SUNCT and SUNA: An Update.

BACKGROUND: Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and short-lasting unilateral neuralgiform headache with autonomic symptoms (SUNA) are rare and disabling primary headache disorders that are subtypes of Short-lasting unilateral neuralgiform headache attacks (SUNHA). AIM: The aim of this narrative review was to provide a comprehensive update on headache phenotype, pathophysiology, and various treatment options available for SUNCT and SUNA. METHODS: References for this review were identified by searches of articles published in the English language in PubMed between 1978 and October 2020 using "short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT)", "short-lasting unilateral neuralgiform headache with autonomic symptoms (SUNA)", "short-lasting unilateral neuralgiform headache attacks (SUNHA)", "trigeminal autonomic cephalalgias" as keywords in various combinations. RESULTS: Of a potential 1103 articles, seven case series describing clinical characteristics of SUNCT/SUNA patients were identified for this review. For symptomatic/secondary SUNCT/SUNA, 53 individual case reports, and one case series were reviewed. One placebo-controlled trial and 11 open-label case series that evaluated various medical and surgical treatments in SUNCT/SUNA were also reviewed. Available literature suggests that SUNCT and SUNA are subtypes of the same disorder characterized by severe side locked short duration headache with ipsilateral prominent cranial autonomic symptoms and signs. Pathophysiology may involve both peripheral and central mechanisms. Lamotrigine is the most effective preventive therapy while intravenous lidocaine is the most efficacious drug as transitional therapy for severe disabling attacks. Surgical options including microvascular decompression in those having neurovascular conflict, occipital nerve stimulation, and hypothalamic deep brain stimulation can be alternative treatment options for medically refractory patients.

[Clinical Aspects of Short-Lasting Unilateral Neuralgiform Headache Attacks].

Short-lasting unilateral neuralgiform headache attacks (SUNHA), within the group of trigeminal autonomic cephalalgias, are characterized by repetitive, brief and severe attacks. The SUNHA includes short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA). SUNCT is diagnosed if both conjunctival injection and tearing occur ipsilateral to the pain, whereas SUNA is diagnosed if only one, or neither, of conjunctival injection and tearing occurs. Previously considered to be rare and rather refractory to treatment, there is increasing awareness of this syndrome and the therapeutic possibilities. During the worst periods, intravenous lidocaine may decrease the intensity of SUNHA. Lamotrigine is the most-effective prophylactic treatment. For medically intractable SUNHA, several surgical approaches, i.e., microvascular decompression of the trigeminal nerve and neuromodulation, have been tried. There is considerable clinical, therapeutic and radiological overlap between SUNCT, SUNA, and trigeminal neuralgia. Despite being considered distinct conditions, the emerging evidence suggests 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. This article discusses the clinical aspects of the syndrome, including pathophysiology, differential diagnosis, radiological imaging and treatment. (Received May 26, 2020; Accepted June 16, 2020; Published November 1, 2020).

Headache in the Older Population: Causes, Diagnoses, and Treatments.

PURPOSE OF REVIEW: Primary headaches are less common and differ in presentation in older versus younger individuals. Secondary headaches become more common among older patients. RECENT FINDINGS: Diagnosis and management of headaches in those > 65 years are discussed. Migraine and tension-type headaches are rarely new onset in this age group and should be a diagnosis of exclusion. In older individuals, migraine is more likely to be bilateral with less sensory sensitivities. Migraine aura may present without headache; careful assessment is needed to exclude stroke. Other primary headaches discussed include cough, hypnic, and other headaches. Secondary causes discussed include giant cell arteritis, trigeminal post-herpetic neuropathy, sleep apnea, cardiac cephalgia, cervicogenic pain, vascular etiologies, medications, and burning-mouth syndrome. In older individuals, primary headaches are diagnoses of exclusion, and treatment is affected by comorbidities and polypharmacy. Secondary headaches are a major consideration requiring appropriate workup. Many treatments can safely be offered regardless of age.

Microvascular decompression or neuromodulation in patients with SUNCT and trigeminal neurovascular conflict?

Objectives To assess the effectiveness of neuromodulation and trigeminal microvascular decompression (MVD) in patients with medically-intractable short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT). Methods Two patients with medically refractory SUNCT underwent MVD following beneficial but incomplete response to neuromodulation (occipital nerve stimulation and deep brain stimulation). MRI confirmed neurovascular conflict with the ipsilateral trigeminal nerve in both patients. Results Although neuromodulation provided significant benefit, it did not deliver complete relief from pain and management required numerous postoperative visits with adjustment of medication and stimulation parameters. Conversely, MVD was successful in eliminating symptoms of SUNCT in both patients with no need for further medical treatment or neuromodulation. Conclusion Neuromodulation requires expensive hardware and lifelong follow-up and maintenance. These case reports highlight that microvascular decompression may be preferable to neuromodulation in the subset of SUNCT patients with ipsilateral neurovascular conflict.

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.

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.

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

BACKGROUND: 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. RESULTS: 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. CLINICAL IMPLICATIONS: 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.

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.

Chronic paroxysmal hemicrania, hemicrania continua and SUNCT: the fate of the three first described cases.

The first patient with chronic paroxysmal hemicrania has been followed for 45 years, and for 33 years with indomethacin treatment. The headache became less severe with time; there was no indomethacin tachyphylaxis. The first patient with SUNCT was followed for 28 years, until his demise at 89. Pain became worse with time. No adequate therapy was found. The first patient with Hemicrania continua was followed for 19 years, until her demise at 81. She was treated with indomethacin during the whole observation time. There was no tachyphylaxis. Both patients treated with indomethacin developed gastric ulcer. And both had gastric surgery. Indomethacin therapy may be a life-long affair. The risk of gastric complications may be substantial.