Gamma Knife Radiosurgery for SUNCT: A Case Series.

BACKGROUND: The treatment of medically refractory patients with chronic short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) is challenging. Stereotactic radiosurgery targeting the trigeminal nerve and sphenopalatine ganglion (SPG) has been used as a less-invasive treatment. The outcomes of this procedure have been described in a few case reports. OBJECTIVES: The objective of the study was to report on the effect of Gamma Knife radiosurgery (GKRS) in 5 patients with chronic SUNCT. METHODS: Retrospective review of our GKRS database identified 5 patients with chronic SUNCT who underwent GKRS targeted to the trigeminal nerve and SPG. A maximum dose of 80-85 Gy and 80 Gy was, respectively, delivered to the trigeminal nerve and SPG. Pain intensity and facial numbness were evaluated using the Barrow Neurological Institute (BNI) scores. RESULTS: These 5 patients were clinically followed for a mean period of 26.2 months. Within a period ranging from 2 days to 9 months, GKRS was successful in reducing pain attacks and autonomic symptoms in all 5 patients. At the last assessments, BNI pain scores of I, II, and IIIa were achieved in 1, 1, and 3 patients, respectively. Two patients developed nonbothersome facial numbness (BNI facial numbness score II). CONCLUSIONS: These 5 cases show that GKRS targeted to both the trigeminal nerve and the SPG is effective in reducing pain and autonomic symptoms of patients with SUNCT, although nonbothersome trigeminal sensory disturbances may occur.

Trigeminal microvascular decompression for short-lasting unilateral neuralgiform headache attacks.

A significant proportion of patients with short-lasting unilateral neuralgiform headache attacks are refractory to medical treatments. Neuroimaging studies have suggested a role for ipsilateral trigeminal neurovascular conflict with morphological changes in the pathophysiology of this disorder. We present the outcome of an uncontrolled open-label prospective single-centre study conducted between 2012 and 2020, to evaluate the efficacy and safety of trigeminal microvascular decompression in refractory chronic short-lasting unilateral neuralgiform headache attacks with MRI evidence of trigeminal neurovascular conflict ipsilateral to the pain side. Primary endpoint was the proportion of patients who achieved an 'excellent response', defined as 90-100% weekly reduction in attack frequency, or 'good response', defined as a reduction in weekly headache attack frequency between 75% and 89% at final follow-up, compared to baseline. These patients were defined as responders. The study group consisted of 47 patients, of whom 31 had short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing, and 16 had short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (25 females, mean age ± SD 55.2 years ± 14.8). Participants failed to respond or tolerate a mean of 8.1 (±2.7) preventive treatments pre-surgery. MRI of the trigeminal nerves (n = 47 patients, n = 50 symptomatic trigeminal nerves) demonstrated ipsilateral neurovascular conflict with morphological changes in 39/50 (78.0%) symptomatic nerves and without morphological changes in 11/50 (22.0%) symptomatic nerves. Postoperatively, 37/47 (78.7%) patients obtained either an excellent or a good response. Ten patients (21.3%, short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing = 7 and short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms = 3) reported no postoperative improvement. The mean post-surgery follow-up was 57.4 ± 24.3 months (range 11-96 months). At final follow-up, 31 patients (66.0%) were excellent/good responders. Six patients experienced a recurrence of headache symptoms. There was no statistically significant difference between short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing and short-lasting unilateral neuralgiform headache attacks in the response to surgery (P = 0.463). Responders at the last follow-up were, however, more likely to not have interictal pain (77.42% versus 22.58%, P = 0.021) and to show morphological changes on the MRI (78.38% versus 21.62%, P = 0.001). The latter outcome was confirmed in the Kaplan-Meyer analysis, where patients with no morphological changes were more likely to relapse overtime compared to those with morphological changes (P = 0.0001). All but one patient, who obtained an excellent response without relapse, discontinued their preventive medications. Twenty-two post-surgery adverse events occurred in 18 patients (46.8%) but no mortality or severe neurological deficit was seen. Trigeminal microvascular decompression may be a safe and effective long-term treatment for patients suffering short-lasting unilateral neuralgiform headache attacks with MRI evidence of neurovascular conflict with morphological changes.

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).

The neurosurgical treatment of craniofacial pain syndromes: current surgical indications and techniques.

Craniofacial pain syndromes are comprised of multiple pathological entities resulting in pain referred to the scalp, face, or deeper cranial structures. In a small subset of patients affected by those syndromes, pharmacological and physical therapies fail in alleviating pain. In some of those refractory patients surgical procedures aimed at relieving pain are indicated and have been adopted with variable results and safety profiles. In this review, the authors describe craniofacial pain syndromes that most commonly fail to respond to pharmacological therapies and may be amenable to tailored surgical procedures. In particular, trigeminal, glossopharyngeal, and occipital neuralgias are considered, as well as some primary headache syndromes such as cluster headache, short unilateral neuralgiform headache with conjunctival injection and tearing/short unilateral neuralgiform headache with autonomic symptoms, and migraine. Surgical techniques, including the implantation of deep brain or peripheral nerve electrodes with subsequent chronic stimulation, microvascular decompression of neurovascular conflicts, and percutaneous lesioning of neural structures are described. Finally, surgical indications, outcomes, and safety of these procedures are presented.

Pathogenesis, Surgical Treatment, and Cure for SUNCT Syndrome.

BACKGROUND: Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) are characterized by attacks of moderate to severe stabbing pain, strictly unilateral, with periorbital or temporal distribution, associated with cranial autonomic symptoms, such as lacrimation and redness of the ipsilateral eye. METHODS: To obtain mechanistic insights into the pathogenesis of SUNCT syndrome, more than 800 cases treated in our institution during the last 7 years were retrospectively reviewed. Two patients showed typical autonomic symptoms of SUNCT. RESULTS: Magnetic resonance imaging suggested potential compression of the trigeminal nerve by the intracranial artery in these cases and complete remission was achieved by microvascular decompression. CONCLUSIONS: Microvascular decompression provides an appropriate therapeutic choice if vascular compression of the trigeminal nerve is identified. From our 2 cases, we propose that, in some cases of SUNCT diagnosed previously, characteristic symptoms were induced by compression of the side surface of the first branch of the trigeminal nerve at the root exit zone by the intracranial artery.

SUNCT/SUNA and neurovascular compression: new cases and critical literature review.

BACKGROUND: Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and short-lasting unilateral neuralgiform headache with cranial autonomic symptoms (SUNA) are primary headache syndromes. A growing body of literature has focused on brain magnetic resonance imaging (MRI) evidence of neurovascular compression in these syndromes. OBJECTIVE: The objective of this article is to assess whether SUNCT is a subset of SUNA or whether the two are separate syndromes and clarify the role of neurovascular compression. METHOD: We describe three new SUNCT cases with MRI findings of neurovascular compression and critically review published SUNCT/SUNA cases. RESULTS: We identified 222 published SUNCT/SUNA cases. Our three patients with neurovascular compression added to the 34 cases previously described (16.9%). SUNCT and SUNA share the same clinical features and therapeutic options. At present, there is no available abortive treatment for attacks. Lamotrigine was effective in 64% of patients; topiramate and gabapentin in about one-third of cases. Of the 34 cases with neurovascular compression, seven responded to drug therapies, 16 patients underwent microvascular decompression of the trigeminal nerve (MVD) with effectiveness in 75%. CONCLUSIONS: We suggest that SUNCT and SUNA should be considered clinical phenotypes of the same syndrome. Brain MRI should always be performed with a dedicated view to exclude neurovascular compression. The high percentage of remission after MVD supports the pathogenetic role of neurovascular compression.

SUNCT and SUNA: medical and surgical treatments.

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 and often disabling primary headache disorders. Their management can be challenging. The abortive therapies are not generally useful as the attacks are relatively short lasting. A myriad of pharmacological preventive treatments have been tried in single case reports or small series in an open-label fashion. Lamotrigine, as an oral preventive treatment, and lidocaine, as an intravenous transitional treatment, seem to be the most effective therapies. For medically intractable chronic forms of SUNCT and SUNA, several surgical approaches have been tried. These include ablative procedures involving the trigeminal nerve or the Gasserian ganglion, microvascular decompression of the trigeminal nerve, and neurostimulation techniques. This review provides an overview of the current pharmacological and surgical options for SUNCT and SUNA syndromes.

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

BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

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.

Microvascular decompression of the trigeminal nerve in the treatment of SUNCT and SUNA.

BACKGROUND: Medical management of short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) syndrome and short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA) is often unsatisfactory. METHODS: The authors report nine cases of SUNCT/SUNA that failed medical treatment and had an aberrant arterial loop either in contact with or compressing the appropriate trigeminal nerve demonstrated on MRI. All underwent microvascular decompression of the ipsilateral trigeminal nerve for intractable pain. RESULTS: Immediate and complete relief of SUNCT and SUNA symptoms occurred in 6/9 (67%) cases. This was sustained for a follow-up period of 9-32 months (mean 22.2). In 3/9 (33%) cases, there was no benefit. Ipsilateral hearing loss was observed in one case. CONCLUSION: Medically intractable SUNCT and SUNA subjects with a demonstrable aberrant arterial loop impinging on the trigeminal nerve on neuroimaging may benefit from microvascular decompression.

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.

El hipotálamo en el SUNCT: similitudes y diferencias con las otras cefaleas trigeminoautonómicas / The hypothalamus in sunct syndrome: similarities and differences with the other trigeminal-autonomic cephalalgias

Introducción. El SUNCT forma parte del grupo de cefaleas trigeminoautonómicas (CTA) –cefalea en racimos y hemicraniasparoxísticas–, al compartir con ellas una serie de características. El impulso final para incluir el SUNCT en estegrupo fue la demostración de que el hipotálamo es pieza clave en su fisiopatología, dato común con las otras CTA. Sin embargo,su parecido clínico con la neuralgia trigeminal de la primera rama también es llamativo, aunque se admite que la génesisde la neuralgia trigeminal es periférica. Desarrollo. Se exponen las evidencias disponibles hasta el momento actual que hanpermitido involucrar al hipotálamo en el SUNCT, así como sus similitudes y diferencias con las otras CTA. Se trata de evidenciasclínicas, hormonales, de neuroimagen funcional (activación del hipotálamo posteroinferior) y de resultado terapéutico(con estimulación hipotalámica profunda). Asimismo, se detallan las bases neuroanatómicas (el hipotálamo como parte de lasredes neurales involucradas en procesos comportamentales, de memoria, de control antinociceptivo, de control del ritmo vigilia-sueño y otros ritmos circadianos, etc.) y neuroquímicas (orexinas, somatostatina y opiáceos endógenos), que sustentaríanlas hipótesis que tratan de establecerse en virtud de las evidencias previamente comentadas, las cuales tendrían muchospuntos de solapamiento entre las distintas CTA. Conclusión. Queda aún abierto el debate sobre si el hipotálamo es el generadorde las CTA o si es un elemento permisivo en su desarrollo, y cuál sería la explicación más plausible para las diferenciasfenotípicas entre ellas. Posteriores estudios podrán ir aclarando el enigma del SUNCT y de las otras CTA(AU)

Introduction. SUNCT belongs to the group of trigeminal-autonomic cephalalgias (TAC) –cluster headache andparoxysmal hemicranias–, since its shares a series of features with them. SUNCT was finally included in this group when thehypothalamus was proved to play a key role in its pathophysiology, an aspect that it has in common with other TAC. However,its clinical resemblance to trigeminal neuralgia of the first branch is notable, although it is accepted that the genesis of thetrigeminal neuralgia is peripheral. Development. The article presents the evidence available to date that has made it possibleto associate the hypothalamus with SUNCT, as well as outlining its similarities and differences with respect to other TAC. Thisevidence is clinical, hormonal, from functional neuroimaging (activation of the posteroinferior hypothalamus) and fromtherapeutic outcomes (with deep hypothalamic stimulation). Likewise, a detailed description is provided of both theneuroanatomical bases (the hypothalamus as part of the neural networks involved in processes concerned with behaviour,memory, antinociceptive control, waking-sleep control and other circadian rhythms, etc.) and the neurochemical bases(orexins, somatostatin and endogenous opiates) that would support the hypotheses which researchers are attempting toestablish to fit the evidence discussed earlier, which would have many points that overlap from one TAC to another.Conclusions. The question as to whether the hypothalamus is the/a generator of TAC or whether it is an element that allows itsdevelopment remains open to debate, as does the issue of which would be the most plausible explanation for the phenotypicdifferences between them. Future studies will allow the enigma of SUNCT and the other TAC to be explained(AU)

Short-lasting, unilateral neuralgiform headache attacks with conjunctival injection and tearing syndrome treated successfully with transsphenoidal resection of a growth hormone-secreting pituitary adenoma.

Short-lasting, unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) syndrome was first described in 1978 as one of the trigeminal autonomic cephalgias. In this paper the authors present a patient with a growth hormone-secreting pituitary adenoma who experienced resolution of SUNCT syndrome after transsphenoidal tumor resection.

Chronic high frequency stimulation of the posteromedial hypothalamus in facial pain syndromes and behaviour disorders.

Chronic high frequency stimulation (HFS) of the posteromedial hypothalamus (PMH) has been the first direct therapeutic application of functional neuroimaging data in a restorative reversible procedure for the treatment of an otherwise refractory neurological condition; in fact, the target coordinates for the stereotactic implantation of the electrodes have been provided by positron emission tomography (PET) studies, which were performed during cluster headache attacks. HFS of PMH produced a significant and marked reduction of pain attacks in patients with chronic cluster headache (CCH) and in one patient with short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT). The episodes of violent behaviour and psychomotor agitation during the attacks of CCH supported the idea that the posteromedial hypothalamus could be also involved in the control of aggressiveness; this has been previously suggested, in the seventies, by the results obtained in Sano's hypothalamotomies for the treatment of abnormal aggression and disruptive behaviour. On the basis of these considerations, we have performed HFS of the PMH and controlled successfully violent and disruptive behaviour in patients refractory to the conventional sedative drugs. Finally, we also tested the same procedure in three patients with refractory atypical facial pain, but unfortunately, they did not respond to this treatment.