Short-Lasting Unilateral Neuralgiform Headache Attacks with Conjunctival Injection and Tearing

Abstract

SUNCT (Short-lasting Unilateral Neuralgiform headache attacks with Conjunctival injection and Tearing) and SUNA (Short-lasting Unilateral Neuralgiform headache attacks with cranial Autonomic symptoms) are rare primary headache syndromes, classified as Trigeminal Autonomic Cephalalgias (TACs). Hypothalamic involvement in the TACs has been suggested by functional imaging data and clinically with deep brain stimulation. Fifty-two patients (43 SUNCT, 9 SUNA) were studied to determine the clinical phenotype of these conditions and response to medications. A functional imaging study explored activation of the posterior hypothalamus in attacks of SUNCT/SUNA. The clinical study characterised SUNCT and SUNA in terms of epidemiology, phenotype and clinical characteristics. Indomethacin is ineffective on single-blind testing. Intravenous lidocaine was effective in all cases. Open-label trails showed the effectiveness of lamotrigine, topiramate and gabapentin. On functional imaging there was hypothalamic activation bilaterally in 5/9 SUNCT patients, and contralaterally in two patients. Two SUNCT patients had ipsilateral negative activation. In SUNA the activation was bilaterally negative. There was no hypothalamic activation in a patient with SUNCT secondary to a brainstem lesion. The data suggests that there should be revised classification for SUNCT and SUNA, with an increased range of attack duration and frequency, cutaneous triggering of attacks, and a lack of refractory period. The concept of 'attack load' is introduced. The lack of response to indomethacin and the response to intravenous lidocaine, are useful in diagnostic and therapeutic terms, respectively. Preventive treatments include lamotrigine, gabapentin and topiramate. The role of hypothalamic involvement in SUNCT and SUNA as TACs is considered.

SUNCT and SUNA: medical and surgical treatments

“…Among the 11 patients with a favourable response, 8 of the 33 patients had a partial response and 3 had a complete or almost complete response [15]. In a recent open-label series of 36 SUNCT and 5 SUNA patients treated with carbamazepine, 40 % of SUNCT and 20 % of SUNA patients reported a favourable response [6].…”

“…Topiramate was reported to be effective in five patients at doses up to 300 mg daily [9,10]. Subsequently, 11 of 21 SUNCT patients (52 %) had a good response to topiramate in an open-label study [6].…”

“…It had a moderate-togood effect in 68 % of SUNCT patients and 25 % of SUNA patients in an open-label study. The range of daily dose varied between 25 and 600 mg [6]. In an Australian series, the response to lamotrigine depended on the subtype of SUNCT; lamotrigine was reported to produce an excellent response in episodic SUNCT, but a poor response in those with the chronic form [2].…”

“…When tried in an open-label fashion in 22 SUNCT and 5 SUNA patients at up to 3,600 mg daily, it was reported to be effective in 60 % of SUNA but only 45 % of SUNCT patients [6].…”

“…The activation of the posterior hypothalamus during attacks, demonstrated in functional neuroimaging studies [5,6], has led to the hypothesis that the posterior hypothalamus plays a crucial role in the pathophysiology of SUNCT syndrome. However, besides the occurrence of ipsilateral autonomic symptoms during attacks, there are few other similarities shared between SUNCT/SUNA and the other TACs syndromes.…”