Epidemiology, burden and clinical spectrum of cluster headache: a global update.

BACKGROUND: This narrative review aims to broaden our understanding of the epidemiology, burden and clinical spectrum of cluster headache based on updated findings with a global perspective. METHODS: We conducted a literature search on the following topics: (a) epidemiology; (b) burden: quality of life, disability, economic burden, job-related burden and suicidality; and (c) clinical spectrum: male predominance and its changes, age, pre-cluster and pre-attack symptoms, aura, post-drome, attack characteristics (location, severity, duration and associated symptoms), bout characteristics (attack frequency, bout duration and bout frequency), circadian and seasonal rhythmicity and disease course. RESULTS: New large-scale population-based reports have suggested a lower prevalence than previous estimations. The impact of cluster headache creates a significant burden in terms of the quality of life, disability, economic and job-related burdens and suicidality. Several studies have reported decreasing male-to-female ratios and a wide age range at disease onset. The non-headache phases of cluster headache, including pre-cluster, pre-attack and postictal symptoms, have recently been revisited. The latest data regarding attack characteristics, bout characteristics, and circadian and seasonal rhythmicity from different countries have shown variability among bouts, attacks, individuals and ethnicities. Studies on the disease course of cluster headache have shown typical characteristics of attacks or bouts that decrease with time. CONCLUSIONS: Cluster headache may be more than a "trigeminal autonomic headache" because it involves complex central nervous system phenomena. The spectrum of attacks and bouts is wider than previously recognised. Cluster headache is a dynamic disorder that evolves or regresses over time.

Outcome of epidural blood patch for imaging-negative spontaneous intracranial hypotension.

BACKGROUND: Spontaneous intracranial hypotension is diagnosed by an abnormal finding in brain MRI, spinal imaging, or lumbar puncture. However, the sensitivity of each test is low. We investigated whether patients with suspected spontaneous intracranial hypotension and negative imaging findings would respond to epidural blood patch. METHODS: We prospectively recruited patients with new-onset orthostatic headache admitted at the Samsung Medical Center from January 2017 to July 2021. In patients without abnormal imaging findings and no history of prior epidural blood patch, treatment outcome-defined as both 50% response in maximal headache intensity and improvement of orthostatic component-was collected at discharge and three months after epidural blood patch. RESULTS: We included 21 treatment-naïve patients with orthostatic headache and negative brain and spinal imaging results who received epidural blood patch. After epidural blood patch (mean 1.3 times, range 1-3), 14 (66.7%) and 19 (90.5%) patients achieved both 50% response and improvement of orthostatic component at discharge and three months post-treatment, respectively. Additionally, complete remission was reported in 11 (52.4%) patients at three-month follow-up, while most of the remaining patients had only mild headaches. Among nine (42.9%) patients who underwent lumbar puncture, none had an abnormally low opening pressure (median 13.8 cm H2O, range 9.2-21.5). CONCLUSION: Given the high responder rates of epidural blood patch in our study, empirical epidural blood patch should be considered to treat new-onset orthostatic headache, even when brain and spinal imaging are negative. The necessity of lumbar puncture is questionable considering the high response rate of epidural blood patch and low rate of "low pressure."

Reversible cerebral Vasoconstriction syndrome intERnational CollaborativE (REVERCE) network: Study protocol and rationale of a multicentre research collaboration.

INTRODUCTION: Reversible cerebral vasoconstriction syndrome (RCVS) is a rare, but increasingly recognised cerebrovascular condition with an estimated annual age-standardised incidence of approximately three cases per million. Knowledge about risk factors and triggering conditions and information about prognosis and optimal treatment in these patients are limited. METHODS: The REversible cerebral Vasoconstriction syndrome intERnational CollaborativE (REVERCE) project aims to elucidate the epidemiological and clinical characteristics of RCVS by collecting individual patient data from four countries (France, Italy, Taiwan and South Korea) in the setting of a multicentric study. All patients with a diagnosis of definite RCVS will be included. Data on the distribution of risk factors and triggering conditions, imaging data, neurological complications, functional outcome, risk of recurrent vascular events and death and finally the use of specific treatments will be collected. Subgroup analyses will be made based on age, gender, aetiology, ethnicity and geographical region of residence. ETHICS AND DISSEMINATION: Ethical approval for the REVERCE study will be obtained from national or local institutional review boards in the participating centres. When needed, a standardised data transfer agreement will be provided for participating centres. We plan dissemination of our results in peer-reviewed international scientific journals and through conference presentations. We expect that the results of this unique study will lead to better understanding of clinical and epidemiological characteristics of RCVS patients.