SUNCT/SUNA: Case series presenting in an orofacial pain clinic.

AIM: Little is known about short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA). We present our experience with SUNCT/SUNA patients to aid identification and management of these disorders. METHODS: A retrospective review of patient records of one orofacial pain clinic was performed. Inclusion criteria was a diagnosis of SUNCT/SUNA confirmed with at least one follow-up visit. RESULTS: Six of the 2464 new patients seen between 2015-2018 met the selection criteria (SUNCT n = 2, SUNA n = 4). Gender distribution was one male to one female and average age of diagnosis was 52 years (range 26-62). Attacks were located in the V1/V2 trigeminal distributions, and five patients reported associated intraoral pain. Pain quality was sharp, shooting, and burning with two patients reporting "numbness". Pain was moderate-severe in intensity, with daily episodes that typically lasted for seconds. Common autonomic features were lacrimation, conjunctival injection, rhinorrhea, and flushing. Frequent triggers were touching the nose or a specific intraoral area. Lamotrigine and gabapentin were commonly used as initial therapy. CONCLUSIONS: Differentiating between SUNCT/SUNA does not appear to be clinically relevant. Presenting symptoms were consistent with those published, except 5/6 patients describing intraoral pain and two patients describing paresthesia.

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.

Tic, Triggering, and Tearing: From CTN to SUNHA.

PREMISE: Classical trigeminal neuralgia (CTN) and the short-lasting unilateral neuralgiform headache attacks (SUNHA) are clinically similar. PROBLEM: The SUNHAs include short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA). Shared clinical signs with CTN include severe, unilateral trigeminal pain that is often triggered by innocuous stimuli and accompanied by a dull persistent background pain. Recent reports on trigeminal neuralgia cases with atypical features such as autonomic signs and prolonged attack duration further blur the clinical distinction between CTN and SUNHAs. POTENTIAL SOLUTIONS: Are the similarities greater than their differences? If so, this may reflect a spectrum of disease ranging from typical CTN attacks to typical SUNHAs with a mixed phenotype in the middle. In this review they will summarize the overlap between these entities and contrast the pathophysiology and treatment approach.

Case Report: Coexistence of SUNCT and Hypnic Headache in the Same Patient.

BACKGROUND: Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and hypnic headache (HH) are two exceedingly rare and distinctly classified primary headaches. The hypothalamus seems to be a crucial region involved in the pathophysiology of both conditions, but no cases of SUNCT and HH co-occurrence have been described so far. CASE RESULTS: A 49-year-old woman who has been suffering from SUNCT for years, with alternation of symptomatic periods and remissions, developed a new headache with different clinical features, presenting exclusively during sleep and with a dramatic responsiveness to caffeine, that met the diagnostic criteria for HH. CONCLUSIONS: The available literature suggests that SUNCT and HH are different conditions but the association in the same patient that we describe supports the concept that they are not mutually exclusive. Further studies are needed to establish if they share a common pathophysiological mechanism.

SUNCT, SUNA and trigeminal neuralgia: different disorders or variants of the same disorder?

PURPOSE OF REVIEW: Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT), short-lasting unilateral neuralgiform headache attacks with autonomic symptoms (SUNA) and trigeminal neuralgia are considered different disorders, thus grouped in separate sections of the International Classification of Headache Disorders 3 beta. However, the clinical, radiological and therapeutic overlap between SUNCT, SUNA, and trigeminal neuralgia has challenged this traditional view. This review summarizes the available clinical and pathophysiological evidence on whether SUNCT, SUNA and trigeminal neuralgia should be considered separate entities or variants of the same disorder. RECENT FINDINGS: Data on the clinical phenotype and effective management strategies in SUNCT and SUNA syndromes have shown striking similarities with trigeminal neuralgia. Moreover, studies exploring radiological findings supported the hypothesis of common aetiological and pathophysiological basis between SUNCT/SUNA and trigeminal neuralgia. However, a limitation of most studies is that they have included small samples of patients and therefore any conclusions need to be drawn cautiously. SUMMARY: Despite being considered distinct conditions, emerging clinical and radiological evidence supports a broader nosological concept of SUNCT, SUNA, and trigeminal neuralgia. These conditions may constitute a continuum of the same disorder, rather than separate clinical entities. Further evidence is required to shed light on this nosological issue, given its potential impact on clinical practice and further research studies in this area.

The effect of posterior hypothalamus region deep brain stimulation on sleep.

BACKGROUND: Early observations by von Economo showed that the posterior part of the hypothalamus (PH) plays a prominent role in sleep-wake regulation. The PH is a candidate area involved in cluster headaches and other trigeminal autonomic cephalalgias (TACs) and is targeted for deep brain stimulation (DBS). CASE REPORTS: Sleep studies in two men, 69- and 39-years-old, with pre-existing sleep disorders, before and after PH-DBS for pharamacoresistant cluster headache and SUNCT syndrome showed that PH-DBS led to a dramatic alteration of the patients' sleep patterns. This coincided with an improvement of the predominantly diurnal TACs, suggesting a PH-DBS-induced change in sleep patterns. Hypnograms after DBS demonstrated disrupted sleep and a prolonged period of wakefulness after midnight in both patients, which was reproduced the second night. CONCLUSIONS: PH-DBS, a promising treatment for severe refractory TACs, affects sleep quality and pre-existing sleep disorders. This needs to be considered when treating patients with PH-DBS.

Role of trigeminal microvascular decompression in the treatment of SUNCT and SUNA.

Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) and short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA) are primary headache disorders. Evidence suggests that SUNCT/SUNA have similar pathophysiology to the trigeminal autonomic cephalalgias and involves the trigeminal autonomic reflex. This review provides an overview of microvascular decompression of the trigeminal nerve and other surgical therapeutic options for SUNCT/SUNA. We have undertaken a mini-meta-analysis of available case reports and case series with the aim of providing recommendations for the use of such therapies in SUNCT/SUNA. There is some evidence supporting microvascular decompression of the trigeminal nerve in selected patients who have medically refractory SUNCT/SUNA and a demonstrable ipsilateral aberrant vessel on magnetic resonance imaging (MRI). We also consider what further investigations could be undertaken to assess the role of surgical interventions in the treatment of these often debilitating conditions.

[Update on trigeminal autonomic cephalalgia]. / Neues zu trigeminoautonomen Kopfschmerzen.

Cluster headache, paroxysmal hemicrania and short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT syndrome) are classified under trigeminal autonomic cephalalgia (TAC). The proposed revision of the international classification of headache disorders (ICDH-3 beta) adds hemicrania continua to this diagnostic group. Moreover, diagnostic criteria of the other TACs were modified and are characterized by persistent headache or headache attacks accompanied by cranial autonomic symptoms. The main difference between the various TACs is the duration of attacks. Differentiation is important because different pharmacological strategies are necessary.

Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) with preserved refractory period: report of three cases.

BACKGROUND: Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) and short-lasting unilateral neuralgiform attacks with cranial autonomic features (SUNA) are rare primary headache syndromes characterized by spontaneous or triggered attacks of unilateral, brief, multiple, orbitofrontal pain associated with ipsilateral autonomic features. SUNCT is considered as a subset of SUNA. In SUNA, there may be cranial autonomic symptoms other than conjunctival injection and lacrimation, or either of two is present. SUNCT/SUNA can be triggered immediately after or at the decrescendo phase of the ongoing attack without any intervening refractory period. Refractory period is usually present in trigeminal neuralgia. Absent refractory period is thought to reliably differentiate SUNCT/SUNA from trigeminal neuralgia and has been proposed for inclusion into the International Classification of Headache Disorders (ICHD) diagnostic criteria for SUNCT. CASE REPORTS: We report three patients of SUNCT syndrome with preserved intervening refractory period of variable duration observed at different times. DISCUSSION: Trigeminal neuralgias with autonomic features, SUNA and SUNCT share a common pathophysiological mechanism and actually represent a continuum. It is well known that patient with trigeminal neuralgia may transform into SUNCT/SUNA. Similarly, being a continuum, the presence or the absence of refractory period and its duration may change in a patient with SUNCT/SUNA at different time points. CONCLUSION: The presence of refractory period should not exclude the diagnosis of SUNCT in a patient with other clinical features suggestive of SUNCT.

SUNCT syndrome successfully treated by gamma knife radiosurgery: case report.

BACKGROUND: The SUNCT syndrome (short-unilateral neuralgiform headache with conjunctival injection and tearing) can be very disabling for affected patients and is often refractory to medical management. We report the first case of SUNCT with a successful response to stereotactic radiosurgery without any adverse effect. CASE: After failing optimal medical treatment, a 82-year old male patient suffering from SUNCT syndrome was treated with Gamma knife radiosurgery. The trigeminal nerve and sphenopalatine ganglion were targeted with a maximum dose of 80 Gy each. The patient had complete pain cessation 2 weeks after the treatment, and remains pain-free with no medication at the latest follow-up 39 months after radiosurgery. He did not have any side effect from the procedure. CONCLUSION: Gamma knife radiosurgery is an option for medically refractory SUNCT patients.

Deep brain stimulation of the posterior hypothalamic area in intractable short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT).

BACKGROUND: SUNCT (short-lasting unilateral neuralgiform headache with conjunctival injection and tearing) is a rare syndrome characterized by the sudden onset of excruciating unilateral periorbital pain that is accompanied by conjunctival injection and lacrimation or further autonomic signs. Similar to patients with chronic cluster headache, Leone and Lyons showed a beneficial effect of deep brain stimulation of the posterior hypothalamic region in two patients with a chronic SUNCT. CASE: Here, we present the case of a man with a chronic SUNCT responding to deep brain stimulation of the posterior hypothalamic area. CONCLUSION: This case supports the idea of a central origin of SUNCT and shows that deep brain stimulation of the hypothalamic region can be effective in the treatment of the chronic form of this rare disorder.

The role of lamotrigine in the treatment of short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing syndrome.

OBJECTIVE: To evaluate the efficacy of lamotrigine for treatment of short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) syndrome. DATA SOURCES: Literature was accessed through MEDLINE (1950-June 2010) using the terms lamotrigine, triazines, SUNCT, and trigeminal autonomic cephalgia. STUDY SELECTION AND DATA EXTRACTION: All articles in English and studies conducted in humans were identified and evaluated. DATA SYNTHESIS: SUNCT syndrome can be an extremely challenging headache type to manage and has been considered refractory to pharmacotherapy. Many anticonvulsants have been evaluated as promising SUNCT treatments, with lamotrigine specifically reported as an effective first-line treatment option. There is a lack of randomized placebo-controlled clinical trials evaluating lamotrigine in SUNCT syndrome therapy; however, 2 observational studies, 3 case series, and 5 case reports were reviewed. Lamotrigine appears to decrease the frequency and severity of SUNCT attacks, leading to complete resolution in some patients. A decrease in symptoms was achieved with doses ranging from 25 to 600 mg/day. In some cases, there was initial response to low doses, but dosage titrations were often necessary when symptoms returned several days after being managed at the same dose. Lamotrigine should be initiated at 25 mg/day and gradually titrated, guided by response and adverse effects. The risk of Stevens-Johnson syndrome, a dose-related adverse effect, can be minimized with gradual titration. CONCLUSIONS: According to case reports and observational studies, lamotrigine therapy has resulted in decreased frequency or resolution of SUNCT syndrome attacks. Randomized, controlled trials are necessary to confirm the efficacy of lamotrigine for this indication.

Methylprednisolone therapy for short-term prevention of SUNCT syndrome.

Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) is characterized by severe and frequent daily pain attacks causing transient physical disability for the patients during the headache period. Currently there is no option for abortive treatment of the attacks, mainly due to the short-lived nature and frequency of the repeated headaches, while highly efficacious therapy is also unavailable for short-term prevention. We report rapidly suppressed headache attacks with orally administered methylprednisolone in eight headache periods of three patients with idiopathic, episodic SUNCT syndrome. The remission was maintained until the period was over in all cases. Although the mechanism of methylprednisolone action is unclear, it is probably based on the anti-inflammatory effects of the drug.

SUNCT syndrome with paroxysmal mydriasis: Clinical and pupillometric findings.

SUNCT syndrome (short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing) is a primary headache characterised by a high frequency of attacks associated with marked autonomic periocular signs and symptoms. Activation of the hypothalamus via the superior salivary nucleus is probably responsible for some of the autonomic involvement observed during SUNCT attacks. We describe a case of SUNCT with unusual autonomic features (e.g., mydriasis) and early onset. Pupillometric studies were performed both in a basal condition (without anisocoria) and after instillation of phenylephrine (a drug with direct sympathomimetic activity) and pilocarpine (a parasympathetic agonist). The findings in this patient seem to indicate involvement of the ocular sympathetic supply in SUNCT, responsible for the mydriasis, and seem to strengthen the possibility that the autonomic phenomena in this syndrome vary with different levels of pain severity.

Trigeminal autonomic cephalalgias: paroxysmal hemicrania, SUNCT/SUNA, and hemicrania continua.

The trigeminal autonomic cephalalgias (TACs) are a group of primary headache disorders that include cluster headache (CH), paroxysmal hemicrania (PH), and short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing/cranial autonomic features (SUNCT/SUNA). Hemicrania continua (HC) is often included with this group, although the second edition of The International Classification of Headache Disorders did not link the entities. Trigeminal autonomic cephalalgias are generally characterized by relatively short-lasting attacks of severe pain and lateralized associated features including the pain, cranial autonomic symptoms, and where present, migrainous symptoms, such as photophobia. Paroxysmal hemicrania has intermediate duration and intermediate attack frequency. Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing has the shortest attack duration and the highest attack frequency. Hemicrania continua has a continuous pain with exacerbations that can include cranial autonomic symptoms as part of the phenotype. The syndromes share much in their pathophysiology and investigation paths; however, their treatment is distinct, so that the accurate differentiation is important for optimal management.

SUNCT responsive to percutaneous balloon compression of the gasserian ganglion--10-year follow-up.

We report the case of a woman with short-lasting unilateral, neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) whose severe headache attacks ceased after percutaneous balloon compression of the Gasserian ganglion. The patient remains pain free after 10-year follow-up. This may be the first literature report of SUNCT in Chile.

A case of early-onset and monophasic trigeminal autonomic cephalalgia: could it be a SUNCT?

A 2-year-old female came to the Neurological Emergency Room of "Giovanni XXIII" Hospital in Bari, 6 h after the onset of severe facial pain, which occurred soon after awakening. Stabbing pain affected the right frontal and periorbital area, with ipsilateral conjunctival injection, swelling of the eyelids and tearing. Except the duration, from 5 to 30 s., the attacks were stereotyped including the occurrence and features of autonomic signs. Based on the typical clinical findings and the normal magnetic resonance imaging (MRI), we diagnosed short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing syndrome (SUNCT). The spontaneous remission within a few hours made prophylactic therapy unnecessary. At the last follow-up, after 3 months, the patient was still symptom free. In our case, after an active period lasting 2 days the disease disappeared completely. However the typical features of the disease (unilateral pain, short duration and high frequency of the attacks, autonomic signs ipsilateral to pain, numbers of attacks) were all present. While the diagnostic criteria of the International Headache Society classification for SUNCT did not include the duration of disease, it is likely that the active period lasting 2 days could be an expression of the clinical variability of the disease.

Different forms of trigeminal autonomic cephalalgias in the same patient: description of a case.

The trigeminal autonomic cephalalgias (TACs), including cluster headache, paroxysmal hemicrania and SUNCT, are characterized by the cardinal combination of short-lasting unilateral pain and autonomic phenomena affecting the head. Hemicrania continua (HC) shares many clinical characteristics with TACs, including unilateral pain and ipsilateral autonomic features. Nevertheless, HC is separately classified in the revised International Classification of Headache Disorders (ICHD-II). Here, we describe the case of a 45-year-old man presenting an unusual concurrence of different forms of primary headaches associated with autonomic signs, including subsequently ipsilateral cluster headache, SUNCT and HC. This report supports the theory that common mechanisms could be involved in pathophysiology of different primary headache syndromes.

SUNA syndrome with seasonal pattern.

SUNA is a trigeminal autonomic cephalalgia (TAC) characterized by short unilateral attacks centered on the ophthalmic trigeminal distribution, and accompanied by at least one of a number of cranial autonomic symptoms that can include lacrimation, redness of the ipsilateral eye, nasal congestion, rhinorrhea, and eyelid edema. It exists in episodic and chronic form. We have described an atypical case of episodic SUNA with an exclusive seasonal pattern as previously reported in other trigeminal autonomic cephalalgia, commonly known as TACs.