valproate at a dosage of 1000 mg b.i.d.. Complete remission of the attacks has been obtained for 1 year, without significant side effects. Received: 24 July 2001, Accepted in revised form: 10 January 2002 Correspondence to P. Meineri     

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.     

Oxygen therapy influences episodic cluster headache and related cutaneous brush and cold allodynia

Cluster headache (CH) is characterized by a series of sudden attacks of short-lasting severe headache pain with ipsilateral autonomic features, including lacrimation, rhinorrhea, localized sweating, eyelid edema, and partial or complete Horner's syndrome. Just like in migraine, brush allodynia has been described for CH and for short lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) syndrome. Administration of normobaric oxygen is part of the standard therapy for CH attacks. Here, we describe a young male with a first CH attack and the influence of oxygen on pain and concomitant cutaneous allodynia.     

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.     

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: a new case with some unusual features

A healthy 22–year–old man complained of primary stabbing headache (PSH) for about two months. The headache recurred after one year and after a month the pain took on the characteristics of short–lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT). This patient shows some unusual features: juvenile onset, miosis during attacks as part of autonomic phenomena and close temporal relationship with stressful events. The association between PSH and SUNCT may be interpreted as the coexistence of two different headaches or that the PSH is a forerunner to the SUNCT.     

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.     

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: 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. 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 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.     

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.     

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 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 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.