Classification, diagnostic criteria, and epidemiology

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 ◽  
2013 ◽  
Vol 34 (3) ◽  
pp. 231-235 ◽  
Author(s):  
Andreas Totzeck ◽  
Hans-Christoph Diener ◽  
Charly Gaul

Introduction The trigeminal autonomic cephalalgias (TACs) subsume four primary headache disorders. Hemicrania continua is increasingly regarded as an additional TAC. In rare cases patients may present with two different TACs or a TAC and hemicrania continua. Cases We report four patients with two different TACs or one TAC and hemicrania continua. Two patients presented with cluster headache and paroxysmal hemicrania, one patient with cluster headache and hemicrania continua, and one patient suffered from cluster headache and SUNCT. Discussion While the International Classification of Headache Disorders (ICHD-II) proposes specific diagnostic criteria, the variability of clinical presentation may make clear diagnosis difficult. All patients fulfilled the ICHD-II criteria. The manifestation of two different TACs or hemicrania continua in one patient is uncommon but possible and should be taken into account especially when chronic headache patients present with changing headache symptoms.


Cephalalgia ◽  
2003 ◽  
Vol 23 (9) ◽  
pp. 929-930 ◽  
Author(s):  
C Lisotto ◽  
F Mainardi ◽  
F Maggioni ◽  
G Zanchin

The trigeminal autonomic cephalgias (TACs) are characterized by short-lasting unilateral headaches with autonomic features (1). They include four headache disorders, cluster headache (CH), paroxysmal hemicrania (PH), SUNCT syndrome and hemicrania continua (HC). The coexistence of different ipsilateral TACs in the same patient has been previously reported in six published cases (2-6). In five of these patients an association of CH and PH was noted (2-5). The two varieties of attacks occurred separately in three patients, while their simultaneous occurrence was observed in two cases. In another patient the successive occurrence of trigeminal neuralgia, SUNCT syndrome, PH and CH in one active headache period was noted (6). All the reported cases concerned male patients. We describe what we believe to be the first case of coexistence of two different contralateral TACs.


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.


2001 ◽  
Vol 59 (4) ◽  
pp. 944-947 ◽  
Author(s):  
Germany Gonçalves Veloso ◽  
Alexandre Ottoni Kaup ◽  
Mario F Pietro Peres ◽  
Eliova Zukerman

Episodic paroxysmal hemicrania (EPH) is a rare disorder characterized by frequent, daily attacks of short-lived, unilateral headache with accompanying ipsilateral autonomic features. EPH has attack periods which last weeks to months separated by remission intervals lasting months to years, however, a seasonal variation has never been reported in EPH. We report a new case of EPH with a clear seasonal pattern: a 32-year-old woman with a right-sided headache for 17 years. Pain occurred with a seasonal variation, with bouts lasting one month (usually in the first months of the year) and remission periods lasting around 11 months. During these periods she had headache from three to five times per day, lasting from 15 to 30 minutes, without any particular period preference. There were no precipitating or aggravating factors. Tearing and conjunctival injection accompanied ipsilaterally the pain. Previous treatments provided no pain relief. She completely responded to indomethacin 75 mg daily. After three years, the pain recurred with longer attack duration and was just relieved with prednisone. We also propose a new hypothesis: the EPH-cluster headache continuum.


Author(s):  
Gennaro Bussone ◽  
Elisabetta Cittadini

Paroxysmal hemicrania is classified as a trigeminal autonomic cephalalgia by the International Classification of Headache Disorders, third edition. The current criteria require at least 20 attacks of severe unilateral orbital, supraorbital, or temporal pain, lasting 2–30 minutes, accompanied by ipsilateral cranial autonomic features such as ptosis, eyelid oedema, conjunctival injection, lacrimation, nasal blockage, or rhinorrhoea. Attacks usually have a frequency of more than five per day, and respond exquisitely to indomethacin.


Cephalalgia ◽  
2020 ◽  
pp. 033310242095452
Author(s):  
Sharoon Qaiser ◽  
Andrew D Hershey ◽  
Joanne Kacperski

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.


Cephalalgia ◽  
2019 ◽  
Vol 39 (12) ◽  
pp. 1488-1499 ◽  
Author(s):  
Sarah Miller ◽  
Susie Lagrata ◽  
Manjit Matharu

Background Multiple cranial nerve blocks of the greater and lesser occipital, supraorbital, supratrochlear and auriculotemporal nerves are widely used in the treatment of primary headaches. We present efficacy and safety data for these procedures. Methods In an uncontrolled open-label prospective study, 119 patients with chronic cluster headache, chronic migraine, short lasting unilateral neuralgiform attack disorders, new daily persistent headaches, hemicrania continua and chronic paroxysmal hemicrania were examined. All had failed to respond to greater occipital nerve blocks. Response was defined as a 50% reduction in either daily attack frequency or moderate-to-severe headache days after 2 weeks. Results The response rate of the whole cohort was 55.4%: Chronic cluster headache, 69.2%; chronic migraine, 49.0%; short lasting unilateral neuralgiform attack disorders, 56.3%; new daily persistent headache, 10.0%; hemicrania continua, 83.3%; and chronic paroxysmal hemicrania, 25.0%. Time to benefit was between 0.50 and 33.58 hours. Benefit was maintained for up to 4 weeks in over half of responders in all groups except chronic migraine and paroxysmal hemicrania. Only minor adverse events were recorded. Conclusion Multiple cranial nerve blocks may provide an efficacious, well tolerated and reproducible transitional treatment for chronic headache disorders when greater occipital nerve blocks have been unsuccessful.


Neurology ◽  
2017 ◽  
Vol 88 (11) ◽  
pp. 1069-1076 ◽  
Author(s):  
Nunu Lund ◽  
Mads Barloese ◽  
Anja Petersen ◽  
Bryan Haddock ◽  
Rigmor Jensen

Objective:To describe differences between the sexes in the phenotype of cluster headache (CH) in a large, well-characterized clinical CH population.Methods:Patients from the Danish CH survey aged 18–65 years, diagnosed with CH according to International Classification of Headache Disorders, second edition, completed questionnaires and structured interviews.Results:A total of 351 patients with CH participated, with a male:female ratio of 2:1. The diurnal variation of attacks showed moments of peak prominence in men’s attack cycle to be advanced by 1 hour compared to women’s, despite no difference in self-reported bedtime or chronotype (p = 0.31). The onset of CH decreased with increasing age for both sexes. Diagnostic delay was numerically longer for men vs women (6.56 vs 5.50 years, p = 0.21); however, more women had previously been misdiagnosed (61.1% vs 45.5%, p < 0.01) and received the correct diagnosis at a tertiary headache center (38.8% vs 20.9%, p < 0.001). Only minor sex differences in clinical characteristics were found but chronic CH was more prevalent in women compared to men (44.0% vs 31.9%, p < 0.05).Conclusions:Despite a similar clinical phenotype, diurnal attack cycle is advanced by 1 hour in men with CH compared to women. Rhythmicity is a defining characteristic of CH and these findings suggest differences in the hypothalamus’ influence on attack occurrence between the sexes. In addition, women were more often misdiagnosed and diagnosis in the primary or secondary sector more often failed. Furthermore, women had chronic CH more frequently than men. A long diagnostic delay and frequent misdiagnosis emphasize the need for increased awareness of CH in both sexes.


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