Cluster headache syndrome

Neurology

Clinicals - History

Fact Explanation
Headache Cluster headache, (also called as Horton's headache/ histaminic cephalalgia/ migrainous neuralgia) is the most painful recurrent headache with the most stereotyped attacks. The exact pathophysiology remains unclear, however it is thought to be due to vascular dilation, trigeminal nerve stimulation, circadian effects, histamine release, genetic factors, and autonomic nervous system activation. A cluster headache attack is characterized by a sudden onset and peaking in 10-15 minutes. The pain is severe to very severe and excruciating, stabbing and sharp in nature. Headache is exclusively unilateral and affects periorbital, retro-orbital, or temporal regions. An attack lasts 5 to 180 minutes. An episode contains several attacks such as 1-8 times in a day for as long as 4 months. The duration of the cluster period is often strikingly consistent for a given patient. A common pattern is one or two cluster periods per year. With time, however, the clusters may become seasonal and then occur more often and last longer. Headache
Cluster headache, (also called as Horton's headache/ histaminic cephalalgia/ migrainous neuralgia) is the most painful recurrent headache with the most stereotyped attacks. The exact pathophysiology remains unclear, however it is thought to be due to vascular dilation, trigeminal nerve stimulation, circadian effects, histamine release, genetic factors, and autonomic nervous system activation. A cluster headache attack is characterized by a sudden onset and peaking in 10-15 minutes. The pain is severe to very severe and excruciating, stabbing and sharp in nature. Headache is exclusively unilateral and affects periorbital, retro-orbital, or temporal regions. An attack lasts 5 to 180 minutes. An episode contains several attacks such as 1-8 times in a day for as long as 4 months. The duration of the cluster period is often strikingly consistent for a given patient. A common pattern is one or two cluster periods per year. With time, however, the clusters may become seasonal and then occur more often and last longer.
Facial pain The characteristic ipsilateral periorbital, retro-orbital, or temporal pain in the cluster headache is accompanied by a radiating pain in the cheek, neck, occipital and nuchal areas. The character of the pain is the same as the headache (severe stabbing, excruciating and sharp) and appears and disappears along with the headache. Facial pain
The characteristic ipsilateral periorbital, retro-orbital, or temporal pain in the cluster headache is accompanied by a radiating pain in the cheek, neck, occipital and nuchal areas. The character of the pain is the same as the headache (severe stabbing, excruciating and sharp) and appears and disappears along with the headache.
Sleep deprivation Onset during the night or 1 to 2 hours after falling asleep is common. In some patients, these may occur at the onset of REM sleep. At times, several attacks per night can result in sleep deprivation in patients with chronic cluster headache, particularly when they avoid sleep for fear of inducing a further attack. Sleep deprivation
Onset during the night or 1 to 2 hours after falling asleep is common. In some patients, these may occur at the onset of REM sleep. At times, several attacks per night can result in sleep deprivation in patients with chronic cluster headache, particularly when they avoid sleep for fear of inducing a further attack.
Nasal congestion Severe headache attacks are typically accompanied by cranial autonomic symptoms due to central disinhibition of the nociceptive and autonomic (sympathetic) receptors of the trigeminal nociceptive pathways. Therefore the patient may complain of a characteristically ipsilateral congestion of the nose and/ or ipsilateral rhinorrhea. These are owing to the unopposed parasympathetic action that activates nasal goblet cells to produce more secretions. Nasal congestion
Severe headache attacks are typically accompanied by cranial autonomic symptoms due to central disinhibition of the nociceptive and autonomic (sympathetic) receptors of the trigeminal nociceptive pathways. Therefore the patient may complain of a characteristically ipsilateral congestion of the nose and/ or ipsilateral rhinorrhea. These are owing to the unopposed parasympathetic action that activates nasal goblet cells to produce more secretions.
Tearing Intense tearing occur in the ipsilateral eye. This is due to parasympathetic activation of the lacrimal glands to produce and secrete more tears in that side. Tearing
Intense tearing occur in the ipsilateral eye. This is due to parasympathetic activation of the lacrimal glands to produce and secrete more tears in that side.
Facial sweating This is also unilateral and is owing to the unopposed parasympathetic activation of the sweat glands. Facial sweating
This is also unilateral and is owing to the unopposed parasympathetic activation of the sweat glands.
Drooping of the eye lid Ptosis or the drooping of the eyelid occurs when the levator and superior tarsal muscles are not innervated by the sympathetic branches of the oculomotor nerve. This is also ipsilateral. Drooping of the eye lid
Ptosis or the drooping of the eyelid occurs when the levator and superior tarsal muscles are not innervated by the sympathetic branches of the oculomotor nerve. This is also ipsilateral.
Agitation The pain is so severe and excruciating that most of the patients usually do not prefer resting on the bed. They prefer to pace or move around, scream in pain, bang themselves against a hard surface or crawl on the floor instead. Agitation
The pain is so severe and excruciating that most of the patients usually do not prefer resting on the bed. They prefer to pace or move around, scream in pain, bang themselves against a hard surface or crawl on the floor instead.
Important negative facts There are several important negative facts in the the history that must be evaluated. Most of them are helpful in differentiating the cluster headache from migraine. Patients usually does not complain of nausea or vomiting. Photophobia and phonophobia are usually absent, or only one is present. Patients with cluster headache do not typically report any form of aura, fever, stiff neck, recent trauma, or bruxism. Important negative facts
There are several important negative facts in the the history that must be evaluated. Most of them are helpful in differentiating the cluster headache from migraine. Patients usually does not complain of nausea or vomiting. Photophobia and phonophobia are usually absent, or only one is present. Patients with cluster headache do not typically report any form of aura, fever, stiff neck, recent trauma, or bruxism.
Past history of similar events Cluster headache is a form of primary headache which is recurring/ episodic or chronic in human. Therefore patients may give a past history of similar events. Based on the duration and frequency of episodes, the disease is divided into chronic and episodic categories. Chronic disease is characterized by at least one cluster period lasting at least one year, with no remission or remission of less than one month. Patients with episodic cluster headaches get
at least two cluster periods of at least one week but less than one year, with remission for at least one month.
Past history of similar events
Cluster headache is a form of primary headache which is recurring/ episodic or chronic in human. Therefore patients may give a past history of similar events. Based on the duration and frequency of episodes, the disease is divided into chronic and episodic categories. Chronic disease is characterized by at least one cluster period lasting at least one year, with no remission or remission of less than one month. Patients with episodic cluster headaches get
at least two cluster periods of at least one week but less than one year, with remission for at least one month.
Risk factors Cluster headache is usually found in the adults who are in their 30s. The incidences are more common in males than in females. Alcohol, tobacco smoke and nitroglycerin are among the most common triggers. Weather changes, high altitudes, heat, tiredness, smells and bright light can trigger headache too. Risk factors
Cluster headache is usually found in the adults who are in their 30s. The incidences are more common in males than in females. Alcohol, tobacco smoke and nitroglycerin are among the most common triggers. Weather changes, high altitudes, heat, tiredness, smells and bright light can trigger headache too.
Complications It has been found that attempts and ideas of suicides are more commoner among the patients with cluster headache than in the general population. Agitation during the acute episode can result in serious traumas. Complications
It has been found that attempts and ideas of suicides are more commoner among the patients with cluster headache than in the general population. Agitation during the acute episode can result in serious traumas.

Clinicals - Examination

Fact Explanation
Restlessness Patient is usually in a severe pain. It is so excruciating that the/ she can't sit in one place. Patient may move around and scream in pain during an acute episode. Restlessness
Patient is usually in a severe pain. It is so excruciating that the/ she can't sit in one place. Patient may move around and scream in pain during an acute episode.
Eyelid edema Ipsilateral eye lid edema can be seen in many patients. This is a result of the associated autonomic dysfunction and increased bodily secretions. Eyelid edema
Ipsilateral eye lid edema can be seen in many patients. This is a result of the associated autonomic dysfunction and increased bodily secretions.
Miosis Excessive constriction of the pupil of the eye (miosis) is a common sign in the ipsilateral eye. Central inhibition of sympathetic nervous system caused the prominance of unopposed parasympathetic innervation via the trigeminal nerve. This results in contracting the iris sphincter muscle, producing miosis. Miosis
Excessive constriction of the pupil of the eye (miosis) is a common sign in the ipsilateral eye. Central inhibition of sympathetic nervous system caused the prominance of unopposed parasympathetic innervation via the trigeminal nerve. This results in contracting the iris sphincter muscle, producing miosis.
Ptosis Parasympathetic innervation causes in relaxation of levator and superior tarsal muscles. This along with the swelling of the upper eye lid in the ipsilateral eye result ptosis. Ptosis
Parasympathetic innervation causes in relaxation of levator and superior tarsal muscles. This along with the swelling of the upper eye lid in the ipsilateral eye result ptosis.
Conjunctival injection Trigeminal autonomic cephalgia or the activation of autonomic (parasympathetic) system results in the dilatation of the superficial conjunctival vessels causing conjunctival injection. Conjunctival injection
Trigeminal autonomic cephalgia or the activation of autonomic (parasympathetic) system results in the dilatation of the superficial conjunctival vessels causing conjunctival injection.
Facial perspiration Parasympathetic activation of the sweat glands in the ipsilateral face and forehead results in excessive sweating in that side. Facial perspiration
Parasympathetic activation of the sweat glands in the ipsilateral face and forehead results in excessive sweating in that side.
Bradycardia Unopposed parasympathetic activity on the heart causes heart rate below 60 beats per minute. Bradycardia
Unopposed parasympathetic activity on the heart causes heart rate below 60 beats per minute.

Investigations - Diagnosis

Fact Explanation
CT scan/ MRI scan Neuroimaging is not needed except those who have abnormal findings on examination or the nature of the headache is changing recently. They are useful to exclude secondary causes of headache such as intracranial hemorrhages and tumors. Otherwise the diagnosis of the cluster headache is mainly via the characteristic clinical presentation and findings . CT scan/ MRI scan
Neuroimaging is not needed except those who have abnormal findings on examination or the nature of the headache is changing recently. They are useful to exclude secondary causes of headache such as intracranial hemorrhages and tumors. Otherwise the diagnosis of the cluster headache is mainly via the characteristic clinical presentation and findings .

Investigations - Management

Fact Explanation
Full blood count This is done in the patients who are refractory to the medical management as a preparation to a surgical intervention. Patients who are anemic are not ideally fit for the surgery. Full blood count
This is done in the patients who are refractory to the medical management as a preparation to a surgical intervention. Patients who are anemic are not ideally fit for the surgery.
Fasting blood sugar Assessing glycemic control is very important before the surgical procedure. patients should ideally be in normoglycemic levels before the surgical procedure. Fasting blood sugar
Assessing glycemic control is very important before the surgical procedure. patients should ideally be in normoglycemic levels before the surgical procedure.
Blood urea and Serum electrolytes During the surgery when the patient is under the influence of anesthesia, patient's kidney function plays a key role in the fluid balance and blood pressure control. Assessing the electrolyte balance and blood urea nitrogen give a collective idea regarding patient's hemostasis. Blood urea and Serum electrolytes
During the surgery when the patient is under the influence of anesthesia, patient's kidney function plays a key role in the fluid balance and blood pressure control. Assessing the electrolyte balance and blood urea nitrogen give a collective idea regarding patient's hemostasis.

Management - Supportive

Fact Explanation
Patient education Patients should be educated regarding the disease. Identification and avoiding the triggering factor play a key role. Proper sleep, emotional control, not involve in excessive physical activities, avoidance of smoking and alcohol are important in prevention. Narcotics may expedite transformation of episodic Clusters to chronic Cluster headache. Psychological support should be provided for those in need. Patient education
Patients should be educated regarding the disease. Identification and avoiding the triggering factor play a key role. Proper sleep, emotional control, not involve in excessive physical activities, avoidance of smoking and alcohol are important in prevention. Narcotics may expedite transformation of episodic Clusters to chronic Cluster headache. Psychological support should be provided for those in need.
Prophylaxis Verapamil is the prophylactic drug of choice for episodic and chronic types of cluster headache.
Initial dose of 120 –240 mg in three divided doses is recommended. Dose can be increased up to 1200 mg per day in case of chronic cluster headache. Hypotension, bradycardia, atrioventricular block, dizziness, fatigue, nausea and constipation are the common side effects. In terminating a Cluster headache cycle, corticosteroids are extremely effective as well as in preventing immediate headache recurrence. Methysergide, lithuim, divalproex sodium, lithium carbonate, topirimate and baclofen are the other options in prophylactic treatment.
Prophylaxis
Verapamil is the prophylactic drug of choice for episodic and chronic types of cluster headache.
Initial dose of 120 –240 mg in three divided doses is recommended. Dose can be increased up to 1200 mg per day in case of chronic cluster headache. Hypotension, bradycardia, atrioventricular block, dizziness, fatigue, nausea and constipation are the common side effects. In terminating a Cluster headache cycle, corticosteroids are extremely effective as well as in preventing immediate headache recurrence. Methysergide, lithuim, divalproex sodium, lithium carbonate, topirimate and baclofen are the other options in prophylactic treatment.

Management - Specific

Fact Explanation
Approach for the pharmacological management Pharmacological therapy is decided based on the presentation of the symptoms. Acute presentation should be treated with drugs that basically give a symptomatic relief. This approach of the treatment is called as abortive therapy. Oxygen, 5HT agonists, Tryptans etc are used for this purpose. Prophylatic therapy should be used in patients who gets frequent episodes interfering their normal life style.
Calcium channel blockers such as Verapamil, lithium, Methysergide, divalproex sodium, lithium carbonate, topirimate and baclofen is used for this purpose.
Approach for the pharmacological management
Pharmacological therapy is decided based on the presentation of the symptoms. Acute presentation should be treated with drugs that basically give a symptomatic relief. This approach of the treatment is called as abortive therapy. Oxygen, 5HT agonists, Tryptans etc are used for this purpose. Prophylatic therapy should be used in patients who gets frequent episodes interfering their normal life style.
Calcium channel blockers such as Verapamil, lithium, Methysergide, divalproex sodium, lithium carbonate, topirimate and baclofen is used for this purpose.
Oxygen therapy Oxygen is the treatment of choice for sypmtomatic relief in a patient with acute episode of cluster headache. 7 L per minute for 15 minutes via face mask is the recommended regime. Oxygen is the best option for the pregnant patients. Oxygen therapy
Oxygen is the treatment of choice for sypmtomatic relief in a patient with acute episode of cluster headache. 7 L per minute for 15 minutes via face mask is the recommended regime. Oxygen is the best option for the pregnant patients.
Sumatriptan Administration of sumatriptan by subcutaneous injection in a dose of 6 mg is an effective means of aborting an individual cluster attack. Sumatriptan nasal spray is less effective than the subcutaneous formulation. It is contraindicated in patients with coronary artery disease, uncontrolled hypertension, angina and in pregnancy. Zolmitriptan, naratriptan, rizatriptan, almotriptan, frovatriptan, and eletriptan are the other triptans that can be considered as alternatives. Sumatriptan
Administration of sumatriptan by subcutaneous injection in a dose of 6 mg is an effective means of aborting an individual cluster attack. Sumatriptan nasal spray is less effective than the subcutaneous formulation. It is contraindicated in patients with coronary artery disease, uncontrolled hypertension, angina and in pregnancy. Zolmitriptan, naratriptan, rizatriptan, almotriptan, frovatriptan, and eletriptan are the other triptans that can be considered as alternatives.
Intravenous dihydroergotamine (DHE) Intravenous dihydroergotamine provide instant relief during an acute attack. It is also available in intranasal and injectable forms. These forms provide slower relief. Intravenous dihydroergotamine (DHE)
Intravenous dihydroergotamine provide instant relief during an acute attack. It is also available in intranasal and injectable forms. These forms provide slower relief.
Topical 4% lidocaine 4% intranasal lidocaine gives a symptomatic relief to the pain. 1 mL of 10% lidocaine is also effective. It should be placed by a cotton swab bilaterally for 5 minutes. Topical 4% lidocaine
4% intranasal lidocaine gives a symptomatic relief to the pain. 1 mL of 10% lidocaine is also effective. It should be placed by a cotton swab bilaterally for 5 minutes.
Intranasal capsaicin Capsaicin applied to the nasal mucosa has shown to significantly decrease the number and the severity of cluster headaches. Sensation of burning of the nasal cavity is the commonest side effect that usually decreases after five applications. Intranasal capsaicin
Capsaicin applied to the nasal mucosa has shown to significantly decrease the number and the severity of cluster headaches. Sensation of burning of the nasal cavity is the commonest side effect that usually decreases after five applications.
Percutaneous radiofrequency retrogasserian rhizotomy Patients with total resistance to medical treatment are permitted for surgical interventions. The current mainstay of surgical intervention for these patients is percutaneous radiofrequency retrogasserian rhizotomy (PRFR). It gives good to excellent results in majority of the patients. Side effects include anesthesia dolorosa, facial dysesthesia and corneal sensory loss. Percutaneous radiofrequency retrogasserian rhizotomy
Patients with total resistance to medical treatment are permitted for surgical interventions. The current mainstay of surgical intervention for these patients is percutaneous radiofrequency retrogasserian rhizotomy (PRFR). It gives good to excellent results in majority of the patients. Side effects include anesthesia dolorosa, facial dysesthesia and corneal sensory loss.
Gamma knife radiosurgery Gamma knife radiosurgery to lesion the trigeminal nerve root may be an effective treatment. It is less invasive though facial sensory disturbances are common afterwards. Gamma knife radiosurgery
Gamma knife radiosurgery to lesion the trigeminal nerve root may be an effective treatment. It is less invasive though facial sensory disturbances are common afterwards.

Concise, fact-based medical articles to refresh your knowledge

Access a wealth of content and skim through a smartly presented catalog of diseases and conditions.

  1. Cluster headache. Am Fam Physician [online] 2013 Jul 15, 88(2):Online [viewed 11 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/23939651
  2. EDVARDSSON B. Symptomatic cluster headache: a review of 63 cases. Springerplus [online] 2014:64 [viewed 11 September 2014] Available from: doi:10.1186/2193-1801-3-64
  3. GAUL C, DIENER HC, MüLLER OM. Cluster headache: clinical features and therapeutic options. Dtsch Arztebl Int [online] 2011 Aug, 108(33):543-9 [viewed 11 September 2014] Available from: doi:10.3238/arztebl.2011.0543
  4. LEROUX E, DUCROS A. Cluster headache. Orphanet J Rare Dis [online] 2008 Jul 23:20 [viewed 11 September 2014] Available from: doi:10.1186/1750-1172-3-20
  5. MATHARU M. Cluster headache. Clin Evid (Online) [online] 2010 Feb 9 [viewed 11 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/21718584
  6. TEIXEIRA MANOEL J., SIQUEIRA SILVIA R.D.T., ALMEIDA GILBERTO M.. Percutaneous radiofrequency rhizotomy and neurovascular decompression of the trigeminal nerve for the treatment of facial pain. Arq. Neuro-Psiquiatr. [online] 2006 December, 64(4):983-989 [viewed 11 September 2014] Available from: doi:10.1590/S0004-282X2006000600018
  7. The International Classification of Headache Disorders, 3rd edition (beta version). Cephalalgia [online] December, 33(9):629-808 [viewed 09 September 2014] Available from: doi:10.1177/0333102413485658 2)
  8. WEAVER-AGOSTONI J. Cluster headache. Am Fam Physician [online] 2013 Jul 15, 88(2):122-8 [viewed 11 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/23939643