SUNCT syndrome: Prolonged attacks, refractoriness and response to topiramate

Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) syndrome is a rare form of strictly unilateral headache accompanied by cranial autonomic features. The pain is abrupt in onset, of short duration, stabbing or burning in quality, and accompanied by marked ipsilateral cranial autonomic features, usually lacrimation and conjunctival injection.1 The natural history of the condition is poorly understood. Generally, a clustering pattern is reported, with active phases erratically alternating with remissions. During symptomatic periods, the frequency of attacks varies from less than 1 per day to more than 30 per hour. Functional MRI in spontaneous attacks has demonstrated activation of the ipsilateral posterior hypothalamic gray.2 We describe a patient with SUNCT syndrome with previously unreported responsiveness to topiramate, who has atypical relatively long-lasting pain paroxysms and refractoriness after attacks. A 63-year-old medical practitioner presented with a 3-year history of headache. He described strictly right-sided episodes of severe, shooting or burning pain centered on the temple and forehead with occasional radiation to the orbit. The pain usually lasted 30 to 45 seconds but could range from 2 seconds to 1 minute. He had up to 15 …