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 or first division trigeminal neuralgia associated with cerebellar hypoplasia

Short-lasting unilateral neuralgiform headache (SUNCT) and first division trigeminal neuralgia (TN) are rare and very similar periorbital unilateral pain syndromes. Few cases of SUNCT are associated with posterior skull lesions. We describe a 54-year-old man with symptoms compatible with both the previous painful syndromes, associated with a small posterior skull and a cerebellar hypoplasia. The short height and the reported bone fractures could be compatible with a mild form of osteogenesis imperfecta, previously described in one case associated with SUNCT. However, a hypoplastic posterior cranial fossa characterizes also Chiari I malformation. The difficult differential diagnosis between SUNCT and TN and their relation with posterior skull malformations is debated.     

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 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.     

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     

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.