Cluster headache
Cluster headache is a neurological disorder consisting of excruciating pain on one side of the head, often around or behind the eye, general restlessness with neurological (autonomic) signs, such as lacrimation of the eye and ptosis (drooping eyelids).
The attacks occur in clusters (hence the name) and can follow both circadian and seasonal patterns.
It is sub-classified into episodic and chronic.
The condition is also known as Horton’s headache, histaminic cephalalgia and sphenopalatine neuralgia.
Cluster headaches are part of the trigeminal autonomic cephalgia (TACs) subgroup , and like migraines and tension headaches, cluster headaches are a primary headache.
Diagnosing cluster headaches can be challenging, often leading to delays in correct diagnosis and incorrect treatment.
Note!
Unbearable pain and/or neurological signs, should always be thoroughly investigated by a neurologist as they can be part of many different diseases, sometimes serious ones.
Especially if the symptoms and signs are new, call the ambulance or emergency department of your local hospital.
1
Migraine headaches are often unilateral, moderate to severe in intensity and described as pulsating.
Not everyone with migraine gets a headache.
2
Most people with migraines will have increased headaches with physical activity, and be sensitive to light (photophobic), feel unwell and nauseous during an attack.
3
Triggers are important, but not all triggers trigger every time.
If you’re tired and hungry, the threshold is lower and you’re more likely to develop a migraine attack from the same trigger.
4
Start preventive measures early, preferably already if you have more than 2 attacks per month, to prevent development into chronic migraine.
Distribution
Cluster headache affects more than 1 in 1000 people at some point in their lives. It is the most common trigeminal autonomic cephalgias (TACs).
Possible mechanisms
The mechanisms behind cluster headaches are not fully understood, but the trigeminovascular system, the parasympathetic system and the hypothalamus are thought to be involved. The parasympathetic system explains the signs of the eye, and the hypothalamus controls the circadian rhythm. As with migraine, CGRP (calcitonin gene-related peptide) also appears to play a role in cluster headaches.
Normal symptom description
The pain of cluster headaches is described as the worst pain imaginable, worse than giving birth. It is experienced as a sharp and/or pulsating pain, behind or around the eye, and/or around the temple region.
Other symptoms
In addition to head pain, an attack may also include changes in and around the eye. One or more signs of ptosis (drooping eyelids), miosis (pupil constriction), lacrimation (tearing) and conjunctival injection (red eyes), facial sweating, nasal congestion and eyelid edema on the same side of the pain are normal. Many people also experience a feeling of restlessness or agitation.
Duration
A cluster headache attack can last for 15-180, if left untreated.
Frequency
The attacks can occur in series, or bouts, for weeks or months, sometimes at the same time of year. The ICHD-3 criteria have set the frequency of attacks from 1 every other day, to 8 times per day.
Triggers
Alcohol consumption, sleep, relaxation, stress, heat, weather changes, high altitude, histamine release and strong odors can trigger headache attacks. Smoking does not trigger an attack, but smokers tend to be more troubled by the disease.
Exacerbating factors
When experiencing an attack, lying down is the most common aggravating factor, even if everything seems painful.
Mitigating factors
During an attack, there are not many mitigating factors. But standing up and moving around seems to be better than lying down.
Treatment
The most effective acute treatment during an attack is oxygen and triptans.
Oxygen significantly relieves symptoms within 30 minutes in 70% of patients, and subcutaneous sumatriptan injection makes 75% of patients pain-free within 20 minutes. Sphenopalatine ganglion stimulation has been added to the guidelines as an effective acute treatment.
According to the 2016 guidelines for cluster headache, the only preventive treatment with a level A recommendation is corticosteroid injected into the top of the neck (suboccipital).
Verapamil (calcium channel blocker) is a very common prophylactic medication for cluster headache, but is only given a level C recommendation in the same guideline. CGRP antibodies may also have a preventive role in cluster headaches, but do not yet have a recommendation in a guideline.
For other acute and preventive medical treatment, see the guidelinesor other recent articles from May et al (2018) or Wei and Goadsby (2021).
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