scholarly journals Cluster headache and the hypothalamus – causal relationship or epiphenomenon?

2012 ◽  
pp. 188-197
Author(s):  
Dagny Holle ◽  
Mark Obermann
Keyword(s):  
Cluster Headache ◽  
Animal Studies ◽  
Primary Headache ◽  
Headache Disorder ◽  
Invasive Treatment ◽  
Primary Headache Disorder ◽  
Underlying Pathophysiology ◽  
Neuroimaging Techniques ◽  
Deep Brain

Typical clinical features of cluster headache (CH) include trigeminal distribution of pain, circadian/circannual rhythmicity, and ipisilateral cranial autonomic features. This presentation led to the assumption that the hypothalamus plays a pivotal role in this primary headache disorder. Several studies using neuroimaging techniques or measuring hormone levels supported the hypothesis of a hypothalamic involvement in the underlying pathophysiology in CH. Animal studies added further evidence regarding this hypothesis. Based on previous data even invasive treatment methods such as hypothalamic deep brain stimulation (DBS) were tried for therapy. However, the principal question whether these alterations are pathognomonic for CH or whether they might be detected in trigeminal pain disorders in general in terms of an epiphenomenon is still unsolved. This review summarizes studies on hypothalamic involvement in CH pathophysiology, demonstrates the involvement of the hypothalamus in other diseases, and tries to illuminate the role of the hypothalamus based on this synopsis.

Genetics of cluster headache

Cephalalgia ◽  
2019 ◽  
Vol 39 (10) ◽  
pp. 1298-1312 ◽  
Author(s):  
Kate F Gibson ◽  
Anita Dos Santos ◽  
Nunu Lund ◽  
Rigmor Jensen ◽  
Ioannis M Stylianou
Keyword(s):  
Cluster Headache ◽  
Candidate Gene ◽  
Gene Selection ◽  
Association Studies ◽  
Twin Studies ◽  
Primary Headache ◽  
Headache Disorder ◽  
Genome Wide Association Studies ◽  
Genetic Studies ◽  
Primary Headache Disorder

Background Cluster headache is the most severe primary headache disorder. A genetic basis has long been suggested by family and twin studies; however, little is understood about the genetic variants that contribute to cluster headache susceptibility. Methods We conducted a literature search of the MEDLINE database using the PubMed search engine to identify all human genetic studies for cluster headache. In this article we provide a review of those genetic studies, along with an overview of the pathophysiology of cluster headache and a brief review of migraine genetics, which have both been significant drivers of cluster headache candidate gene selection. Results The investigation of cluster headache genetic etiology has been dominated by candidate gene studies. Candidate selection has largely been driven by the pathophysiology, such as the striking rhythmic nature of the attacks, which spurred close examination of the circadian rhythm genes CLOCK and HCRTR2. More recently, unbiased genetic approaches such as genome-wide association studies (GWAS) have yielded new genetic avenues of interest including ADCYAP1R1 and MME. Conclusions The majority of candidate genes studied for cluster headache suffer from poor reproducibility. Broader genetic interrogation through larger unbiased GWAS, exome, and whole genome studies may provide more robust candidates, and in turn provide a clearer understanding of the causes of cluster headache.

Headache

2015 ◽  
Author(s):  
Peter J. Goadsby
Keyword(s):  
Cluster Headache ◽  
Severe Pain ◽  
Primary Headache ◽  
Headache Disorder ◽  
Headache Disorders ◽  
Important Work ◽  
Pain Experience ◽  
Global Impact ◽  
Primary Headache Disorder ◽  
The World

Headache disorders are the dominant cause of neurological disability in the world and the most common reason for neurological referral in any country studied. Yet for much of the first half the twentieth century, research was mired in peripheral mechanistic sideshows. Migraine, the most common disabling primary headache disorder, has been established as primarily a brain problem, with important advances in classification, treatment, and biological understanding. Cluster headache, perhaps the most severe pain experience of humans, has found its nidus in the diencephalon; treatments are evolving and biology being unravelled. Contributions to headache disorders resonate across humanity, so important work here has a global impact for good.

scholarly journals Update on Treatment of Cluster Headache

2021 ◽  
Vol 39 (3) ◽  
pp. 113-120
Author(s):  
Jong-Hee Sohn ◽  
Mi Ji Lee ◽  
Soo-Jin Cho
Keyword(s):  
Cluster Headache ◽  
Preventive Treatment ◽  
Primary Headache ◽  
Headache Disorder ◽  
Preventive Therapy ◽  
Systemic Steroid ◽  
Novel Treatments ◽  
Primary Headache Disorder ◽  
Cranial Autonomic Symptoms ◽  
High Flow Oxygen

Cluster headache (CH) is characterized by severe unilateral short-lasting headache attacks, accompanying ipsilateral cranial autonomic symptoms or the sense of restlessness and agitation, or both. CH is a highly disabling primary headache disorder but often not optimally treated. High-flow oxygen and parenteral triptans are the most effective treatment to treat an acute CH attack. Transitional treatments include systemic steroid therapy and sub-occipital steroid injection. For preventive therapy, verapamil and lithium are recommended as first-line treatments. Novel treatments have appeared, such as neuromodulation and medication targeting calcitonin gene-related peptide (CGRP) or its receptor. Galcanezumab, the only anti-CGRP receptor monoclonal antibody with proven efficacy for the preventive treatment of episodic CH, represents an important advance for pharmacological treatment of CH. Neuromodulation strategies, such as the non-invasive vagus nerve stimulation and the sphenopalatine ganglion stimulation, have been proven effective in reducing the intensity and frequency of attacks, and also to be safe and well tolerated.

Complete alleviation of treatment refractory primary SUNCT syndrome with clomiphene citrate (a medicinal deep brain hypothalamic modulator)

Cephalalgia ◽  
2014 ◽  
Vol 34 (12) ◽  
pp. 1021-1024 ◽  
Author(s):  
Todd D Rozen
Keyword(s):  
Clomiphene Citrate ◽  
Treatment Options ◽  
Primary Headache ◽  
Headache Disorder ◽  
Decompression Surgery ◽  
Sunct Syndrome ◽  
Primary Headache Disorder ◽  
Treatment Refractory ◽  
Putative Mechanism ◽  
Deep Brain

Objective To report the first ever case of primary short-lasting unilateral neuralgiform headache attacks (SUNCT) syndrome completely responsive to clomiphene citrate. Methods Case report. Results SUNCT is a primary headache disorder marked by frequent attacks of one-sided headache with cranial autonomic associated symptoms. When SUNCT is deemed medicinally treatment refractory, it can cause tremendous patient-related disability. Surgical treatment options are available including hypothalamic deep brain stimulation, occipital nerve stimulator placement or arterial decompression surgery, but these procedures carry significant morbidity. A patient presented with a 10 month complaint of multiple, daily short-lasting, right-sided headaches each lasting from 60 to 120 seconds in duration and occurring from 100 to 200 times per day. The head pain was associated with ipsilateral eyelid ptosis and conjunctival injection. The patient was diagnosed with SUNCT but was unresponsive to multiple recognized medicinal treatments. He had complete alleviation of his attacks with clomiphene citrate, a synthetic, non-steroidal, ovulatory stimulant that directly binds to hypothalamic estrogen receptors. The clomiphene was tolerated without any adverse events. A putative mechanism of action for clomiphene in the prevention of SUNCT will be presented. Conclusion Clomiphene citrate is a unique treatment for SUNCT and appears to be very safe and effective.

CACNA1A Gene Polymorphisms in Cluster Headache

Cephalalgia ◽  
2001 ◽  
Vol 21 (10) ◽  
pp. 953-958 ◽  
Author(s):  
C Sjöstrand ◽  
V Giedratis ◽  
K Ekbom ◽  
E Waldenlind ◽  
J Hillert
Keyword(s):  
Cluster Headache ◽  
Primary Headache ◽  
Headache Disorder ◽  
Familial Hemiplegic Migraine ◽  
Hemiplegic Migraine ◽  
Promising Candidate ◽  
Primary Headache Disorder ◽  
Increased Risk ◽  
Ihs Criteria ◽  
Cacna1a Gene

Cluster headache (CH) is a primary headache disorder where the aetiological and pathophysiological mechanisms still are largely unknown. An increased risk of CH in first- and second-degree relatives suggests the importance of genetic factors. Mutations of the P/Q type calcium channel alpha 1 subunit (CACNA1A) gene on chromosome 19p13 have been shown to cause several neurological disorders with a wide clinical spectrum, mainly episodic diseases. Missence mutations of the gene cause familial hemiplegic migraine (FHM) and it is also likely to be involved in the more common forms of migraine. The CACNA1A gene is thus a promising candidate gene for CH. In this study we performed an association analysis of an intragenic polymorphic (CA)n-repeat with marker D19S1150 and a (CAG)n-repeat in the 3′UTR region, in 75 patients with CH according to IHS criteria and 108 matched controls. Genotypes and allele frequencies were similarly distributed in patients and controls. Linkage disequilibrium between the two markers was similar in patients and controls. We conclude that an importance of the CACNA1A gene in sporadic CH is unlikely.

Responsiveness of short-lasting unilateral neuralgiform headache with conjunctival injection and tearing to hypothalamic deep brain stimulation

2009 ◽  
Vol 110 (2) ◽  
pp. 279-281 ◽  
Author(s):  
Mark K. Lyons ◽  
David W. Dodick ◽  
Virgilio Gerald H. Evidente
Keyword(s):  
Deep Brain Stimulation ◽  
Functional Imaging ◽  
Brain Stimulation ◽  
Primary Headache ◽  
Headache Disorder ◽  
Trigeminal Autonomic Cephalalgias ◽  
Conjunctival Injection ◽  
Imaging Studies ◽  
Primary Headache Disorder ◽  
Deep Brain

Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) is a severe primary headache disorder that is often refractory to medical therapy. Although the pathogenesis of this and other trigeminal autonomic cephalalgias is not completely understood, ipsilateral activation of the posterior and inferior hypothalamus has been identified on functional imaging studies during attacks. The authors report on a case of SUNCT syndrome successfully treated with hypothalamic deep brain stimulation and discuss the current literature.

Cluster headache

2020 ◽  
pp. 182-189
Author(s):  
Ilse F. de Coo ◽  
Leopoldine A. Wilbrink ◽  
Joost Haan
Keyword(s):  
Nervous System ◽  
Cluster Headache ◽  
Sensory System ◽  
Primary Headache ◽  
Headache Disorder ◽  
Primary Headache Disorder ◽  
Oxygen Inhalation ◽  
First Choice ◽  
Temporal Course ◽  
Subcutaneous Sumatriptan

Cluster headache is a primary headache disorder characterized by attacks of severe-to-very severe unilateral pain, located in the orbital, supraorbital, and/or temporal regions, with ipsilateral autonomic symptoms and/or restlessness. By definition, cluster headache attacks last between 15 and 180 minutes. Based on the temporal course, cluster headache can be categorized into episodic and chronic. A subdivision can also be made in primary and secondary chronic and secondary episodic. The cause of cluster headache is unknown, but probably the hypothalamus, trigeminal sensory system, some cranial blood vessels, and the autonomic nervous system are involved. A combination of acute (oxygen inhalation and/or subcutaneous sumatriptan) and prophylactic (first choice verapamil) treatment is usually necessary. For treatment-resistant patients, some experimental invasive options exist.

Pathophysiology of hypnic headache

Cephalalgia ◽  
2014 ◽  
Vol 34 (10) ◽  
pp. 806-812 ◽  
Author(s):  
Dagny Holle ◽  
Steffen Naegel ◽  
Mark Obermann
Keyword(s):  
Electrophysiological Study ◽  
Primary Headache ◽  
Headache Disorder ◽  
Primary Headaches ◽  
Hypnic Headache ◽  
Primary Headache Disorder ◽  
Study Results ◽  
Symptomatic Headache ◽  
Underlying Mechanisms ◽  
Underlying Pathophysiology

Background Hypnic headache (HH) is a rare primary headache disorder that is characterized by strictly sleep related headache attacks. Purpose The underlying pathophysiology of HH is mainly enigmatic but some clinical characteristics such as circadian rhythmicity and caffeine responsiveness may point toward possible underlying mechanisms. Method Current studies that deal with the pathophysiology of HH are summarized. Data on cerebral imaging, sleep, electrophysiology studies, effectiveness of drugs, and symptomatic headache types are discussed to illuminate underlying pathophysiologic mechanisms. Conclusion HH can be clearly differentiated by its clinical presentation as well as imaging and electrophysiological study results from other primary headaches such as migraine or cluster headache. The underlying pathophysiology is still enigmatic but a hypothalamic involvement seems to be likely.

Device review: Pulsante™ sphenopalatine ganglion microstimulator

2019 ◽  
Vol 9 (6) ◽  
pp. 535-541
Author(s):  
Nenna Nwazota ◽  
Srinivas Pyati ◽  
Kyle Fisher ◽  
Lance Roy
Keyword(s):  
Remote Control ◽  
Cluster Headache ◽  
Treatment Option ◽  
Primary Headache ◽  
Headache Disorder ◽  
Sphenopalatine Ganglion ◽  
Primary Headache Disorder ◽  
Fixation Plate ◽  
Stimulating Electrodes ◽  
Autonomic Reflex

Cluster headache (CH) is a primary headache disorder. The use of neuromodulation in treatment of CH is well documented. The sphenopalatine ganglion (SPG) has long been a target for management of CH. Intervention at the level of the SPG can interrupt the trigemino-autonomic reflex, which mediates CH pain. The Pulsante system is the only device on the market created for SPG stimulation. The Pulsante device consists of the device body, a lead with six stimulating electrodes placed in the pterygopalatine fossa, and a fixation plate to allow anchoring of the device to the maxilla. Stimulation is administered via a patient-controlled handheld remote control held over the cheek. SPG stimulation is an important treatment option for CH patients.

Cluster Headache

Cephalalgia ◽  
2000 ◽  
Vol 20 (9) ◽  
pp. 787-803 ◽  
Author(s):  
DW Dodick ◽  
TD Rozen ◽  
PJ Goadsby ◽  
SD Silberstein
Keyword(s):  
Gender Differences ◽  
Cluster Headache ◽  
Treatment Options ◽  
Clinical Manifestations ◽  
Primary Headache ◽  
Headache Disorder ◽  
Autonomic Symptoms ◽  
Head Pain ◽  
Primary Headache Disorder

Cluster headache is a stereotypic, primary headache disorder that is marked by repeated short-lasting attacks of severe, unilateral head pain and associated autonomic symptoms. Cluster headache is probably due to an abnormality in the circadian hypothalamic generator with subsequent trigeminovascular activation. We have reviewed the clinical manifestations, pathophysiology, gender differences, and treatment options in cluster headache.

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