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Neurology India 2021Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and short-lasting unilateral neuralgiform headache with autonomic... (Review)
Review
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.
Topics: Headache; Humans; Lamotrigine; Microvascular Decompression Surgery; SUNCT Syndrome; Trigeminal Autonomic Cephalalgias
PubMed: 34003160
DOI: 10.4103/0028-3886.315990 -
Journal of Neurology, Neurosurgery, and... Jan 2023Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and short-lasting unilateral neuralgiform headache with autonomic... (Review)
Review
Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and short-lasting unilateral neuralgiform headache with autonomic symptoms (SUNA) are disabling primary headache disorders. The advent of advanced imaging technologies and surgical techniques has translated to a growing arsenal of interventional therapies capable of treating headache disorders. This literature review sheds light on the current evidence available for interventional therapies in medically intractable SUNCT/SUNA. PubMed and EMBASE were searched for publications between 1978 and 2022. Inclusion criteria were SUNCT/SUNA studies reporting outcomes following occipital nerve stimulation (ONS), pulsed radiofrequency (PRF) of sphenopalatine ganglion (SPG), stereotactic radiosurgery (SRS), deep brain stimulation (DBS) or microvascular decompression (MVD) of the trigeminal nerve. A greater than 50% reduction in severity or a greater than 50% reduction in the number of attacks was defined as a successful response. The rate of successful responses for the various treatment modalities were as follows: ONS 33/41 (80.5%), PRF of SPG 5/9 (55.6%), DBS of the ventral tegmental area 14/16 (86.7%), SRS to the SPG and/or trigeminal nerve 7/9 (77.8%) and MVD 56/73 (76.7%). Mean follow-up time in months was 42.5 (ONS), 24.8 (PRF), 25.3 (DBS), 20.8 (SRS) and 42.4 (MVD). A significant proportion of SUNCT/SUNA patients remain refractory to medical therapy (45%-55%). This review discusses existing literature on interventional approaches, including neuromodulation, radiofrequency ablation, gamma knife radiosurgery and MVD. The outcomes are promising, yet limited data exist, underscoring the need for further research to develop a robust surgical management algorithm.
Topics: Humans; SUNCT Syndrome; Headache; Trigeminal Nerve; Neuralgia; Headache Disorders
PubMed: 35977820
DOI: 10.1136/jnnp-2022-329588 -
Neurology Sep 2019
Topics: Humans; Prospective Studies; SUNCT Syndrome; Trigeminal Nerve
PubMed: 31427500
DOI: 10.1212/WNL.0000000000008121 -
Neurological Sciences : Official... Dec 2020Cluster headache (CH), paroxysmal hemicrania (PH), short-lasting unilateral neuralgiform headache attacks (including SUNCT and SUNA), and hemicrania continua (HC)... (Review)
Review
Cluster headache (CH), paroxysmal hemicrania (PH), short-lasting unilateral neuralgiform headache attacks (including SUNCT and SUNA), and hemicrania continua (HC) compose the group of trigeminal autonomic cephalalgias (TACs). Here, we review the recent advances in the field and summarize the current knowledge about the origin of these headaches. Similar to the other primary headaches, the pathogenesis is still much obscure. However, advances are being made in both animal models and humans studies. Three structures clearly appear to be crucial in the pathophysiology of TACs: the trigeminal nerve, the facial parasympathetic system, and the hypothalamus. The physiologic and pathologic functioning of each of these elements and their interactions is being progressively clarified, but critical questions are still open.
Topics: Animals; Cluster Headache; Headache; Humans; Paroxysmal Hemicrania; SUNCT Syndrome; Trigeminal Autonomic Cephalalgias
PubMed: 32845489
DOI: 10.1007/s10072-020-04639-4 -
Cephalalgia : An International Journal... Jan 2021Trigeminal autonomic cephalalgias (TACs) are characterized by paroxysmal attacks of unilateral primary headaches associated with ipsilateral craniofacial autonomic... (Review)
Review
INTRODUCTION
Trigeminal autonomic cephalalgias (TACs) are characterized by paroxysmal attacks of unilateral primary headaches associated with ipsilateral craniofacial autonomic symptoms. In this pediatric case series, 13 cases of short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT)/short-lasting unilateral neuralgiform headache with autonomic symptoms (SUNA), including children ages 3-18 years, are discussed. This paper reviews the application of International Classification of Headache Disorders, 3rd edition (ICHD-3) criteria especially in children presenting with SUNCT or SUNA. This is the largest pediatric case series of SUNCT/SUNA reported in the literature.
BACKGROUND
Trigeminal autonomic cephalalgias are rare in children and adolescents, with SUNCT/SUNA having the least reported cases. We will discuss the application of ICHD-3 criteria to diagnose SUNCT/SUNA in children and review overlapping cases and their response to different treatment options including indomethacin, which is typically reserved for specific subtypes of TACs; for example, paroxysmal hemicrania.
CONCLUSION
This case series presents a unique opportunity to aid in the diagnosis and treatment of similar pediatric cases in the future. It helps us to broaden the ICHD-3 criteria to diagnose and treat different overlapping trigeminal autonomic cephalalgia cases in children.
Topics: Adolescent; Child; Child, Preschool; Headache; Headache Disorders; Humans; Paroxysmal Hemicrania; SUNCT Syndrome; Trigeminal Autonomic Cephalalgias
PubMed: 32867532
DOI: 10.1177/0333102420954525 -
The Journal of Headache and Pain Oct 2022Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and short-lasting unilateral neuralgiform headache attacks with...
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) have not been evaluated sufficiently due to limited data, particularly in China.
METHODS
Patients with SUNCT or SUNA treated in a tertiary headache centre or seven other headache clinics of China between April 2009 and July 2022 were studied; we compared their demographics and clinical phenotypes.
RESULTS
The 45 patients with SUNCT and 31 patients with SUNA had mean ages at onset of 37.22 ± 14.54 years and 42.45 ± 14.72 years, respectively. The mean ages at diagnosis of SUNCT and SUNA were 41.62 ± 12.70 years and 48.68 ± 13.80 years, respectively (p = 0.024). The correct diagnosis of SUNCT or SUNA was made after an average of 2.5 (0-20.5) years or 3.0 (0-20.7) years, respectively. Both diseases had a female predominance (SUNCT: 1.14:1; SUNA: 2.10:1). The two diseases differed in the most common attack site (temporal area in SUNCT, p = 0.017; parietal area in SUNA, p = 0.002). Qualitative descriptions of the attacks included stabbing pain (44.7%), electric-shock-like pain (36.8%), shooting pain (25.0%), and slashing pain (18.4%). Lacrimation was the most common autonomic symptom in both SUNCT and SUNA patients, while eyelid oedema, ptosis, and miosis were less frequent. Triggers such as cold air and face washing were shared by the two diseases, and they were consistently ipsilateral to the attack site.
CONCLUSIONS
In contrast to Western countries, SUNCT and SUNA in China have a greater female predominance and an earlier onset. The shared core phenotype of SUNCT and SUNA, despite their partial differences, suggests that they are the same clinical entity.
Topics: Female; Male; Humans; Cross-Sectional Studies; SUNCT Syndrome; Headache; Neuralgia; China
PubMed: 36289482
DOI: 10.1186/s10194-022-01509-6 -
Cephalalgia : An International Journal... Feb 2024Short-lasting unilateral neuralgiform headache attacks (SUNHA) have the features of both short-lasting unilateral neuralgiform pain, such as trigeminal neuralgia or... (Review)
Review
BACKGROUND
Short-lasting unilateral neuralgiform headache attacks (SUNHA) have the features of both short-lasting unilateral neuralgiform pain, such as trigeminal neuralgia or stabbing headache, and associated trigeminal autonomic symptoms, such as paroxysmal hemicrania or cluster headache. Recognizing and adequately treating SUNHA is essential but current treatment methods are ineffective in treating SUNHA.
METHODS
We reviewed the changes in the concept of short-lasting unilateral neuralgiform headache attacks and provide a narrative review of the current medical and surgical treatment options, from the first choice of treatment for patients to treatments for selective intractable cases.
RESULTS
Unlike the initial impression of an intractable primary headache disorder affecting older men, SUNHA affects both sexes throughout their lifespan. One striking feature of SUNHA is that the attacks are triggered by cutaneous or intraoral stimulation. The efficacy of conventional treatments is disappointing and challenging, and preventive therapy is the mainstay of treatment because of highly frequent attacks of a very brief duration. Amongst them, lamotrigine is effective in approximately two-third of the patients with SUNHA, and intravenous lidocaine is essential for the management of acute exacerbation of intractable pain. Topiramate, oxcarbazepine and gabapentin are considered good secondary options for SUNHA, and botulinum toxin can be used in selective cases. Neurovascular compression is commonly observed in SUNHA, and surgical approaches, such as neurovascular compression, have been reported to be effective for intractable cases.
CONCLUSIONS
Recent advances in the understanding of SUNHA have improved the recognition and treatment approaches for this unique condition.
Topics: Male; Female; Humans; Aged; SUNCT Syndrome; Headache; Anticonvulsants; Gabapentin; Lamotrigine; Neuralgia; Trigeminal Autonomic Cephalalgias
PubMed: 38415675
DOI: 10.1177/03331024241232256 -
Frontiers in Neurology 2020Opalski syndrome is a rare variation of lateral medullary syndrome (LMS) accompanied by ipsilateral hemiparesis. Short-lasting unilateral neuralgiform headaches with...
Opalski syndrome is a rare variation of lateral medullary syndrome (LMS) accompanied by ipsilateral hemiparesis. Short-lasting unilateral neuralgiform headaches with conjunctival injection and tearing (SUNCT) is a rare headache syndrome which belongs to the trigeminal autonomic cephalalgias. SUNCT syndrome has been previously described in association with LMS. We here describe a case of SUNCT syndrome with Opalski syndrome caused by dorsolateral medullary infarction.
PubMed: 32117015
DOI: 10.3389/fneur.2020.00052 -
Revista de Neurologia Jul 2023Identify the number of cases with a possible diagnosis of short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) or...
OBJECTIVE
Identify the number of cases with a possible diagnosis of short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) or short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA) in patients with a previous diagnosis of Trigeminal Neuralgia (TN) at the Neurology Service of the National Medical Center 20 de Noviembre. This will confirm that these trigeminal-autonomic cephalalgias should be ruled out and considered as differential diagnoses of trigeminal neuralgia.
PATIENTS AND METHODS
Cross-sectional and retrospective study. The complete electronic medical records of 100 patients with a diagnosis of TN were evaluated during the period from April 2010 to May 2020. Autonomic symptoms were intentionally searched for in these patients and compared with the diagnostic criteria of SUNCT and SUNA of the 3rd edition of the International Classification of Headache Disorders. Chi-square tests and subsequent bivariate regression were performed to determine the association between variables.
RESULTS
One hundred patients with a diagnosis of TN were included. After reviewing the clinical manifestations, 12 patients with autonomic symptoms were found and compared with the diagnostic criteria of SUNCT and SUNA. However, they did not meet the absolute criteria to be diagnosed with the previously mentioned diseases, nor to be ruled out.
CONCLUSIONS
TN is a painful and frequent entity that can present with autonomic symptoms, therefore making it important to identify SUNCT and SUNA as differential diagnoses, to recognize them and treat them appropriately.
Topics: Humans; Trigeminal Neuralgia; Retrospective Studies; Cross-Sectional Studies; SUNCT Syndrome; Trigeminal Autonomic Cephalalgias; Headache; Diagnostic Errors
PubMed: 37403242
DOI: 10.33588/rn.7702.2023166