Cluster headache: review of the literature

Current Pain and Headache Reports, 2007

When conventional treatment approaches to cluster headache are unsuccessful, expert recommendations are relevant but may not be easily accessible to treating clinicians. We conducted a study of expert recommendations in response to standardized vignettes. Ten expert headache clinicians were asked what treatment they would recommend for a hypothetical 55-year-old male cluster headache patient in the following five situations: 1) known coronary artery disease with response only to sumatriptan; 2) strictly unilateral headaches unresponsive to preventive treatment; 3) effective abortive treatment not covered by insurance; 4) patient request to obtain methysergide from Canada; and 5) headaches responsive only to steroid treatment.

Deutsches Ärzteblatt international, 2011

Cluster headache is the most common type of trigemino-autonomic headache, affecting ca. 120 000 persons in Germany alone. The attacks of pain are in the periorbital area on one side, last 90 minutes on average, and are accompanied by trigemino-autonomic manifestations and restlessness. Most patients have episodic cluster headache; about 15% have chronic cluster headache, with greater impairment of their quality of life. The attacks often possess a circadian and seasonal rhythm. Selective literature review Oxygen inhalation and triptans are effective acute treatment for cluster attacks. First-line drugs for attack prophylaxis include verapamil and cortisone; alternatively, lithium and topiramate can be given. Short-term relief can be obtained by the subcutaneous infiltration of local anesthetics and steroids along the course of the greater occipital nerve, although most of the evidence in favor of this is not derived from randomized clinical trials. Patients whose pain is inadequatel...

Annals of Indian Academy of Neurology, 2018

Review Article IntroductIon Cluster headache is a primary headache disorder, belonging to the trigeminal autonomic cephalalgias (TACs). Descriptions of the disorder in the literature dates as far back as 1641, where the Dutch physician Nicolaes Tulp, famous from Rembrandt's painting, "The anatomy lesson," described a recurring severe unilateral headache lasting no longer than 2 h in the Observationes Medicae. [1] However, cranial autonomic features were not described therein. Wilfred Harris (1869-1960), a Madras-born London neurologist, described cluster headache in his classic monograph Neuritis and Neuralgia in 1926; [2] this was probably the earliest clear recognition of it as a separate entity from migraine and trigeminal neuralgia. [3] In 1936, Harris named these headaches migrainous neuralgia or ciliary (migrainous) neuralgia, [4] where he recorded the unilaterality of attacks, severity, associated autonomic features, and frequency of attacks. His description was the first recorded reports of cluster headache in the English medical literature. The same clinical features are detailed in the International Classification Headache Disorder-3 (ICHD-3). [5] This review will cover the epidemiology, pathophysiology, clinical features, and diagnosis of the disorder. EpIdEmIology Given the low prevalence of cluster headache compared to migraine, it is difficult to assess accurately the prevalence of cluster headache in the community. Nonetheless, given the specific features of cluster headache, it is possible to identify possible cases in the community, using questionnaires based on the ICHD criteria. Community-based studies have been performed to ascertain the prevalence of cluster headache. They are generally modeled on a two-step process. The first step is to screen for possible cluster headache cases either through mailed questionnaires or structured interviews based on the ICHD Cluster headache is a primary headache disorder affecting up to 0.1% of the population. Patients suffer from cluster headache attacks lasting from 15 to 180 min up to 8 times a day. The attacks are characterized by the severe unilateral pain mainly in the first division of the trigeminal nerve, with associated prominent unilateral cranial autonomic symptoms and a sense of agitation and restlessness during the attacks. The male-to-female ratio is approximately 2.5:1. Experimental, clinical, and neuroimaging studies have advanced our understanding of the pathogenesis of cluster headache. The pathophysiology involves activation of the trigeminovascular complex and the trigeminal-autonomic reflex and accounts for the unilateral severe headache, the prominent ipsilateral cranial autonomic symptoms. In addition, the circadian and circannual rhythmicity unique to this condition is postulated to involve the hypothalamus and suprachiasmatic nucleus. Although the clinical features are distinct, it may be misdiagnosed, with patients often presenting to the otolaryngologist or dentist with symptoms. The prognosis of cluster headache remains difficult to predict. Patients with episodic cluster headache can shift to chronic cluster headache and vice versa. Longitudinally, cluster headache tends to remit with age with less frequent bouts and more prolonged periods of remission in between bouts.

Headache: The Journal of Head and Face Pain, 2005

Background.-In the absence of biological markers, the diagnosis of cluster headache (CH) rests on clinical evidence as reported through ad-hoc interviews. Objective.-The aim of this study was to validate a 16-item self-administered questionnaire designed to screen CH cases. Methods.-The questions were based on the second edition of the International Headache Society classification (ICHD-II) criteria for CH (n = 12) and on specific disease features (n = 3). Answers to each question were either "Yes," "No," or "Don't know." The validity of this screening tool was assessed using a two-step procedure. In Step 1, the 16 questions were submitted to 30 healthy subjects with different cultural backgrounds to verify content clarity. In Step 2, the questionnaire was given to 71 patients (32 women and 39 men) aged 15 to 78 years (mean 37.5 years), who were seen at the University of Parma Headache Center with a diagnosis of CH (episodic, 17; chronic, 13), migraine (without aura, 18; with aura, 3), and tension-type headache (episodic, 16; chronic, 4) according to the ICHD-II criteria. Sensitivity, specificity, and the positive and negative predictive values were calculated for each question as measures of validity. Results.-Severity, unilaterality, and location of pain had the highest sensitivity (100%), but low (34.1%) or fairly low specificity (61.0% and 58.5%, respectively). In contrast, a positive response to lithium or verapamil had a 66.7% sensitivity and a 97.6% specificity. Sensitivity and specificity were 100% and 90.2% for duration of attacks (<180 to 240 minutes), and 90.0% and 92.7% for compulsory movements, respectively. The best discriminatory pattern for symptom detection was unilaterality of pain and the presence of at least five of the following seven features: pain severity and location, duration, frequency and daily recurrence of attacks, rhinorrhea and restlessness as accompanying symptoms. Conclusions.-Our findings confirm that this questionnaire is a useful method for CH screening in epidemiological studies.

Journal of neurosciences in rural practice, 2014

Cluster headache (CH) is uncommon and most painful of all primary headaches, and continues to be managed suboptimally because of wrong diagnosis. It needs to be diagnosed correctly and specifically treated. There are few studies and none from this region on CH. To study the detailed clinical profile of CH patients and to compare them among both the genders. Study was conducted at Mahatma Gandhi hospital, Jodhpur (from January 2011to December 2013). Study comprises 30 CH patients diagnosed according to International Headache Society guidelines (ICHD-II). Routine investigations and MRI brain was done in all patients. All measurements were reported as mean ± SD. Categorical variables were compared using the Chi-square test, and continuous variables were compared using Student&#39;s t-test. SPSS for Windows, Version 16.0, was used for statistical analyses with the significance level set at P = 0.05. M: F ratio was 9:1. Age at presentation was from 22-60 years (mean - 38 years). Latency ...

Neurological Sciences, 2023

Background The Cluster Headache Impact Questionnaire (CHIQ) is a specific and easy-to-use questionnaire to assess the current impact of cluster headache (CH). The aim of this study was to validate the Italian version of the CHIQ. Methods We included patients diagnosed with episodic CH (eCH) or chronic CH (cCH) according to the ICHD-3 criteria and included in the "Italian Headache Registry" (RICe). The questionnaire was administered to patients through an electronic form in two sessions: at first visit for validation, and after 7 days for test-retest reliability. For internal consistency, Cronbach's alpha was calculated. Convergent validity of the CHIQ with CH features and the results of questionnaires assessing anxiety, depression, stress, and quality of life was evaluated using Spearman's correlation coefficient. We included 181 patients subdivided in 96 patients with active eCH, 14 with cCH, and 71 with eCH in remission. The 110 patients with either active eCH or cCH were included in the validation cohort; only 24 patients with CH were characterized by a stable attack frequency after 7 days, and were included in the test-retest cohort. Internal consistency of the CHIQ was good with a Cronbach alpha value of 0.891. The CHIQ score showed a significant positive correlation with anxiety, depression, and stress scores, while showing a significant negative correlation with quality-of-life scale scores. Conclusion Our data show the validity of the Italian version of the CHIQ, which represents a suitable tool for evaluating the social and psychological impact of CH in clinical practice and research. Keywords Cluster headache • Questionnaire • Cluster Headache Impact Questionnaire • Quality of life • Disability Abbreviations CH Cluster headache cCH Chronic cluster headache eCH Episodic cluster headache PRO(s) Patient-reported outcome(s) CHIQ Cluster Headache Impact Questionnaire ICHD-3 International Classification of Headache Disorders, 3rd Edition RICe Italian Headache Registry (Registro Italiano delle Cefalee) REDCap Research Electronic Data Capture DASS-21 Depression, Anxiety, and Stress Scale Short Version SF-36 Short Form Health Survey IQR Interquartile range ICC Intraclass correlation coefficients Background Cluster headache (CH) is a primary headache disorder that affects approximately 0.1% of the population [1]. Approximately 85% of individuals afflicted by CH have episodic CH

˜The œJournal of headache and pain, 2024

Background New guidelines for cluster headache clinical trials were recently published. We welcome these new guidelines and raise additional considerations in trial methodologies. We present non-inferiority trials to overcome ethical issues with placebo use, and additionally discuss issues with trial recruitment. We highlight some possible issues and solutions to be considered with the recently published cluster headache trial guidelines. Keywords Cluster headache trial, Methodology cluster headache design, Future directions cluster headache trials

Seminars in Neurology, 2010

Cluster headache is a rare yet exquisitely painful primary headache disorder occurring in either episodic or chronic patterns. The unique feature of cluster headache is the distinctive circadian and circannual periodicity in the episodic forms. The attacks are stereotypic-they are of extreme intensity and short duration, occur unilaterally, and are associated with robust signs and symptoms of autonomic dysfunction. Although the pathophysiology of cluster headache remains to be fully understood, there have been a number of recent seminal observations. To exclude structural mimics, patients presenting with symptoms suggestive of cluster headache warrant at least a brain magnetic resonance imaging (MRI) scan in their work-up. The medical treatment of cluster headache includes acute, transitional, and maintenance prophylaxis. Agents used for acute therapy include inhalation of oxygen, triptans, such as sumatriptan, and dihydroergotamine. Transitional prophylaxis refers to the short-term use of fast-acting agents. This typically involves either corticosteroids or an occipital nerve block. The mainstay of prophylactic therapy is verapamil. Yet, other medications, including lithium, divalproex sodium, topiramate, methysergide, gabapentin, and even indomethacin, may be useful when the headache fails to respond to verapamil. For medically refractory patients, surgical interventions, occipital nerve stimulation, and deep brain stimulation remain an option. As the sophistication of functional neuroimaging increases, better insight into the pathophysiological mechanisms that underlie cluster headache is expected.

The journal of headache and pain, 2014

Chronic cluster headache (CCH) often resists to prophylactic pharmaceutical treatments resulting in patients&#39; life damage. In this rare but pragmatic situation escalation to invasive management is needed but framing criteria are lacking. We aimed to reach a consensus for refractory CCH definition for clinical and research use. The preparation of the final consensus followed three stages. Internal between authors, a larger between all European Headache Federation members and finally an international one among all investigators that have published clinical studies on cluster headache the last five years. Eighty-five investigators reached by email. Proposed criteria were in the format of the International Classification of Headache Disorders III-beta (description, criteria, notes, comments and references). Following this evaluation eight drafts were prepared before the final. Twenty-four (28.2%) international investigators commented during two rounds. Refractory CCH is described in...

Revista Médica de Minas Gerais, 2014

Introduction: cluster headaches (CS) are a subtype of primary headache disorder characterized by daily pain attacks of 15-180 minutes for weeks to months, usually interspersed with periods of remission. Painful crises are very intense, of periorbital or orbital location, associated with symptoms of autonomic dysfunction. Despite its unique clinical presentation, CS remains under-recognized and underdiagnosed. Objectives: To describe and discuss the diagnostic and therapeutic challenges of CS based on clinical cases. Methods: this is a series of five cases of CS under clinical monitoring. Results: Of the 467 patients treated at the neurological clinic five had a diagnosis of CS, corresponding to 1.07% of the total. The clinical presentation varied little in relation to the description in the literature; it affected predominantly men, episodically. Time elapsed between onset and diagnosis was usually long. Conclusion: Even in a tertiary care neurological center, the number of diagnosed patients is small, which reflects on ignorance about CS and contributes to late diagnosis and lack of specific treatment.

Cephalalgia, 2000

During 1981–96 a series of 60 consecutive out-patients was examined in relation to an assumed first period of cluster headache (CH). On follow up in 1998 we found that six were deceased at a mean age of 56.5 years (range 45–74 years), of whom one had a definitive CH diagnosis and five had one documented headache period only. Six patients were lost to follow up because they could not be reached. In the final group for evaluation ( n = 49) it was found that 13 (26.5%) patients had had one cluster period only during a mean observation time of 8.9 years. Out of 36 patients with a definitive CH diagnosis according to International Headache Society (IHS) criteria, 31 patients had episodic CH, four patients had primary chronic CH and one patient had secondary chronic CH. Of the patients with a definitive CH diagnosis, 83% on follow up had had a recurrence of a second period of CH within 3 years or continuous attacks (chronic/semichronic CH) from the onset. Evidently some patients may suffe...

Frontiers in pain research, 2024

Cluster headache (CH) is one of the worst primary headaches that remain underdiagnosed and inappropriately treated. There are recent advances in the understanding of this disease and available treatments. This paper aims to review CH's recent clinical and pathophysiological findings, diagnosis, and treatment. We performed a narrative literature review on the sociodemographics, clinical presentations, pathophysiological findings, and diagnosis and treatment of CH. CH affects 0.1% of the population with an incidence of 2.07-9.8/100,00 person-years-habitants, a mean prevalence of 53/100,000 inhabitants (3-150/100,000 inhabitants). The male-to-female ratio remains inconclusive, as the ratio of 4.3:1 has recently been modified to 1.3-2.6, possibly due to previous misdiagnosis in women. Episodic presentation is the most frequent (80%). It is a polygenetic and multifactorial entity that involves dysfunction of the trigeminovascular system, the trigeminal autonomic reflex, and the hypothalamic networks. An MRI of the brain is mandatory to exclude secondary etiologies. There are effective and safe pharmacological treatments oxygen, sphenopalatine, and great occipital nerve block, with the heterogeneity of clinical trial designs for patients with CH divided into acute, transitional, or bridge treatment (prednisone) and preventive interventions. In conclusion, CH remains underdiagnosed, mainly due to a lack of awareness within the medical community, frequently causing TYPE

Cephalalgia Reports, 2019

Objective: To present the methodology and to describe the sample of a large, Internet-based survey on the burden of cluster headache (CH). Methods: Participants filled out a questionnaire online. Inclusion criteria were a reported diagnosis of CH and a residency in a European country; exclusion criteria were refusal to give informed consent and to complete the questionnaire. Results: A total of 1514 participants completed the questionnaire. Of these, 66.2% were male and 31.2% reported suffering from chronic CH. The diagnosis was validated based upon the responses in 92.9% of the participants. Other diagnoses seemed more likely in 0.8%. Among the participants with self-reported chronic CH, the International Classification of Headache Disorders-3 beta criteria for chronicity were fulfilled by 90.8%. Conclusions: In this article, we discuss the applied methodology as well as the properties of the sample. The overall accuracy of the self-reported diagnoses was very good as judged by our...

Neurological Sciences, 2019

This paper overviews available literature addressing behavioral and psychological aspects of cluster headache. Behavioral correlates of sleep and drug use are explored, as are the psychological correlates pertaining to psychopathology and cognitive functioning. We conclude with a review of the few investigations addressing adjunctive behavioral treatments for cluster headache, and provide suggestions for possible ways to enhance effects of behavioral interventions for this painful and difficult to treat headache disorder.

Neurological Sciences, 2013

The peripheral and central origin of pain in cluster headache (CH) and trigeminal autonomic cephalgias (TACs) has been matter of debate. In the last decade, a number of information came from both animal and human studies. This paper briefly highlights main data from these studies. Taken together, there is now sufficient body of evidence indicating that CH and TACs can be regarded as a unique headache spectrum-syndrome, due to involvement of specific brain areas.