SUNCT syndrome

Last updated
SUNCT syndrome
Specialty Neurology   OOjs UI icon edit-ltr-progressive.svg
Frequency50 described cases (as of 2015) [1]

Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT syndrome) is a rare headache disorder that belongs to the group of headaches called trigeminal autonomic cephalalgia (TACs). [1] Symptoms include excruciating burning, stabbing, or electrical headaches mainly near the eye and typically these sensations are only on one side of the body. The headache attacks are typically accompanied by cranial autonomic signs that are unique to SUNCT. Each attack can last from five seconds to six minutes and may occur up to 200 times daily.

Contents

TACs are caused by activation of the autonomic nervous system of the trigeminal nerve in the face.

As of 2015 about 50 cases have been described in the medical literature. [1] Onset of the symptoms usually come later in life, at an average age of about 50. Although the majority of patients are men over the age of 50, it is not uncommon to find SUNCT present among other age groups, including children and infants.

Signs and symptoms

People affected by SUNCT often describe their headache attacks as excruciating pain. The attacks are severe enough to disrupt daily activities, but hospitalization is not necessary for most affected individuals.[ citation needed ]

Frequency and duration

The average number of attacks per day is around 60, ranging from 3 to 200 times. [2]

The attacks can be divided into three groups: single stabs, groups of stabs, and attacks with saw-tooth pattern, from the shortest to the longest duration respectively. The attacks usually last from five seconds to 240 seconds. Pain is described as excruciating and stabbing, pulsating, electric, or burning in nature. In attacks of longer duration, the pain changes, following a sawtooth pattern characterized by multiple stabs. Typically, longer attacks are more painful due to psychological effects, and patients often feel agitated before and during the attack. They occur mostly in the orbital, supraorbital, or temporal region, but can also occur in the retro-orbital (behind the orbit of the eye) region, side, top, and back of head, second and third trigeminal divisions, teeth, neck, and ear. Only a negligible percentage of attacks (less than 2%) occur at night. [3]

Cranial autonomic symptoms associated with headache attacks

SUNCT often accompanies cranial autonomic symptoms, including lacrimation (tear flow), ipsilateral ptosis (drooping of the eyelid which is on the same side as the attacks), eyelid edema (swelling due to fluid accumulation), nasal blockage, and conjunctival injection (redness of eye). Depending on which division of the trigeminal nerve innervates the area affected, autonomic symptoms may be less obvious. Short-lasting unilateral neuralgiform headache attacks with cranial autonomic features (SUNA) is a subset of SUNCT that constitutes less than 20% of all reported cases, and may be diagnosed when fewer autonomic symptoms are observed during attacks. Due to the rarity of these conditions, the small number of properly diagnosed patients does not allow for establishing a clear definition. The main difference between SUNCT and SUNA is the clinical presentation of the secondary autonomic symptoms. In SUNCT, both conjunctival injection (red eyes) and lacrimation on the same side of the headache should be present. In SUNA, one or both of these two symptoms are absent, and other autonomic symptoms may be present instead. SUNA attacks may also sometimes be longer, up to 10 minutes in total. [3]

The majority of SUNCT attacks occur chronically; however, some occur episodically. In chronic SUNCT, attacks can occur regularly or irregularly without a distinct refractory period, whereas in episodic SUNCT, attacks occur only for a certain period of time and then cease for another period. Episodes of attacks last from a few days to a few months, and up to 22 attack episodes in one year have been observed. SUNCT can go into remission even after a chronic period, and relapse either spontaneously or due to some stimulus. [2]

Triggers of headache attacks

Most of the time SUNCT occurs spontaneously; however, attacks can be triggered by simple daily stimuli such as touching the face or scalp, washing, chewing, eating, talking, coughing, blowing nose, or showering. They also can be triggered by light (including sunlight and fluorescent lights) and injuries. Stimuli capable of triggering a cluster headache, including alcohol, smoke, strong smells and a warm environment, can also trigger SUNCT in a few patients. [2] In some cases, patients with episodic SUNCT only exhibit symptoms biannually, in spring and fall. This periodicity suggests that the hypothalamus, which is responsive to day length and photoperiod for regulating circadian and seasonal rhythms, has a role in its triggering. [4]

Diagnosis

SUNCT must be properly distinguished from cluster headaches, since cluster headaches also occur several times per day with separate attacks, and share some common symptoms. However, cluster headaches usually last longer (up to three hours), occur less often (three to five attacks per day), and do not accompany cranial autonomic symptoms. IHS standard criteria for the diagnosis of SUNCT specifically includes pain in the trigeminal division of the face, especially in the orbital region, often with cranial autonomic symptoms which last for relatively short periods of time (from five seconds to several minutes) up to 100 times per day. SUNCT is a major subset of SUNA, [5] which does not accompany cranial symptoms; complete separation between the two is inappropriate since SUNCT does not necessarily always accompany cranial autonomic signs. Exact statistical data is not available due to common mis-diagnosis, and setting up diagnostic criteria is important. [2]

The International Headache Classification established by the International Headache Society criteria for diagnosing SUNCT for therapeutic purposes is:[ citation needed ]

(i) Type of attack – Attacks of unilateral orbital, supraorbital, or temporal areas from stabbing or pulsating pain accompanied by ipsilateral conjunctival injection and lacrimation
(ii) Number of attacks per day – 3 to 200 attacks per day
(iii) Attack load in minutes per day – 5–240 seconds

Symptoms of SUNCT often lead to misdiagnosis as paroxysmal hemicrania, which is also categorized in the same group. Inefficiency of indomethacin usually indicates SUNCT over paroxysmal hemicrania. [6]

Misdiagnosis and indecisive diagnosis in the past has made it difficult to obtain accurate statistics about SUNCT. Proper diagnosis will broaden data availability and facilitate discovery of new treatment options and useful statistics.

Pathophysiology

The pathophysiology of SUNCT is incompletely understood. Recent studies suggest the existence of a relationship between the hypothalamus and the condition. Functional MRI and deep brain stimulation of a large patient pool showed activation of the posterior hypothalamus during attacks of primary SUNCT, on both sides and contralaterally (on the opposite side). [7] Functional MRI studies suggest brain stem activation during attacks as well. [8] Activation of the trigeminocervical complex and other related structures results from innervation from branches of trigeminal and upper cervical nerves. [9] There exists a direct connection between trigeminal nucleus caudalis, which is located in the brain stem, and the posterior hypothalamus. Therefore, it is possible that stimulation of the peripheral trigeminal nerve activates the hypothalamus and the hypothalamus in turn communicates with the trigeminal nucleus caudalis via neurotransmitters such as orexin.[ citation needed ]

Elevated levels of prolactin, secretion of which is regulated by the hypothalamus, seem to be associated with SUNCT attacks. Some patients with a pituitary tumor only experience attacks at night. These patients only exhibit higher levels of prolactin at night, even where hormone levels, including prolactin, were normal throughout the day. [10]

SUNCT is considered a primary headache (or condition), but can also occur as a secondary symptom of other conditions. However, a patient can only be diagnosed with SUNCT as a primary condition.[ citation needed ]

A pituitary tumor causes SUNCT as a secondary headache. Some patients with a pituitary tumor complain of short-lasting heachaches. Upon removal of the tumor, the symptoms of SUNCT may disappear permanently. [11]

Although it occurs very rarely, paranasal sinusitis can also cause secondary SUNCT. The symptoms of SUNCT in such cases can be removed permanently with endoscopic sinus surgery. [12]

Treatment

As diagnostic criteria have been indecisive and its pathophysiology remains unclear, no permanent cure is available. Antiepileptic medications (membrane-stabilizing drugs) such as pregabalin, gabapentin, topiramate, and lamotrigine improve symptoms, but there is no effective permanent or long-term treatment for SUNCT. [13] Lamotrigine exhibits some long-term prevention and reduction in some patients according to case reports and observational studies; however, titration of dose is necessary due to adverse skin reactions. [14] Lamotrigine was found to be the most effective treatment according to a later open-label study, with 56 percent of patients responding to treatment compared to oxcarbazepine (46%), duloxetine (30%), carbamazepine (26%), topiramate (25%), pregabalin and gabapentin (10%). [15]

Intravenous lidocaine can abolish symptoms during its administration, or reduce frequency and duration of attacks. [16] Around 90 percent of patients responded to this treatment in one study. [15] However, administration of intravenous lidocaine requires careful monitoring of ECG and blood pressure. [16] Methylprednisolone therapy shows some promise in short-term prevention of attacks, even though its mechanism of action is yet to be discovered. [17]

The calcium channel blocker verapamil is reported to be useful in alleviating symptoms (lower frequency and duration of attacks), even though some patients experience worsened symptoms. [6]

Various medications that are often used in other headache syndromes such as nonsteroidal anti-inflammatory drugs, acetaminophen, tricyclic antidepressants, calcium channel antagonists do not relieve the symptoms of SUNCT.

There have been attempts to alter oxygen supply during attacks to alleviate the symptoms since some of the headaches are caused by decreased oxygen supply; however, elevated blood oxygen level did not affect the symptoms.

Researchers now[ when? ] focus on the administration of various combination of medications and therapies to treat symptoms of SUNCT. [18]

History

In 1977, a 62-year-old male patient was the first to be diagnosed with SUNCT. The patient had experienced unilateral, mild pain in the ocular and periorbital areas since he was 30 years old. The patient started to experience more severe attacks after being struck by a fishing rod in the lower medial supraorbital area when he was 58 years old. The attacks could be provoked by mechanical stimuli and occurred more frequently with symptoms of conjuctival injection, lacrimation, and rhinorrhea. The definitive diagnosis and determination of the role of the trauma (fishing rod strike) could not be made until 10 years later when another similar case occurred. Conditions of the next case ruled out the possibility of SUNCT being a post-traumatic headache. [19]

Bad Brains singer H.R. has this disorder, as revealed by his wife. [20]

See also

Related Research Articles

<span class="mw-page-title-main">Headache</span> Pain in the head, neck, or face

Headache, also known as cephalalgia, is the symptom of pain in the face, head, or neck. It can occur as a migraine, tension-type headache, or cluster headache. There is an increased risk of depression in those with severe headaches.

<span class="mw-page-title-main">Cluster headache</span> Neurological disorder

Cluster headache (CH) is a neurological disorder characterized by recurrent severe headaches on one side of the head, typically around the eye(s). There is often accompanying eye watering, nasal congestion, or swelling around the eye on the affected side. These symptoms typically last 15 minutes to 3 hours. Attacks often occur in clusters which typically last for weeks or months and occasionally more than a year.

<span class="mw-page-title-main">Trigeminal neuralgia</span> Neurological pain disorder

Trigeminal neuralgia, also called Fothergill disease, tic douloureux, trifacial neuralgia, or suicide disease, is a long-term pain disorder that affects the trigeminal nerve, the nerve responsible for sensation in the face and motor functions such as biting and chewing. It is a form of neuropathic pain. There are two main types: typical and atypical trigeminal neuralgia. The typical form results in episodes of severe, sudden, shock-like pain in one side of the face that lasts for seconds to a few minutes. Groups of these episodes can occur over a few hours. The atypical form results in a constant burning pain that is less severe. Episodes may be triggered by any touch to the face. Both forms may occur in the same person. It is regarded as one of the most painful disorders known to medicine, and often results in depression and suicide.

<span class="mw-page-title-main">Triptan</span> Class of pharmaceutical drugs

Triptans are a family of tryptamine-based drugs used as abortive medication in the treatment of migraines and cluster headaches. This drug class was first commercially introduced in the 1990s. While effective at treating individual headaches, they do not provide preventive treatment and are not considered a cure. They are not effective for the treatment of tension–type headache, except in persons who also experience migraines. Triptans do not relieve other kinds of pain.

Chronic paroxysmal hemicrania (CPH) is a severe debilitating unilateral headache usually affecting the area around the eye. It normally consists of multiple severe, yet short, headache attacks affecting only one side of the cranium. It is more commonly diagnosed in women than in men, but, unlike a migraine, has no neurological symptoms associated with it. CPH headaches are treated through the use of non-steroidal anti-inflammatory drugs, with indomethacin found to be usually effective in eliminating symptoms.

Hemicrania continua (HC) is a persistent unilateral headache that responds to indomethacin. It is usually unremitting, but rare cases of remission have been documented. Hemicrania continua is considered a primary headache disorder, meaning that another condition does not cause it.

<span class="mw-page-title-main">Tolosa–Hunt syndrome</span> Medical condition

Tolosa–Hunt syndrome is a rare disorder characterized by severe and unilateral headaches with orbital pain, along with weakness and paralysis (ophthalmoplegia) of certain eye muscles.

Geniculate ganglionitis or geniculate neuralgia (GN), also called nervus intermedius neuralgia, Ramsay Hunt syndrome, or Hunt's neuralgia, is a rare disorder characterized by severe paroxysmal neuralgic pain deep in the ear, that may spread to the ear canal, outer ear, mastoid or eye regions. GN may also occur in combination with trigeminal or glossopharyngeal neuralgia.

<span class="mw-page-title-main">Orthostatic headache</span> Medical condition

Orthostatic headache is a medical condition in which a person develops a headache while vertical and the headache is relieved when horizontal. Previously it was often misdiagnosed as different primary headache disorders such as migraine or tension headaches. Increasing awareness of the symptom and its causes has prevented delayed or missed diagnosis.

New daily persistent headache (NDPH) is a primary headache syndrome which can mimic chronic migraine and chronic tension-type headache. The headache is daily and unremitting from very soon after onset, usually in a person who does not have a history of a primary headache disorder. The pain can be intermittent, but lasts more than 3 months. Headache onset is abrupt and people often remember the date, circumstance and, occasionally, the time of headache onset. One retrospective study stated that over 80% of patients could state the exact date their headache began.

The International Classification of Headache Disorders (ICHD) is a detailed hierarchical classification of all headache-related disorders published by the International Headache Society. It is considered the official classification of headaches by the World Health Organization, and, in 1992, was incorporated into the 10th edition of their International Classification of Diseases (ICD-10). Each class of headache contains explicit diagnostic criteria—meaning that the criteria include quantities rather than vague terms like several or usually—that are based on clinical and laboratory observations.

<span class="mw-page-title-main">Cerebrospinal fluid leak</span> Medical condition

A cerebrospinal fluid leak is a medical condition where the cerebrospinal fluid (CSF) that surrounds the brain and spinal cord leaks out of one or more holes or tears in the dura mater. A CSF leak is classed as either nonspontaneous (primary), having a known cause, or spontaneous (secondary) where the cause is not readily evident. Causes of a primary CSF leak are those of trauma including from an accident or intentional injury, or arising from a medical intervention known as iatrogenic. A basilar skull fracture as a cause can give the sign of CSF leakage from the ear nose or mouth. A lumbar puncture can give the symptom of a post-dural-puncture headache.

Vestibular migraine (VM) is vertigo with migraine, either as a symptom of migraine or as a related neurological disorder.

The classification of all headaches, including migraines, is organized by the International Headache Society, and published in the International Classification of Headache Disorders (ICHD). The current version, the ICHD-3 beta, was published in 2013.

<span class="mw-page-title-main">Red ear syndrome</span> Medical condition

Red ear syndrome (RES) is a rare disorder of unknown etiology which was originally described in 1994. The defining symptom of red ear syndrome is redness of one or both external ears, accompanied by a burning sensation. A variety of treatments have been tried with limited success.

Abdominal migraine(AM) is a functional disorder that usually manifests in childhood and adolescence, without a clear pathologic mechanism or biochemical irregularity. Children frequently experience sporadic episodes of excruciating central abdominal pain accompanied by migrainous symptoms like nausea, vomiting, severe headaches, and general pallor. Abdominal migraine can be diagnosed based off clinical criteria and the exclusion of other disorders.

Migraine treatment may be either prophylactic (preventive) or abortive (rescue) or acute. Prevention is better than cure, so the ideal treatment goal is to prevent migraine attacks. Because migraine is an exceedingly complex condition, there are various preventive treatments which have their effect by disrupting different links in the chain of events that occur during a migraine attack. As rescue treatments also target and disrupt different processes occurring during migraine, these are summarized, with their relative merits and demerits.

Trigeminal autonomic cephalalgia (TAC) refers to a group of primary headaches that occurs with pain on one side of the head in the trigeminal nerve area and symptoms in autonomic systems on the same side, such as eye watering and redness or drooping eyelids.

Neck-tongue syndrome (NTS), which was first recorded in 1980, is a rare disorder characterized by neck pain with or without tingling and numbness of the tongue on the same side as the neck pain. Sharp lateral movement of the head triggers the pain, usually lasting from a few seconds to a few minutes. Headaches may occur with the onset of NTS. The typical age of onset is around adolescence and may occur as early as 8–15 years old. However, it is worth noting that clinical onset can occur earlier or later and NTS onset related to trauma can occur at any age, beginning after the incident.

<span class="mw-page-title-main">Recurrent painful ophthalmoplegic neuropathy</span> Medical condition

Recurrent painful ophthalmoplegic neuropathy (RPON), previously known as ophthalmoplegic migraine (OM), is a rare neurological disorder that is characterized by repeated headache attacks and reversible ipsilateral paresis of one or more ocular cranial nerves (CN). Oculomotor nerve (CNIII) is by far the most common cranial nerve involves in RPON, while abducens nerve (CNVI) and trochlear nerve (CNIV) involvements are also reported. Globally, RPON was estimated to have an annual incidence rate of 0.7 per million as of 1990, no further epidemiological studies have been conducted. It occurs more often in children and females.

References

  1. 1 2 3 Pomeroy, JL; Nahas, SJ (August 2015). "SUNCT/SUNA: A Review". Current Pain and Headache Reports. 19 (8): 38. doi:10.1007/s11916-015-0511-2. PMID   26092511. S2CID   29646696.
  2. 1 2 3 4 Cohen AS, Matharu MS, Goadsby PJ (October 2006). "Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) or cranial autonomic features (SUNA)—a prospective clinical study of SUNCT and SUNA". Brain. 129 (Pt 10): 2746–60. doi: 10.1093/brain/awl202 . PMID   16905753.
  3. 1 2 Buttice', Claudio (2022). What you need to know about headaches. Santa Barbara, California: Greenwood Publishing Group. p. 59. ISBN   978-1-4408-7531-1. OCLC   1259297708.
  4. ↑ Irimia P, Gállego-Pérez Larraya J, Martinez-Vila E (January 2008). "Seasonal periodicity in SUNCT syndrome". Cephalalgia. 28 (1): 94–6. doi:10.1111/j.1468-2982.2007.01434.x. PMID   17868283. S2CID   19188443.
  5. ↑ Tada Y, Ikuta N, Negoro K (2009). "Short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA)". Intern. Med. 48 (24): 2141–4. doi: 10.2169/internalmedicine.48.2602 . PMID   20009409.
  6. 1 2 Narbone MC, Gangemi S, Abbate M (June 2005). "A case of SUNCT syndrome responsive to verapamil". Cephalalgia. 25 (6): 476–8. doi:10.1111/j.1468-2982.2004.00876.x. PMID   15910576. S2CID   10190618.
  7. ↑ Cohen, A. S., M. S. Matharu, et al. (2006). "Functional MRI in SUNCT (Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing) and SUNA (Short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms) shows differential hypothalamic activation with increasing pain." Journal of Neurology, Neurosurgery, and Psychiatry 77(12): 041.
  8. ↑ Auer T, Janszky J, Schwarcz A, et al. (June 2009). "Attack-related brainstem activation in a patient with SUNCT syndrome: an ictal fMRI study". Headache. 49 (6): 909–12. doi:10.1111/j.1526-4610.2009.01348.x. PMID   19220497. S2CID   39514918.
  9. ↑ Goadsby PJ, Lipton RB (January 1997). "A review of paroxysmal hemicranias, SUNCT syndrome and other short-lasting headaches with autonomic feature, including new cases". Brain. 120 (Pt 1): 193–209. doi: 10.1093/brain/120.1.193 . PMID   9055807.
  10. ↑ Bosco D, Labate A, Mungari P, Vero S, Fava A (April 2007). "SUNCT and high nocturnal prolactin levels: some new unusual characteristics". J Headache Pain. 8 (2): 114–8. doi:10.1007/s10194-007-0370-2. PMC   3476135 . PMID   17497264.
  11. ↑ Rocha Filho PA, Galvão AC, Teixeira MJ, et al. (June 2006). "SUNCT syndrome associated with pituitary tumor: case report". Arq Neuropsiquiatr. 64 (2B): 507–10. doi: 10.1590/s0004-282x2006000300029 . PMID   16917628.
  12. ↑ Choi JY, Seo WK, Kim JH, Oh K, Yu SW (2008). "Symptomatic SUNCT syndrome associated with ipsilateral paranasal sinusitis". Headache. 48 (10): 1527–30. doi:10.1111/j.1526-4610.2008.01243.x. PMID   19076651. S2CID   27878564. It is observed that TACs share some common phenotypes but have different pathophysiology.
  13. ↑ Pareja JA, Cuadrado ML, Caminero AB, Barriga FJ, Barón M, Sánchez-del-Río M (April 2005). "Duration of attacks of first division trigeminal neuralgia". Cephalalgia. 25 (4): 305–8. doi: 10.1111/j.1468-2982.2004.00864.x . PMID   15773828. S2CID   42285100.
  14. ↑ Rosselli, Jennifer L; Karpinski, Julie P (January 2011). "The Role of Lamotrigine in the Treatment of Short-Lasting Unilateral Neuralgiform Headache Attacks with Conjunctival Injection and Tearing Syndrome". Annals of Pharmacotherapy. 45 (1): 108–113. doi:10.1345/aph.1P462.
  15. 1 2 Lambru, Giorgio; Stubberud, Anker; Rantell, Khadija; Lagrata, Susie; Tronvik, Erling; Matharu, Manjit Singh (1 March 2021). "Medical treatment of SUNCT and SUNA: a prospective open-label study including single-arm meta-analysis". Journal of Neurology, Neurosurgery & Psychiatry. 92 (3): 233–241. doi: 10.1136/jnnp-2020-323999 . ISSN   0022-3050.
  16. 1 2 Arroyo AM, Durán XR, Beldarrain MG, Pinedo A, García-Moncó JC (January 2010). "Response to intravenous lidocaine in a patient with SUNCT syndrome". Cephalalgia. 30 (1): 110–2. doi:10.1111/j.1468-2982.2009.01871.x. PMID   19438923. S2CID   9801531.
  17. ↑ Trauninger A, Alkonyi B, Kovács N, Komoly S, Pfund Z (June 2010). "Methylprednisolone therapy for short-term prevention of SUNCT syndrome". Cephalalgia. 30 (6): 735–9. doi:10.1111/j.1468-2982.2009.01971.x. PMID   19673911. S2CID   42721204.
  18. ↑ Smit, Rupert D; Mouchtouris, Nikolaos; Kang, KiChang; Reyes, Maikerly; Sathe, Anish; Collopy, Sarah; Prashant, Giyarpuram; Yuan, Hsiangkuo; Evans, James J (17 August 2022). "Short-lasting unilateral neuralgiform headache attacks (SUNCT/SUNA): a narrative review of interventional therapies". Journal of Neurology, Neurosurgery & Psychiatry. 94 (1): jnnp–2022–329588. doi:10.1136/jnnp-2022-329588. PMID   35977820. S2CID   251627159.
  19. ↑ Sjaastad O, Russell D, Hørven I, et al. (1978). "Multiple, neuralgiform, unilateral headache attacks associated with conjunctival injection and appearing in clusters. A nosological problem". Proceedings of the Scandinavian Migraine Society. p. 31.
  20. ↑ Kreps, Daniel (March 16, 2016). "Bad Brains' H.R. Raising Money to Combat Headache Disorder". Rolling Stone . Retrieved 21 October 2016.
This page is based on this Wikipedia article
Text is available under the CC BY-SA 4.0 license; additional terms may apply.
Images, videos and audio are available under their respective licenses.