SUNCT, SUNA and short-lasting unilateral neuralgiform headache attacks: Debates and an update.

BACKGROUND: Short-lasting unilateral neuralgiform headache attacks (SUNHA) have the features of both short-lasting unilateral neuralgiform pain, such as trigeminal neuralgia or stabbing headache, and associated trigeminal autonomic symptoms, such as paroxysmal hemicrania or cluster headache. Recognizing and adequately treating SUNHA is essential but current treatment methods are ineffective in treating SUNHA. METHODS: We reviewed the changes in the concept of short-lasting unilateral neuralgiform headache attacks and provide a narrative review of the current medical and surgical treatment options, from the first choice of treatment for patients to treatments for selective intractable cases. RESULTS: Unlike the initial impression of an intractable primary headache disorder affecting older men, SUNHA affects both sexes throughout their lifespan. One striking feature of SUNHA is that the attacks are triggered by cutaneous or intraoral stimulation. The efficacy of conventional treatments is disappointing and challenging, and preventive therapy is the mainstay of treatment because of highly frequent attacks of a very brief duration. Amongst them, lamotrigine is effective in approximately two-third of the patients with SUNHA, and intravenous lidocaine is essential for the management of acute exacerbation of intractable pain. Topiramate, oxcarbazepine and gabapentin are considered good secondary options for SUNHA, and botulinum toxin can be used in selective cases. Neurovascular compression is commonly observed in SUNHA, and surgical approaches, such as neurovascular compression, have been reported to be effective for intractable cases. CONCLUSIONS: Recent advances in the understanding of SUNHA have improved the recognition and treatment approaches for this unique condition.

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

BACKGROUND: Short-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. METHODS: Patients 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. RESULTS: The 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. CONCLUSIONS: In 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.

Pain Relief in Short-Lasting Unilateral Neuralgiform Headache with Conjunctival inJection and Tearing Syndrome with Intravenous Ketamine: A Case Report.

PURPOSE: Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) is a rare form of primary headache, classified as trigeminal autonomic cephalalgia. Since the underlying mechanism of the pathogenesis has not yet been determined, a standardized therapeutic strategy for SUNCT is unavailable. We present a case of SUNCT syndrome with successful pain relief by intravenous administration of ketamine, an N-methyl-D-aspartate receptor (NMDAR) antagonist. CASE REPORT: A 56-year-old male patient reported severe throbbing and shooting pain in forehead, temporal and periorbital region. We confirmed conjunctival injection, lacrimation, blepharoptosis, and miosis as symptoms related to autonomic activity, and made a diagnosis of SUNCT based on ICHD-3 beta. Numerous treatments were attempted, including pregabalin, gabapentine, nonsteroidal antiinflammatory drugs, acetaminophen, steroids, antidepressants, triptans, nerve blocks, and intravenous lidocaine with unsatisfactory results. Intravenous administration of ketamine (0.4 mg/kg) for one hour, was found to relieve the severe pain. CONCLUSION: Intravenous ketamine can effectively treat SUNCT syndrome. This case demonstrated that involvement of NMDAR could be one of the mechanisms of SUNCT syndrome pathogenesis and establish a therapeutic strategy for this pain syndrome.

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

INTRODUCTION: The 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. METHODS: In 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. RESULTS: The 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. CONCLUSIONS: We 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.

Ketamine as a potential option in the treatment of short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing.

A number of treatment options have been used over the years in short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) with variable results. The most common preventive treatments include carbamazepine, lamotrigine, indomethacin, gabapentin and topiramate. Ketamine is being increasingly used in the treatment of neuropathic pain. The parentral formulations are generally used as oral preparations have poor bioavailability. Recently, ketamine lozenges have been shown to have sufficiently high bioavailability to support their use as a preventive treatment in a number of conditions causing intractable neuropathic pain. We report a 58-year-old man whose symptoms of SUNCT were not responsive to conventional preventive treatments but responded well to a subcutaneous, sub-anaesthetic ketamine infusion and subsequently, sublingual ketamine lozenges.

An unusual case of short-lasting unilateral neuralgiform headache attacks.

BACKGROUND: Short-lasting unilateral neuralgiform headaches include those with conjunctival injection and tearing and with cranial autonomic symptoms. Most frequently reported as idiopathic, there is a growing number of symptomatic cases described. CASE REPORT: A 57-year old man presented a 16-year history of right hemifacial short-lasting pain attacks accompanied by ipsilateral autonomic symptoms and simultaneous malar contractions. Brain MRI disclosed a right acoustic neuroma compressing the right facial nerve and a venous developmental anomaly perpendicular to the right facial nerve root entry zone, without lesions affecting the trigeminal nerve. He was started on lamotrigine, resulting in complete remission of pain attacks, autonomic signs and facial contractions. CONCLUSIONS: This patient presents a typical short-lasting unilateral neuralgiform headache with response to lamotrigine. The uniqueness of the case is the co-occurring malar contractions, evocative of facial nerve involvement. We speculate whether facial nerve compression renders this nerve more susceptible to triggering during a short-lasting unilateral neuralgiform headache attack.

[A case of short-lasting unilateral neuralgiform headache with conjunctival injection and tearing triggered by mumps meningitis in a patient with recurrent primary stabbing headache].

A 32-year-old man with a 16-year history of recurrent primary stabbing headache was admitted to our hospital, owing to mumps meningitis. On day 2 of admission, he began experiencing episodes of unbearable intermittent stabbing pain, each lasting few seconds, with conjunctival injection and tearing, on the temporal side of the left orbit. We suspected trigeminal autonomic cephalgias, and administered non-steroidal anti-inflammatory drugs (NSAIDs), oxygen, and sumatriptan; however, the pain episodes persisted. Subsequently, after the start of intravenous lidocaine administration, the pain episodes stopped. We diagnosed a short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT), triggered by mumps meningitis. This is a valuable case report, involving a patient with a history of primary stabbing headache who was diagnosed with SUNCT triggered by mumps meningitis.

SUNCT like syndrome preceding acute ophthalmic- distribution zoster: A case report and review of the literature.

BACKGROUND: SUNCT like syndrome has been observed as a post zoster condition, as a syndrome associated with overt ophthalmic zoster after appearance of herpetic lesions and in varicella zoster virus meningoencephalitis without rash. However, SUNCT like syndrome fully consistent and congruent with the criteria of the ICHD immediately before onset of an ophthalmic-distribution zoster has not been reported. CASE PRESENTATION: We report 73-year-old patient with a SUNCT like syndrome as the first presentation of an acute ophthalmic-distribution zoster. Symptomatic treatment was highly effective, after antiviral treatment with acyclovir no further medication was required. CONCLUSIONS: SUNCT like syndrome can be the initial clinical presentation immediately preceding an acute ophthalmic-distribution zoster. This is congruent with previous observations of zoster - related SUNCT like syndromes with different temporal relationship to zoster. Zoster - related SUNCT like syndromes seem to respond well to symptomatic treatment. Clinicians should ask and look for history and signs of ophthalmic zoster in SUNCT like headaches.

Post herpes zoster SUNCT like syndrome: Insights from two case reports.

Background SUNCT like syndrome secondary to post herpes zoster infection has not been reported in literature. Case We are reporting two cases of SUNCT like syndrome secondary to post herpes zoster infection of the V1 distribution of the trigeminal nerve. Treatment with pregabalin and lamotrigine achieved complete symptomatic relief in both patients. Conclusion SUNCT like syndrome can occur after herpetic infection of the trigeminal nerve. Unlike primary SUNCT syndrome, post-herpetic SUNCT like syndrome seems to respond well to pharmacological treatment and has a good prognosis.

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

Background 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 two rare headache syndromes classified broadly as Trigeminal Autonomic Cephalalgias (TACs). Methods Here, 65 SUNCT (37 males) and 37 SUNA (18 males) patients were studied to describe their clinical manifestations and responses to treatment. Results Pain 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. Conclusions SUNCT and SUNA are rare primary headache disorders that are distinct and very often tractable to medical therapy.

Therapeutical approaches to paroxysmal hemicrania, hemicrania continua and short lasting unilateral neuralgiform headache attacks: a critical appraisal.

BACKGROUND: Hemicrania continua (HC), paroxysmal hemicrania (PH) and short lasting neuralgiform headache attacks (SUNCT and SUNA) are rare syndromes with a difficult therapeutic approach. The aim of this review is to summarize all articles dealing with treatments for HC, PH, SUNCT and SUNA, comparing them in terms of effectiveness and safety. METHODS: A survey was performed using the pubmed database for documents published from the 1st January 1989 onwards. All types of articles were considered, those ones dealing with symptomatic cases and non-English written ones were excluded. RESULTS: Indomethacin is the best treatment both for HC and PH. For the acute treatment of HC, piroxicam and celecoxib have shown good results, whilst for the prolonged treatment celecoxib, topiramate and gabapentin are good options besides indomethacin. For PH the best drug besides indomethacin is piroxicam, both for acute and prolonged treatment. For SUNCT and SUNA the most effective treatments are intravenous or subcutaneous lidocaine for the acute treatment of active phases and lamotrigine for the their prevention. Other effective therapeutic options are intravenous steroids for acute treatment and topiramate for prolonged treatment. Non-pharmacological techniques have shown good results in SUNCT and SUNA but, since they have been tried on a small number of patients, the reliability of their efficacy is poor and their safety profile mostly unknown. CONCLUSIONS: Besides a great number of treatments tried, HC, PH, SUNCT and SUNA management remains difficult, according with their unknown pathogenesis and their rarity, which strongly limits the studies upon these conditions. Further studies are needed to better define the treatment of choice for these conditions.

A case of secondary SUNCT syndrome.

SUNCT syndrome, a rare form of primary headaches, may be secondary to pituitary tumours. The secondary forms usually related with prolactinomas. The response of dopamin agonists could be variable. In this study, we reported a case of SUNCT syndrome secondary to prolactinoma. Cranial magnetic resonance imaging was performed for this patient because of the increase in pain severity and frequency. A hemorrhage was detected into the prolactinoma ipsilateral to the pain. The headache attacks were taken undercontrol and remission was ensured with cabergoline in a short time.

Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing-like attacks in a pediatric patient found to have a pontine capillary telangiectasia and developmental venous anomaly: A case report exploring the root of the problem.

Introduction Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT)-like attacks are rarely reported in the pediatric population and may remain undiagnosed and under-investigated as a result. Case presentation We present a case of a 15-year-old male with intermittent, episodic, right-sided brief headaches most in keeping with SUNCT, initially diagnosed as paroxysmal hemicrania, but with no response to indomethacin. The pain was likewise not responsive to typical migraine treatments or steroids. Management and outcome Contrast-enhanced magnetic resonance imaging demonstrated a right pontine capillary telangiectasia with an associated developmental venous anomaly that was adjacent to the root of the right trigeminal nerve. Differential diagnosis included first division trigeminal neuralgia with autonomic features. The patient's pain was partially alleviated by oxygen administration and responded well to carbamazepine; he remained pain free on carbamazepine a year later. Conclusion This case highlights the diagnostic dilemma of differentiating SUNCT from trigeminal neuralgia with autonomic features, both of which are rare diagnoses in pediatric patients, and the importance of appropriate neuroimaging to rule out secondary causes in patients presenting with trigeminal autonomic cephalalgias, recognizing that abnormalities identified on neuroimaging, such as vessels adjacent to the trigeminal nerve, may not be causative findings.

A boy with bilateral SUNA: A case report.

Background Short-lasting unilateral neuralgiform headache attacks (SUNA) is a primary headache characterized by frequent attacks of severe headaches in association with ipsilateral cranial autonomic features. SUNA is defined as a strictly unilateral pain and bilateral cases are very unusual, so secondary causes should be searched for vigorously if there are bilateral symptoms. Despite a number of therapeutic trials, effective management for the majority of SUNA patients is not available at present. Management of SUNA is often difficult. Case We report the case of a young boy with bilateral SUNA attacks, with no detected underlying cause, who is responsive to indomethacin. Conclusion Rarely, primary SUNA can present with bilateral symptoms. According to our experience in this case, indomethacin should always be offered to patients with suspected SUNA.

Botulinum Toxin A for the Treatment of a Child with SUNCT Syndrome.

Background. Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) syndrome is an unusual cause of headache, mainly described in older adults, and is rare in children. Pain attacks may be severe, frequent, and prolonged. The therapeutic benefits of many drugs are disappointing. Patient and Methods. A 12-year-old boy suffered severe headache and toothache for 20 days. As treatment with nonsteroidal anti-inflammatory drugs, anticonvulsants, and steroids proved ineffective, he was treated with ipsilateral multisite subcutaneous injections of botulinum toxin A 70 U around the orbit, the temporal area, and the upper gum. Results. The pain had reduced in frequency and severity by the fourth day after treatment and had completely disappeared after 7 days. There were no side effects or recurrence during a subsequent 17-month follow-up period. Conclusion. Botulinum toxin A can be used to treat the first episode of SUNCT in children over the age of 12 years.

SUNCT headaches after ipsilateral ophthalmic-distribution zoster.

Nine days after left ophthalmic-distribution zoster, a 47-year-old man developed SUNCT headaches (short-lasting unilateral neuralgiform headache with conjunctival injection and tearing). In contrast to two prior cases of SUNCT that developed after varicella zoster virus (VZV) meningoencephalitis without rash, this case describes an association of SUNCT with overt zoster, thus adding to the spectrum of headache and facial pain syndromes caused by VZV reactivation.