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The short-lasting primary headache syndromes may be conveniently divided into those exhibiting marked autonomic activation and those without autonomic activation. The former group comprise chronic and episodic paroxysmal hemicrania, short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT syndrome) and cluster headache. These headache syndromes are compared with other short-lasting headache disorders, such as hypnic headache, and persistent headache with milder autonomic features such as hemicrania continua. Cluster headache is included with the shorter-lasting headaches to attempt a nosological analysis of these syndromes. The paroxysmal hemicranias are characterized by frequent short-lasting attacks of unilateral pain usually in the orbital, supraorbital or temporal region that typically last minutes. The attack frequency usually ranges from 5 to 40 attacks per day. The pain is severe and associated with autonomic symptoms such as conjunctival injection, lacrimation, nasal congestion, rhinorrhoea, ptosis or eyelid oedema. Almost all reported cases respond to treatment with indomethacin, but respond poorly to other treatments including other nonsteroidal anti-inflammatory drugs. A recent case study demonstrated the release of both trigeminal and parasympathetic neuropeptides during a bout of pain in the same pattern previously described in cluster headache. The SUNCT syndrome is a distinctive rare condition characterized by less severe pain but marked autonomic activation during attacks. Consistent with previous reports, the present case of SUNCT syndrome was intractable to therapy. The similarites of these syndromes suggests a considerable shared pathophysiology. It is suggested that the syndromes are sufficiently well established for inclusion in the International Headache Society Classification system and that trigeminal-autonomic cephalalgias should be classified as a group together. A proposed re-classification is presented.
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PMID:A review of paroxysmal hemicranias, SUNCT syndrome and other short-lasting headaches with autonomic feature, including new cases. 905 7

Two patients suffering from SUNCT syndrome are presented. Some features are remarkable. The first patient was a 69-year-old man whose first crisis was located in the right supraorbital region. After a 4-month spontaneous remission, the pain returned to the upper part of the cheek, radiating to the supraciliary region on the same side, with lacrimation and conjunctival injection. Rhinorrhea was absent. The painful attacks were triggered by head movements. Clinical improvement occurred with carbamazepine treatment. The second patient was a 48-year-old woman whose painful attacks lasted from 30 to 45 seconds followed by a burning sensation lasting 2 hours. Autonomic signs such as conjunctival injection, lacrimation, and edema and ipsilateral ptosis of the upper lid were rather marked. There was never any rhinorrhea. Her attacks were triggered by head and eye movements. She responded to the administration of corticosteroids and carbamazepine. According to these features, the two patients had SUNCT syndrome, and the positive carbamazepine response suggests a relationship with trigeminal neuralgia.
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PMID:SUNCT syndrome. Two cases in Argentina. 963 Jul 90

The syndrome of short-lasting unilateral, neuralgiform attacks of pain in the peri orbital area associated with conjunctival injection and tearing (SUNCT) is a rare disorder affecting mainly males. We report two French patients (1 male and 1 female) with SUNCT syndrome, 27 and 28 years of age respectively. Both patients had short (30 sec), frequent (30-100/day) excruciating pain located at the peri orbital area, associated with conjunctival injection, tearing, rhinorrhea, ptosis and others vasomotor symptoms. Clinical examination and imaging were normal. Most drugs used in the treatment of migraine, cluster headache, trigeminal neuralgia, and other short-lasting headaches were not successful.
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PMID:[Idiopathic SUNCT (short lasting unilateral neuralgiform headache attacks with conjunctival injection, tearing, sweating and rhinorrhea) syndrome: 2 new cases]. 1192 48

Cluster headache (CH) is a primary headache disease characterized by recurrent short-lasting attacks (15 to 180 minutes) of excruciating unilateral periorbital pain accompanied by ipsilateral autonomic signs (lacrimation, nasal congestion, ptosis, miosis, lid edema, redness of the eye). It affects young adults, predominantly males. Prevalence is estimated at 0.5-1.0/1,000. CH has a circannual and circadian periodicity, attacks being clustered (hence the name) in bouts that can occur during specific months of the year. Alcohol is the only dietary trigger of CH, strong odors (mainly solvents and cigarette smoke) and napping may also trigger CH attacks. During bouts, attacks may happen at precise hours, especially during the night. During the attacks, patients tend to be restless. CH may be episodic or chronic, depending on the presence of remission periods. CH is associated with trigeminovascular activation and neuroendocrine and vegetative disturbances, however, the precise cautive mechanisms remain unknown. Involvement of the hypothalamus (a structure regulating endocrine function and sleep-wake rhythms) has been confirmed, explaining, at least in part, the cyclic aspects of CH. The disease is familial in about 10% of cases. Genetic factors play a role in CH susceptibility, and a causative role has been suggested for the hypocretin receptor gene. Diagnosis is clinical. Differential diagnoses include other primary headache diseases such as migraine, paroxysmal hemicrania and SUNCT syndrome. At present, there is no curative treatment. There are efficient treatments to shorten the painful attacks (acute treatments) and to reduce the number of daily attacks (prophylactic treatments). Acute treatment is based on subcutaneous administration of sumatriptan and high-flow oxygen. Verapamil, lithium, methysergide, prednisone, greater occipital nerve blocks and topiramate may be used for prophylaxis. In refractory cases, deep-brain stimulation of the hypothalamus and greater occipital nerve stimulators have been tried in experimental settings. The disease course over a lifetime is unpredictable. Some patients have only one period of attacks, while in others the disease evolves from episodic to chronic form.
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PMID:Cluster headache. 1865 39

The term "Trigeminal Autonomic Cephalalgia (TAC)" was first coined by Goadsby and Lipton[1] to include a group of relatively rare primary headache disorders characterized by moderate to severe, short-lived head pain in the trigeminal distribution with unilateral cranial parasympathetic autonomic features, such as lacrimation, rhinorrhea, conjunctival injection, eyelid edema, and ptosis. In the current International Classification of Headache Disorders (ICHD-3 beta),[2] the TAC group includes cluster headache (CH), paroxysmal hemicrania (PH), short-lasting unilateral neuralgiform headache attacks (SUNHAs) and their 2 subforms - SUNHAs with conjunctival injection and tearing (SUNCT), SUNHAs with cranial autonomic symptoms (SUNA). Hemicrania Continua (HC) is also now included in the TAC group. Although the entities included under TACs seem broadly similar, they differ in attack duration, frequency and their response to different treatments. At one end of the spectrum lies CH, the prototypic TAC where the duration of attacks is the longest and at the other end is the SUNCT syndrome where the duration is shortest. There is some overlap across the entities; they are not difficult to recognize and subclassify. The umbrella term "TAC" for the short-lasting headaches with autonomic features was for the first time introduced in The ICHD, 2nd edition (ICHD-2) published in 2004.[3] The beta version of the 3rd edition of The ICHD[2] was published in 2013. Headache classification being an evolving process, there have been some changes within the TAC group between ICHD-2 and ICHD-3 beta.[45] Diagnostic criteria have been revised to reflect pathophysiological and clinical observations. Neuroimaging has provided insights into the pathophysiology of TACs. Functional neuroimaging has helped to elucidate key structures activated during attacks of TACs. Correct diagnosis remains the key to correct management of the TACs because treatment options vary. The aim of this article will be to highlight the changes in ICHD-3 beta to this group and to emphasize the clinical implications of these changes. Description of individual entities included under TACs are included elsewhere and will therefore not be detailed here.
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PMID:Classification of Trigeminal Autonomic Cephalalgia: What has Changed in International Classification of Headache Disorders-3 Beta? 2972 Aug 18