Cluster tic syndrome and other combinations of primary headaches with trigeminal neuralgia

Author(s):  
Leopoldine A. Wilbrink ◽  
Joost Haan ◽  
Juan A. Pareja

Cluster tic syndrome (cluster headache and trigeminal neuralgia) and other combined tic syndromes are very rare and heterogeneously described as case reports in the literature. Three types of cluster tic syndromes can be distinguished: (i) simultaneously occurring trigeminal neuralgia and cluster headache according to the ICHD-2 criteria (most frequent); (2) attacks not fulfilling criteria for either cluster headache or trigeminal neuralgia but having features of both; (iii) secondary cluster tic syndrome. Trigeminal neuralgia can also co-occur with paroxysmal hemicrania (paroxysmal hemicrania tic) and with SUNCT. In case of co-occurrence of two primary headache syndromes, a combination of separate treatments aimed at the two headache syndromes is recommended. The pathophysiology of the tic combination syndromes is unknown.

Author(s):  
Thijs H. Dirkx ◽  
Peter J. Koehler

The trigeminal autonomic cephalalgias (TACs), including cluster headache, paroxysmal hemicrania, SUNCT (short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing), SUNA (with cranial autonomic symptoms), and hemicrania continua, belong to the primary headaches. They are characterized by severe unilateral headache attacks in association with ipsilateral cranial autonomic features. Cluster headache is the most frequent of the TACs. The other TACs are rare, but epidemiological data are scarce and variable. The various types of TAC are distinguished not only by differences in attack frequency and duration, but also by differences with respect to treatment response. The typical headache syndromes, fulfilling the International Classification of Headache Disorders-3 criteria, have also been described in association with other disorders and imaging is required to exclude intracranial pathology in all newly diagnosed patients.


Cephalalgia ◽  
2008 ◽  
Vol 28 (6) ◽  
pp. 626-630 ◽  
Author(s):  
P Irimia ◽  
E Cittadini ◽  
K Paemeleire ◽  
AS Cohen ◽  
PJ Goadsby

Our objective was to compare the presence of self-reported unilateral photophobia or phonophobia, or both, during headache attacks comparing patients with trigeminal autonomic cephalalgias (TACs)—including cluster headache, shortlasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and paroxysmal hemicrania—or hemicrania continua, and other headache types. We conducted a prospective study in patients attending a referral out-patient clinic over 5 months and those admitted for an intramuscular indomethacin test. Two hundred and six patients were included. In episodic migraine patients, two of 54 (4%) reported unilateral photophobia or phonophobia, or both. In chronic migraine patients, six of 48 (13%) complained of unilateral photophobia or phonophobia, or both, whereas none of the 24 patients with medication-overuse headache reported these unilateral symptoms, although these patients all had clinical symptoms suggesting the diagnosis of migraine. Only three of 22 patients (14%) suffering from new daily persistent headache (NDPH) experienced unilateral photophobia or phonophobia. In chronic cluster headache 10 of 21 patients (48%) had unilateral photophobia or phonophobia, or both, and this symptom appeared in four of five patients (80%) with episodic cluster headache. Unilateral photophobia or phonophobia, or both, were reported by six of 11 patients (55%) with hemicrania continua, five of nine (56%) with SUNCT, and four of six (67%) with chronic paroxysmal hemicrania. Unilateral phonophobia or photophobia, or both, are more frequent in TACs and hemicrania continua than in migraine and NDPH. The presence of these unilateral symptoms may be clinically useful in the differential diagnosis of primary headaches.


Neurology ◽  
2019 ◽  
Vol 93 (12) ◽  
pp. e1138-e1147 ◽  
Author(s):  
Christian Ziegeler ◽  
Arne May

ObjectiveTo assess the prevalence of facial pain (V2 and/or V3) presentations among nearly 3,000 patients with headache treated in a university tertiary care center.MethodsBetween 2010 and 2018, we routinely assessed the prevalence of facial pain presentations of all patients with primary headaches.ResultsOf 2,912 patient datasets, 291 patients reported facial pain either as an independent or as an additional symptom. Among patients with migraine, 2.3% (44 of 1,935) reported a facial involvement, most commonly in V2. Of these, 18 patients (40.9%) experienced the pain predominantly in the face. In patients with cluster headache, 14.8% (42 of 283) reported a facial involvement, of which 31.0% perceived the pain predominantly in the face. A facial involvement was seen in 45.0% of patients with paroxysmal hemicrania (9 of 20), 21.4% of patients with hemicrania continua (9 of 42), and 20.0% of patients with short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing/short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (3 of 15). In addition, we present 6 patients who reported a constant side-locked facial pain with superseded well-defined facial pain attacks of 10- to 30-minute duration that appeared several times per day.ConclusionOur data suggest that a facial involvement in primary headaches is infrequent but not uncommon. A sole facial presentation of primary headache symptomatology seems to be exceptionally rare. We describe 3 different types of facial pain involvement and, in this context, distinguish patients with paroxysmal orofacial pain syndromes that have not been previously described. These patients may represent a new entity that could tentatively be called constant unilateral facial pain with added attacks.


Cephalalgia ◽  
2002 ◽  
Vol 22 (9) ◽  
pp. 725-729 ◽  
Author(s):  
TD Rozen

Recently, cluster headache has been shown to occur with aura, suggesting that as more cluster patients are seen by headache specialists new forms of this well-defined primary headache syndrome will be identified. This study presents three atypical presentations of cluster headache: persistent or unremitting cluster, periodic cluster, and reflex or event-related cluster. Case reports are presented with an explanation as to why these headaches should be considered cluster headache.


1999 ◽  
Vol 19 (2) ◽  
pp. 115-127 ◽  
Author(s):  
Arne May ◽  
Peter J. Goadsby

Primary headache syndromes, such as cluster headache and migraine, are widely described as vascular headaches, although considerable clinical evidence suggests that both are primarily driven from the brain. The shared anatomical and physiologic substrate for both of these clinical problems is the neural innervation of the cranial circulation. Functional imaging with positron emission tomography has shed light on the genesis of both syndromes, documenting activation in the midbrain and pons in migraine and in the hypothalamic gray in cluster headache. These areas are involved in the pain process in a permissive or triggering manner rather than as a response to first-division nociceptive pain impulses. In a positron emission tomography study in cluster headache, however, activation in the region of the major basal arteries was observed. This is likely to result from vasodilation of these vessels during the acute pain attack as opposed to the rest state in cluster headache, and represents the first convincing activation of neural vasodilator mechanisms in humans. The observation of vasodilation was also made in an experimental trigeminal pain study, which concluded that the observed dilation of these vessels in trigeminal pain is not inherent to a specific headache syndrome, but rather is a feature of the trigeminal neural innervation of the cranial circulation. Clinical and animal data suggest that the observed vasodilation is, in part, an effect of a trigeminoparasympathetic reflex. The data presented here review these developments in the physiology of the trigeminovascular system, which demand renewed consideration of the neural influences at work in many primary headaches and, thus, further consideration of the physiology of the neural innervation of the cranial circulation. We take the view that the known physiologic and pathophysiologic mechanisms of the systems involved dictate that these disorders should be collectively regarded as neurovascular headaches to emphasize the interaction between nerves and vessels, which is the underlying characteristic of these syndromes. Moreover, the syndromes can be understood only by a detailed study of the cerebrovascular physiologic mechanisms that underpin their expression.


Cephalalgia ◽  
2010 ◽  
Vol 30 (8) ◽  
pp. 975-982 ◽  
Author(s):  
Sanjay Prakash ◽  
Nilima D Shah ◽  
Bhavna V Chavda

Introduction: Response to indomethacin is an essential feature for the diagnosis of both paroxysmal hemicrania (PH) and hemicrania continua (HC). Cluster headache (CH) is widely considered to be a disease unresponsive to indomethacin. Case reports: We report four patients with CH who responded to indomethacin. Two patients, who were refractory to the usual therapy for CH, fulfilled the criteria for chronic CH. Conversely, two patients had a history of episodic CH and showed response to both indomethacin and the usual therapy for CH. Literature review: We also reviewed the literature for the presence of indomethacin response in patients with CH. We noted a large number of cases labeled as CH by the authors which showed a response to indomethacin. Discussion: Many cases of definite or possible CH were wrongly labeled as PH because of patients' responding to indomethacin. Conclusion: The response to indomethacin in patients with CH may not be as immediate as in other indomethacin-responsive headaches, and many patients may need larger doses.


2000 ◽  
Vol 58 (2B) ◽  
pp. 518-521 ◽  
Author(s):  
PAULO HÉLIO MONZILLO ◽  
WILSON LUIZ SANVITO ◽  
AGNALDO R. DA COSTA

The so-called short lasting primary headaches include heterogenic entities that can be divided between those without pronounced autonomic activation and those where this activation is evident, which includes the cluster-tic syndrome. We report five new cases with age closer to the trigeminal neuralgia's one, and concomitance of cluster headache and trigeminal neuralgia, which is less frequent in the literature. We also discuss briefly the pathophysiology of these clinical entities, suggesting that the trigeminus nerve is a common pathway of pain manifestation.


2020 ◽  
Vol 10 (1) ◽  
pp. 30 ◽  
Author(s):  
Andrea Carmine Belin ◽  
Caroline Ran ◽  
Lars Edvinsson

Cluster headache (CH) is a severe primary headache with a prevalence of 1/1000 individuals, and a predominance in men. Calcitonin gene-related peptide (CGRP) is a potent vasodilator, originating in trigeminal neurons and has a central role in CH pathophysiology. CGRP and the CGRP receptor complex have recently taken center stage as therapeutic targets for primary headaches, such as migraine. Multiple CGRP and CGRP receptor monoclonal antibodies, as well as small molecule antagonists (gepants) are on their way constituting a new frontier of migraine and possibly CH medication. During a CH attack, there is an activation of the trigeminal-autonomic reflex with the release of CGRP, and inversely if CGRP is administered to a CH patient in an active disease phase, it triggers an attack. Increased levels of CGRP have been found in ipsilateral jugular vein blood during the active phase of CH. This process is hypothesized to have a key role in the intense pain perception and in the associated distinctive vasodilation. So far, clinical tests of CGRP antibodies have been inconclusive in CH patients. This review summarizes the current state of knowledge on the role of CGRP in CH pathology, and as a target for future treatments.


Cephalalgia ◽  
1992 ◽  
Vol 12 (5) ◽  
pp. 318-320 ◽  
Author(s):  
Alain Djacoba Tehindrazanarivelo ◽  
Jean Marc Visy ◽  
Marie-Germaine Bousser

We report two patients with ipsilateral attacks of cluster headache and chronic paroxysmal hemicrania. The first patient, a 33-year-old man, started having attacks of chronic cluster headache at the age of 27. At 33, they were replaced by typical attacks of ipsilateral chronic paroxysmal hemicrania which showed a dramatic improvement with indomethacin 150 mg daily. After two days of complete remission, cluster headache attacks reappeared and persisted until verapamil, 360 mg a day, was added to indomethacin. The second patient, a 45-year-old man, first developed attacks of episodic cluster headache at the age of 35. At 44, he experienced ipsilateral typical attacks of chronic paroxysmal hemicrania, and two months later attacks of cluster headache. Under verapamil 240 mg daily, attacks of cluster headache disappeared, but those of chronic paroxysmal hemicrania increased in frequency until indomethacin 150 mg daily was added. These observations suggest a close relationship but not a similarity between cluster headache and chronic paroxysmal hemicrania, and show the practical therapeutic interest of maintaining this distinction. Cluster headache, chronic paroxysmal hemicrania, indomethacin, verapamil


Cephalalgia ◽  
2013 ◽  
Vol 33 (6) ◽  
pp. 384-389 ◽  
Author(s):  
Anke Hambach ◽  
Stefan Evers ◽  
Oliver Summ ◽  
Ingo W Husstedt ◽  
Achim Frese

Background Headache associated with sexual activity is a well-known primary headache disorder. In contrast, some case reports in the literature suggest that sexual activity during a migraine or cluster headache attack might relieve the pain in at least some patients. We performed an observational study among patients of a tertiary headache clinic. Methods A questionnaire was sent to 800 unselected migraine patients and 200 unselected cluster headache patients. We asked for experience with sexual activity during a headache attack and its impact on headache intensity. The survey was strictly and completely anonymous. Results In total, 38% of the migraine patients and 48% of the patients with cluster headache responded. In migraine, 34% of the patients had experience with sexual activity during an attack; out of these patients, 60% reported an improvement of their migraine attack (70% of them reported moderate to complete relief) and 33% reported worsening. In cluster headache, 31% of the patients had experience with sexual activity during an attack; out of these patients, 37% reported an improvement of their cluster headache attack (91% of them reported moderate to complete relief) and 50% reported worsening. Some patients, in particular male migraine patients, even used sexual activity as a therapeutic tool. Conclusions The majority of patients with migraine or cluster headache do not have sexual activity during headache attacks. Our data suggest, however, that sexual activity can lead to partial or complete relief of headache in some migraine and a few cluster headache patients.


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