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European Journal of Neurology Jul 2021Many clinicians lack experience in managing trigeminal autonomic cephalalgias (TACs) in pregnancy and lactation. In addition to cluster headache, TACs include hemicrania... (Review)
Review
Many clinicians lack experience in managing trigeminal autonomic cephalalgias (TACs) in pregnancy and lactation. In addition to cluster headache, TACs include hemicrania continua, paroxysmal hemicrania, and short-lasting unilateral neuralgiform headache with conjunctival injection and tearing/autonomic symptoms (SUNCT/SUNA). Treating these rare, severe headache conditions often requires off-label drugs that have uncertain teratogenic potential. In the last few years, several new treatment options and safety documentation have emerged, but clinical guidelines are lacking. This narrative review aimed to provide an updated clinical guide and good clinical practice recommendations for the management of these debilitating headache disorders in pregnancy and lactation.
Topics: Breast Feeding; Cluster Headache; Diagnosis, Differential; Female; Humans; Pregnancy; SUNCT Syndrome; Trigeminal Autonomic Cephalalgias
PubMed: 33852763
DOI: 10.1111/ene.14864 -
Revue Neurologique Oct 2021
Topics: Cranial Nerves; Headache; Humans; Neuralgia; SUNCT Syndrome
PubMed: 33781563
DOI: 10.1016/j.neurol.2020.11.011 -
British Journal of Neurosurgery Oct 2023Dural arteriovenous fistula represents a rare cause of secondary Trigeminal Neuralgia. To date, there have been 18 reported cases of successful treatment of trigeminal...
Dural arteriovenous fistula represents a rare cause of secondary Trigeminal Neuralgia. To date, there have been 18 reported cases of successful treatment of trigeminal neuralgia with endovascular embolization. Here we describe a unique case of a 51-year-old man with right-sided petrous ridge dural arteriovenous fistula causing ipsilateral V1 pain and cranial autonomic dysfunction. Cerebral angiography confirmed a Cognard type 3 right-sided tentorial dAVF supplied by an enlarged meningo-hypophpyseal vessel from the extradural ICA and by the middle meningeal artery. The fistula drains towards the midline via deep cerebral veins, the internal cerebral vein and the straight sinus with likely variceal compression of the right trigeminal nerve root entry zone. Transarterial Onyx embolization resulted in complete obliteration of the dural arteriovenous fistula with immediate resolution of facial pain and autonomic symptoms. After 24 months, the patient remains symptom-free and has no radiographic or clinical evidence of fistula recurrence. To date, there have been no other cases reported of trigeminal neuralgia with cranial autonomic symptoms or SUNCT syndrome due to a dural arteriovenous fistula. The presence of cranial autonomic symptoms with trigeminal neuralgia or a diagnosis of SUNCT should not deter endovascular treatments of dural arteriovenous fistulas or treatment of other compressive vascular lesions.
Topics: Male; Humans; Middle Aged; Trigeminal Neuralgia; Trigeminal Nerve; Central Nervous System Vascular Malformations; Embolization, Therapeutic; Cerebral Angiography; Fistula
PubMed: 33467937
DOI: 10.1080/02688697.2021.1874295 -
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 -
Cephalalgia : An International Journal... May 2021Little is known about short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and short-lasting unilateral neuralgiform...
AIM
Little is known about short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA). We present our experience with SUNCT/SUNA patients to aid identification and management of these disorders.
METHODS
A retrospective review of patient records of one orofacial pain clinic was performed. Inclusion criteria was a diagnosis of SUNCT/SUNA confirmed with at least one follow-up visit.
RESULTS
Six of the 2464 new patients seen between 2015-2018 met the selection criteria (SUNCT n = 2, SUNA n = 4). Gender distribution was one male to one female and average age of diagnosis was 52 years (range 26-62). Attacks were located in the V1/V2 trigeminal distributions, and five patients reported associated intraoral pain. Pain quality was sharp, shooting, and burning with two patients reporting "numbness". Pain was moderate-severe in intensity, with daily episodes that typically lasted for seconds. Common autonomic features were lacrimation, conjunctival injection, rhinorrhea, and flushing. Frequent triggers were touching the nose or a specific intraoral area. Lamotrigine and gabapentin were commonly used as initial therapy.
CONCLUSIONS
Differentiating between SUNCT/SUNA does not appear to be clinically relevant. Presenting symptoms were consistent with those published, except 5/6 patients describing intraoral pain and two patients describing paresthesia.
Topics: Adult; Autonomic Nervous System Diseases; Facial Pain; Female; Gabapentin; Headache Disorders; Humans; Lamotrigine; Magnetic Resonance Imaging; Male; Middle Aged; Migraine Disorders; Retrospective Studies; SUNCT Syndrome; Trigeminal Nerve
PubMed: 33269943
DOI: 10.1177/0333102420977292 -
Brain and Nerve = Shinkei Kenkyu No... Nov 2020Short-lasting unilateral neuralgiform headache attacks (SUNHA), within the group of trigeminal autonomic cephalalgias, are characterized by repetitive, brief and severe...
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).
Topics: Headache; Humans; Microvascular Decompression Surgery; SUNCT Syndrome; Trigeminal Autonomic Cephalalgias; Trigeminal Nerve
PubMed: 33191307
DOI: 10.11477/mf.1416201681 -
The Journal of International Medical... Oct 2020Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) is a rare primary headache syndrome. However, some cases of secondary...
Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) is a rare primary headache syndrome. However, some cases of secondary SUNCT are attributed to underlying diseases such as demyelination. We herein report a case of SUNCT with progression to neuromyelitis optica spectrum disorder (NMOSD). A 43-year-old woman developed headaches; 6 weeks later, she developed bilateral visual loss and numbness on the left side of her body. She was ultimately diagnosed with NMOSD.
Topics: Adult; Female; Headache; Humans; Neuralgia; Neuromyelitis Optica; SUNCT Syndrome
PubMed: 33070690
DOI: 10.1177/0300060520964349 -
Headache Nov 2020Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) is a severe headache disorder characterized by clustered episodes of...
BACKGROUND
Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) is a severe headache disorder characterized by clustered episodes of extreme pain. Refractory to most interventions, ipsilateral unilateral ventral tegmental area (VTA) deep brain stimulation (DBS) has been previously reported to be efficacious in 14 cases.
METHODS AND RESULTS
Herein, we report the first case of an individual with medically refractory SUNCT who underwent bilateral VTA DBS. The patient experienced better improvement of his headaches with bilateral stimulation compared to unilateral stimulation. He also had a return of severe headaches within a few hours after his stimulator was switched off during sleep, with rebound worsening of his headaches over several days. The main side effects were double vision and difficulty focusing while reading, which were observed primarily with unipolar stimulation that required high voltages, high frequencies, and multiple negative contacts to control the headaches. The side effects were minimized with bipolar and interleaving stimulation with comparable control of his headaches.
CONCLUSION
Bilateral VTA DBS may be effective in alleviating medical refractory SUNCT.
Topics: Deep Brain Stimulation; Humans; Male; Middle Aged; SUNCT Syndrome; Ventral Tegmental Area
PubMed: 33038268
DOI: 10.1111/head.13989 -
Ideggyogyaszati Szemle Sep 2020Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing/short-lasting unilateral neuralgiform headache with cranial autonomic features...
Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing/short-lasting unilateral neuralgiform headache with cranial autonomic features (SUNCT/SUNA) is a rare severe headache. At the time of an attack, it can hinder a patient from eating and requires acute intervention. The sphenopalatine ganglion is an extracranial parasympathetic ganglion with both sensory and autonomic fibers. Sphenopalatine ganglion block has long been used in the treatment of headache, particularly when conventional methods have failed. Here, we present a patient who was resistant to intravenous lidocaine, but responded rapidly to sphenopalatine ganglion block during an acute episode of SUNCT/SUNA.
Topics: Headache; Humans; Lidocaine; Neuralgia; SUNCT Syndrome; Sphenopalatine Ganglion Block; Treatment Outcome
PubMed: 33035414
DOI: 10.18071/isz.73.0345