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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 -
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 -
Revista de Neurologia Aug 2022Headache is one of the most frequent reasons for medical consultation. The SUNCT type is classified as a primary trigeminal-autonomic headache and should be considered...
INTRODUCTION
Headache is one of the most frequent reasons for medical consultation. The SUNCT type is classified as a primary trigeminal-autonomic headache and should be considered in cases of short-lasting, hemifacial pain associated with autonomic dysfunction. Additionally, in the approach to this disease, secondary causes that facilitate its onset must be ruled out.
CASE REPORT
We describe a case of a new-onset SUNCT-type headache in which involvement of the first branch of the trigeminal nerve due to herpes zoster virus infection was documented six days after the onset of pain.
CONCLUSION
Trigeminal autonomic headaches are usually primary; yet, multiple conditions have been described that are associated with SUNCT-type headaches and have a secondary etiology. Among these, herpes zoster virus infection should be considered at the onset of episodes consistent with SUNCT-type headache unresponsive to medical treatment.
Topics: Herpes Zoster; Humans; SUNCT Syndrome
PubMed: 35879882
DOI: 10.33588/rn.7503.2021410 -
The Neurohospitalist Jul 2022Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) and short-lasting unilateral neuralgiform headache attacks with cranial...
Central Nervous System Involvement of Multiple Myeloma Presenting as Short-lasting Unilateral Neuralgiform Headache with Conjunctival Injection and Tearing: A Case Report.
Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) and short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA) are part of the trigeminal autonomic cephalalgia (TAC) group of headache disorders. Attacks present with repeated, severe, sharp, stabbing, or throbbing pain. Patients may experience a single attack, recurrent attacks with pain-free interictal periods, or a sawtooth pattern background pain with superimposed stabs. Although SUNCT typically presents as a primary headache disorder, it may be secondary to an underlying pathology, such as pituitary tumors or posterior fossa lesions, both intra and extra-axial (vascular lesion, tumor, or bony abnormalities). Multiple Myeloma (MM) with central nervous system involvement (CNS MM) most commonly presents with visual changes (36%), radiculopathy (27%), headache (25%), confusion (21%), dizziness (7%) and seizures (6%). Secondary SUNCT cases have been sparsely described (less than 60), and CNS MM presenting as SUNCT has not been previously described in the literature. Our case describes a previously unreported clinical presentation of CNS MM. The report highlights the need for a timely and thorough diagnostic work-up of headache in patients with risk factors for a secondary etiology, which in this case included new-onset, autonomic features, older age, and history of malignancy. A misdiagnosis will preclude a potentially life-extending or saving targeted therapy for the underlying illness. We also aim to remind practitioners of the variability in the clinical symptoms of SUNCT, which are known to occur in a significant number of cases, including migrainous features and dull interictal pain.
PubMed: 35755231
DOI: 10.1177/19418744221086681 -
Journal of Neuro-ophthalmology : the... Dec 2022
Topics: Humans; SUNCT Syndrome
PubMed: 35482913
DOI: 10.1097/WNO.0000000000001549 -
Brain : a Journal of Neurology Aug 2022A significant proportion of patients with short-lasting unilateral neuralgiform headache attacks are refractory to medical treatments. Neuroimaging studies have...
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.
Topics: Adult; Aged; Female; Humans; Male; Microvascular Decompression Surgery; Middle Aged; Prospective Studies; Recurrence; SUNCT Syndrome
PubMed: 35325067
DOI: 10.1093/brain/awac109 -
Acta Neurologica Taiwanica Mar 2021Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) is a rare form of primary headache, classified as trigeminal autonomic...
PURPOSE
Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) is a rare form of primary headache, classified as trigeminal autonomic cephalalgia. Since the underlying mechanism of the pathogenesis has not yet been determined, a standardized therapeutic strategy for SUNCT is unavailable. We present a case of SUNCT syndrome with successful pain relief by intravenous administration of ketamine, an N-methyl-D-aspartate receptor (NMDAR) antagonist.
CASE REPORT
A 56-year-old male patient reported severe throbbing and shooting pain in forehead, temporal and periorbital region. We confirmed conjunctival injection, lacrimation, blepharoptosis, and miosis as symptoms related to autonomic activity, and made a diagnosis of SUNCT based on ICHD-3 beta. Numerous treatments were attempted, including pregabalin, gabapentine, nonsteroidal antiinflammatory drugs, acetaminophen, steroids, antidepressants, triptans, nerve blocks, and intravenous lidocaine with unsatisfactory results. Intravenous administration of ketamine (0.4 mg/kg) for one hour, was found to relieve the severe pain.
CONCLUSION
Intravenous ketamine can effectively treat SUNCT syndrome. This case demonstrated that involvement of NMDAR could be one of the mechanisms of SUNCT syndrome pathogenesis and establish a therapeutic strategy for this pain syndrome.
Topics: Administration, Intravenous; Blepharoptosis; Headache; Humans; Ketamine; Male; Middle Aged; SUNCT Syndrome
PubMed: 34549399
DOI: No ID Found -
Cephalalgia : An International Journal... Nov 2021There are five headache disorders composing the trigeminal autonomic cephalalgias (cluster headache, paroxysmal hemicrania, short-lasting unilateral neuralgiform... (Meta-Analysis)
Meta-Analysis
BACKGROUND AND OBJECTIVE
There are five headache disorders composing the trigeminal autonomic cephalalgias (cluster headache, paroxysmal hemicrania, short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT), short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA), and hemicrania continua). Little is known about these disorders in the pediatric population. The objectives of this study are to report the full age ranges of pediatric trigeminal autonomic cephalalgias and to determine if pediatric-onset trigeminal autonomic cephalalgias display similar signs and symptoms as adult onset.
METHODS
Search criteria in Medline Ovid, Embase, PsycINFO, and Cochrane Library were created by a librarian. The remainder of the steps were independently performed by two neurologists using PRISMA guidelines. Inclusion criteria for titles and abstracts were articles discussing cases of trigeminal autonomic cephalalgias with age of onset 18 or younger, as well as any epidemiological report on trigeminal autonomic cephalalgias (as age of onset data was often found in the results section but not in the title or abstract). Data extracted included age of onset, sex, and International Classification of Headache Disorders criteria for trigeminal autonomic cephalalgias (including pain location, duration, frequency, autonomic features, restlessness) and some migraine criteria (photophobia, phonophobia, and nausea). Studies that did not meet full criteria for trigeminal autonomic cephalalgias were examined separately as "atypical trigeminal autonomic cephalalgias"; secondary headaches were excluded from this category.
RESULTS
In all, 1788 studies were searched, 86 met inclusion criteria, and most (56) examined cluster headache. In cluster headache, onset occurred at every pediatric age (range 1-18 years) with a full range of associated features. Autonomic and restlessness features were less common in pediatric patients, while migrainous features (nausea, photophobia, and phonophobia) were found at similar rates. The sex ratio of pediatric-onset cluster headache (1.8, 79 male and 43 female) may be lower than that of adult-onset cluster headache. Data for other trigeminal autonomic cephalalgias, while more limited, displayed most of the full range of official criteria. The data for atypical trigeminal autonomic cephalalgias were also limited, but the most common deviations from the official criteria were abnormal frequencies and locations of attacks.
CONCLUSIONS
Trigeminal autonomic cephalalgias can start early in life and have similar features to adult-onset trigeminal autonomic cephalalgias. Specifically, pediatric-onset cluster headache patients display the full range of each criterion for cluster headache (except maximum frequency of six instead of eight attacks per day). However, cranial autonomic features and restlessness occur at a lower rate in pediatrics. Additional information is needed for the other trigeminal autonomic cephalalgias. As for expanding the ICHD-3 criteria for pediatric-onset trigeminal autonomic cephalalgias, we have only preliminary data from atypical cases, which suggests that the frequency and location of attacks sometimes extend beyond the official criteria. This study was registered as a systematic review in PROSPERO (registration number CRD42020165256).
Topics: Adolescent; Adult; Child; Child, Preschool; Female; Headache; Headache Disorders; Humans; Infant; Male; Paroxysmal Hemicrania; SUNCT Syndrome; Trigeminal Autonomic Cephalalgias
PubMed: 34407646
DOI: 10.1177/03331024211027560 -
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 -
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