Interictal Burden of Cluster Headache: Results of the EUROLIGHT Cluster Headache Project, an Internet-Based, Cross-Sectional Study of People With Cluster Headache.

OBJECTIVE: The aim of this study was to measure the interictal burden (IIB) associated with cluster headache (CH). BACKGROUND: CH is characterized by an alternation of excruciatingly painful attacks and pain-free periods. Absence of pain does not necessarily imply absence of symptoms, though. Some may persist or improve more slowly than pain; others may arise in between attacks. METHODS: Participants filled out an online survey based on the EUROLIGHT questionnaire. Inclusion criteria were a reported diagnosis of CH and residency in a European country; exclusion criteria were refusal to give informed consent and to complete the questionnaire. RESULTS: Most of the burden was related to higher headache frequencies: ongoing symptoms despite pain freedom, impaired autonomy due to avoidance of triggers, reluctance to tell others about the disease, and the feeling of not being understood by family and friends, employers and colleagues. Irreversible, potentially accumulating burden may occur if headache frequency is high; examples are impairment of career options, relationships, and family planning issues. Worrying about future attacks and avoiding potential triggers occur independently from attack frequency and disease duration. There were no differences between the in-bout and the out-bout period among participants with episodic CH. Participants with chronic CH reported greater IIB. CONCLUSIONS: Even between attacks CH can have a huge and potentially irreversible impact on life. Different types of IIB were identified, all of which may contribute to the total burden of disease. As cumulative burden might be irreversible, prevention strategies need to be developed.

New treatments for headache.

Migraine and cluster headache are primary headache disorders commonly encountered in clinical practice. Despite the profound disability caused by these primary headache disorders, available acute and preventive treatment options are limited. Recent understanding of headache pathophysiology has led to the development of new drug formulations and novel drug targets that are extremely promising. This article will highlight several of the new treatments that are currently under investigation including novel delivery mechanisms of already existing medications, calcitonin gene-related peptide (CGRP) receptor antagonists, antibodies to CGRP and its receptor, serotonin receptor agonists, transient receptor potential vanilloid receptor modulators, orexin receptor antagonists, glial cell modulators, and neuromodulation. If data is supportive, these therapies will be welcome additions to the headache specialist's armamentarium.

Cluster headache in the United States of America: demographics, clinical characteristics, triggers, suicidality, and personal burden.

OBJECTIVE: To present results from the United States (US) Cluster Headache Survey including data on cluster headache demographics, clinical characteristics, suicidality, diagnostic delay, triggers, and personal burden. BACKGROUND: There are few large-scale studies looking at cluster headache patients and none from the USA. This manuscript will present data from The US Cluster Headache Survey, the largest survey ever completed of cluster headache patients living in the USA. METHODS: The total survey was composed of 187 multiple-choice questions that dealt with issues related to cluster headache including demographics, clinical characteristics, comorbid medical conditions, family history, triggers, smoking history, and personal burden. The survey was placed on a Web site from October through December 2008. RESULTS: A total of 1134 individuals completed the survey (816 male, 318 female). Some key highlights from the survey include the following: (1) diagnostic delay: there remains a significant diagnostic delay for cluster headache patients on average 5+ years with only 21% receiving a correct diagnosis at time of initial presentation. (2) Suicidality: suicidal ideations are substantial, occurring in 55%. (3) Eye color: the predominant eye color in cluster headache patients is brown and blue, not hazel as suggested in previous descriptions. (4) Laterality: cluster headache has a right-sided predominance. (5) Attack profile: in US cluster headache sufferers, most attacks occur between early evening and early morning hours with peak time of headache onset between midnight and 3 am; the circadian periodicity for cluster headache is present but is not as predominant in the population as previously thought. (6) Triggers: beer is the most common type of alcohol trigger in US cluster headache patients; noted migraine triggers such as weather changes and smells are also very common cluster headache triggers. (7) Medical comorbidities: peptic ulcer disease does not have a high prevalence in US cluster headache patients as suggested by previous literature; cluster headache is associated with a low prevalence of cardiac disease as well as cerebrovascular disease even though the majority of patients are chronic heavy smokers. In US cluster headache sufferers, there appears to be comorbidity with restless leg syndrome, and this has not been demonstrated in non-US cluster headache populations. (8) Personal burden: cluster headache is disabling to the individual as almost 20% of cluster headache patients have lost a job secondary to cluster headache, while another 8% are out of work or on disability secondary to their headaches. CONCLUSION: Some findings from the US Cluster Headache Survey expound on what is currently known about cluster headache, while some of the results contradict what has been previously written, while other information is completely new about this fascinating headache disorder.

Assessment of the CO2 response by means of non diffusible contrast media and angio-CT in patients with cluster headache.

We analyzed the possibility of assessing functional vasomotor changes by means of Arm-Brain Circulation Time (rABCT) and Vascular volume images (Vv) obtained with Angio-CT, in basal condition and following CO2 inhalation, in a sample of 48 patients with cluster headache. CO2 inhalation resulted in the appearance of local changes, which were detected in 28 regions. Analysis by indicator images of Vv-dependent rABCT distribution showed two main patterns: abnormal rABCT mostly evident at the smallest Vv pixels and abnormal rABCT dependent on abnormal Vv distribution. The former pattern was linked to abnormality at the circle of Willis; the latter to abnormal local vasomotor responses. Patients with cluster headache showed both patterns, which prompted us to conclude for the presence of low-degree stenosis in carotid arteries and vasomotor instability in peripheral brain vessels.

Cluster headache: transcranial Doppler assessment of dynamic cerebral circulatory changes during hypocapnia and attack.

Transcranial Doppler ultrasound (TCD) investigations have been carried out in cluster headache patients (8 during remission and 6 during bout) and 14 healthy subjects, to assess cerebral vasomotor reactivity (VMR) to hypocapnia induced by voluntary hyperventilation. VMR was expressed as the relative change in blood flow velocity (V) (%) as a function of the reduction in end-tidal PCO2 (PETCO2) (kPa), i.e. V/P ETCO2. TCD with simultaneous PETCO2 monitoring, was also performed in 5 patients during spontaneous attacks. Prior to hyperventilation, there was bilaterally lower anterior cerebral artery velocity (VACA) during the bout than during remission (P < 0.05 on the symptomatic side), and also lower than in the controls. During remission, VACA was higher on the symptomatic side than on the other side (P < 0.05). ACA also showed a lower VMR during the bout than during remission, and it was also lower than in controls (bout vs. remission on the non-symptomatic side, P < 0.01; on the symptomatic side, P > 0.1). Approximately 30 minutes after the onset of attack, PETCO2 started to decrease gradually from 4.65 to 4.10 kPa in one patient with severe attack. The VACA decreased markedly and bilaterally already at an early stage of the attack, i.e. prior to the hyperventilation. Middle cerebral artery velocity tended to decrease 30 minutes after the onset of attack on the symptomatic side, and 50 minutes after onset on the non-symptomatic side. It is concluded that the vascular changes observed most likely are secondary phenomena during the cluster headache attack.

Cluster headache: combined assessment with pupillometry and evaporimetry.

Twenty-nine patients with cluster headache have been examined with the pupillometer and the evaporimeter. Pupils were dilated by sympathicomimetic drugs instilled into the conjunctival sacs, and responses of the two sides were compared. Forehead sweating was stimulated by body heating and by pilocarpine injection, and sweat evaporation on the two sides was compared. Most patients demonstrated the known patterns of hyposecretion on heating, of pilocarpine supersensitivity, and of deficient pupillary dilatation on OH-amphetamine stimulation on the symptomatic side and a supersensitivity of this pupil to phenylephrine. There were deviations from the rule for all methods of testing. Sixteen patients demonstrated a typical supersensitivity response of the pupil and of the sweat glands on the symptomatic side. Six patients had no such response, and seven patients had a discrepancy between the pupillary and the sweat gland response to stimulation, supersensitivity being present in one system only. There was a high degree of concordance between the results of the various methods of examination.