Overview
An acute onset of severe neurovascular headache characterized by frequent episodes of severe unilateral pain around the eye, with oxygen and medication as the main treatment, occurring in men, with the majority of cases occurring in the 20-40 year olds.
What is cluster headache?
Definition
Cluster headache is a primary neurovascular headache with a fixed location and periodicity of attacks.
The headache is usually fixed in and around one eye socket and occurs almost at the same time every day, often at night.
The headaches occur frequently and in bunches over a short period of time, hence the name cluster headache, and the period of attack is called the cluster phase.
Classification
According to the duration of clinical symptoms, they can be categorized into the following 2 types.
Episodic cluster headache
Cluster headache attacks last for 7 to 30 days and recur within 1 year.
Headache remission lasts for at least 1 month.
Chronic cluster headache
Cluster headache without periods of remission for at least 1 year, or with periods of remission of less than 1 month.
Morbidity
About 48 people per 100,000 people in China suffer from this disease.
The age of onset is 20-40 years old.
There are more males than females, and the number of patients is about 6 times that of females.
Questions you may be concerned about
Are cluster headaches and migraines the same thing?
Cluster headaches and migraines have their own characteristics in terms of onset and symptoms.
Migraines are far more common than cluster headaches and are more common in women, while cluster headaches are more common in men.
Migraine pain is usually much less severe than cluster headache, but lasts for more than 4 hours without a distinct rhythm.
Cluster headache pain is intense, usually lasting no more than 3 hours, with annual and daily rhythms, and remissions usually lasting months to years.
How to stop a cluster headache attack quickly?
The preferred pain management for cluster headache attacks, which are very painful, includes oxygen and medication.
Medications: Oral medications are slow to work, so subcutaneous injections of sumatriptan, sumatriptan, and zolmitriptan nasal spray are commonly used. The medications are more effective but can only be given up to 2 times in a 24 hour period.
Oxygen therapy: Inhalation of high-flow pure oxygen using a mask for 15 to 20 minutes, usually effective within 5 minutes, with significant efficacy within 30 minutes, is effective in most patients and is particularly suitable for patients with contraindications to medication or frequent attacks on the same day.
Can cluster headache be operated?
Nerve block, nerve stimulation and surgery can be performed for cluster headaches that are not well treated with medication, but the efficacy is not yet certain.
Nerve block treatment: common occipital nerve closure treatment, injection of prednisone and lidocaine into the occipital nerve on the same side of the headache, can relieve the headache symptoms, but it is easy to recur.
Nerve stimulation treatment: such as occipital nerve stimulation, pterygopalatine ganglion stimulation, deep brain stimulation.
Surgical treatment: such as percutaneous radiofrequency trigeminal nerve rhizotomy and other surgical treatments.
Causes
Causes
The cause of the disease is still unclear and may be related to the following factors.
Genetic factors play a role in the development of cluster headache, about 5% of patients may be autosomal dominant.
Activation of gray matter areas in the posterior thalamus may also be associated with the development of cluster headaches.
There are other etiologic hypotheses, such as the neurovascular origin hypothesis and the histamine accumulation hypothesis, but all of these hypotheses cannot independently explain the etiology of cluster headache.
Predisposing factors
The following factors can trigger the above etiology and cause an attack or exacerbation of the disease.
Alcohol consumption.
Application of vasodilators such as nitroglycerin.
Seasonal changes, with attacks often occurring in the spring and fall of the year.
Mental stress, tension, etc.
Strong odor stimulation, such as paint odor, tobacco odor, perfume odor, etc.
Symptoms
Main Symptoms
The main manifestation is repeated intensive episodes of unilateral severe headache, and the pain is characterized as follows.
The headache often occurs suddenly without aura.
The pain is overwhelmingly located in one orbital, supraorbital, and/or temporal region and can radiate to other areas of the head.
The headache is a sharp, explosive, nonpulsatile, severe pain. During the most severe episodes, the level of headache can be so excruciating that the patient is often unable to lie down.
The headache occurs at almost the same time every day, often at night, and lasts 15 to 180 minutes, with the frequency of attacks ranging from one every other day to eight per day. Most patients experience one to two attacks per day.
Other symptoms
The patient’s headache is often accompanied by autonomic symptoms in the ipsilateral facial area, and the following symptoms are common.
Conjunctival congestion (redness of the “whites” of the eyes), involuntary tearing, and runny eyes.
Sweating and redness of the forehead and face.
A feeling of fullness in the ears and edema of the eyelids.
Ptosis (drooping of the upper eyelid), with the upper lid moving down when the eyes are naturally open and looking forward, narrowing of the fissure of the lid, and partial or complete inability to lift the upper lid.
Irritability or restlessness.
Seek medical attention
Department of Medicine
Neurology
Prompt medical attention is recommended in case of recurrent intensive episodes of severe unilateral headache with or without conjunctival congestion, involuntary tearing and runny eyes, sweating and redness of the forehead and face, and ptosis.
Preparation for medical treatment
Preparation for the consultation: registration, preparation of documents, common problems
Tips
Avoid self-medicating with painkillers before going to the doctor, as this may aggravate the symptoms or cover up the condition.
If you usually keep a headache diary, you can give it to the doctor during the consultation for more reference.
When the headache is severe, it is recommended that your family members accompany you to the doctor, and avoid driving or riding to the doctor by yourself.
Preparation Checklist
Symptom list
Particular attention should be paid to the time of onset of symptoms, special manifestations, etc.
When did the headache first start? Which side of the headache?
How many times a day can a headache occur? When do they usually occur? How long does it last?
Is the level of headache tolerable?
Medical History Checklist
Does anyone in the family also suffer from cluster headaches?
Was there any alcohol consumption, stress, or smell of irritating odors prior to the onset of symptoms?
Checklist
Test results from the last six months that can be brought to the doctor’s office
CT head, magnetic resonance imaging (MRI) of the head
CT angiography, MRI angiography
Medication list
Medication in the last 3 months, if available in boxes or packages, may be brought to the doctor’s office
Vasodilators: nitroglycerin
Triptans: sumatriptan, zolmitriptan
Diagnosis
Diagnosis based on
Medical history
Family history of cluster headaches.
There is alcohol consumption, stress, and smell of irritating odors prior to the attack.
History of vasodilator medication such as nitroglycerin.
Clinical manifestations
The main manifestations are repeated intensive episodes of unilateral severe headache with or without involuntary tearing and runny nose, sweating and redness of the forehead and face, and ptosis.
The headache usually lasts 15 to 180 minutes at a time, and the frequency of attacks ranges from one every other day to eight per day.
Ocular examination may reveal miosis, conjunctival congestion, and ptosis.
Imaging
Cranial CT, MRI
Helps to understand the intracranial lesions.
Organic intracranial disorders that cause headaches can be ruled out.
CT angiography or magnetic resonance angiography
Can directly show vascular lesions in the brain.
Helps to screen for lesions such as aneurysms or arteriovenous malformations.
Diagnostic Criteria
Diagnostic criteria for cluster headache
More than 5 episodes of the following, which cannot be better explained by other diagnoses in the International Classification of Headache Disorders, 3rd edition.
Severe or very severe pain occurring unilaterally in the orbital, supraorbital and/or temporal regions. If untreated, the pain can last from 15 to 180 minutes.
Headache episodes meet at least 1 of the following 2 items
Accompanied by at least 1 of the following signs or symptoms (on the same side as the headache) Conjunctival congestion and/or tearing; Nasal congestion and/or runny nose; Eyelid edema; Sweating of forehead and face; Redness of forehead and face; Feeling of fullness in the ears; Reduced pupils and/or ptosis.
Restlessness or agitation.
Frequency of attacks from 1 every other day to 8 per day for more than half of the time during the cluster (attack) period.
Diagnostic Criteria for Episodic Cluster Headache
Episodes meet the diagnostic criteria for cluster headache and occur within the cluster period.
Duration of at least 2 cluster periods lasting from 7 days to 1 year (untreated) and headache remission for ≥1 month.
Diagnostic criteria for chronic cluster headache
Episodes meet the diagnostic criteria for cluster headache and the conditions listed below.
No periods of remission for at least 1 year or periods of remission <1 month.
Differential Diagnosis
Clinical features Cluster headache trigeminal neuralgia migraine paroxysmal migrainous headache
Headache site Fixed unilateral headache, preferably in the orbital, supraorbital and/or temporal regions, which may radiate to other areas of the head Cheeks, upper and lower jaws, and tongue Fixed unilateral headache in the frontal-temporal region, preferably in the orbital, supraorbital and/or temporal regions of the eye
Headache site
Fixed unilateral headache, preferably in the orbital, supraorbital and/or temporal regions, which may radiate to other areas of the head
Cheeks, upper and lower jaw, and tongue
One or both frontal-temporal regions
Fixed unilateral headache, preferably in the orbital, supraorbital and/or temporal regions
Level of headache Sharp, explosive, non-dyskinetic headache Sharp electric shock, pins and needles, cutting or tearing pain Dyskinetic headache Non-dyskinetic severe headache
Headache Level
Sharp, explosive, non-throbbing headache
Sharp electric shock, pins and needles, cutting or lacerating pain
Throbbing headache
Non-throbbing headache
Episode duration 15 to 180 minutes lasting seconds or 1 to 2 minutes usually lasting 4 to 72 hours lasting 2 to 30 minutes
Attack Duration
15-180 minutes
A few seconds or 1 to 2 minutes
Usually lasts 4 to 72 hours
2 to 30 minutes
Seizure frequency 1 every other day to 8 times a day as the disease progresses, the number of seizures gradually increased, seizures prolonged seizure frequency varies, can be a few days, weeks, or months seizures once or more times a day several times or dozens of times
Frequency of attacks
1 every other day to 8 times a day
As the disease progresses, the number of attacks gradually increases and the duration of attacks is prolonged.
The frequency of attacks varies, and may occur once or more in a few days, weeks, or months.
Several times a day or tens of times a day
Accompanying symptoms: conjunctival congestion, involuntary tears, runny nose, forehead and facial sweating, reflexive twitching of facial muscles such as ptosis, poor facial and oral hygiene, emaciation, depression, nausea, vomiting, blurred vision, numbness of limbs, and other aura symptoms similar to cluster headache in some patients.
Accompanying symptoms
Conjunctival congestion, involuntary tears, runny nose, forehead and facial sweating, ptosis, etc.
Reflex twitching of facial muscles, poor facial and oral hygiene, emaciation, and depression
Nausea, vomiting, and in some patients, aura symptoms such as blurred vision and numbness of the limbs may precede the attack
Similar to cluster headache
Treatment
Oxygen therapy
Oxygen therapy is the treatment of choice for cluster headache attacks and is effective in relieving headache attacks.
Generally, pure oxygen inhalation is given for 10-20 minutes. The specific oxygen flow rate, frequency and duration of oxygen inhalation should be strictly followed by the doctor’s instructions, and should not be adjusted or terminated on one’s own.
Medication
Acute phase drug treatment
Triptans
Help to relieve headache symptoms quickly.
Commonly used drugs include sumatriptan and zolmitriptan.
Contraindicated in patients with cardiovascular disease and hypertension.
Prophylactic medications
Since oxygen therapy or medication can only provide temporary relief in the acute phase, patients should be given prophylactic medications as soon as cluster headache is diagnosed to help shorten the duration of the attack and reduce the number of attacks.
Verapamil
Verapamil is effective in preventing cluster headache attacks and is most effective within 2 to 3 weeks of administration.
Common adverse effects include lower extremity edema, hypotension, and dizziness.
Glucocorticoids
Commonly used prophylactic drugs, but long-term use may occur serious adverse effects, so generally short-term use, followed by gradual reduction.
Commonly used drug is prednisone.
Long-term use of glucocorticosteroids should pay attention to infection, osteoporosis, hypokalemia and other adverse effects. During the period of taking glucocorticosteroids, the side effects of hormones can be reduced by replenishing calcium, potassium, gastric protection, smoking and alcohol cessation and other measures.
Lithium carbonate
It can prevent cluster headache attacks, with a slower onset of action than verapamil, and is suitable for those for whom other drugs are ineffective or contraindicated.
Common adverse effects include nausea, vomiting, loss of appetite, and dry mouth.
Since the commonly used therapeutic dose of lithium carbonate is close to the toxic dose, blood lithium concentration should be tested regularly to prevent toxicity.
Other drugs
Topiramate, sodium valproate, phenothiazine, and indomethacin may also be used in the prevention of cluster headaches.
Other treatments
If the efficacy of all the drug treatments is unsatisfactory, the treatment of occipital nerve closure by injecting prednisone and lidocaine at the occipital nerve on the same side of the headache may be considered, which can relieve the symptoms of the headache, but is prone to recurrence.
Neurophysiological treatment, such as occipital nerve stimulation, deep brain stimulation.
If all treatment modalities are ineffective, surgical treatment such as percutaneous radiofrequency trigeminal rhizotomy may be carefully considered.
Prognosis
Cure
Although the incidence of cluster headache is lower than that of other primary headaches such as migraine and tension headache, it has a greater impact on the patient’s quality of life, and only provides short-term relief through timely treatment, and is prone to recurrence.
Harmfulness
Long-term frequent and repeated severe headaches can easily trigger anxiety, depression and other adverse emotions, which seriously affects the quality of life of patients.