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      Cluster Headache: Epidemiology, Pathophysiology, Clinical Features, and Diagnosis


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          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.

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          Most cited references 67

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          Cluster headache: a prospective clinical study with diagnostic implications.

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            Human in vivo evidence for trigeminovascular activation in cluster headache. Neuropeptide changes and effects of acute attacks therapies.

            Cluster headache is a rare very severe disorder that is clinically well characterized with a relatively poorly understood pathophysiology. In this study patients with episodic cluster headache fulfilling the criteria of the International Headache Society were examined during an acute spontaneous attack of headache to determine the local cranial release of neuropeptides. Blood was sampled from the external jugular vein ipsilateral to the pain before and after treatment of the attack. Samples were assayed for calcitonin gene-related peptide (CGRP), vasoactive intestinal polypeptide (VIP), substance P and neuropeptide Y. Attacks were treated with either oxygen inhalation, sumatriptan or an opiate. Thirteen patients were studied of whom 10 were male and three female. All had well-established typical attacks of cluster headache when blood was sampled. During the attacks external jugular vein blood levels of CGRP and VIP were raised while there was no change in neuropeptide Y or substance P. Calcitonin gene-related peptide levels rose to 110 +/- 7 pmol/l (normal: < 40) while VIP levels rose to 20 +/- 3 pmol/l (normal: < 7). Treatment with both oxygen and subcutaneous sumatriptan reduced the CGRP level to normal, while opiate administration did not alter the peptide levels. These data demonstrate for the first time in vivo human evidence for activation of the trigeminovascular system and the cranial parasympathetic nervous system in an acute attack of cluster headache. Furthermore, it is shown that both oxygen and sumatriptan abort the attacks and terminate activity in the trigeminovascular system.
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              The incidence and prevalence of cluster headache: a meta-analysis of population-based studies.

              Cluster headache is a trigemino-autonomic cephalgia with a low prevalence. Several population-based studies on its prevalence and incidence have been performed, but with different methodology resulting in different figures. We analysed all available population-based epidemiological studies on cluster headache and compared the data in a meta-analysis. The pooled data showed a lifetime prevalence of 124 per 100,000 [confidence interval (CI) 101, 151] and a 1-year prevalence of 53 per 100,000 (CI 26, 95). The overall sex ratio was 4.3 (male to female), it was higher in chronic cluster headache (15.0) compared with episodic cluster headache (3.8). The ratio of episodic vs. chronic cluster headache was 6.0. Our analysis revealed a relatively stable lifetime prevalence, which suggests that about one in 1000 people suffers from cluster headache, the prevalence being independent of the region of the population study. The sex ratio (male to female) is higher than published in several patient-based epidemiological studies.

                Author and article information

                Ann Indian Acad Neurol
                Ann Indian Acad Neurol
                Annals of Indian Academy of Neurology
                Medknow Publications & Media Pvt Ltd (India )
                April 2018
                : 21
                : Suppl 1
                : S3-S8
                [1 ]Department of Basic and Clinical Neuroscience, Headache Group, Institute of Psychiatry, Psychology and Neuroscience, King's College London, London, UK
                [2 ]NIHR-Wellcome Trust King's Clinical Research Facility, King's College Hospital, London, UK
                [3 ]Department of Medicine, Division of Neurology, National University Health System, University Medicine Cluster, Singapore
                Author notes
                Address for correspondence: Dr. Peter James Goadsby, King's College London, Wellcome Foundation Building, Denmark Hill Campus West, London SE5 9PJ, UK. E-mail: peter.goadsby@ 123456kcl.ac.uk
                Copyright: © 2006 - 2018 Annals of Indian Academy of Neurology

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