Abstract
SUNCT syndrome (short-lasting, unilateral neuralgiform headache with conjuctival injection and tearing) is a rare primary headache syndrome, originally described in 1989 by Sjaastad et al. (1). It is characterized by short lasting attacks of unilateral neuralgiform headache and prominent autonomic features in the ipsilateral eye. Diagnostic criteria (2) include at least 20 attacks of unilateral orbital or temporal pulsating or stabbing pain, lasting for 5–240 s. The frequency of the attacks must be between three and 200 per day and they should be accompanied by ipsilateral conjuctival injection and lacrimation. SUNCT has a mean age of onset at around 50 (3, 4), and only a few patients with late-onset SUNCT are described in the literature (5–8), the oldest being 77 years at onset (8). We present a case of SUNCT with onset at the age of 88 years. To the best of our knowledge this is the first report of a Greek patient with SUNCT syndrome and the first report of SUNCT onset after the age of 80 in the literature.
Case report
An 88-year-old man was admitted to the Emergency Department of Nikea General Hospital with a 3-month history of headaches. No precipitating events such as head injury or infection prior to the onset of attacks were reported. The pain was stabbing, strictly left-sided within the orbital and temporal area, lasted from 30 to 45 s and repeated 10–30 times daily. No pain triggers or refractory period after each attack were reported. The pain was accompanied by ipsilateral conjuctival injection and lacrimation, but there was no associated ptosis or rhinorrhoea. Headache was severe (average ranking 8 on a 0–10 visual scale). There was no nausea, photophobia, phonophobia or osmophobia. During attacks the patient preferred to stand still but movement did not exacerbate the pain. Past medical history included hypertension, which was well controlled with β-blockers. No past personal or family history of headaches was reported. The patient was a heavy smoker (20 cigarettes per day for 65 years). Clinical examination (both physical and neurological), routine blood tests (including erythrocyte sedimentation rate) and brain magnetic resonance imaging and magnetic resonance angiography were all normal. Previous diagnoses included trigeminal neuralgia and atypical facial pain. Carbamazepine (200 mg daily) was ineffective and withdrawn (it was pre-given by others). Lamotrigine 125 mg daily completely controlled headaches. The patient remains headache free 1 year later.
Discussion
SUNCT is a rare primary headache syndrome, slightly predominant in males and usually presenting in middle life. Its clinical characteristics and diagnostic criteria include strictly unilateral headache attacks accompanied by prominent autonomic features ipsilateral to the pain (2, 4).
SUNCT should be differentially diagnosed from trigeminal neuralgia (TN) (9). Differentiation can be clinically difficult, although autonomic symptoms are not usually met in cases of TN (4, 10), with the exception of mild lacrimation (11). TN is more common in elderly patients and usually shows good response to anticonvulsants such as carbamazepine. In contrast, SUNCT was initially considered refractory to various treatments (12). However, carbamazepine was effective in some SUNCT cases (4), though newer antiepileptics such as lamotrigine (13–15) and gabapentin (16, 17) have shown better results in case reports or small series of patients. Posterior fossa lesions have also been described to mimic the phenotype of SUNCT (18), therefore detailed neuroimaging is necessary, especially if there is a late onset of the headache.
To the best of our knowledge, there are no reports of SUNCT with onset after the age of 77 years. Our patient, aged 88, represents the oldest patient with SUNCT onset, indicating the need for clinical consideration of elderly patients with refractory facial pain.