Coexistence of SUNCT and aura? A case report

In the February issue of Cephalalgia, Lambru et al. describe 10 patients in whom SUNCT (short-lasting unilateral neuralgiform headache with conjunctival injection and tearing) or SUNA (short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms) coexisted with hemiplegic migraine. As both are very rare conditions, the authors suggest that these two primary headache disorders could respond to a common underlying mechanism, with sodium channelopathy being an attractive potential candidate (1).

We report on a case associating SUNCT syndrome and symptoms suggestive of migraine aura. An otherwise healthy 39-year-old woman without a previous headache history was sent to our headache clinic by a general neurologist due to a refractory headache. She presented a three-year history of recurrent episodes (average 10–15 per day) of severe, lancinating/electrical pain located over the left periocular region. The episodes lasted between one and two minutes (median around 90 seconds when timing) and were accompanied by ipsilateral, intense lacrimation, conjunctival injection and rhinorrhea. At least two days per month and always subjectively coinciding with days in which she experienced a higher number of these episodes, she referred to progressive blurring of vision, reaching vision loss, in her right hemifield, lasting one to two hours followed by speech difficulties (she could not find words or speak fluently), also lasting one to two hours. Neurological examination, routine laboratory determinations and neuroimaging (brain CT, MRI with and without gadolinium and angioMRI) were all unremarkable. The patient had been treated with therapeutic doses of beta blockers, indomethacin, valproic acid and even pericranial injections of botulinum toxin type A with no response. She could not tolerate topiramate. She had to give up her work as a professional photographer. We recommended trying lamotrigine up to 100 mg/12 hours. After a six-month follow-up, she has spectacularly improved, referring to an average of two to four “mild” episodes per week and being able to work again.

Although we cannot state a definite association between SUNCT and aura from this isolated clinical observation, our patient would be the first SUNCT case coexisting with a “migraine” aura, which could be considered as typical in development and clinical content, except for its rather prolonged duration. This association would support the hypothesis that SUNCT syndrome and aura could share a common pathophysiological mechanism. Considering the rarity of SUNCT, these findings are not totally surprising, as there are well-documented cases of typical aura co-occurring with all the remaining trigeminal autonomic cephalalgias, (2) including cluster headache, (3) paroxysmal hemicrania (4,5) and hemicrania continua (6,7). Finally, the clear response of both pain episodes and aura to lamotrigine (8) also supports Lambru et al.’s hypothesis that a sodium channel dysfunction could be the underlying mechanism explaining the pathophysiology of both SUNCT and aura.