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. [1,2,3,4,5,6]
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. [2,3,5]
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. [1,2,3,5]
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. [2,3,5,6]
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. [1,2,3,4]
Facial sweating This is also unilateral and is owing to the unopposed parasympathetic activation of the sweat glands. [1,3,5]
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. [1,3,7]
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. [1,2,3,5]
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. [1,2,3,6]
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. [2,4,5,6,7]
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. [2,3,5,6]
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. [2,3,4]
References
  1. 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
  2. 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)
  3. 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
  4. 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
  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. 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
  7. 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

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. [1,2,3,4]
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. [2,3,5,6]
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. [2,3,6,7]
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. [1,2,3]
Conjunctival injection Trigeminal autonomic cephalgia or the activation of autonomic (parasympathetic) system results in the dilatation of the superficial conjunctival vessels causing conjunctival injection. [1,3,4,5]
Facial perspiration Parasympathetic activation of the sweat glands in the ipsilateral face and forehead results in excessive sweating in that side. [1,2,3,6]
Bradycardia Unopposed parasympathetic activity on the heart causes heart rate below 60 beats per minute. [4,6,7]
References
  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. 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)
  3. 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
  4. 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
  5. 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
  6. 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
  7. 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

Differential Diagnoses

Fact Explanation
Migraine Migraine is a chronic neurological disorder characterized by recurrent moderate to severe headache. This is usually associated with several other symptoms such as nausea, vomiting and sensitivity to light, sound, or smell. There are several other symptoms which are common to both migraine and sinusitis namely facial pain, sinus pressure, nasal congestion, rhinorrhoea and teary eyes. [1]
Tolosa-Hunt Syndrome Tolosa-Hunt syndrome is a rare disorder caused by a nonspecific inflammation of the superior orbital fissure or cavernous sinus. It is characterized by severe periorbital headaches, along with ophthalmoplegia. These are accompanied by mostly unilateral diplopia, visual loss and paresthesias along the forehead. [2]
Trigeminal neuralgia Trigeminal neuralgia is a chronic condition that affects the trigeminal nerve characterized by unilateral facial pain following the sensory distribution of the maxillary and mandibular areas of the nerve. There are many triggers such as chewing, talking, smiling, drinking cold or hot fluids etc. [3]
Sinusitis Sinusitis is an infectious or non-infectious inflammation of one or more para nasal sinuses. The infection may be bacterial, viral or fungal. Non-infectious sinusitis is allergic in origin. Acute sinusitis is defined as the infection lasts less than 4 weeks and symptoms resolves completely with treatment. In sub acute cases, the infection lasts 4 - 12 weeks whereas in chronic sinusitis it lasts more than 12 weeks with or without treatment. The majority of cases follow a viral upper respiratiory tract infection which involves the whole upper respiratory epithelium including the para nasal sinuses. Such infections cause hyperaemia and edema of the mucosa which block the ostia. There will be a cellular infiltration and an increase in mucous production which blocks the nasal passage. Excessive mucous production causes initial watery nasal discharge. Stasis of secretion occurs due to paralyzed cilia. This leads to secondary bacterial infection which makes the nasal discharge purulent. [4]
Intracranial hemorrhage Intracranial hemorrhage (ICH) is a hemorrhage, or bleeding, within the skull. Intracranial bleeding occurs as a result of a physical trauma in head injury or nontraumatic causes (as occurs in hemorrhagic stroke) such as a ruptured aneurysm. It presents with altered level of consciousness, nausea, vomiting and headache. Sometimes they may also have seizures and focal neurological signs. [5]
Subarachnoid hemorrhage Blood spilling in the subarachnoid space located in between the pial and arachnoid membranes secondary to traumatic or non-traumatic events such as aneurysms and arterial venous malformations cause Subarachnoid hemorrhage. Patients usually present with headache, dizziness, orbital pain, diplopia and sometimes visual loss. [6]
Pitutary tumors Pitiutary tumor is a rare condition. Most pituitary tumors are benign an d asymptomatic. Some of these tumors can present with facial flushing, muscle weakness, hypertention, irregular heart beat, headache, visual disturbances and acromegaly. [7]
References
  1. AL-HASHEL JY, AHMED SF, ALROUGHANI R, GOADSBY PJ. Migraine misdiagnosis as a sinusitis, a delay that can last for many years. J Headache Pain [online] 2013 Dec 12:97 [viewed 26 May 2014] Available from: doi:10.1186/1129-2377-14-97
  2. BERALDIN BS, FELIPPU A, MARTINELLI F, PATRICIO HC. Tolosa-Hunt syndrome mimicking cavernous sinus tumor. Braz J Otorhinolaryngol [online] 2013 Mar-Apr, 79(2):256 [viewed 11 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/23670334
  3. CONFORTI R, PARLATO RS, DE PAULIS D, CIRILLO M, MARRONE V, CIRILLO S, MORACI A, PARLATO C. Trigeminal neuralgia and persistent trigeminal artery. Neurol Sci [online] 2012 Dec, 33(6):1455-8 [viewed 11 September 2014] Available from: doi:10.1007/s10072-012-0942-z
  4. DESROSIERS M, EVANS GA, KEITH PK, WRIGHT ED, KAPLAN A, BOUCHARD J, CIAVARELLA A, DOYLE PW, JAVER AR, LEITH ES, MUKHERJI A, SCHELLENBERG RR, SMALL P, WITTERICK IJ. Canadian clinical practice guidelines for acute and chronic rhinosinusitis Allergy Asthma Clin Immunol [online] , 7(1):2 [viewed 27 May 2014] Available from: doi:10.1186/1710-1492-7-2
  5. CACERES JA, GOLDSTEIN JN. Intracranial hemorrhage. Emerg Med Clin North Am [online] 2012 Aug, 30(3):771-94 [viewed 12 September 2014] Available from: doi:10.1016/j.emc.2012.06.003
  6. CIUREA AV, PALADE C, VOINESCU D, NICA DA. Subarachnoid hemorrhage and cerebral vasospasm - literature review. J Med Life [online] 2013 Jun 15, 6(2):120-5 [viewed 12 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/23904869
  7. SANTOS AB, FRANçA MM, HIROSAWA RM, MARIVO M, ZANINI MA, NUNES VS. Conservative management of pituitary tumor apoplexy. Arq Bras Endocrinol Metabol [online] 2011 Jun, 55(5):345-8 [viewed 12 September 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/21881818

Investigations - for 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 . [1,2]
References
  1. 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
  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

Investigations - Fitness for 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. [1,2]
Fasting blood sugar Assessing glycemic control is very important before the surgical procedure. patients should ideally be in normoglycemic levels before the surgical procedure. [1,2]
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. [1,2]
References
  1. 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)
  2. 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

Management - General Measures

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. [1,2,3,4]
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. [1,2,3]
References
  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. 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
  3. 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)
  4. 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

Management - Specific Treatments

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. [1,2,3]
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. [1,2,3,4]
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. [2,3,5,6]
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. [2,4,6]
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. [1,3,4]
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. [2,3,5,6]
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.[1,2,6,7]
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. [1,2,7]
References
  1. 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)
  2. 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
  3. 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
  4. 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
  5. 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
  6. 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
  7. 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