Episodic and Chronic Cluster Headache: Differences in Family History, Traumatic Head Injury, and Chronorisk.

OBJECTIVE AND BACKGROUND: The diagnostic criteria of episodic and chronic cluster headache (cCH) were recently modified, yet pathophysiological differences between the two are still unclear. The aim of this cross-sectional study is to identify and characterize other differences between episodic and cCH. METHODS: Data from a retrospective, questionnaire- and interview-based study were analyzed with a focus on associated factors including traumatic head injury (THI), familial history, and change of phenotype. Attack patterns were analyzed using Gaussian and spectral modeling. RESULTS: 400 patients and 200 controls participated. A positive family history was more prevalent in chronic than episodic cluster headache (eCH) (34/146 (23%) vs 33/253 (13%), respectively, P = .008). A history of THI was more common in patients than controls (173/400 (43%) vs 51/200 (26%), respectively, P < .0001) and in chronic compared to eCH (77/146 (53%) vs 96/253 (37%), respectively, P = .004). Patients with a positive family history had a unique diurnal attack pattern with twice the risk of nocturnal attacks as patients who did not report family history. Patients reporting phenotype change had a chronobiological fingerprint similar to the phenotype they had experienced a transition into. A higher attack frequency in chronic patients was the only difference in symptom manifestation across all analyzed subgroups of patients. CONCLUSIONS: cCH is associated with a positive family history and THI. In familial CH, a peak in nocturnal chronorisk may implicate genes involved in diurnal-, sleep- and homeostatic regulation. The stereotypical nature of the CH attacks themselves is confirmed and differences between subgroups should be sought in other characteristics.

Clinical features of chronic cluster headache based on the third edition of the International Classification of Headache Disorders: A prospective multicentre study.

The criterion for the remission period of chronic cluster headache (CCH) was recently revised from < 1 month to < 3 months in the third edition of the International Classification of Headache Disorders (ICHD-3). However, information on the clinical features of CCH based on the ICHD-3 criteria is currently limited. The present study aimed to investigate the clinical features of CCH based on ICHD-3 using data from the Korean Cluster Headache Registry (KCHR). The KCHR is a multicentre prospective registry of patients with cluster headache (CH) from 15 hospitals. Among the 250 participants with CH, 12 and 176 participants were classified as having CCH and episodic cluster headache (ECH), respectively. Among 12 participants with CCH, 6 (50%) had remission periods of < 1 month, and the remaining 6 (50%) had a remission period of 1-3 months. Six participants had CCH from the time of onset of CH, and in the other 6 participants, CCH evolved from ECH. CCH subjects had later age of onset of CH, developed the condition after a longer interval after CH onset, and had more migraine and less nasal congestion and/or rhinorrhoea than ECH subjects. Clinical features of CCH with remission periods < 1 month were not significantly different from those of CCH with remission periods of 1-3 months, except for the total number of bouts. More current smoking and less diurnal rhythmicity were observed in participants with CCH evolved from ECH compared to those with ECH. In conclusion, the number of subjects with CCH doubled when the revised ICHD-3 criteria were used. Most of clinical characteristics of CCH did not differ when the previous and current version of ICHD was applied and compared. Some clinical features of CCH were different from those of ECH, and smoking may have a role in CH chronification.

More precise phenotyping of cluster headache using prospective attack reports.

BACKGROUND AND PURPOSE: The clinical characteristics of cluster headache (CH) are based mainly on retrospective attack descriptions of 'usual' attacks, but whether these reports are reliable is uncertain. The aim was to compare retrospective and prospective attack descriptions and describe the within- and between-patient variability of attacks. METHOD: Fifty-seven CH patients underwent a semi-structured interview obtaining a retrospective account of usual CH attacks. Patients thereafter prospectively recorded the clinical characteristics of up to 10 attacks per patient in a headache diary. Four different attack characteristics were investigated: (i) severity, (ii) duration, (iii) number of autonomic symptoms and (iv) number of migrainous symptoms. Retrospective and prospective data were compared. Within- and between-patient variability of attacks was assessed. RESULTS: Retrospective attack descriptions (n = 57) were significantly longer (P = 0.046) and more severe (P < 0.0001) for untreated attacks compared with prospective reports (n = 500). The number of autonomic symptoms was significantly higher in the retrospective reports compared to the prospective reports (P < 0.0001). Within-patient variability for attack duration, pain severity and number of autonomic and migrainous symptoms was low. Compared to men, more women reported longer (P = 0.026) and more severe (P = 0.028) attacks with more migrainous symptoms (P = 0.033). CONCLUSIONS: Important differences were found between prospectively and retrospectively reported attacks with duration and severity of untreated attacks overestimated in retrospective attack descriptions. CH attacks display low within-patient variability, but the presentation of CH attacks varies between patients. The high prevalence of symptoms typically associated with migraine should raise more diagnostic awareness for CH, especially in women who are more often misdiagnosed as having migraine.

The validation of the Hungarian version of the ID-migraine questionnaire.

BACKGROUND: Despite its high prevalence, migraine remains underdiagnosed and undertreated. ID-Migraine is a short, self-administrated questionnaire, originally developed in English by Lipton et al. and later validated in several languages. Our goal was to validate the Hungarian version of the ID-Migraine Questionnaire. METHODS: Patients visiting two headache specialty services were enrolled. Diagnoses were made by headache specialists according to the ICHD-3beta diagnostic criteria. There were 309 clinically diagnosed migraineurs among the 380 patients. Among the 309 migraineurs, 190 patients had only migraine, and 119 patients had other headache beside migraine, namely: 111 patients had tension type headache, 3 patients had cluster headache, 4 patients had medication overuse headache and one patient had headache associated with sexual activity also. Among the 380 patients, 257 had only a single type headache whereas 123 patients had multiple types of headache. Test-retest reliability of the ID-Migraine Questionnaire was studied in 40 patients. RESULTS: The validity features of the Hungarian version of the ID-Migraine questionnaire were the following: sensitivity 0.95 (95% CI, 0.92-0.97), specificity 0.42 (95% CI, 0.31-0.55), positive predictive value 0.88 (95% CI, 0.84-0.91), negative predictive value 0.65 (95% CI, 0.5-0.78), missclassification error 0.15 (95% CI, 0.12-0.19). The kappa coefficient of the questionnaire was 0.77. CONCLUSION: The Hungarian version of the ID-Migraine Questionnaire had adequate sensitivity, positive predictive value and misclassification error, but a low specificity and somewhat low negative predictive value.

Cluster headache - clinical pattern and a new severity scale in a Swedish cohort.

Background The aim of this study was to investigate clinical features of a cluster headache cohort in Sweden and to construct and test a new scale for grading severity. Methods Subjects were identified by screening medical records for the ICD 10 code G44.0, that is, cluster headache. Five hundred participating research subjects filled in a questionnaire including personal, demographic and medical aspects. We constructed a novel scale for grading cluster headache in this cohort: The Cluster Headache Severity Scale, which included number of attacks per day, attack and period duration. The lowest total score was three and the highest 12, and we used the Cluster Headache Severity Scale to grade subjects suffering from cluster headache. We further implemented the scale by defining a cluster headache maximum severity subgroup with a high Cluster Headache Severity Scale score ≥ 9. Results A majority (66.7%) of the patients reported that attacks appear at certain time intervals. In addition, cluster headache patients who were current tobacco users or had a history of tobacco consumption had a later age of disease onset (31.7 years) compared to non-tobacco users (28.5 years). The Cluster Headache Severity Scale score was higher in the patient group reporting sporadic or no alcohol intake than in the groups reporting an alcohol consumption of three to four standard units per week or more. Maximum severity cluster headache patients were characterised by higher age at disease onset, greater use of prophylactic medication, reduced hours of sleep, and lower alcohol consumption compared to the non-cluster headache maximum severity group. Conclusion There was a wide variation of severity grade among cluster headache patients, with a very marked impact on daily living for the most profoundly affected.

Strictly unilateral headaches: considerations of a clinician.

The aim of the lecture is to draw attention to the role that clinical practice and clinical observation have had in stimulating research on the pathophysiology of cluster headache (CH) and other trigeminal autonomic cephalalgias (TACs). The symptoms of cluster headache-in particular the typical circadian periodicity of the headaches and the seasonal recurrence of cluster periods-were fundamental in shifting attention away from peripheral pathogenetic hypotheses to the idea that cluster headache could have a central origin. Initially, solid neuroendocrinological data pointed to hypothalamic involvement. For example, CH patients were shown to have alterations in biorhythms. Subsequently, modern functional neuroimaging techniques were able to demonstrate that the homolateral posterior hypothalamus is activated during TAC headaches, so implicating this region in TAC pathogenesis. It is known that the hypothalamus has a modulatory effect on nociceptive and autonomic pathways, particularly on the nociceptive trigeminovascular system. Future research should clarify whether the hypothalamus is the generator of TAC headaches, or whether it is activated in response to an alteration of the homeostatic equilibrium between limbic emotional-affective components and autonomic-nociceptive components modulated by the hypothalamus.

Diagnosing and understanding adult headache.

Primary headaches are the most common headache disorders. The most common forms are tension-type headaches, migraine, and cluster headache. Knowing the clinical presentation coupled with taking a thorough history taking and performing a thorough physical examination usually helps in arriving at a correct diagnosis. Special attention should be paid to unusual clinical presentations. Further diagnostic work-up should be performed in the presence of atypical and worrisome signs.

Evaluation and management of "sinus headache" in the otolaryngology practice.

Patients, primary care doctors, neurologists and otolaryngologists often have differing views on what is truly causing headache in the sinonasal region. This review discusses common primary headache diagnoses that can masquerade as "sinus headache" or "rhinogenic headache," such as migraine, trigeminal neuralgia, tension-type headache, temporomandibular joint dysfunction, giant cell arteritis (also known as temporal arteritis) and medication overuse headache, as well as the trigeminal autonomic cephalalgias, including cluster headache, paroxysmal hemicrania, and hemicrania continua. Diagnostic criteria are discussed and evidence outlined that allows physicians to make better clinical diagnoses and point patients toward better treatment options.

Clinical profile of cluster headaches in China - a clinic-based study.

BACKGROUND: The clinical profile of cluster headache in Chinese patients have not been fully studied. METHODS: The classification and clinical features of 120 consecutive patients with cluster headache (105 males, 15 females; mean age, 34.9 ± 10.5 years) visiting at International Headache Center from May 2010 to August 2012 were analyzed. RESULTS: Patients came from 16 different regions of China. Mean age at onset of cluster headache was 26.7 ± 10.9 years. Only 13 patients (10.8%) had previously been diagnosed with cluster headache. Mean time to diagnosis from first symptoms was 8.2 ± 7.1 years (range, 0-35 years). Chronic cluster headache was observed in only 9 patients (7.5%). The most commonly reported location of cluster headache was temporal region (75.0%), followed by retro-orbital region (68.3%), forehead (32.5%), vertex (32.5%) and occipital (22.5%). Lacrimation was the most consistently reported autonomic feature (72.5%). During acute attacks, 60.0% of patients experienced nausea, and 41.7% experienced photophobia and 40.8% experienced phonophobia. In addition, 38.3% reported restless behavior and 45.8% reported that physical activity exacerbated the pain. None of patients experienced visual or other kinds of aura symptoms before cluster attacks. We found that 38.3% of patients had <1 cluster period and 35.8% for 1-2 cluster periods per year with these periods occurring less frequently during the summer than during other seasons. Cluster duration was 1-2 months in 32.5% of patients. During cluster periods, 73.3% of patients had 1-2 attacks per day, and 39.2% experienced cluster attacks ranging in duration from 1 h to less than 2 h. The duration of attacks were 1.5 (1-2.25) hours for males and 1.5 (1-3) for females respectively. The World Health Organization quality of life-8 questionnaire showed that cluster headache reduced life quality. CONCLUSIONS: Compared to Western patients, Chinese patients showed a relatively low prevalence of chronic cluster headaches, pain sites mainly focused on areas distributed by the first division of the trigeminal nerve, a low frequency of restlessness and absent aura. These clinical features may be more common in Eastern populations, including mainland Chinese, Japanese and Taiwanese patients, than in Western patients.

Cluster headache and neuropsychological functioning.

BACKGROUND: Despite significant advances in unravelling the pathophysiology of cluster headache (CH), little is known about neuropsychological functioning. Apart from neuroimaging studies indicating involvement of posterior hypothalamic and other areas frequently involved in nociception, some studies suggest involvement of prefrontal areas. Among others, these mediate executive functioning (EF). METHODS: Therefore, three neuropsychological tests (Trail Making Test (TMT), Go/Nogo Task and Stroop Task) were completed by four headache patient samples (chronic CH, episodic CH in the active or inactive period, and migraine patients) and compared to healthy controls. RESULTS: Analyses revealed that patients especially with chronic and active episodic CH were particularly impaired in tests relying more on intact EF (i.e. TMT-B, Stroop interference) than on basal cognitive processes (i.e. TMT-A, Stroop naming). Within the CH groups performance decreased linearly with increasing severity. DISCUSSION: These findings are in line with a recently proposed involvement of prefrontal structures in CH pathophysiology as patients performed worse on neuropsychological tasks relying on these structures. Impaired EF could also result from medication and sleep disturbances due to active CH. Because the decreased performance was also present outside the attacks it may hint at generally altered brain functions, but do not necessarily reflect clinically relevant behaviour.

Ascending cluster headache: a description of three cases and a review of the literature.

INTRODUCTION: It has been previously shown that cluster headache (CH) can involve some extratrigeminal areas. Occipital pain has been recognized in several patients, even as the origin of the attacks. Nevertheless, the proposals of topographic variations of CH have been mainly focused on the location of pain in either supraorbital or infraorbital regions. CASE REPORTS: We report three patients fulfilling International Classification of Headache Disorders criteria for CH whose attacks started with mild or moderate headache at the occipital region and gradually moved forward over 10 to 30 minutes, finally reaching the ipsilateral orbital area. There the pain acquired typical CH features, with severe intensity and ipsilateral autonomic accompaniments. CONCLUSIONS: These descriptions of ascending CH probably reflect pathophysiological mechanisms involving the trigemino-cervical complex, and also offer theoretical support for some new therapeutic approaches such as great occipital nerve blockades or occipital neurostimulation.

Cluster-like headache. A comprehensive reappraisal.

Among the primary headaches, cluster headache (CH) presents very particular features allowing a relatively easy diagnosis based on criteria listed in Chapter 3 of the International Classification of Headache Disorders (ICHD-II). However, as in all primary headaches, possible underlying causal conditions must be excluded to rule out a secondary cluster-like headache (CLH). The observation of some cases with clinical features mimicking primary CH, but of secondary origin, led us to perform an extended review of CLH reports in the literature. We identified 156 CLH cases published from 1975 to 2008. The more frequent pathologies in association with CLH were the vascular ones (38.5%, n = 57), followed by tumours (25.7%, n = 38) and inflammatory infectious diseases (13.5%, n = 20). Eighty were excluded from further analysis, because of inadequate information. The remaining 76 were divided into two groups: those that satisfied the ICHD-II diagnostic criteria for CH, 'fulfilling' group (F), n = 38; and those with a symptomatology in disagreement with one or more ICHD-II criteria, 'not fulfilling' group (NF), n = 38. Among the aims of this study was the possible identification of clinical features leading to the suspicion of a symptomatic origin. In the differential diagnosis with CH, red flags resulted both for F and NF, older age at onset; for NF, abnormal neurological/general examination (73.6%), duration (34.2%), frequency (15.8%) and localization (10.5%) of the attacks. We stress the fact that, on first observation, 50% of CLH presented as F cases, perfectly mimicking CH. Therefore, the importance of accurate, clinical evaluation and of neuroimaging cannot be overestimated.

Sluder's neuralgia: a trigeminal autonomic cephalalgia?

The objective was to formulate distinctive criteria to substantiate our opinion that Sluder's neuralgia and cluster headache are two different clinical entities. A systematic review was carried out of all available, original literature on Sluder's neuralgia. Pain characteristics, periodicity and associated signs and symptoms were studied and listed according to frequency of appearance. Eleven articles on Sluder's neuralgia were evaluated. Several differences between Sluder's neuralgia and cluster headache became evident. Based on described symptoms, new criteria for Sluder's neuralgia could be formulated. Sluder's neuralgia and cluster headache could possibly be regarded as two different headache syndromes, and Sluder's neuralgia could be a trigeminal autonomic cephalalgia.

Application of ICHD-II criteria for headaches in a TMJ and orofacial pain clinic.

The aim of this study was to identify and diagnose headache in a temporomandibular joint and orofacial pain clinic population using the second edition of The International Classification of Headache Disorder criteria. In 502 temporomandibular disorder and orofacial pain patients, 246 patients (49%) were diagnosed with tension-type headache (TTH), followed by migraine without aura (14.5%), probable migraine (12.9%), migraine with aura (7%), probable TTH (4.8%) and cluster headache (0.2%). The prevalence of headaches was compared between male and female patients, and the prevalence of migraine was found to be higher in women than in men. In evaluating by age, the prevalence of migraine was highest in patients in their 20s and 30s and declined as age increased above 40. TTH showed the highest rate throughout all age groups, but it also decreased as age increased. In this study, the prevalence of migraine was lower than that reported in Dr Kim et al.'s study, and the prevalence of TTH much higher than that reported in the previous study. Of the headache patients, 81.1% presented with masseter muscle pain and 47.8% with temporal muscle pain. This finding suggests that pericranial muscle pain may be an inducing factor of primary headache.

Redefining primary headaches.

In the light of the pathophysiologic knowledge acquired in the recent years, a tentative redefinition is now possible of some types of headache until now defined as idiopathic, and indistinctly described as primary headaches. Cluster headache and trigeminal neuralgia are known examples of diseases classified as primary, which are, in contrast, well-defined diseases to be distinguished from headaches without any recognized anatomic site of lesion or pathogenesis. Another still debated condition, chronic migraine, is proposed here as the consequence of "processes" to be ascribed to mechanisms activated by other comorbid conditions. The observations supporting the possibility that allodynia represents the implicit process leading to pain progression, which occurs in some migraineurs, are discussed.

Use of complementary and alternative medicine by patients with cluster headache: results of a multi-centre headache clinic survey.

OBJECTIVES: To evaluate the rates, pattern, satisfaction with, and presence of predictors of complementary and alternative medicine (CAM) use in a clinical population of patients with cluster headache (CH). DESIGN AND SETTING: One hundred CH patients attending one of three headache clinics were asked to undergo a physician-administered structured interview designed to gather information on CAM use. RESULTS: Past use of CAM therapies was reported by 29% of the patients surveyed, with 10% having used CAM in the previous year. Only 8% of the therapies used were perceived as effective, while a partial effectiveness was reported in 28% of CAM treatments. The most common source of recommendation of CAM was a friend or relative (54%). Approximately 62% of CAM users had not informed their medical doctors of their CAM use. The most common reason for deciding to try a CAM therapy was that it offered a "potential improvement of headache" (44.8%). Univariate analysis showed that CAM users had a higher income, had a higher lifetime number of conventional medical doctor visits, had consulted more headache specialists, had a higher number of CH attacks per year, and had a significantly higher proportion of chronic CH versus episodic CH. A binary logistic regression analysis was performed and two variables remained as significant predictors of CAM use: income level (OR=5.7, CI=1.6-9.1, p=0.01), and number of attacks per year (OR=3.08, CI=1.64-6.7, p<0.0001). CONCLUSION: Our findings suggest that CH patients, in their need of and quest for care, seek and explore both conventional and CAM approaches, even though only a very small minority finds them very satisfactory.

Trigeminal autonomic cephalalgias. Part 1: cluster headache.

Cluster headache is characterized by severe, strictly unilateral pain attacks lasting 15 to 180 minutes localized to orbital, temporal, and midface areas accompanied by ipsilateral autonomic features. It represents 1 of 3 primary headaches classified as trigeminal autonomic cephalalgias. While its prevalence is small, it is not uncommon for cluster headache patients to present at dental offices seeking relief for their pain. It is important for oral health care providers to recognize cluster headache and render an accurate diagnosis. This will avoid the pitfall of implementing unnecessary and inappropriate traditional dental treatments in hopes of alleviating this neurovascular pain. The following article is part 1 of a review on trigeminal autonomic cephalalgias and focuses on cluster headache. Aspects of cluster headache including its prevalence and incidence, genetics, pathophysiology, clinical presentation, classification and variants, diagnosis, medical management, and dental considerations are discussed.

Treatment of medically intractable cluster headache by occipital nerve stimulation: long-term follow-up of eight patients.

BACKGROUND: Cluster headache is a form of primary headache that features repeated attacks of excruciatingly severe headache usually occurring several times a day. Patients with chronic cluster headache have unremitting illness that necessitates daily preventive medical treatment for years. When medically intractable, the condition has previously been treatable only with cranially invasive or neurally destructive methods. METHODS: Eight patients with medically intractable chronic cluster headache were implanted in the suboccipital region with electrodes for occipital nerve stimulation. Other than the first patient, who was initially stimulated unilaterally before being stimulated bilaterally, all patients were stimulated bilaterally during treatment. FINDINGS: At a median follow-up of 20 months (range 6-27 months for bilateral stimulation), six of eight patients reported responses that were sufficiently meaningful for them to recommend the treatment to similarly affected patients with chronic cluster headache. Two patients noticed a substantial improvement (90% and 95%) in their attacks; three patients noticed a moderate improvement (40%, 60%, and 20-80%) and one reported mild improvement (25%). Improvements occurred in both frequency and severity of attacks. These changes took place over weeks or months, although attacks returned in days when the device malfunctioned (eg, with battery depletion). Adverse events of concern were lead migrations in one patient and battery depletion requiring replacement in four. INTERPRETATION: Occipital nerve stimulation in cluster headache seems to offer a safe, effective treatment option that could begin a new era of neurostimulation therapy for primary headache syndromes.