SUNA Patients Research Articles

A Retrospective Comparative Study in Patients with SUNA and SUNCT.

Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA) are rare primary headache disorders. The aim of the study is to describe and compare the clinical characteristics of patients with SUNA and SUNCT. Patients with SUNCT or SUNA observed in a neurology clinic of a tertiary hospital in India between January 2017 and December 2022 were evaluated. Thirteen patients with SUNA (seven female, 54%) and 16 patients with SUNCT (nine female, 56%) were identified for the evaluation. The mean ages at the onset of SUNA and SUNCT were 36.8.5 ± 8.1 years and 37.2 ± 8.4 years, respectively. The age of onset in our patients was somewhat younger than that of other large series. The demographic and clinical features of SUNA patients were comparable to those of SUNCT patients. Orbital/retro-orbital area was the most common site of pain in both types of headaches. The pattern of pain was noted as single stab (in all patients), repetitive stabs (SUNA vs. SUNCT: 77% vs. 75%), and sawtooth patterns (SUNA vs. SUNCT: 23% vs. 25%). The majority of attacks in both groups lasted less than two minutes. Conjunctival injection and tearing were present in all SUNCT patients (as a part of the diagnostic criteria). The prevalence of conjunctival injection and tearing in SUNA was 46% and 31%, respectively. All patients reported spontaneous attacks. Triggers were reported in seven (54%) patients with SUNA and nine (56%) with SUNCT. Only one patient in each group had a refractory period following a trigger-induced episode. Two patients in the SUNCT group had compression of the trigeminal nerve by a vascular loop. This is the largest case series from India. There were no significant differences between patients with SUNA and SUNCT.

Similarities and differences between SUNCT and SUNA: a cross-sectional, multicentre study of 76 patients in China

BackgroundShort-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA) have not been evaluated sufficiently due to limited data, particularly in China.MethodsPatients with SUNCT or SUNA treated in a tertiary headache centre or seven other headache clinics of China between April 2009 and July 2022 were studied; we compared their demographics and clinical phenotypes.ResultsThe 45 patients with SUNCT and 31 patients with SUNA had mean ages at onset of 37.22 ± 14.54 years and 42.45 ± 14.72 years, respectively. The mean ages at diagnosis of SUNCT and SUNA were 41.62 ± 12.70 years and 48.68 ± 13.80 years, respectively (p = 0.024). The correct diagnosis of SUNCT or SUNA was made after an average of 2.5 (0–20.5) years or 3.0 (0–20.7) years, respectively. Both diseases had a female predominance (SUNCT: 1.14:1; SUNA: 2.10:1). The two diseases differed in the most common attack site (temporal area in SUNCT, p = 0.017; parietal area in SUNA, p = 0.002). Qualitative descriptions of the attacks included stabbing pain (44.7%), electric-shock-like pain (36.8%), shooting pain (25.0%), and slashing pain (18.4%). Lacrimation was the most common autonomic symptom in both SUNCT and SUNA patients, while eyelid oedema, ptosis, and miosis were less frequent. Triggers such as cold air and face washing were shared by the two diseases, and they were consistently ipsilateral to the attack site.ConclusionsIn contrast to Western countries, SUNCT and SUNA in China have a greater female predominance and an earlier onset. The shared core phenotype of SUNCT and SUNA, despite their partial differences, suggests that they are the same clinical entity.

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

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...

Trigeminal neurovascular contact in SUNCT and SUNA: a cross-sectional magnetic resonance study.

Emerging data-points towards a possible aetiological and therapeutic relevance of trigeminal neurovascular contact in short lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and perhaps in short lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA). We aimed to assess the prevalence and significance of trigeminal neurovascular contact in a large cohort of consecutive SUNCT and SUNA patients and evaluate the radiological differences between them. The standard imaging protocol included high spatial and nerve-cistern contrast resolution imaging acquisitions of the cisternal segments of the trigeminal nerves and vessels. MRI studies were evaluated blindly by two expert evaluators and graded according to the presence, location and degree of neurovascular contact. The degree of contact was graded as with or without morphological changes. Neurovascular contact with morphological changes was defined as contact with distortion and/or atrophy. A total of 159 patients (SUNCT = 80; SUNA = 79) were included. A total of 165 symptomatic and 153 asymptomatic trigeminal nerves were analysed. The proportion of neurovascular contact on the symptomatic trigeminal nerves was higher (80.0%) compared to the asymptomatic trigeminal nerves (56.9%). The odds on having neurovascular contact over the symptomatic nerves was significantly higher than on the asymptomatic nerves [odds ratio (OR): 3.03, 95% confidence interval (CI) 1.84-4.99; P < 0.0001]. Neurovascular contact with morphological changes were considerably more prevalent on the symptomatic side (61.4%), compared to the asymptomatic side (31.0%) (OR 4.16, 95% CI 2.46-7.05; P < 0.0001). On symptomatic nerves, neurovascular contact with morphological changes was caused by an artery in 95.0% (n = 77/81). Moreover, the site of contact and the point of contact around the trigeminal root were respectively proximal in 82.7% (67/81) and superior in 59.3% (48/81). No significant radiological differences emerged between SUNCT and SUNA. The multivariate analysis of radiological predictors associated with the symptomatic side, indicated that the presence of neurovascular contact with morphological changes was strongly associated with the side of the pain (OR: 2.80, 95% CI 1.44-5.44; P = 0.002) even when adjusted for diagnoses. Our findings suggest that neurovascular contact with morphological changes is involved in the aetiology of SUNCT and SUNA. Along with a similar clinical phenotype, SUNCT and SUNA also display a similar structural neuroimaging profile, providing further support for the concept that the separation between them should be abandoned. Furthermore, these findings suggest that vascular compression of the trigeminal sensory root, may be a common aetiological factor between SUNCT, SUNA and trigeminal neuralgia thereby further expanding the overlap between these disorders.

Phenotypic and treatment outcome data on SUNCT and SUNA, including a randomised placebo-controlled trial.

BackgroundShort-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA) are two rare headache syndromes classified broadly as Trigeminal Autonomic Cephalalgias (TACs).MethodsHere, 65 SUNCT (37 males) and 37 SUNA (18 males) patients were studied to describe their clinical manifestations and responses to treatment.ResultsPain was almost always unilateral and side-locked. There were three types of attack: Single stabs, stab groups, and a saw-tooth pattern, with some patients experiencing a mixture of two types. As to cranial autonomic symptoms, SUNA patients mainly had lacrimation (41%) and ptosis (40%). Most cases of the two syndromes had attack triggers, and the most common triggers were touching, chewing, or eating for SUNCT, and chewing/eating and touching for SUNA. More than half of each group had a personal or family history of migraine that resulted in more likely photophobia, phonophobia and persistent pain between attacks. For short-term prevention, both syndromes were highly responsive to intravenous lidocaine by infusion; for long-term prevention, lamotrigine and topiramate were effective for SUNCT, and lamotrigine and gabapentin were efficacious in preventing SUNA attacks. A randomized placebo-controlled cross-over trial of topiramate in SUNCT using an N-of-1 design demonstrated it to be an effective treatment in line with clinical experience.ConclusionsSUNCT and SUNA are rare primary headache disorders that are distinct and very often tractable to medical therapy.

EHMTI-0272. Microvascular decompression of the trigeminal nerve in the treatment of medically intractable SUNCT and SUNA

Aims We report the outcome of MVD, using Jannetta procedure, in nine cases of medically intractable chronic SUNCT and SUNA patients. Methods Nine patients with chronic SUNCT and SUNA with a vascular loop indenting or distorting the trigeminal nerve ipsilaterally to the side of the pain demonstrable on MRI scan were offered MVD of the trigeminal nerve. All the patients failed to respond or tolerate adequate dosages of: lamotrigine, topiramate, oxcarbazepine, carbamazepine, duloxetine, pregabalin or gabapentin and greater occipital nerve blocks. Intravenous lidocaine improved the pain only during infusion in some of them. Results Seven SUNCT and two SUNA patients underwent MVD of the trigeminal nerve. They all had multiple daily severe headache attacks at baseline. At a median follow-up of 15 months (range: 8-30 months) after the operation, four patients became and remained pain free, one patient each had 90%, 70% and 50% improvement, respectively. The patient who improved by 50% was rendered pain free on preventive treatments. Two patients became pain free for 12 months, before pain returned. One patient suffered a CSF leak after the procedure. Conclusions MVD of the trigeminal nerve was effective in 7/9 SUNCT/ SUNA patients (78%). Although longer follow-up is needed to establish the long term outcome of this procedure, these preliminary data support the role of MVD in the surgical management of SUNCT and SUNA. No conflict of interest.

EHMTI-0198. Importance of neurovascular conflict with the trigeminal nerve in SUNCT and SUNA

Recently, a small case series showed the presence of neurovascular conflict with the trigeminal nerve in a significant proportion of SUNCT and SUNA patients, suggesting a possible pathophysiological overlap between SUNCT, SUNA and trigeminal neuralgia (TN).

Occipital Nerve Stimulation in the Treatment of Medically Intractable SUNCT and SUNA

Background: Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) or with cranial autonomic symptoms (SUNA) are primary headaches characterized by frequent attacks of severe headaches in association with cranial autonomic features. Patients with chronic SUNCT or SUNA have unremitting symptoms that necessitate prolonged use of medical preventive treatments, many of which are prone to causing side effects. They can be medically intractable, in which case neurally destructive or cranially invasive surgical treatments can be offered, though these have hitherto yielded conflicting results. Occipital nerve stimulation (ONS) offers a nondestructive and relatively low risk surgical alternative. Objective: To assess the efficacy and safety of ONS in chronic, medically intractable SUNCT and SUNA patients. Study design: Prospective open-label study. Methods: Nine medically intractable, chronic SUNCT and SUNA patients were implanted with electrodes for bilateral occipital nerve stimulation. Data were collected prospectively for pre- and postimplantation headache characteristics, including frequency, intensity and duration of attacks. Diaries were used to assess headache improvement. Results: At a median follow-up of 38 months (range 24–55 months), all but one patient reported substantial improvement. Four patients became pain-free, 3 were almost pain-free (96 – 98% improvement), and one had a marked reduction in attack frequency and severity (81% improvement). After an initial rapid improvement, the maximum benefit of ONS was attained after a lag of a few months. Device malfunction was followed by recurrence or worsening of the attacks within a few days in most patients. Adverse events included lead migration, exposure of the electrode, and pain due to muscle recruitment over the leads. One patient developed hemicrania continua one month after implantation and was successfully treated with indomethacin. Conclusion: ONS appears to offer an effective and safe treatment option, without significant morbidity, for medically intractable SUNCT and SUNA. Given the variable results with cranially invasive or neurally destructive surgery, ONS might be considered the surgical treatment of choice for medically intractable SUNCT and SUNA. Key words: SUNCT, SUNA, occipital nerve stimulation, trigeminal autonomic cephalalgias, neuromodulation, peripheral nerve stimulation, trigeminal neuralgia, greater occipital nerve blockade

POH04 Treatment of medically intractable SUNCT and SUNA with occipital nerve stimulation

ObjectivesTo report on the outcome and follow-up of 7 medically intractable SUNCT and SUNA patients treated with ONS.BackgroundSUNCT and SUNA are primary headaches characterised by repeated attacks of very severe...