Abstract
Introduction
SUNCT syndrome is a clinical condition described by Sjaastad et al. (1) characterized, as the acronym SUNCT denotes, by Shortlasting, Unilateral, Neuralgiform headache attacks, with Conjunctival injection, Tearing and rhinorrhoea. This clinical entity could be classified into Goadsby and Lipton's ‘trigeminal-autonomic cephalalgias’ (2).
The SUNCT syndrome is generally refractory to drug therapy. We present a patient, with a 17-year history of pain, compatible with SUNCT syndrome, and refractory to multiple drugs, but with a persistent therapeutic response to surgical treatment.
Case report
A 68-year-old woman presented a 17-year history of severe left-side pain, involving the eye, front and temple. Her general health was otherwise good, and there was no a family history of migraine or other primary headaches.
The pain was excruciating, with a burning quality but no throbbing. The attacks lasted from 5 s to 1 min. The pain was associated with ipsilateral conjunctival injection, lacrimation, rhinorrhoea and eyelid oedema. Some attacks were provoked by speaking, swallowing, chewing, yawning, facial movements and physical efforts. There was no nausea or vomiting. The frequency of the attacks was from 30 to more than 100 a day with complete pain resolution between attacks. She referred no period of remission.
The general exploration was unremarkable, as were the ear, nose, and ophthalmological examinations. The neurological findings were without pathological significance. In the attacks observed by us, eyelid oedema and ptosis were prominent symptoms.
Routine blood and urine analyses were unremarkable. The x-ray study of the sinuses and chest were normal. Magnetic resonance imaging (MRI) of the brain showed no significant findings.
In her long-lasting evolution, the patient had been treated with many drugs (analgesics, indomethacin, naproxen, amitriptyline, neuroleptics, corticoesteroids) without improvement. Carbamazepine had little effect, and was abandoned because of side effects. Gabapentine was tried for 3 months with no response. An anaesthetic block proved equally ineffective.
Neurosurgical treatment was proposed and accepted by the patient. In June 1998 she was operated upon in the Department of Neurosurgery (Ciutat Sanitaria i Universitaria de Bellvitge, Barcelona, Spain). Percutaneous left trigeminal ganglion compression (Mullan's technique) (3) was performed, with complete pain relief from the early post-operative period. In the 18 months since surgery, the patient has remained asymptomatic, with the exception of a single attack 6 months ago that lasted approximately 15 s, followed by spontaneous resolution.
Discussion
We present a patient with strictly unilateral short-lasting pain, associated with autonomic symptoms (conjunctival injection, tearing, eyelid oedema, etc.). Autonomic features are found in cluster headache (CH), chronic paroxysmal hemicrania (CPH) and rarely in first division (V1) trigeminal neuralgia (4–6). However, in CH and CPH there are generally no trigger points in the attacks, and the symptoms are more persistent; on the other hand in V1 trigeminal neuralgia the attacks are shorter and the autonomic manifestations and signs are less pronounced (7). We think that our case, because of the short duration of the pain attack, the presence of autonomic disorders and numerous triggering mechanisms, is compatible with SUNCT (8). Our case, in agreement with other cases described, proved highly refractory to drug therapy. The patient has been practically free of pain since the surgery. To our knowledge this is the first fully reported case of SUNCT to respond to surgical treatment.