SUNCT Syndrome in the Elderly

SUNCT syndrome (Short-lasting unilateral Neuralgiform headache attacks with Conjunctival Injection and Tearing) is a rare form of strictly unilateral headache with prominent autonomic symptoms (1). Diagnostic criteria are now established which require at least 20 attacks of unilateral, moderately severe, orbital or temporal, stabbing of throbbing pain, lasting for 5–240 s and associated with ipsilateral conjunctival injection and lacrimation (2).

The syndrome is slightly predominant in males, with a mean age of onset at around 50 (3). Most primary headache syndromes present in early adulthood to middle age, yet there are case reports of SUNCT or SUNCT–like syndrome with onset in later life. Five patients with onset after the age of 70 have been reported; the reported ages of onset are 71 (4), 73 (5), 74 (6, 7), and 77 (8). We report a case of an elderly woman whose SUNCT syndrome started at the age of 75, and who presented to our clinic at the age of 85. Her misdiagnosis as trigeminal neuralgia illustrates the importance of recognizing SUNCT in this age group.

Case report

An 85-year-old-woman attended our clinic in May 2003. She had been suffering from headaches for 10 years, since the age of 75. She had yearly bouts of pain for 2–3 weeks since then, apart from a 2-month bout in the last year. The pain was strictly left sided, periorbital facial pain with radiation to the back of the head. The pain would come as rapid-onset electric-shock-like jabs which would last for 2 min in a saw-tooth pattern and continue with 2–5 minute intervals over 3 h. She would have these groups of attacks 3 times a day, and they could also wake her at night. There was associated ipsilateral conjunctival injection, lacrimation, rhinorrhoea and ptosis. There was no nausea, photophobia, phonophobia or feeling of agitation. Movement would not exacerbate the pain. Triggers included touching the left side of her face and eating. There was no refractory period after each attack.

Past medical history was significant for hypercholesterolaemia, which was well controlled, and a hiatus hernia. Family history was significant for migraine in her daughter although there was no history of headaches in the patient apart from the current complaint. She was a lifelong nonsmoker who lived alone although her son-in-law was involved in her care. Neurological examination, including facial sensation, was entirely normal.

She was taking simvastatin, omeprazole and carbamazepine 100 mg; the latter had a mild effect in reducing the attacks during her last bout but caused her significant unsteadiness. At the time of her presentation to clinic she was coming to the end of a bout of attacks and was therefore not started on any further treatment.

She had an MRI scan of the brain including posterior fossa structures and trigeminal nerves, which was normal. Full blood count and erythrocyte sedimentation rate were normal.

Discussion

SUNCT syndrome is one of the Trigeminal Autonomic Cephalgias (TACs) which are characterized as being unilateral headache attacks with prominent autonomic symptoms such as ptosis, eyelid oedema, conjunctival injection, lacrimation, nasal congestion and rhinorrhoea (9). SUNCT is included in the second edition of the International Headache Society Classification (2).

The differential diagnosis of SUNCT includes trigeminal neuralgia (10). SUNCT is differentiated by its predominance of autonomic symptoms, which are relatively lacking in the latter syndrome (11, 12), although not entirely absent (13). Trigeminal neuralgia is commoner in elderly patients and the idiopathic form is often highly responsive to antiepileptics such as carbamazepine (14). SUNCT, on the other hand, is generally considered to be more difficult to treat (15), although recently open clinical trials suggest that lamotrigine may be useful (7, 16–18), and certainly many patients with SUNCT can have a limited response to carbamazepine (3).

This patient may have been diagnosed as having trigeminal neuralgia on account of her age of onset. However given the phenotype of her attacks, including the lack of refractory period and the prominence of autonomic features, the diagnosis is much more in keeping with SUNCT (3). It is necessary both in trigeminal neuralgia and SUNCT in this age group to perform detailed imaging studies of the posterior fossa and trigeminal nerve, to detect any mass or vascular lesions on the nerve which may account for the pain.

As far as we are aware there are no reports of SUNCT syndrome presenting at the age 85. In one case the patient's attacks resolved after a contralateral hemispheric stroke at the age of 80 (6). Our patient would then represent the oldest patient with ongoing SUNCT syndrome. Other patients in the literature in their eighties would include those at the ages of 80 (6), 81 (1, 8) and 83 (5). This is not simply exotica as the case illustrates the need for detailed history taking in elderly patients who have been diagnosed as having trigeminal neuralgia, who may indeed have SUNCT, and would be better treated with alternate strategies.