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.

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.

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 pituitary tumors: clinical characteristics and treatment.

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 rare types of trigeminal autonomic cephalalgias (TACs). Objective To describe a series of patients with SUNCT and SUNA including relationship to pituitary tumors. METHOD: All patients diagnosed with SUNCT or SUNA in the Calgary Headache Assessment and Management Program were reviewed. RESULTS: Six patients (five SUNCTs and one SUNA) were identified. The pain was severe, sharp, showed fixed-laterality, involved mainly the orbito-fronto-temporal region and was associated with autonomic symptoms. Attack duration ranged from 3 to 300 seconds and frequency was 1-200 paroxysms/day. MRI showed ipsilateral pituitary adenomas to the pain in five out of five of the SUNCT patients. Patients with adenomas underwent surgery. Pathology included three prolactinomas, and one mixed adenoma and gangliocytoma. One patient has remained headache free for 4 years after surgery. One was pain free for a year, and then headaches returned with tumor recurrence. Another had major improvement, and two have not improved. Patients were generally refractory to medications. CONCLUSION: All five of our patients with typical SUNCT had pituitary tumors, with headache ipsilateral to the pituitary tumors in all cases. Tumor removal provided major improvement in three out of five patients. Medical treatment was only partially effective.

Where SUNCT contacts TN: a case report.

BACKGROUND: 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. CASE: In this paper, we report a new case with the main purpose to shine a light on the pathophysiology of these conditions. CONCLUSION: 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.

SUNA and red ear syndrome: a new association and pathophysiological considerations.

Red ear syndrome (RES) is characterised by attacks of unilateral or bilateral burning ear pain associated with erythema. Primary and secondary forms have been described. Primary RES appears to have a frequent association with primary headaches especially migraine. Here, we describe the case of a woman with short-lasting unilateral neuralgiform attacks with cranial autonomic symptoms (SUNA) and recurrent episodes of ipsilateral red ear triggerable by cutaneous stimulation. Lamotrigine was beneficial for her SUNA but not for the RES. Both these disorders are extremely rare therefore their coexistence in the same individual may suggest similar pathophysiological mechanisms rather than a chance association.

Coexistence of hemiplegic migraine with SUNCT or SUNA: a case series.

BACKGROUND: Hemiplegic migraine (HM) is a rare subtype of migraine with aura that includes transient hemiparesis of different degrees. The dominantly inherited type, called familial hemiplegic migraine, along with the sporadic type, in which no family history of similar attacks exists, have been recently recognized by the revised International Classification of Headache Disorders (ICHD-2). 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 also very rare conditions that are classified as trigeminal autonomic cephalalgias. This report provides the first description of an association between HM and SUNCT/SUNA. CASES: Eight females and two males with both these disorders were identified between 2007 and 2010. CONCLUSION: The coexistence of such rare primary headache disorders is probably due to a common underlining mechanism, with a sodium channelopathy being an attractive potential candidate.

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.

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.

A case of ocular hypertension complicated by SUNCT syndrome.

We report a 53-year-old woman with laser iridotomy (LI)-resistant angle-closure and conjunctival injection, which was thought to be the cause of ciliochoroidal effusion associated with short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) syndrome. LI had no effect on any of the symptoms except for intraocular pressure. The symptoms disappeared after a subsequent procedure for SUNCT syndrome. MRI of the left eye showed ciliochoroidal effusion at paroxysm and was normalized upon relief.

SUNCT and optic nerve hypoplasia.

SUNCT has been reported in association with abnormalities of the brainstem and pituitary region. We present a patient with a history of left optic nerve hypoplasia, mild hypothalamic-pituitary dysfunction, and SUNCT starting in adolescence. SUNCT with an early age of onset may be associated with congenital abnormality of the hypothalamic-pituitary axis.

Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing: response to antiepileptic dual therapy.

Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) is included among trigeminal autonomic cephalalgias in the International Classification of Headache Disorders-2. Available literature suggests that it responds to anticonvulsants, particularly lamotrigine. However, management of partial responders is difficult and antiepileptic duo-therapy may be an answer to it. Nonetheless, to our knowledge, anticonvulsant combination has never been tried in partial responders to monotherapy. We are presenting a case of SUNCT that had overlapping symptoms with Short-lasting Unilateral Neuralgiform headache attacks with cranial Autonomic features and responded well only to the combination of lamotrigine with carbamazepine. However, lamotrigine had to be stopped as patient developed leucopenia and it resulted in partial recurrence of symptoms.

[Symptomatic SUNCT with cerebral abscess and subdural empyema]. / Syndrome SUNCT symptomatique d'abcès et d'empyème cérébraux.

SUNCT syndrome is a rare form of a primary headache disorder, although secondary causes, particularly posterior fossa abnormalities, are well known. We report a new case in a 67-year-old man suffering SUNCT syndrome secondary to pyogenic cerebral abscess and empyema localized in the convexity portion of the right frontal lobe.

[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.

Congestión nasal como síntoma autonómico acompañante de crisis migrañosas / Nasal congestion as an autonomic symptom accompanying migraine attacks

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Whiplash injury-induced atypical short-lasting unilateral neuralgiform headache with conjunctival injection and tearing syndrome treated by greater occipital nerve block.

BACKGROUND: Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) syndrome is a rare type of trigeminal autonomic cephalalgia. SUNCT syndrome is typically idiopathic, and secondary SUNCT syndrome after indirect injury is very rare. We report a case of SUNCT syndrome manifested dramatically after a whiplash injury and treated by greater occipital nerve (GON) block. CASE: A 62-year-old woman presented with headache associated with ipsilateral orbitofacial pain, lacrimination, and conjunctival injection after a car accident. Imaging study revealed no acute lesions. The symptoms were triggered and worsened by touch on the ipsilateral scalp and posterior neck region, and also by talking, swallowing, or chewing. We performed GON blocks for headache 3 times. The patient experienced pain relief after a GON block. CONCLUSIONS: Secondary SUNCT syndrome induced by whiplash injury is rare. Our case showed good outcome of SUNCT syndrome treated by GON.