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Cluster Headache

Hemicrania continua

Cluster headache is a condition, characterized by recurrent severe headaches particularly on one side of the head. It is a neurological disorder, wherein the affected individuals would also experience associated symptoms such as conjunctival injection, nasal congestion, rhinorrhea, miosis or ptosis.


Presentation

Development of severe, unilateral and sudden headache marks the onset of cluster headaches. Individuals usually experience headaches 2 to 3 hours after they fall asleep. Attacks can occur almost every day and can continue for several months. Headaches typically occur at the same time of the day, every day and last for about 15 to 180 minutes. Affected individuals experience the following signs and symptoms [8]:

Severe Pain
  • All six included studies used a single dose of triptan to treat an attack of moderate to severe pain intensity.[ncbi.nlm.nih.gov]
  • Cluster headache (CH) is associated with the most severe pain of the primary headache disorders. Barriers to optimal care include misdiagnosis, diagnostic delay, undertreatment, and mismanagement.[ncbi.nlm.nih.gov]
  • One possible explanation for the severe pain described in these cases is hyperalgesia induced by high doses of methadone. 2014 Société Française de Pharmacologie et de Thérapeutique.[ncbi.nlm.nih.gov]
  • Cluster headache is an uncommon, severely painful, and disabling condition, with rapid onset. Validated treatment options are limited; first-line therapy includes inhaled oxygen.[cochrane.org]
  • This headache syndrome involves what is described as “attacks” of severe pain on one side of the head lasting 15-180 minutes and occurring as little as once every day or as often as 8 times a day.[coloradoheadache.com]
Nocturnal Awakening
  • Ligia Mendonça Lucchesi, Jose Geraldo Speciali, Rogerio Santos-Silva, Jose Augusto Taddei, Sergio Tufik and Lia Rita Azeredo Bittencourt, Nocturnal awakening with headache and its relationship with sleep disorders in a population-based sample of adult[doi.org]
Nasal Discharge
  • discharge and tearing of the eye is on the same side as the pain Excessive sweating Face may become flushed on the affected side Cluster headaches are not associated with the gastrointestinal disturbances or sensitivity to light that is found in migraine[headaches.org]
  • There may be nasal discharge or congestion and tearing of the eye during an attack, which occur on the same side as the pain. Excessive sweating may occur, and the face may become flushed on the affected side.[my.clevelandclinic.org]
  • discharge/”runny nose”), forehead and facial sweating, miosis (excessive constriction/tightening of the eye’s pupil), ptosis (drooping of the upper eyelid), eyelid edema (swelling/”puffiness” of the eyelid from excessive watery fluid collection).[clusterbusters.org]
Hyposmia
  • Dysgeusia has only rarely been reported as a side effect of lithium in other conditions and hyposmia has not previously been reported.[ncbi.nlm.nih.gov]
Nausea
  • Our female cluster headache population had more nausea and significantly more vomiting than our men. Manzoni et al 12 also found that nausea was more common in women than men. They did not find any other sex differences in cluster headache symptoms.[dx.doi.org]
  • Photophobia or phonophobia and nausea were in part responsible for this delay, and should be recognised as part of the clinical spectrum of CH.[ncbi.nlm.nih.gov]
  • Confusion, dizziness, blurry vision, diabetes insipidus, headache, nausea, polyuria In a double-blind crossover study 33 of 30 patients, 50 percent of patients responded in two weeks.[aafp.org]
  • Interictal headache or concomitant symptoms, such as photophobia or nausea during episodes, may distract from the appropriate diagnosis.[doi.org]
Bulimia
  • DBS had no effect on a pathological weight gain from 70 kg to 150 kg due to bulimia at night, usually during headache attacks. This case illustrates that cluster headache is, in some patients, only one symptom of a complex hypothalamic syndrome.[ncbi.nlm.nih.gov]
Motor Restlessness
  • A recent review that focuses mostly on the behavior of motor restlessness during the CH attack, it also addresses the issue of suicidality. PubMed Google Scholar 20. Mitsikostas DD, Thomas AM: Comorbidity of headache and depressive disorders.[doi.org]
Lacrimation
  • METHODS/RESULTS: Ophthalmic features such as conjunctival injection, lacrimation, ptosis and miosis occur in the vast majority of patients with cluster headache, whereas co-existent ocular movement disorders are rare.[ncbi.nlm.nih.gov]
  • Abstract Cluster headache (CH) is characterized by a series of sudden attacks of short-lasting severe headache pain with ipsilateral autonomic features, including lacrimation, rhinorrhea, localized sweating, eyelid edema, and partial or complete Horner's[ncbi.nlm.nih.gov]
  • Cluster headache, an excruciating, unilateral headache usually accompanied by conjunctival injection and lacrimation, can occur episodically or chronically, and can be difficult to treat.[ncbi.nlm.nih.gov]
  • We report the case of an 11-year-old boy who suffered from sudden attacks of shock-like, intense pain, localized in the right orbital region, with associated photophobia, phonophobia, conjunctival injection, lacrimation, nasal congestion, rhinorrhoea[ncbi.nlm.nih.gov]
Eye Pain
  • July 2013 Article – “Cluster Headaches – a Medical Enigma” Symptoms Symptoms may include Excruciating, boring, burning pain (much more severe than migraine) localized around one eye pain very pronounced behind one eye, commonly radiating to forehead,[headacheaustralia.org.au]
  • These are common symptoms of a cluster headache: Sudden onset of pain, generally around or behind the eye Pain builds to a peak in about 10 to 15 minutes Restlessness or agitation Red or watering eyes Nasal congestion Sweating on the forehead Eyelid drooping[hopkinsmedicine.org]
  • When activated, the trigeminal nerve leads to the eye pain associated with cluster headaches.[my.clevelandclinic.org]
  • Most people with cluster headaches describe their pain as occurring around or behind the eye. Pain may radiate along the forehead, into the jaw or along the gum line and into the teeth , or across the cheek of the affected side.[medicinenet.com]
Red Eye
  • Cluster headache is an intense and powerful, one-sided headache accompanied by involuntary symptoms such as red eye, droopy eyelids, flow of tears, small pupils and one-sided facial sweating.[clinicaltrials.gov]
  • Symptoms can include: Swelling under or around the eye (may affect both eyes) Excessive tearing Red eye Rhinorrhea (runny nose) or one-sided stuffy nose (same side as the head pain) Red, flushed face Cluster headaches may occur daily for months, alternating[web.archive.org]
  • But attacks can also cause red eyes, tearing, a puffy and drooping eyelid, runny nose, and even facial sweating. Are There Any Modern Medical Cluster Headache Remedies? Yes, there are.[amazing-solutions.com]
  • Strengthen your vocabulary For those who aren’t doctors, here are some definitions of the medical terms used by the IHS: conjunctival injection – red eye edema – swelling or puffiness from fluid collection ipsilateral – on the same side lacrimation –[clusterbusters.org]
  • CH attacks also include one or more cranial autonomic symptoms (CAS) on the same side of the head as the pain (ipsilaterally) such as red eye (conjunctival injection), eyelid swelling (edema), forehead and facial sweating, tearing (lacrimation), abnormally[rarediseases.org]
Excessive Tearing
  • Affected individuals also experience excessive tearing along with stuffed nose. Diagnosis: A preliminary physical examination would be done at the initial level.[symptoma.com]
  • Symptoms can include: Swelling under or around the eye (may affect both eyes) Excessive tearing Red eye Rhinorrhea (runny nose) or one-sided stuffy nose (same side as the head pain) Red, flushed face Cluster headaches may occur daily for months, alternating[web.archive.org]
  • Common signs and symptoms during a headache include: Excruciating pain, generally situated in or around one eye, but may radiate to other areas of your face, head, neck and shoulders One-sided pain Restlessness Excessive tearing Redness in your eye on[mayoclinic.org]
  • Common signs and symptoms during a headache include: Excruciating pain that is generally situated in, behind or around one eye, but may radiate to other areas of your face, head and neck One-sided pain Restlessness Excessive tearing Redness of your eye[mayoclinic.org]
  • Other signs and symptoms may be evident on the painful side of the head, including: a droopy eyelid a constricted pupil excessive tearing from your eye eye redness sensitivity to light swelling under or around one or both of your eyes a runny nose or[healthline.com]
Flushing
  • Symptoms: Symptoms of cluster headaches include sudden and severe headache, followed by flushing and redness of face. Affected individuals also experience excessive tearing along with stuffed nose.[symptoma.com]
  • Explicitly, they asked about lacrimation, conjunctival injection, nasal congestion, rhinorrhoea, eyelid oedema and forehead/facial flushing.[doi.org]
  • Autonomic features, including nasal congestion, rhinorrhea, lacrimation, facial flushing, and Horner syndrome, are prominent and occur on the same side as the headache.[merckmanuals.com]
  • […] can switch sides) and the main defining feature is the association with one or more of the 'cranial autonomic' features normally described as follows: Reddening and tearing of the eye A runny or blocked nostril Droopy eyelid Constriction of the pupil Flushing[ouchuk.org]
  • Like all medicines, verapamil has side-effects: constipation flushing dizziness ankle swelling Other preventive medicines If verapamil doesn’t work for you, there are other medicines that can help.[bupa.co.uk]
Hyperhidrosis
  • Labor, DR, Mohr, JP, Nichols, FT, Tatemichi, TK Unilateral hyperhidrosis after cerebral infarction. Neurology 1988 ; 38: 1679 – 82 Google Scholar Crossref Medline 12.[dx.doi.org]
Facial Pain
  • Abstract BACKGROUND: Chronic cluster headache (CCH) is characterized by recurrent bouts of facial pain lasting up to 180 minutes in the absence of a long remission period.[ncbi.nlm.nih.gov]
  • Headache and facial pain. Stuttgart : G. Thieme Verlag, 1981 : 1 – 274. Google Scholar 11. D’Alessandro, R, Gamerini, G, Benassi, G, Morganti, G, Cortelli, P, Lugaresi, E. Cluster headache in the Republic of San Marino.[oadoi.org]
  • Abstract Calcitonin gene-related peptide (CGRP) is a signaling neuropeptide released from activated trigeminal sensory afferents in headache and facial pain disorders.[ncbi.nlm.nih.gov]
  • Atypical facial pain. Headache 1969 ;9: 27 – 30. Google Scholar Crossref Medline Schiller, F. Prophylactic and other treatment for “histaminic”, “cluster” or “limited variant of migraine”. JAMA 1960 ;173: 1907 – 11. Google Scholar Crossref ISI[oadoi.org]
Cheek Pain
  • People who suffer from cluster headaches--especially people who have tooth or cheek pain with the cluster attacks--have an increased risk of developing a stomach ulcer.[my.clevelandclinic.org]
Headache
  • […] of cluster headache over time.[doi.org]
  • Keywords Migraine Cluster Headache Cavernous Sinus International Headache Society Chronic Cluster Headache These keywords were added by machine and not by the authors.[oadoi.org]
  • Geweke LO: Misdiagnosis of cluster headache. Curr Pain Headache Rep 2002, 6 :76–82. PubMed Google Scholar 19. Torelli P, Manzoni GC: Behavior during cluster headache. Curr Pain Headache Rep 2005, 9 :113–119.[doi.org]
  • Other common types of headaches include migraine headache and tension headache. Causes Cluster headaches are a fairly common form of chronic, repeated headaches. They are four times more common in men than women.[web.archive.org]
Recurrent Headache
  • headaches, and pain facilitation due to glial activation.[hdl.handle.net]
  • Recurrent headache occurred in 4.4% of the cases: migraine in 1.7%; cluster headache in 0.09%; other, non‐migrainous headache (MNH) in 2.6%.[oadoi.org]
  • Alessandro S Zagami and Sian L Goddard, Practical neurology Part 7 ‐ Recurrent headaches with visual disturbance, Medical Journal of Australia, 196, 3, (178-183), (2012).[doi.org]
  • Non-invasive physical treatments for chronic/recurrent headache. Cochrane Database Syst Rev. 2004;(3):CD001878. Copyright 2013 by the American Academy of Family Physicians. This content is owned by the AAFP.[aafp.org]
Excruciating Headache
  • The excruciating headaches tend to turn up in bouts lasting six to eight weeks.[scientificamerican.com]
  • Triptans for acute cluster headache Cluster headaches are excruciating headaches of extreme intensity. They can last for several hours, are usually on one side of the head only, and affect men more than women.[doi.org]
  • Paroxysmal hemicrania (PH), like CH, is characterized by strictly unilateral, brief, excruciating headaches that occur in association with cranial autonomic features.[dx.doi.org]

Workup

The following procedures are employed for diagnosing cluster headaches:

  • Neurological exam: This would be done to detect the physical signs of cluster headaches. The examination would reveal ptosis and miosis and other associated sign.
  • Imaging: Imaging studies such as CT scan of the brain and MRI of the blood vessels and brain would help in diagnosing the cause of cluster headaches. These tests would also help in ruling out conditions of tumor and aneurysm.

Treatment

Cluster headache cannot be cured; it can however be effectively managed by reducing the severity of the symptoms. The major objectives for treatment of the condition involve shortening of the duration of attacks, reducing the frequency and decreasing the severity of headaches. Following are the methods which are employed for treating acute attacks of cluster headaches:

  • Oxygen therapy: In acute conditions, inhaling 100% oxygen at rate of 12 liters per minute would provide relief within 15 minutes [9]. 
  • Triptans: Triptans can be given through injections or nasal spray. It has been observed that the one given through injections is more effective than nasal spray.
  • Octreotide: This medication is given through injection and is a synthetic form of the brain chemical somatostatin. 
  • Local anesthetics: Local anesthetics given through intranasal route is an effective treatment regime for cluster headaches.
  • Dihydroergotamine: This form of medication is given through the intravenous route and is an effective treatment regime for some percentage of individuals suffering from cluster headaches [10].
  • Surgery: This is often the last resort when other forms of aggressive treatment do not work. However, this is usually not an effective way of treatment because the surgical procedures employed may cause damage to the neighboring nerve cells.

Prognosis

Cluster headaches can be severe enough to interfere with daily work and also affects the quality of life; however the condition is not life threatening. With appropriate treatment, it can be effectively managed. Cluster headaches do not permanently damage the brain [7].

Etiology

The exact cause of cluster headache is not known. The disease is sporadic in nature; however some pieces of evidence suggest an autosomal dominant pattern. Abnormalities in the hypothalamus are known to play foul in the development of cluster headache. An increase in the activity of the hypothalamus has been noted in the affected individuals during the course of cluster headaches. The various risk factors for cluster headache include alcohol consumption, smoking and certain medications such as nitroglycerin [2].

Epidemiology

The exact incidence rate of cluster headache in the US is unknown. Middle aged individuals are more prone to develop such a type of condition. However, in many instances, cluster headache can even affect small children aged 1 year and adults aged 79 years and above [3]. Cluster headache is a rare phenomenon, with an estimated prevalence rate of 0.4% in men and 0.08% in women. Males are more prone to develop this condition compared to females, with male to female ratio being 6:1 [4].

Sex distribution
Age distribution

Pathophysiology

The pathophysiology of cluster headaches is not properly understood. The periodic attacks suggest the involvement of human biological clock along with increase in activity of the hypothalamus [5]. Diagnostic procedures have revealed that the major area of defect that gives rise to cluster headaches is the posterior hypothalamic grey matter. One of the mechanisms which have been understood, is that the attack is caused due to dilation of blood vessels which in turn exert pressure on the trigeminal nerve. Such a theory is known to give rise to the condition of cluster headaches [6].

Prevention

Individuals are advised to avoid the various trigger factors such as smoking and alcohol. Maintaining a headache diary would also help in identifying the day and time of attacks which would help in preventing the onset of future bouts. Certain medications have also proved to be effective in preventing the development of cluster headaches. These include allergic medications, seizure medicines and hypertensive medicines.

Summary

Cluster headache is often confused with conditions of migraines, tension headache, sinus or the usual headache. Affected individuals may experience regular attacks for a period of 1 week to a year on a regular basis. This type of neurological condition belongs to a group of trigeminal autonomic cephalalgias. So far there is no known cure to treat cluster headaches. However, these can be prevented and the development of acute attacks can be effectively managed [1].

Patient Information

  • Definition: Cluster headaches are a type of neurological disorder which involve onset of sudden and severe headaches usually on one side of head. Males are more prone to contract this disease condition in comparison to females.
  • Cause: The exact cause of cluster headache is unknown. Certain trigger factors include alcohol consumption, smoking and medications such as nitroglycerin for treating heart disease.
  • Symptoms: Symptoms of cluster headaches include sudden and severe headache, followed by flushing and redness of face. Affected individuals also experience excessive tearing along with stuffed nose.
  • Diagnosis: A preliminary physical examination would be done at the initial level. In addition, neurological examination would be done followed by imaging studies such as CT scan and MRI of the brain. This would help in ruling out other conditions of tumor and aneurysm.
  • Treatment: There is no known cure for cluster headaches. The condition can be effectively managed with appropriate treatment regime. Pharmacologic treatment forms the basis of the treatment regime. Medications such as triptans and steroids are given through the intravenous route. In addition, oxygen therapy has also been proven to be helpful for providing instant relief. Surgery is often the last resort and not always helpful in treating cluster headaches.

References

Article

  1. Mendizabal JE, Umana E, Zweifler RM. Cluster headache: Horton's cephalalgia revisited. South Med J. Jul 1998;91(7):606-17.
  2. Manzoni GC. Cluster headache and lifestyle: remarks on a population of 374 male patients. Cephalalgia 1999; 19:88.
  3. Evers S, Frese A, Majewski A, et al. Age of onset in cluster headache: the clinical spectrum (three case reports). Cephalalgia 2002; 22:160.
  4. Rozen TD, Fishman RS. Female cluster headache in the United States of America: what are the gender differences? Results from the United States Cluster Headache Survey. J Neurol Sci. Jun 15 2012;317(1-2):17-28.
  5. Lodi R, Pierangeli G, Tonon C, et al. Study of hypothalamic metabolism in cluster headache by proton MR spectroscopy. Neurology. 2006;66(8):1624-6.
  6. Favier I, van Vliet JA, Roon KI, et al. Trigeminal autonomic cephalgias due to structural lesions: a review of 31 cases. Arch Neurol 2007; 64:25.
  7. May A. Cluster headache: pathogenesis, diagnosis, and management. Lancet 2005; 366:843.
  8. Sjaastad, O (Ed). Cluster Headache Syndrome. W B Saunders Company Ltd, London 1992.
  9. Petersen AS, Barloese MC, Jensen RH. Oxygen treatment of cluster headache: a review. Cephalalgia 2014; 34:1079.
  10. Tfelt-Hansen P. Acute pharmacotherapy of migraine, tension-type headache, and cluster headache. J Headache Pain. Apr 2007;8(2):127-34.

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Last updated: 2019-07-11 21:44