Short-lasting unilateral neuralgiform headache with conjuctival injection and tearing (SUNCT) is a rare headache syndrome characterised by unilateral orbital or temporal pain that is stabbing or throbbing in quality and of moderate severity, lasting 5–240 s, associated with conjuctival injection and tearing. Less common autonomic symptoms include sweating of the forehead and rhinorrhoea. There should be at least 20 attacks per day to fulfil the International Headache Society criteria for the diagnosis of SUNCT [1]. It is differentiated from paroxysmal hemicranias by its unresponsiveness to indomethacin. SUNCT is also refractory to most drugs used in the treatment of other short-lasting headaches. While most cases are primary (idiopathic), SUNCT can be symptomatic and due to cerebellopontine angle arteriovenous malformations [2], brainstem cavernous angioma [3], cerebellopontine angle astrocytoma or pituitary adenomas [4, 5]. We report a patient with symptomatic SUNCT due to vascular compression of the trigeminal nerve, who had the ability to ‘‘switch off’’ the paroxysms of pain by neck flexion. The patient underwent microvascular decompression resulting in a complete cure. A 55-year-old man presented with a 10-year history of paroxysms of ‘‘electric shock-like’’ pain which affected the left side of the face, particularly the left eye, but sometimes spreading to affect the temporal region. Triggers included eating, talking, shaving and touching the face, although some of the attacks occurred spontaneously. The attacks were short-lived lasting only seconds and were associated with lacrimation and injection of the left eye; on average, he experienced approximately 20–60 attacks of pain per day. He discovered that he was often able to ‘‘switch off’’ the pain by a sudden movement of combined flexion and rotation of the neck. The initial movement was lateral rotation either to the left or the right and then rapid neck flexion, bringing his chin down to the sternum. This would stop the occurrence of any pain for the next few minutes to 1 h. The attacks were unresponsive to indomethacin, gabapentin, prednisolone, amitriptyline, phenytoin and pregabalin. He had a definite but inadequate response to carbamazepine and lamotrigine. Topiramate gave him good pain relief but the effect wore off after 6 months. He did not derive any benefit from radiofrequency ablation. The pain was so severe that he had to give up his job 5 years after symptom-onset. Neurological examination was normal. Magnetic resonance imaging of the brain, using thin slices (0.7 mm) of the heavily weighted T2 sequence showed compression of the left fifth cranial nerve by the left superior cerebellar artery (see Fig. 1). The patient underwent microvascular decompression. Intra-operatively, the left superior cerebellar artery was noted to be abutting the left trigeminal nerve root, with obvious pulsatile lateral displacement of the root. The patient had complete symptom relief after surgery, was successfully weaned off all medication and remains asymptomatic 18 months later. There have been a few case reports of symptomatic SUNCT in association with compression of the trigeminal nerve [6, 7]. The association, however, is not as robust as in trigeminal neuralgia and the imaging findings are sometimes E. Chaila (&) E. Ali M. Hutchinson Department of Neurology, St Vincent’s University Hospital, Elm Park, Dublin 4, Ireland e-mail: cholachaila@yahoo.ie
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Jan 1, 2022
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