Medical treatment of SUNCT and SUNA: a prospective open-label study including single-arm meta-analysis

Abstract

IntroductionThe management of short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA) remains challenging in view of the paucity of data and evidence-based treatment recommendations are missing.MethodsIn this single-centre, non-randomised, prospective open-label study, we evaluated and compared the efficacy of oral and parenteral treatments for SUNCT and SUNA in a real-world setting. Additionally, single-arm meta-analyses of the available reports of SUNCT and SUNA treatments were conducted.ResultsThe study cohort comprised 161 patients. Most patients responded to lamotrigine (56%), followed by oxcarbazepine (46%), duloxetine (30%), carbamazepine (26%), topiramate (25%), pregabalin and gabapentin (10%). Mexiletine and lacosamide were effective in a meaningful proportion of patients but poorly tolerated. Intravenous lidocaine given for 7–10 days led to improvement in 90% of patients, whereas only 27% of patients responded to a greater occipital nerve block. No statistically significant differences in responders were observed between SUNCT and SUNA. In the meta-analysis of the pooled data, topiramate was found to be significantly more effective in SUNCT than SUNA patients. However, a higher proportion of SUNA than SUNCT was considered refractory to medications at the time of the topiramate trial, possibly explaining this isolated difference.ConclusionsWe propose a treatment algorithm for SUNCT and SUNA for clinical practice. The response to sodium channel blockers indicates a therapeutic overlap with trigeminal neuralgia, suggesting that sodium channels dysfunction may be a key pathophysiological hallmark in these disorders. Furthermore, the therapeutic similarities between SUNCT and SUNA further support the hypothesis that these conditions are variants of the same disorder.

Trigeminal neuralgia: a practical guide

“…30 A recent large prospective open-label study conducted in 161 patients on the medical treatments of SUNCT/SUNA confirmed the efficacy of sodium channel blockers, indicating also a therapeutic overlap with TN. 31 Taken together these pieces of evidence suggest that SUNCT, SUNA and TN may constitute a continuum of the same disorder. 32 Given the challenges in differentiating between them, table 2 summarises the differences between TN and SUNCT/SUNA.…”

Central Nervous System Involvement of Multiple Myeloma Presenting as Short-lasting Unilateral Neuralgiform Headache with Conjunctival Injection and Tearing: A Case Report

“…Cases of SUNCT/SUNA have demonstrated exquisite response to IV lidocaine therapy (with efficacy in up to 90% of patients). 12 Our patient tolerated both IV lidocaine and IV DHE and experienced transient resolution of headache which rapidly recurred following cessation of the infusions. Ultimately, treatment with intrathecal chemotherapy resulted in sustained pain relief.…”

Trigeminal microvascular decompression for short-lasting unilateral neuralgiform headache attacks

“…This was a single-centre, non-randomized, prospective open-label study aiming to evaluate the efficacy of trigeminal MVD to medically intractable chronic SUNHA patients who had failed medical treatments and who showed ipsilateral trigeminal NVC on MRI with dedicated trigeminal nerve sequences. 9 …”

“… 7 , 8 A recent large prospective open-label study conducted in 161 patients on the medical treatments of SUNCT/SUNA confirmed the efficacy of sodium channel blockers, also indicating a therapeutic overlap with TN. 9 Given the known tolerability issues of sodium channel blockers, especially at high doses often required to control SUNHA symptoms, an unknown although probably high proportion of patients become refractory to medical treatments, thereby justifying surgical approaches.…”