Cluster headache overview
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Cluster headaches are rare, extremely painful and debilitating headaches that occur in groups or clusters. Often appearing during seasonal changes. They may also be described as suicide headaches, a reference to the excruciating pain and resulting desperation that has culminated in actual suicide.
Cluster headaches have been called by several other names in the past including Erythroprosopalgia of Bing, ciliary neuralgia, migrainous neuralgia, erythromelagia of the head, Horton's headache (named after Bayard T. Horton, an American neurologist who was the first to accurately describe the headache in 1939), histaminic cephalalgia, petrosal neuralgia, sphenopalatine neuralgia, Vidian neuralgia, Sluder's neuralgia, and hemicrania angioparalyticia. Sluder's neuralgia (syndrome) and cluster pain can often be temporarily stopped with nasal lidocaine spray. If successful, outpatient nasal septoplasty and splinting can resolve the condition.
Differentiating Cluster Headache from other Diseases
Epidemiology and Demographics
While migraines are diagnosed more often in women, cluster headaches are diagnosed more often in men. The male-to-female ratio in cluster headache ranges from 4:1 to 7:1. It primarily occurs between the ages of 20 to 50 years. This gap between the sexes has narrowed over the past few decades, and it is not clear whether cluster headaches are becoming more frequent in women, or whether they are merely being better diagnosed. Limited epidemiological studies have suggested prevalence rates of between 56 and 326 people per 100,000. Latitude plays a role in the occurrence of cluster headaches, which are more common as one moves away from the equator towards the poles. It is believed that greater changes in day length are responsible for the increase.
Patients with cluster headaches usually have an underlying secondary cause of headaches such as structural brain lesion, TBI and genetic predisposition that may increase the risk of cluster headache.
Natural History, Complications and Prognosis
Cluster headache is one of the most common trigeminal autonomic cephalalgias. It is a life long condition and usually results in reduced quality of life and marked functional disability. Although, the frequency of episodes usually decrease with age, 80% of patients continue to experience the attacks even after 15 years of onset.
Diagnostic criteria for cluster headache require the following as per ICHD-3: International Classification of Headache Disorders, 3rd edition.
History and Symptoms
Cluster headache sufferers typically experience very severe headaches of a piercing quality near one eye or temple that last for fifteen minutes to three hours with some lasting days (rarely more than three days). The headaches are typically unilateral and rarely change sides during the same cycle (see episodic). Cluster headaches have a characteristic circadian periodicity and usually present with autonomic symptoms.
Examination findings in cluster headache are usually autonomic related; suggesting parasympathetic hyperactivity and sympathetic impairment. Exam findings may include ptosis, lacrimation, conjuctival injection, miosis, nasal congestion, rhinorrhea, neck stiffness and photophobia.
Laboratory investigations, such as electrophysiologic testing (eg, evoked potential, electroencephalography) and examination of the cerebrospinal fluid, are not found to be helpful and needs more studies for further evaluation.
Neuro-imaging with a non-contrast computed tomography (CT) scan is usually indicated to rule out underlying structural brain lesion or to evaluate brain and pituitary gland in patient presenting with typical features or highly suspicious of cluster headache.
Neuro-imaging with an magnetic resonance imaging (MRI) scan with and without contrast is preferred to non-contrast computed tomography (CT) scan. MRI is usually indicated to rule out underlying structural brain lesion or to evaluate brain and pituitary gland in patient presenting with typical features or highly suspicious of cluster headache.
Cluster headache treatment is generally divided into acute therapy or abortive therapy focused at aborting individual attacks and preventive or prophylactic therapy aimed at preventing recurrent attacks during the cluster period. Cluster headaches often go undiagnosed for many years, being confused with migraine or other causes of headache. Because of the relative rareness of the condition and ambiguity of the symptoms, some sufferers may not receive treatment in the emergency room and patients may even be mistaken as exhibiting drug-seeking behavior. Over-the-counter pain medications (such as aspirin, paracetamol, and ibuprofen) typically have no effect on the pain from a cluster headache. Unlike other headaches such as migraines and tension headaches, cluster headaches do not respond to biofeedback. Some have reported partial relief from narcotic pain killers. Percocet (Oxycodone with paracetamol) has had widespread success amongst some cluster headache patients, especially males. Anecdotal evidence indicates that cluster headaches can be so excruciating that even morphine does little to ease the pain. However, some newer medications like fentanyl (and Percocet) have shown promise in early studies and use.
The drug of choice for primary prevention of cluster headache is Verapamil. Other agents that can be used also include glucocorticoids, topiramate and lithium.