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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 -
Current Pain and Headache Reports Aug 2015Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and short-lasting neuralgiform headache attacks with cranial... (Review)
Review
Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and short-lasting neuralgiform headache attacks with cranial autonomic features (SUNA) are rare headache disorders characterized by severe, short-lasting headaches. These headache disorders are often refractory to treatment and can be secondary phenomena. This article reviews the history, pathophysiology, and treatment of these disorders. Both pharmacotherapy and procedural interventions are discussed in context of historical and more recent reports.
Topics: Animals; Autonomic Nervous System; Headache; Headache Disorders; Humans; Nervous System Diseases; SUNCT Syndrome; Treatment Outcome
PubMed: 26092511
DOI: 10.1007/s11916-015-0511-2 -
Current Pain and Headache Reports Jun 2018The purpose of this review is to provide an update on the clinical features, diagnosis, pathogenesis, epidemiology, and treatment of the rare primary headache disorders... (Review)
Review
PURPOSE OF REVIEW
The purpose of this review is to provide an update on the clinical features, diagnosis, pathogenesis, epidemiology, and treatment of the rare primary headache disorders short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and short-lasting unilateral neuralgiform headache attacks with autonomic symptoms (SUNA). Together these entities are known as short-lasting unilateral neuralgiform headache attacks (SUNHA).
RECENT FINDINGS
Recent case reports of secondary SUNCT and SUNA due to medullary infarcts support the theory that the trigeminohypothalamic pathway is involved in the pathophysiology of SUNHA. While medical therapy for SUNHA has not significantly changed, surgical therapy for refractory SUNCT and SUNA has made advancements with a recent case series demonstrating the efficacy of deep brain stimulation. We will discuss the pathophysiology of both the pain and the autonomic symptoms experienced in SUNCT and SUNA attacks as well the medical, procedural, and surgical options for treatment with emphasis on recent advances. Specific secondary causes reported in the recent literature will be discussed in brief.
Topics: Humans; SUNCT Syndrome; Trigeminal Autonomic Cephalalgias
PubMed: 29931416
DOI: 10.1007/s11916-018-0707-3 -
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 -
Headache Jun 2017Classical trigeminal neuralgia (CTN) and the short-lasting unilateral neuralgiform headache attacks (SUNHA) are clinically similar. (Review)
Review
PREMISE
Classical trigeminal neuralgia (CTN) and the short-lasting unilateral neuralgiform headache attacks (SUNHA) are clinically similar.
PROBLEM
The SUNHAs include short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA). Shared clinical signs with CTN include severe, unilateral trigeminal pain that is often triggered by innocuous stimuli and accompanied by a dull persistent background pain. Recent reports on trigeminal neuralgia cases with atypical features such as autonomic signs and prolonged attack duration further blur the clinical distinction between CTN and SUNHAs.
POTENTIAL SOLUTIONS
Are the similarities greater than their differences? If so, this may reflect a spectrum of disease ranging from typical CTN attacks to typical SUNHAs with a mixed phenotype in the middle. In this review they will summarize the overlap between these entities and contrast the pathophysiology and treatment approach.
Topics: Autonomic Nervous System Diseases; Humans; SUNCT Syndrome; Trigeminal Neuralgia
PubMed: 28188632
DOI: 10.1111/head.13040 -
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 -
Journal of Oral & Facial Pain and...Trigeminal autonomic cephalalgias (TACs) are primary headaches that include cluster headache (CH), paroxysmal hemicrania (PH), and short-lasting unilateral neuralgiform... (Review)
Review
Trigeminal autonomic cephalalgias (TACs) are primary headaches that include cluster headache (CH), paroxysmal hemicrania (PH), and short-lasting unilateral neuralgiform headache attacks (SUNHAs) with conjunctival injection and tearing (SUNCT) or cranial autonomic features (SUNA). Hemicrania continua (HC) is another form that has been ascribed to TACs for clinical and pathophysiologic reasons. Cluster headache is the most common of these syndromes, even if comparatively rare, with a lifetime prevalence of around 1 in 1,000. TACs share many aspects from a pathophysiologic standpoint (a hypothalamic activation may be involved in all forms initiating the attacks), but differences in attack duration and frequency and in extent of treatment response distinguish one from the other. This review focuses on the treatments currently available for these headaches according to the most recent guidelines. Due to the low frequency of most TACs, there are little data from randomized controlled trials; therefore, evidence from simple open studies in small case series or single-case observations are reported. Promising results have been recently obtained with novel modes of drug administration, invasive pericranial interventions, and different strategies such as neurostimulation. There are also some future treatments being studied at present.
Topics: Cluster Headache; Headache; Humans; SUNCT Syndrome; Trigeminal Autonomic Cephalalgias
PubMed: 30703174
DOI: 10.11607/ofph.1922