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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 -
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 -
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 -
Neurology India 2021Key structures for the pathophysiology of primary headache disorders such as migraine, cluster headache, and other trigeminal autonomic cephalalgias were identified by... (Review)
Review
BACKGROUND
Key structures for the pathophysiology of primary headache disorders such as migraine, cluster headache, and other trigeminal autonomic cephalalgias were identified by imaging in the past years.
OBJECTIVE
Available data on functional imaging in primary headache disorders are summarized in this review.
MATERIAL AND METHODS
We performed a MEDLINE search on December 27, 2020 using the search terms "primary headache" AND "imaging" that returned 453 results in English, out of which 137 were labeled reviews. All articles were evaluated for content and relevance for this narrative review.
RESULTS
The structure depicted most consistently using functional imaging in different states of primary headaches (without and with pain) was the posterior hypothalamus. Whole-brain imaging techniques such as resting-state functional resonance imaging showed a wide-ranging association of cortical and subcortical areas with human nociceptive processing in the pathophysiological mechanisms underlying the different TACs. Similarities of distinct groups of primary headache disorders, as well as their differences in brain activation across these disorders, were highlighted.
CONCLUSION
The importance of neuroimaging research from clinical practice point of view remains the reliable and objective distinction of each individual pain syndrome from one another. This will help to make the correct clinical diagnosis and pave the way for better and effective treatment in the future. More research will be necessary to fulfill this unmet need.
Topics: Cluster Headache; Functional Neuroimaging; Headache; Humans; Migraine Disorders; Trigeminal Autonomic Cephalalgias
PubMed: 34003144
DOI: 10.4103/0028-3886.315987 -
Journal of Neurology, Neurosurgery, and... Mar 2021The management of short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and short-lasting unilateral neuralgiform... (Comparative Study)
Comparative Study 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.
Topics: Administration, Oral; Adolescent; Adult; Aged; Analgesics; Anesthetics, Local; Anticonvulsants; Female; Humans; Infusions, Parenteral; Male; Middle Aged; Prospective Studies; SUNCT Syndrome; Young Adult
PubMed: 33361408
DOI: 10.1136/jnnp-2020-323999 -
Brain : a Journal of Neurology Dec 2020Emerging data-points towards a possible aetiological and therapeutic relevance of trigeminal neurovascular contact in short lasting unilateral neuralgiform headache...
Emerging data-points towards a possible aetiological and therapeutic relevance of trigeminal neurovascular contact in short lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and perhaps in short lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA). We aimed to assess the prevalence and significance of trigeminal neurovascular contact in a large cohort of consecutive SUNCT and SUNA patients and evaluate the radiological differences between them. The standard imaging protocol included high spatial and nerve-cistern contrast resolution imaging acquisitions of the cisternal segments of the trigeminal nerves and vessels. MRI studies were evaluated blindly by two expert evaluators and graded according to the presence, location and degree of neurovascular contact. The degree of contact was graded as with or without morphological changes. Neurovascular contact with morphological changes was defined as contact with distortion and/or atrophy. A total of 159 patients (SUNCT = 80; SUNA = 79) were included. A total of 165 symptomatic and 153 asymptomatic trigeminal nerves were analysed. The proportion of neurovascular contact on the symptomatic trigeminal nerves was higher (80.0%) compared to the asymptomatic trigeminal nerves (56.9%). The odds on having neurovascular contact over the symptomatic nerves was significantly higher than on the asymptomatic nerves [odds ratio (OR): 3.03, 95% confidence interval (CI) 1.84-4.99; P < 0.0001]. Neurovascular contact with morphological changes were considerably more prevalent on the symptomatic side (61.4%), compared to the asymptomatic side (31.0%) (OR 4.16, 95% CI 2.46-7.05; P < 0.0001). On symptomatic nerves, neurovascular contact with morphological changes was caused by an artery in 95.0% (n = 77/81). Moreover, the site of contact and the point of contact around the trigeminal root were respectively proximal in 82.7% (67/81) and superior in 59.3% (48/81). No significant radiological differences emerged between SUNCT and SUNA. The multivariate analysis of radiological predictors associated with the symptomatic side, indicated that the presence of neurovascular contact with morphological changes was strongly associated with the side of the pain (OR: 2.80, 95% CI 1.44-5.44; P = 0.002) even when adjusted for diagnoses. Our findings suggest that neurovascular contact with morphological changes is involved in the aetiology of SUNCT and SUNA. Along with a similar clinical phenotype, SUNCT and SUNA also display a similar structural neuroimaging profile, providing further support for the concept that the separation between them should be abandoned. Furthermore, these findings suggest that vascular compression of the trigeminal sensory root, may be a common aetiological factor between SUNCT, SUNA and trigeminal neuralgia thereby further expanding the overlap between these disorders.
Topics: Adult; Aged; Aged, 80 and over; Anatomy, Cross-Sectional; Atrophy; Cohort Studies; Female; Functional Laterality; Headache Disorders; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Neuroimaging; Pain; Prevalence; SUNCT Syndrome; Trigeminal Nerve; Trigeminal Neuralgia; Young Adult
PubMed: 33301567
DOI: 10.1093/brain/awaa331 -
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 -
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 -
Annals of Indian Academy of Neurology 2022
PubMed: 36560988
DOI: 10.4103/aian.aian_219_22