SUNCT Research Articles

Trigeminal autonomic cephalgias

Review of literature on the trigeminal autonomic cephalgias are presented. Trigeminal autonomic cephalgias are primary headaches with phenotype consisting of trigeminal pain with autonomic sign including lacrimation, rhinorrhea and miosis. Discussed are issues of classification, pathogenesis, clinical picture, diagnosis, differential diagnosis and treatment of this headache. Special attention is paid to cluster headache, paroxysmal hemicrania, SUNCT syndrome, hemicrania continua.

Protocolo diagnóstico y terapéutico de la cefalea crónica

Chronic cephalea is defined as a headache that occurs 15 or more days a month and for more than 3 months. Chronic migraine, in addition to this frequency of headaches, has migraine characteristics. Prophylactic treatment is based on the use of amitriptyline, propranolol, antiepileptic drugs, verapamil and/or tizanidine. Chronic tension cephalea involves oppressive pain, mild phonophobia or photophobia but with no clear general malaise. The symptoms improve with amitriptyline, mirtazapine, venlafaxine, antiepileptic drugs and tizanidine. Chronic neuralgiform cephalea lasts more than a year without periods of remission or with periods of remission that last less than a month. This condition includes the following forms: cephalea in chronic clusters, which is treated with verapamil, lithium carbonate, valproic acid, steroids or topiramate;chronic paroxysmal hemicrania, which is treated with indomethacin; chronic SUNCT (short-lasting unilateral neuralgiform headache with conjunctival injection and tearing), which is treated with antiepileptic drugs, lithium and steroids; continuous paroxysmal hemicrania (characterized by unilateral pain, conjunctival injection, autonomic symptoms, miosis and unilateral palpebral ptosis and restlessness), which responds well to indomethacin.

EHMTI-0302. SUNCT syndrome, case report

The short-lasting primary headache syndromes may be conveniently divided into those exhibiting marked autonomic activation and those without autonomic activation. The former group comprises chronic and episodic paroxysmal hemicrania, short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT syndrome) and cluster headache. SUNCT is relatively rare, with a recent study showing a prevalence of 6.6/100,000 and an incidence of 1.2/100,000. The disorder has a male preponderance, with a sex ratio of 2:1.The typical age of onset is between 40 and 70 years, with a mean age of onset at 48 years.

Therapy of trigeminal autonomic headaches

Trigeminal autonomic cephalgias (TAC) are characterized by severe and strictly unilateral headaches with a frontotemporal and periorbital preponderance in combination with ipsilateral cranial autonomic symptoms, such as lacrimation, conjunctival injection, rhinorrhea, nasal congestion, and restlessness or agitation. One main differentiating factor is the duration of painful attacks. While attacks typically last 5s to 10min in SUNCT syndrome (short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing), paroxysmal hemicrania lasts 2-30min and cluster headaches 15-180min. Hemicrania continua represents a continuous TAC variant. From a therapeutic view, TACs differ substantially. Lamotrigine is used as first-choice prevention in SUNCT syndrome and indometacin in paroxysmal hemicrania. For cluster headaches, acute therapy with inhaled pure oxygen and fast-acting triptans (sumatriptan s.c. and intranasal zolmitriptan) is equally important to short-term preventive therapy with methysergide and cortisone and long-term prophylactic treatment comprising verapamil as drug of first choice and lithium carbonate and topiramate as drugs of second choice. In refractory cases of chronic cluster headache, neuromodulatory approaches such as occipital nerve stimulation and sphenopalatine ganglion stimulation are increasingly applied.

First Reported Case of SUNCT Syndrome Resolved by Resection of Pituitary Tumor in Pediatric Patient

First Reported Case of SUNCT Syndrome Resolved by Resection of Pituitary Tumor in Pediatric Patient

Lidocaína endovenosa no tratamento da síndrome SUNCT secundária a tumor de hipófise: seguimento de uma paciente por um período de quatro anos. Relato de caso

JUSTIFICATIVA E OBJETIVOS: A sindrome SUNCT (short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing syndrome) e descrita como dor de localizacao orbitaria, supraorbitaria ou temporal unilateral, caracterizada como em pontada ou pulsatil, de forte intensidade, incapacitante, de curta duracao, variando entre 5 e 240 segundos. E uma sindrome dolorosa de dificil tratamento, no entanto, no presente caso obteve-se resposta analgesica satisfatoria com lidocaina endovenosa. RELATO DO CASO: Paciente do genero feminino, 32 anos portadora de sindrome SUNCT secundaria a tumor de hipofise (submetida a hipofisectomia transesfenoidal e radiocirurgia), passou a apresentar 30 crises de dor diariamente. Nao obteve resposta analgesica com o procedimento cirurgico. Foi iniciado tratamento farmacologico com lamotrigina, clorpromazina e amitriptilina sem alivio da dor. Submetida a ciclos de infusao de lidocaina venosa obteve adequado alivio da dor. CONCLUSAO: Neste caso de sindrome SUNCT, a lidocaina endovenosa foi capaz de proporcionar analgesia adequada e duradoura, podendo ser considerada como parte integrante nos pacientes portadores dessa de sindrome.

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.

The effect of posterior hypothalamus region deep brain stimulation on sleep

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). 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. 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.

Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing caused by a pituitary adenoma

Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) syndrome is a rare primary headache syndrome first described in 1978. We report on a 43-year-old man with a 10 year history of SUNCT in whom a pituitary macroadenoma was eventually detected. His pain rapidly improved with medical treatment of the prolactinoma and we propose that this is a case of symptomatic SUNCT.

Frameless linac-based stereotactic radiosurgery treatment for SUNCT syndrome targeting the trigeminal nerve and sphenopalatine ganglion

The short-lasting unilateral neuralgiform headache associated with conjunctival injection and tearing or SUNCT syndrome was first described in the 1970s. This paper is the first in the literature that describes the successful use of stereotactic radiosurgery (SRS) using a non-invasive frameless technique, targeting both the trigeminal nerve and the sphenopalatine ganglion in the management of intractable SUNCT. We also discuss the role of selecting peripheral targets in the management of this rare headache syndrome. Among patients treated for functional pain disorders in our radiosurgery unit using the frameless technique since August 2011, one patient with symptoms matching the International Classification of Headache Disorders-2 (ICHD-II) criteria of SUNCT syndrome was identified. The multi-disciplinary case records of this patient were retrospectively reviewed and reported. Our patient had symptoms resembling the ICHD-II diagnostic criteria of SUNCT, which was refractory to medical treatment. Ninety Gy was delivered to the trigeminal root entry zone and 80 Gy was delivered to the sphenopalatine ganglion. At 16 months' follow-up, she was pain free with minimal side effects. Frameless linear accelerator (linac)-based SRS targeting the trigeminal nerve and sphenopalatine ganglion remained successful in our patient at 16 months. Longer follow-up and further experience will determine the efficacy and safety of this approach. We suggest that frameless SRS is a convenient and attractive non-invasive option for patients with medically refractory SUNCT.

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.

Both trigeminal autonomic cephalalgia and trigeminal neuralgia in a child

Short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA) are an uncommon syndrome [1] similar in frequency, duration, setting and severity to short-lasting unilateral neuralgiform headache attack with conjunctival injection and tearing (SUNCT syndrome). SUNCT and SUNA belong to a group of headache syndromes classified as Trigeminal Autonomic Cephalalgias (TACs) and were included in the second edition of the International Classification of Headache Disorders [2]. Some studies have focused on the association between Trigeminal Neuralgia (TN) and TACs with short-lasting attacks [3]. To our best knowledge, no cases of SUNA in childhood have been reported so far. We describe a child with a SUNA syndrome associated with TN probably triggered by inflammatory lesion of nasal mucosa. In April 2006, a 9-year-old child with Norrie disease [4] became agitated and complained of facial pain. Those attacks persisted for approximately half an hour, had a frequency of 2–3 per day and were refractory to NSAIDs. A few days before the beginning of attacks, the patient presented purulent rhinorrhea. An otorhinolaryngoiatric examination evidenced a piece of a toy into the right nostril. The toy was removed but it did not cure the pain. Ophthalmologic examination, brain magnetic resonance imaging, dental scan and EEG were normal. From September to November 2006, the child was treated with Pregabalin and then with Topiramate, without benefit on pain. At the time of our observation, in November 2006, the pain started to be well localized: it involved the forehead, the orbital and temporal region, prevalently on the right-side. Two types of attack were observed: single stabs (clusters with length of attack in range from a few seconds to 5 min) and another one lasting about 30 min. Some of the pain accesses were self-controlled by the patient by massaging the painful area. He had two to three attacks every day, with alternate prevalence of side: pain was accompanied by ipsilateral conjunctival injection (but not tearing), rhinorrhea, eyelid oedema and sweating. The patient was treated with various drugs (Pregabalin, Carbamazepine, Gabapentin), but only the association of Gabapentin and Lamotrigine modified the type of painful attacks. In fact, attacks affected the second division of the trigeminal nerve (V2) without cranial autonomic symptoms. Most of the attacks were still right-sided, lasting from a fraction of a second to 2 min, showing TN characteristics [2]. In June 2007, the accesses became more frequent and intense. The pain accesses were spontaneous or were triggered by the wind, chew, brushing, hot or cold feed. Oxcarbazepine (1200 mg/die) associated with Lamotrigine (200 mg/die) was introduced with a gradual discontinuation of Gabapentin, showing an almost complete remission. Reduction of therapy caused the reappearance of attacks so, at the moment, the child continues this therapy showing sporadic episodes of pain. The headache of this child fulfils the diagnostic criteria for chronic SUNA [2]. He has had more than 20 attacks R. Valeria P. Concetta P. Mariotti (&) Unit of Child Neuropsychiatry, Catholic University, L.go A. Gemelli, 8, 00168 Rome, Italy e-mail: pm@mclink.it

Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing syndrome secondary to an epidermoid tumor in the cerebellopontine angle

Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) syndrome is classified under trigeminal autonomic cephalalgias. This rare headache syndrome is infrequently associated with secondary pathologies. In this paper the authors report on a patient with paroxysmal left retroorbital pain with associated autonomic symptoms of ipsilateral conjunctival injection and lacrimation, suggestive of SUNCT syndrome. After failed medical treatment an MRI sequence was obtained in this patient, demonstrating an epidermoid tumor in the left cerebellopontine angle. The patient's symptoms completely resolved after a gross-total resection of the tumor. This case demonstrates the effectiveness of resection as definitive treatment for SUNCT syndrome associated with tumoral compression of the trigeminal nerve. Early MRI studies should be considered in all patients with SUNCT, especially those with atypical signs and symptoms.

Where SUNCT Contacts TN: A Case Report

Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) and trigeminal neuralgia (TN) are unilateral painful conditions that can share the same triggering factors, autonomic features and the main location, as well as the cyclically recurrent crises. Both these syndromes are associated with a high percentage of findings of vascular malformation touching the trigeminal nerve, suggesting a pathophysiological relationship. In this paper, we report a new case with the main purpose to shine a light on the pathophysiology of these conditions. Many authors described a SUNCT case deriving from TN or vice versa, suggesting that these conditions are strongly related. Every case of transformed TN or SUNCT should therefore be reported to gather and compare further information.

Complete remission of SUNCT syndrome by intravenous glucocorticoid treatment

Complete remission of SUNCT syndrome by intravenous glucocorticoid treatment

Trigemino-autonome Kopfschmerzen – neue Aspekte und Konzepte

Cluster headache, paroxysmal hemicrania and SUNCT syndrome are a group of primary headaches classified as "trigeminal autonomic cephalgias" (TACs) and characterised by relatively short attacks of unilateral head pain associated with ipsilateral craniofacial autonomic manifestations. Duration of attacks is the main feature to distinguish between the three forms of TACs. Modern functional neuroimaging indicates that the hypothalamus may play a crucial role in the pathophysiology of these headaches. Therapeutic regimes consist of acute treatment as well as application of prophylactic medication. After unsuccessful application of conservative treatment options, invasive neuromodulative procedures are finally justified. These treatments should be rather of neurostimulative than of neurodestructive intentions.

SUNCT syndrome findings accompanied by cavernous segment aneurysm

SUNCT syndrome findings accompanied by cavernous segment aneurysm

Orofacial pain due to trigeminal autonomic cephalgia with features of short-lasting neuralgiform headache attacks with conjunctival injection and tearing: a case report

We present a case of a 64-year-old woman with a presumptive diagnosis of short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) syndrome with telangiectasia. Dental procedures were not successful in alleviating the condition. The patient's symptoms of short unilateral severe pain episodes abated after geographic relocation, although orofacial pain continued. Sphenoid sinus surgery further decreased the patient's chronic pain complaints. The patient's current pain condition is controlled with gabapentin therapy. Diagnostic, etiologic, and therapeutic issues related to SUNCT syndrome are discussed. This case represents the first case report of trigeminal autonomic cephalgia with SUNCT syndrome-like features illustrating possible problematic dental therapies. It is only the third SUNCT case report in the dental literature, and the third case reporting a correlation between SUNCT syndrome and sinusitis.

Sustained response to botulinum toxin in SUNCT syndrome

Short-lasting, unilateral, neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) is a rare condition grouped in the category of trigeminal autonomic cephalalgia. The syndrome is characterized by very frequent, unilateral attacks of pain in the ocular and periocular region accompanied by ipsilateral conjunctival injection and lacrimation. The aetiology is unknown, although there have been reports of cases secondary to structural lesions, and treatment is usually ineffective. We present the case of a patient diagnosed with SUNCT refractory to pharmacological treatment; duration of the SUNCT was 20 years. OnabotulinumtoxinA was infiltrated at four points around the orbit. The pain showed a dramatic response to onabotulinumtoxinA infiltration. Efficacy has been maintained for 18 months with 3-monthly infiltrations, with no adverse effects. OnabotulinumtoxinA should be added to the limited therapeutic arsenal available for the treatment of refractory cases of SUNCT.

SUNCT syndrome treated with gamma knife targeting the trigeminal nerve and sphenopalatine ganglion

SUNCT syndrome, an abbreviation for short lasting-unilateral neuralgiform headache attacks with conjunctival injection and tearing is one of the most debilitating unilateral headache syndromes often refractory to medical therapy [1]. We report a case of a 50-year-old man diagnosed with refractory SUNCT syndrome having a near complete response to gamma knife surgery targeting the trigeminal nerve and sphenopalatine ganglion.