Post-traumatic short-lasting unilateral headache with cranial autonomic symptoms (SUNA)

Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) syndrome is a rare headache syndrome classified among the trigeminal autonomic cephalalgias. It is usually idiopathic, although infrequent secondary forms have been described. Recently the term short-lasting unilateral headache with cranial autonomic symptoms (SUNA) has been defined by the International Headache Society (ICHD-2) as similar to SUNCT with less prominent or absent conjunctival injection and lacrimation. We report two patients with paroxysmal orbito-fronto-temporal pains, phenotypically suggesting SUNA, occurring after traumatic head injury.     

Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) with preserved refractory period: report of three cases

Background  

Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) and short-lasting unilateral neuralgiform attacks with cranial autonomic features (SUNA) are rare primary headache syndromes characterized by spontaneous or triggered attacks of unilateral, brief, multiple, orbitofrontal pain associated with ipsilateral autonomic features. SUNCT is considered as a subset of SUNA. In SUNA, there may be cranial autonomic symptoms other than conjunctival injection and lacrimation, or either of two is present. SUNCT/SUNA can be triggered immediately after or at the decrescendo phase of the ongoing attack without any intervening refractory period. Refractory period is usually present in trigeminal neuralgia. Absent refractory period is thought to reliably differentiate SUNCT/SUNA from trigeminal neuralgia and has been proposed for inclusion into the International Classification of Headache Disorders (ICHD) diagnostic criteria for SUNCT.     

Bilateral SUNCT-like headache in a patient with prolactinoma responsive to lamotrigine

Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) syndrome is a rare trigeminal autonomic cephalalgia. The cases of SUNCT with attacks that affected both sides simultaneously have only rarely been reported and some of them had underlying pathology. We have reported a case of bilateral SUNCT-like headache secondary to a prolactinoma and responsive to lamotrigine treatment.     

SUNCT and SUNA: an Update and 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.

Summary

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.

     

SUNCT Syndrome: A Hungarian Case

A Hungarian patient with short-lasting, unilateral, neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) is presented in this paper. This male patient was first diagnosed as having first division trigeminal neuralgia. The location and duration of the attacks and the prominent accompanying autonomic feature on the symptomatic aide, such as conjunctival injection, lacrimation, nasal stuffiness, and the inefficacy of drugs, led to a reconsideration of the diagnosis. The pain paroxysms occurred frequently during a 3-to 4-month period, followed by a longer remission phase. Mechanical precipitating maneuvers were observed during bouts of pain. The clinical picture is reminiscent of the SUNCT syndrome, first described by Sjaastad et al in 1978.
SUNCT and trigeminal neuralgia are in many ways similar, although, some decisive differences have also been noted. Further observations are needed to distinguish the two disorders and to clarify this syndrome as a new headache type or as a trigeminal neuralgia variant.     

SUNCT syndrome in a patient with prolactinoma and cabergoline-induced attacks

Short-lasting unilateral neuralgiform headache with conjuntival injection and tearing (SUNCT) syndrome is a rare trigeminal autonomic cephalalgia. We report a patient with prolactinoma and cabergoline-induced SUNCT attacks and the literature is reviewed for a better understanding of the pathophysiology.     

Clomiphene citrate as a new treatment for SUNCT: hormonal manipulation for hypothalamic-influenced trigeminal autonomic cephalalgias

We present a patient with treatment refractory short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) who was found to have low levels of serum testosterone supporting the hypothalamic connection to this trigeminal autonomic cephalalgia. Clomiphene citrate therapy induced a significant elevation of testosterone levels (by its effect on hypothalamic estrogen receptors) and led to a dramatic reduction in SUNCT attacks. Hormonal manipulation may be a treatment strategy for hypothalamic-influenced trigeminal autonomic cephalalgias.     

SUNCT: bilateral hypothalamic activation during headache attacks and resolving of symptoms after trigeminal decompression

Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) is a primary head-pain syndrome, which is often refractory to any medical treatment. Concerning the pathophysiology of SUNCT, hypothalamic involvement ipsilaterally to the pain has been suggested based on the clinical features and one functional imaging case report. Here we now report a new case with SUNCT and the concomitant cerebral activation pattern (fMRI) during the pain attacks. In addition to an activation of several brain structures known to be generally involved in pain processing, bilateral hypothalamic activation occurred during the pain attacks, arguing for a central origin of the headache. Interestingly, this patient became completely pain free after surgical decompression of the ipsilateral trigeminal nerve. We hypothesize that in this case with a central predisposition for trigeminal autonomic cephalgias, a peripheral trigger with ectopic excitation might have contributed to the clinical picture of SUNCT.     

Case report on a patient with SUNCT-syndrome

Case report on a patient with SUNCT-syndrome (short lasting, unilateral neuralgiform headache attacks with conjunctival injection, sweating, and rhinorrhoea) who was successfully treated with gabapentin. SUNCT, a still relatively unknown headache syndrome, is characterized by attacks of periorbital pain with accompanying ipsilateral autonomic symptoms. Along with this case report the differences of SUNCT to similar headaches are emphasized. Due to clear diagnostic criteria the inclusion of SUNCT in the IHS classification (International Headache Society) as a separate clinical entity should be favoured.     

A case of SUNCT syndrome responsive to zonisamide

Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) is a rare headache syndrome that represents a subtype of trigeminal autonomic cephalalgia thought to be highly refractory to treatment. More recently, numerous anticonvulsant agents including lamotrigine, topiramate, gabapentin, and carbamazepine have been reported to be partially or completely effective for treating SUNCT. We report the case of a patient with SUNCT in whom symptoms were completely relieved with carbamazepine at 600?mg/day. However, carbamazepine had to be discontinued due to severe rash. Zonisamide was selected for continued treatment, as a Na-channel blocker like carbamazepine but with lower risk of producing skin rashes as caused by carbamazepine. Attacks ceased completely with 300?mg/day of zonisamide achieving a blood serum level of 19?μg/ml. This is the first case report to describe zonisamide alone completely eliminating SUNCT symptoms. Zonisamide should be considered a viable candidate drug for the treatment of SUNCT.     

Familial SUNCT

Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) is a rare type of primary headache. In this report we describe the occurrence of SUNCT in a family. Unfortunately, one of the siblings was already dead. However, clear and detailed information from close relatives and her general practitioner confirmed the diagnosis of SUNCT. It is likely that genetic factors contribute to all types of trigeminal autonomic cephalalgias.     

Therapie und Prophylaxe von Cluster-Kopfschmerzen und anderen trigemino-autonomen Kopfschmerzen

Following the new IHS classification, cluster headache, paroxysmal hemicrania, and short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT syndrome) are included in the classification as trigeminal autonomic cephalgias (TAC). The similarities of these syndromes suggest a considerable shared pathophysiology. These syndromes have in common that they involve activation of trigeminovascular nociceptive pathways with reflex cranial autonomic activation. Clinically, this physiology predicts pain with some combination of lacrimation, conjunctival injection, nasal congestion, or eyelid edema. Broadly the management of TAC comprises acute and prophylactic treatment. Some types of trigeminal autonomic headaches such as paroxysmal hemicrania and hemicrania continua have, unlike cluster headaches, a very robust response to indomethacin, leading to a consideration of indomethacin-sensitive headaches. This review covers the clinical picture and therapeutic options. Although studies following the criteria of evidence-based medicine (EBM) are rare, most patients can be treated sufficiently.     

Neues zur Pathophysiologie und Therapie der trigeminoautonomen Kopfschmerzsyndrome

Trigeminal autonomic cephalalgias (TACs) are a group of primary headache disorders, which are characterized by strictly unilateral pain, together with ipsilateral cranial autonomic symptoms. TACs include cluster headache (CH), paroxysmal hemicrania (PH) and short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT syndrome). These diseases all have one thing in common: an activation of trigeminal nociceptive afferentia with a reflex-like activation of cranial autonomic efferentia via the facial nerve. TACs show differences not only in the length and frequency of attacks but also in the response to drug treatment. It is important to recognize and differentiate between these syndromes because they react very well, but very selectively to therapy.     

Chronic paroxysmal hemicrania in paediatric age: report of two cases

Chronic paroxysmal hemicrania (CPH) is a rare primary headache syndrome, which is classified along with hemicrania continua and short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) as trigeminal autonomic cephalalgia (TACs). CPH is characterised by short-lasting (2-30 min), severe and multiple (more than 5/day) pain attacks. Headache is unilateral, and fronto-orbital-temporal pain is combined with cranial autonomic symptoms. According to the International Classification of Headache Disorders, 2nd edition, the attacks are absolutely responsive to indomethacin. CPH has been only rarely and incompletely described in the developmental age. Here, we describe two cases concerning a 7-year-old boy and a 11-year-old boy with short-lasting, recurrent headache combined with cranial autonomic features. Pain was described as excruciating, and was non-responsive to most traditional analgesic drugs. The clinical features of our children's headache and the positive response to indomethacin led us to propose the diagnosis of CPH. Therefore, our children can be included amongst the very few cases of this trigeminal autonomic cephalgia described in the paediatric age.     

Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing

SUNCT (Short-lasting Unilateral Neuralgiform headache attacks with Conjunctival injection and Tearing) and SUNA (Short-lasting Unilateral Neuralgiform headache attacks with cranial Autonomic symptoms) are rare primary headache syndromes, classified as Trigeminal Autonomic Cephalalgias (TACs). Hypothalamic involvement in the TACs has been suggested by functional imaging data and clinically with deep brain stimulation. Fifty-two patients (43 SUNCT, 9 SUNA) were studied to determine the clinical phenotype of these conditions and response to medications. A functional imaging study explored activation of the posterior hypothalamus in attacks of SUNCT/SUNA. The clinical study characterised SUNCT and SUNA in terms of epidemiology, phenotype and clinical characteristics. Indomethacin is ineffective on single-blind testing. Intravenous lidocaine was effective in all cases. Open-label trails showed the effectiveness of lamotrigine, topiramate and gabapentin. On functional imaging there was hypothalamic activation bilaterally in 5/9 SUNCT patients, and contralaterally in two patients. Two SUNCT patients had ipsilateral negative activation. In SUNA the activation was bilaterally negative. There was no hypothalamic activation in a patient with SUNCT secondary to a brainstem lesion. The data suggests that there should be revised classification for SUNCT and SUNA, with an increased range of attack duration and frequency, cutaneous triggering of attacks, and a lack of refractory period. The concept of 'attack load' is introduced. The lack of response to indomethacin and the response to intravenous lidocaine, are useful in diagnostic and therapeutic terms, respectively. Preventive treatments include lamotrigine, gabapentin and topiramate. The role of hypothalamic involvement in SUNCT and SUNA as TACs is considered.     

Pathophysiology and treatment of trigeminal autonomic cephalalgias

Trigeminal autonomic cephalalgias (TACs) are a group of primary headache disorders, which are characterized by strictly unilateral pain, together with ipsilateral cranial autonomic symptoms. TACs include cluster headache (CH), paroxysmal hemicrania (PH) and short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT syndrome). These diseases all have one thing in common: an activation of trigeminal nociceptive afferentia with a reflex-like activation of cranial autonomic efferentia via the facial nerve. TACs show differences not only in the length and frequency of attacks but also in the response to drug treatment. It is important to recognize and differentiate between these syndromes because they react very well, but very selectively to therapy.     

Objective assessment of autonomic signs during triggered first division trigeminal neuralgia

A total of 26 episodes of V-1 trigeminal neuralgia attacks have been recorded in two female patients. Autonomic phenomena were assessed according to a semiquantitative scale. Attacks lasted 17 +/- 5 s. Mild lacrimation without conjunctival hyperaemia, rhinorrhea or ptosis was observed, even in relatively long lasting episodes. This is in clear contradiction with SUNCT (shortlasting, unilateral, neuralgiform headache with conjunctival injection, tearing and rhinorrhea) attacks that are always dramatically accompanied by both lacrimation and conjunctival injection of the symptomatic side from the very onset of symptoms. Carbamazepine provided complete and sustained relief of symptoms in both patients. Herein we will show differential autonomic features of V-1 trigeminal neuralgia vs. SUNCT that will both aid the clinician to distinguish both syndromes and stress that both entities are nosologicaly different.     

Meningioma causing gabapentin-responsive secondary SUNCT syndrome

Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) is one of the rarest and most serious headache disorders. Cases of symptomatic SUNCT syndromes are reported, which demonstrate that brain imaging is very important for diagnosis. In this study, we describe the first case of secondary SUNCT syndrome caused by a meningioma. So far, a clearly effective therapy for SUNCT syndrome has not been known. In this case, however, SUNCT was completely responsive to gabapentin. This underlines that this drug is worthy of being considered as a potential therapeutic option in the treatment of SUNCT syndrome.     

少见的三叉神经自主神经性头痛临床特点

目的:探讨伴有结膜充血及流泪的单侧短暂持续性神经痛样头痛(SUNCT)和阵发性偏侧头痛(PH)的临床特点.方法:回顾性分析2例SUNCT患者和3例PH患者的临床表现及治疗方法.结果:5例患者均表现为单侧眼眶、颞部的剧烈疼痛,并伴有同侧的颅内副交感神经激活症状,持续时间从20秒到30分钟,每天发作频率从8次到100次;S...     

SUNCT: a new case with some unusual features

A healthy 22–year–old man complained of primary stabbing headache (PSH) for about two months. The headache recurred after one year and after a month the pain took on the characteristics of short–lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT). This patient shows some unusual features: juvenile onset, miosis during attacks as part of autonomic phenomena and close temporal relationship with stressful events. The association between PSH and SUNCT may be interpreted as the coexistence of two different headaches or that the PSH is a forerunner to the SUNCT.