| Hemicrania continua | |
|---|---|
| Specialty | Neurology  |
Hemicrania continua (HC) is a persistent unilateral headache that responds to indomethacin. It is usually unremitting, but rare cases of remission have been documented. [1] Hemicrania continua is considered a primary headache disorder, meaning that another condition does not cause it.
In hemicrania continua, basal pain is a dull aching pressure similar to that of TTHs (Tension-Type Headaches) that occurs nearly always on the same side of the head and face. Pain ranges from mild to severe and is characterized by fluctuations that increase in intensity up to three to five times per 24-hour cycle. The range of duration of exacerbations has no boundaries and varies from a few seconds to up to two weeks. While attacks tend to be more frequent at night, no circadian periodicity such as in cluster headache can be observed.
The nature of pain changes during the exacerbation phase, becoming more piercing, throbbing, and intense, generally paired with other highly debilitating symptoms such as nausea, vomiting, dizziness, and sensitivity to light and sounds. During these exacerbation phases, hemicrania continua may mimic other primary and secondary headache disorders, with up to 70% of patients fulfilling the diagnostic criteria for migraine. Physical exertion, changes in sleep patterns, stress, or alcohol consumption can make the headache pain more severe in some patients. [2]
In addition to the persistent daily headache of HC, which is usually mild to moderate (and frequently severe), HC can present other symptoms. [3] These additional symptoms of HC can be divided into three main categories:
The cause of hemicrania continua is unknown.[ citation needed ]
The following diagnostic criteria are given for hemicrania continua: [4]
A variant on hemicrania continua has also been described, in which the attacks may shift sides, although meeting the above criteria in all other respects. [6] [7] [8] [9]
There is no definitive diagnostic test for hemicrania continua. Diagnostic tests such as imaging studies may be ordered to rule out other causes for the headache. When the symptoms of hemicrania continua are present, it's considered "diagnostic" if they respond completely to indomethacin. The efficacy of indomethacin may not be long term for all patients, as can eventually become ineffective. [2]
The factor that allows hemicrania continua and its exacerbations to be differentiated from migraine and cluster headache is that hemicrania continua is characterized by complete response to therapeutic doses (25–300 mg) of indomethacin. The positive response to this drug is, in fact, a fundamental sine qua non criterion used in differential diagnosis. Triptans and other abortive medications do not affect hemicrania continua. [2]
The International Headache Society's International Classification of Headache Disorders classifies hemicrania continua as a primary headache disorder. [4]
Hemicrania continua generally responds only to indomethacin 25–300 mg daily, which must be continued long term. Unfortunately, gastrointestinal side effects are a common problem with indomethacin, which may require additional acid-suppression therapy to control. [10]
In patients who are unable to tolerate indomethacin, the use of celecoxib 400–800 mg per day (Celebrex) and rofecoxib 50 mg per day (Vioxx - no longer available) have both been shown to be effective and are likely to be associated with fewer GI side effects. [11] There have also been reports of two patients who were successfully managed with topiramate 100–200 mg per day (Topamax) although side effects with this treatment can also prove problematic. [9] [12]
Greater occipital nerve (GON) block comprising 40 mg Depomedrone and 10 mls of 1% Lignocaine injected into the affected nerve is effective, up to a period of approximately three months. Changing the 'cocktail' to include (for example) 10 mls of .5% Marcaine and changing to 2% Lignocaine, whilst in theory should increase the longevity, renders the injection completely ineffective. See 4.2 Posology and method of administration (flocculation). [13] [14]
Occipital nerve stimulation may be highly effective when other treatments fail to relieve the intractable pain. [15]
Hemicrania was mentioned in 1881 in The Therapeutic Gazette Vol. 2, by G.S.Davis, [16] and the incident has been cited in King's American Dispensatory (1898 and later editions) [17] in the description of the strong analgesic Jamaican Dogwood, a relatively low dose of which reportedly produced convulsions and prolonged respiratory depression over six hours in an elderly woman with this condition.[ citation needed ]
In newer times, Hemicrania continua was described in 1981; [18] at that time around 130 cases were described in the literature. [18] However, rising awareness of the condition has led to increasingly frequent diagnosis in headache clinics, and it seems that it is not as rare as these figures would imply. The condition occurs more often in women than men and tends to present first in adulthood, although it has also been reported in children as young as 5 years old. [19]
Migraine is a genetically influenced complex neurological disorder characterized by episodes of moderate-to-severe headache, most often unilateral and generally associated with nausea and light and sound sensitivity. Other characterizing symptoms may include vomiting, cognitive dysfunction, allodynia, and dizziness. Exacerbation of headache symptoms during physical activity is another distinguishing feature. Up to one-third of migraine sufferers experience aura, a premonitory period of sensory disturbance widely accepted to be caused by cortical spreading depression at the onset of a migraine attack. Although primarily considered to be a headache disorder, migraine is highly heterogenous in its clinical presentation and is better thought of as a spectrum disease rather than a distinct clinical entity. Disease burden can range from episodic discrete attacks to chronic disease.
Headache, also known as cephalalgia, is the symptom of pain in the face, head, or neck. It can occur as a migraine, tension-type headache, or cluster headache. There is an increased risk of depression in those with severe headaches.
Cluster headache is a neurological disorder characterized by recurrent severe headaches on one side of the head, typically around the eye(s). There is often accompanying eye watering, nasal congestion, or swelling around the eye on the affected side. These symptoms typically last 15 minutes to 3 hours. Attacks often occur in clusters which typically last for weeks or months and occasionally more than a year.
A medication overuse headache (MOH), also known as a rebound headache, usually occurs when painkillers are taken frequently to relieve headaches. These cases are often referred to as painkiller headaches. Rebound headaches frequently occur daily, can be very painful and are a common cause of chronic daily headache. They typically occur in patients with an underlying headache disorder such as migraine or tension-type headache that "transforms" over time from an episodic condition to chronic daily headache due to excessive intake of acute headache relief medications. MOH is a serious, disabling and well-characterized disorder, which represents a worldwide problem and is now considered the third-most prevalent type of headache. The proportion of patients in the population with Chronic Daily Headache (CDH) who overuse acute medications ranges from 18% to 33%. The prevalence of medication overuse headache (MOH) varies depending on the population studied and diagnostic criteria used. However, it is estimated that MOH affects approximately 1-2% of the general population, but its relative frequency is much higher in secondary and tertiary care.
A headache is often present in patients with epilepsy. If the headache occurs in the vicinity of a seizure, it is defined as peri-ictal headache, which can occur either before (pre-ictal) or after (post-ictal) the seizure, to which the term ictal refers. An ictal headache itself may or may not be an epileptic manifestation. In the first case it is defined as ictal epileptic headache or simply epileptic headache. It is a real painful seizure, that can remain isolated or be followed by other manifestations of the seizure. On the other hand, the ictal non-epileptic headache is a headache that occurs during a seizure but it is not due to an epileptic mechanism. When the headache does not occur in the vicinity of a seizure it is defined as inter-ictal headache. In this case it is a disorder autonomous from epilepsy, that is a comorbidity.
Triptans are a family of tryptamine-based drugs used as abortive medication in the treatment of migraines and cluster headaches. This drug class was first commercially introduced in the 1990s. While effective at treating individual headaches, they do not provide preventive treatment and are not considered a cure. They are not effective for the treatment of tension–type headache, except in persons who also experience migraines. Triptans do not relieve other kinds of pain.
Indometacin, also known as indomethacin, is a nonsteroidal anti-inflammatory drug (NSAID) commonly used as a prescription medication to reduce fever, pain, stiffness, and swelling from inflammation. It works by inhibiting the production of prostaglandins, endogenous signaling molecules known to cause these symptoms. It does this by inhibiting cyclooxygenase, an enzyme that catalyzes the production of prostaglandins.
Occipital neuralgia (ON) is a painful condition affecting the posterior head in the distributions of the greater occipital nerve (GON), lesser occipital nerve (LON), third occipital nerve (TON), or a combination of the three. It is paroxysmal, lasting from seconds to minutes, and often consists of lancinating pain that directly results from the pathology of one of these nerves. It is paramount that physicians understand the differential diagnosis for this condition and specific diagnostic criteria. There are multiple treatment modalities, several of which have well-established efficacy in treating this condition.
Chronic paroxysmal hemicrania (CPH) is a severe debilitating unilateral headache usually affecting the area around the eye. It normally consists of multiple severe, yet short, headache attacks affecting only one side of the cranium. Retrospective surveys indicated that paroxysmal hemicrania was more common in women. However, subsequent prospective research showed an equal prevalence between females and males, with a ratio close to 1:1. Unlike in migraine, it has no neurological symptoms associated with it. CPH headaches are treated through the use of non-steroidal anti-inflammatory drugs, with indomethacin found to be especially effective in eliminating symptoms.
Sexual headache is a type of headache that occurs in the skull and neck during sexual activity, including masturbation or orgasm. These headaches are usually benign, but occasionally are caused by intracranial hemorrhage and cerebral infarction, especially if the pain is sudden and severe. They may be caused by general exertion, sexual excitement, or contraction of the neck and facial muscles. Most cases can be successfully treated with medication.
Tolosa–Hunt syndrome is a rare disorder characterized by severe and unilateral headaches with orbital pain, along with weakness and paralysis (ophthalmoplegia) of certain eye muscles.
Migraine surgery is a surgical operation undertaken with the goal of reducing or preventing migraines. Migraine surgery most often refers to surgical nerve decompression of one or several nerves in the head and neck which have been shown to trigger migraine symptoms in many migraine sufferers. Following the development of nerve decompression techniques for the relief of migraine pain in the year 2000, these procedures have been extensively studied and shown to be effective in appropriate candidates. The nerves that are most often addressed in migraine surgery are found outside of the skull, in the face and neck, and include the supra-orbital and supra-trochlear nerves in the forehead, the zygomaticotemporal nerve and auriculotemporal nerves in the temple region, and the greater occipital, lesser occipital, and third occipital nerves in the back of the neck. Nerve impingement in the nasal cavity has additionally been shown to be a trigger of migraine symptoms.
New daily persistent headache (NDPH) is a primary headache syndrome which can mimic chronic migraine and chronic tension-type headache. The headache is daily and unremitting from very soon after onset, usually in a person who does not have a history of a primary headache disorder. The pain can be intermittent, but lasts more than 3 months. Headache onset is abrupt and people often remember the date, circumstance and, occasionally, the time of headache onset. One retrospective study stated that over 80% of patients could state the exact date their headache began.
Vestibular migraine (VM) is vertigo with migraine, either as a symptom of migraine or as a related neurological disorder.
Preventive treatment of migraine can be an important component of migraine management. The goals of preventive therapy are to reduce the frequency, painfulness, and/or duration of migraine attacks, and to increase the effectiveness of abortive therapy. Another reason to pursue prevention is to avoid medication overuse headache (MOH), otherwise known as rebound headache, which can arise from overuse of pain medications, and can result in chronic daily headache. Preventive treatments of migraine include medications, nutritional supplements, lifestyle alterations, and surgery. Prevention is recommended in those who have headaches more than two days a week, cannot tolerate the medications used to treat acute attacks, or those with severe attacks that are not easily controlled.
Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing is a rare headache disorder that belongs to the group of headaches called trigeminal autonomic cephalalgia (TACs). Symptoms include excruciating burning, stabbing, or electrical headaches mainly near the eye and typically these sensations are only on one side of the body. The headache attacks are typically accompanied by cranial autonomic signs that are unique to SUNCT. Each attack can last from five seconds to six minutes and may occur up to 200 times daily.
Occipital nerve stimulation (ONS), also called peripheral nerve stimulation (PNS) of the occipital nerves, is used to treat chronic migraine patients who have failed to respond to pharmaceutical treatments.
The Migraine Specific Quality of Life (MSQoL) is a patient-reported outcome measure which assesses the quality of life of migraineurs. It is a 25-item questionnaire which is filled out by the patient and is used to determine how the patient's life has been affected by their migraines.
Neck-tongue syndrome (NTS), which was first recorded in 1980, is a rare disorder characterized by neck pain with or without tingling and numbness of the tongue on the same side as the neck pain. Sharp lateral movement of the head triggers the pain, usually lasting from a few seconds to a few minutes. Headaches may occur with the onset of NTS. The typical age of onset is around adolescence and may occur as early as 8–15 years old. However, it is worth noting that clinical onset can occur earlier or later and NTS onset related to trauma can occur at any age, beginning after the incident.
Recurrent painful ophthalmoplegic neuropathy (RPON), previously known as ophthalmoplegic migraine (OM), is a rare neurological disorder that is characterized by repeated headache attacks and reversible ipsilateral paresis of one or more ocular cranial nerves (CN). Oculomotor nerve (CNIII) is by far the most common cranial nerve involves in RPON, while abducens nerve (CNVI) and trochlear nerve (CNIV) involvements are also reported. Globally, RPON was estimated to have an annual incidence rate of 0.7 per million as of 1990, no further epidemiological studies have been conducted. It occurs more often in children and females.
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