The first category within the ICHD is Migraine. Migraines in general are considered to be a neurological syndrome. It is estimated that 11% (303 million) of the global population,[2][3][4] including 43 million Europeans[5] and 28 million Americans,[6] experience migraines.
Organization of migraine subtypes
The ICHD-3 beta classification includes 6 main subtypes of migraine (ICHD-1: 7 main subtypes, ICHD-2: 6 main subtypes), most of which are further subdivided. Overall ICHD-3 beta distinguishes 29 migraine subtypes. The following table outlines the main subtypes and their ICHD-1, -2, -3 beta and ICD-10 codes.[1][7][8][9]
Migraine without aura, classified as common migraine, represents a recurrent headache disorder characterized by moderate to severe throbbing pain, typically unilateral, and devoid of preceding visual or sensory disturbances known as auras. This neurological condition, affecting a significant portion of the population, manifests with a range of symptoms, including nausea, vomiting, and heightened sensitivity to light and sound.
The etiology of migraines without aura remains multifactorial, involving genetic predispositions, environmental triggers, and complex neurobiological mechanisms. While the exact pathophysiology is not fully elucidated, a cascade of events involving neurotransmitters, vascular changes, and cortical spreading depression is believed to contribute to the onset of migraine headaches.
Clinical management of migraines without aura encompasses a comprehensive approach, emphasizing both preventive strategies and acute treatments. Lifestyle modifications, such as maintaining a consistent sleep schedule, stress management, and hydration, play a pivotal role in reducing the frequency and intensity of episodes. Additionally, identifying and avoiding individual trigger factors forms an integral part of personalized management plans.
In cases of recurrent or severe migraines, healthcare providers may recommend preventive medications tailored to the patient's specific needs. These medications aim to modulate neurochemical imbalances and minimize the frequency of migraine attacks. Acute treatments, on the other hand, focus on providing relief during active episodes and may include analgesics, anti-nausea medications, and, in some cases, triptans.
Patient engagement in their care is paramount, often involving the maintenance of a headache diary to track triggers, symptom patterns, and treatment efficacy. Collaborative efforts between healthcare professionals and patients facilitate the development of individualized care plans, optimizing outcomes and enhancing the quality of life for those affected by migraines without aura.
If there is a suspicion of migraines without aura, seeking consultation with a healthcare professional is essential for accurate diagnosis, appropriate treatment initiation, and ongoing management. A tailored and holistic approach ensures the effective mitigation of symptoms and empowers individuals to navigate the challenges posed by this chronic neurological condition.[7]
Migraine with aura
This section needs to be updated. Please help update this article to reflect recent events or newly available information.(December 2017)
Aura precedes headache, occurring within 60 minutes
Headache phase
Must include at least three of the following: Unilateral location, Pulsating quality, Moderate to severe pain, Aggravation by routine physical activity.
Migraine with aura represents a distinct neurological disorder characterized by recurrent headaches preceded or accompanied by sensory disturbances, commonly referred to as auras. These transient symptoms, typically manifesting as visual abnormalities like flashing lights or zigzag lines, serve as distinctive precursors to the ensuing headache. Other sensory experiences, including tingling, numbness, difficulty speaking, or temporary alterations in hearing or smell, may also occur during the aura phase.
The pathophysiology of migraines with aura is multifaceted, involving a complex interplay of genetic predispositions, environmental influences, and intricate neurobiological mechanisms. While the precise etiology remains elusive, the condition significantly impacts affected individuals, necessitating a comprehensive and tailored approach to its management.
Clinical management strategies for migraines with aura encompass both preventive measures and acute treatments. Lifestyle modifications, such as adherence to regular sleep patterns, effective stress management, and identification of trigger factors, constitute integral components of a holistic therapeutic approach. Preventive medications may be prescribed for those experiencing recurrent or severe episodes, while acute treatments, often involving analgesics and specific migraine medications like triptans, aim to alleviate symptoms during active migraine episodes.
Collaboration between healthcare professionals and individuals affected by migraines with aura is imperative for the development of personalized treatment plans. Maintaining a meticulous migraine diary facilitates the tracking of symptom patterns, identification of triggers, and evaluation of intervention efficacy. Early recognition of prodromal symptoms further enables timely and targeted interventions, potentially mitigating the severity of ensuing headaches.
Migraine with brainstem aura
Migraine with brainstem aura (abbreviated MBA; aka basilar artery migraine, basilar migraine, basilar-type migraine) is a subtype of migraine with aura in which symptoms clearly originate from the brainstem, but no motor weakness. When motor symptoms are present, the subtype is coded as 1.2.3 Hemiplegic migraine. Originally the terms basilar artery migraine or basilar migraine were used but, since involvement of the basilar artery is unlikely, the term migraine with brainstem aura is preferred in ICHD-3 beta. There are typical aura symptoms in addition to the brainstem symptoms during most attacks. Many patients who have attacks with brainstem aura also report other attacks with typical aura and should be coded for both 1.2.1 Migraine with typical aura and 1.2.2 Migraine with brainstem aura. Many of the symptoms like dysarthria, vertigo, tinnitus, hypacusis, diplopia, ataxia and decreased level of consciousness may occur with anxiety and hyperventilation, and therefore are subject to misinterpretation. Serious episodes of migraine with brainstem aura can lead to stroke, coma, and death. Using triptans and other vasoconstrictors as abortive treatments for migraine with brainstem aura is contraindicated. Abortive treatments for migraine with brainstem aura address vasodilation and restoration of normal blood flow to the vertebrobasilar territory to restore normal brainstem function.
Familial hemiplegic migraine (FHM) is migraine with a possible polygenetic cause—in fact, FHM can only be diagnosed when at least one close relative has it too.[7] The patient experiences typical migraine with aura headache either preceded or accompanied with one-sided, reversible limb weakness and/or sensory difficulties and/or speech difficulties. FHM is associated with ion channel mutations.
When no close family show symptoms, it is known as sporadic hemiplegic migraine.
Effecting a differential diagnosis between basilar migraine and hemiplegic migraine is difficult. Often, the decisive symptom is either motor weakness or unilateral paralysis, which occur in FHM and SHM. Basilar migraine can present tingling and numbness, but true motor weakness and paralysis occur only in hemiplegic migraine.
Retinal migraines are a kind of optical migraine. Those affected will experience a scotoma—a patch of vision loss in one eye surrounded by normal vision—for less than one hour before vision returns to normal. Retinal migraines may be accompanied by a throbbing unilateral headache, nausea, or photophobia.
Abdominal migraine
Abdominal migraine is a recurrent disorder of unknown origin, principally affecting children. Sometimes early on, it can be misdiagnosed in an ER setting as appendicitis. Episodes feature nausea, vomiting, and moderate-to-severe central, abdominal pain. The child is well between episodes. The International Classification of Headache Disorders definition is:
Diagnostic criteria:
A. At least 5 attacks fulfilling criteria B-D.
B. Attacks of abdominal pain lasting 1-72 hours (untreated or unsuccessfully treated)
C. Abdominal pain has all of the following characteristics:
1. midline location, periumbilical or poorly localized
2. dull or "just sore" quality
3. moderate or severe intensity
D. During abdominal pain at least 2 of the following:
1. anorexia
2. nausea
3. vomiting
4. pallor
E. Not attributed to another disorder
—International Classification of Headache Disorders[7]
Most children with abdominal migraines will develop migraine headache in adult life; the two propensities might co-exist during the child's adolescence.
Treating an abdominal migraine can often be difficult;[10] medications used to treat other forms of migraines are usually employed.[11] These include Elavil,[12] Wellbutrin SR,[13] and Topamax.[14]
In some cases, the abdominal migraine is a symptom linked to cyclic vomiting syndrome.[15] There may be a history of migraines in the family of the patient.[16]
A. Attacks, in a menstruating woman, fulfilling the criteria for migraine without aura B. Attacks that occur exclusively from days -2 to +3 of menstruation in at least two out of three menstrual cycles and at no other times of the cycle.
Note: The first day of menstruation is day +1, and the preceding day is day -1; there is no day 0.
It is well documented that migraine occurs nearly three times as often in women than in men,[6][7] and is one of the top five most common disabling conditions in women.[2][17] In over half these women, their headaches are strictly related to their menstrual cycle.[7]
A clinicalepidemiological study suggests that 60% of women with migraine without aura have attacks almost only while menstruating. One in ten had their migraines begin with their first period. Two-thirds do not get migraines while pregnant.[18]
This relationship was noted by the IHS in both versions of the ICHD, and particularly that this disorder fell under migraine without aura. The ICHD-1 referred to this as menstrual migraine, noting that there were no strict guidelines for this diagnosis, but that at least 90% of a woman's attacks should occur within two days of the beginning or end of menstruation. When the ICHD-2 was published, explicit guidelines for a diagnosis of two distinct types of menstruation-related migraine were released, and appear to the right. However, because the nature of the relationship is still unclear, and because the IHS was still uncertain as to whether these were a subset of migraine without aura or a distinct class of migraine, the criteria were delegated to an appendix, while anticipating that they would appear within the main text in the next revision.[7]
The ICHD-2 specifies two different forms of the previously dubbed "menstrual migraine": pure menstrual migraine without aura and menstrually-related migraine without aura. The sole difference between these diagnoses is the occurrence of headache attacks outside of the 5-day period described in the diagnostic criteria. If a woman experiences no attacks outside of this 5-day period, she may be diagnosed with pure menstrual migraine with aura; if she does experience other attacks, however, she may develop menstrually-related migraine without aura. This distinction is made solely for treatment purposes; a woman who only experiences migraines in that 5-day period is likely to benefit more from hormone therapy than a traditional migraine medication such as a triptan.[7]
One defining characteristic of these menstrual migraines is that the woman does not experience an aura. Clinical research has shown migraine with aura to be unrelated to the menstrual cycle, and, in women who have headaches sometimes with aura and sometimes without, the presence or absence of aura does not appear to be related to the menstrual cycle.[7]
As well as being split into two classes, menstrual migraines may have two different pathophysiologies, based on whether or not a woman is taking any oral contraceptives or another form of cyclicalhormone replacement therapy. When these medications are being used, the regular hormonal changes that take place and result in ovulation and other events in the menstrual cycle are suppressed, and menstruation is instead the result of withdrawal from abnormal progestogen concentrations.[7]
Menstrual migraines may also be linked to oestrogen withdrawal. Under the category of headache attributed to a substance or its withdrawal, the ICHD specifies the diagnostic criteria for oestrogen-withdrawal headache (8.4.3, G44.83 and Y42.4), and suggests that both that diagnosis and one of the menstrual migraine diagnoses be used in case of migraines related to oestrogen withdrawal occurring mainly at menstruation.[7]
Acephalgic migraine is a neurological syndrome. It is a variant of migraine in which the patient may experience aura symptoms such as scintillating scotoma, nausea, photophobia, hemiparesis and other migraine symptoms but does not experience headache. Acephalgic migraine is also referred to as amigrainous migraine, ocular migraine, ophthalmic migraine or optical migraine, the last three of which are misnomers.
People with acephalgic migraine are more likely than the general population to develop classical migraine with headache.
The prevention and treatment of acephalgic migraine is broadly the same as for classical migraine. However, because of the absence of "headache", diagnosis of acephalic migraine is apt to be significantly delayed and the risk of misdiagnosis significantly increased.
If symptoms are primarily visual, it may be necessary to consult an optometrist or ophthalmologist to rule out potential eye disease before considering this diagnosis.
Related Research Articles
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 nausea, 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, consisting of as little as several lifetime 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.
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.
Zolmitriptan, sold under the brand name Zomig among others, is a triptan used in the acute treatment of migraine attacks with or without aura and cluster headaches. It is a selective serotonin receptor agonist of the 1B and 1D subtypes.
Cyclic vomiting syndrome (CVS) is a chronic functional condition of unknown pathogenesis. CVS is characterized as recurring episodes lasting a single day to multiple weeks. Each episode is divided into four phases: inter-episodic, prodrome, vomiting, and recovery. Inter-episodic phase, is characterized as no discernible symptoms, normal everyday activities can occur, and this phase typically lasts one week to one month. The prodrome phase is known as the pre-emetic phase, characterized by the initial feeling of an approaching episode, still able to keep down oral medication. Emetic or vomiting phase is characterized as intense persistent nausea, and repeated vomiting typically lasting hours to days. Recovery phase is typically the phase where vomiting ceases, nausea diminishes or is absent, and appetite returns. "Cyclic vomiting syndrome (CVS) is a rare abnormality of the neuroendocrine system that affects 2% of children." This disorder is thought to be closely related to migraines and family history of migraines.
An aura is a perceptual disturbance experienced by some with epilepsy or migraine. An epileptic aura is a seizure.
Acephalgic migraine is a neurological syndrome. It is a relatively uncommon variant of migraine in which the patient may experience some migraine symptoms such as aura, nausea, photophobia, and hemiparesis, but does not experience headache. It is generally classified as an event fulfilling the conditions of migraine with aura with no headache. It is sometimes distinguished from visual-only migraine aura without headache, also called ocular migraine.
Almotriptan is a triptan medication discovered and developed by Almirall for the treatment of heavy migraine headache.
Eletriptan, sold under the brand name Relpax and used in the form of eletriptan hydrobromide, is a second-generation triptan medication intended for treatment of migraine headaches. It is used as an abortive medication, blocking a migraine attack which is already in progress. Eletriptan is marketed and manufactured by Pfizer Inc.
Familial hemiplegic migraine (FHM) is an autosomal dominant type of hemiplegic migraine that typically includes weakness of half the body which can last for hours, days, or weeks. It can be accompanied by other symptoms, such as ataxia, coma, and paralysis. Migraine attacks may be provoked by minor head trauma. Some cases of minor head trauma in patients with hemiplegic migraine can develop into delayed cerebral edema, a life-threatening medical emergency. Clinical overlap occurs in some FHM patients with episodic ataxia type 2 and spinocerebellar ataxia type 6, benign familial infantile epilepsy, and alternating hemiplegia of childhood.
Hemiplegic migraine is a type of migraine headache characterized by motor weakness affecting only one side of the body, accompanied by aura. There is often an impairment in vision, speech, or sensation. It can run in the family, called familial hemiplegic migraine, or in a single individual, called sporadic hemiplegic migraine. The symptoms can be similar to a stroke, and may be precipitated by minor head trauma. People with FHM are advised to avoid activities that may trigger their attacks.
Sporadic hemiplegic migraine (SHM) is a form of hemiplegic migraine headache isolated cases of which are observed. It is a rare disease. It is considered to be a separate type of migraine.
Antimigraine drugs are medications intended to reduce the effects or intensity of migraine headache. They include drugs for the treatment of acute migraine symptoms as well as drugs for the prevention of migraine attacks.
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.
The International Classification of Headache Disorders (ICHD) is a detailed hierarchical classification of all headache-related disorders published by the International Headache Society. It is considered the official classification of headaches by the World Health Organization, and, in 1992, was incorporated into the 10th edition of their International Classification of Diseases (ICD-10). Each class of headache contains explicit diagnostic criteria—meaning that the criteria include quantities rather than vague terms like several or usually—that are based on clinical and laboratory observations.
Vestibular migraine (VM) is vertigo with migraine, either as a symptom of migraine or as a related neurological disorder.
Abdominal aura, also known as visceral aura and epigastric aura, is a type of somatosensory aura that typically manifests as abdominal discomfort in the form of nausea, malaise, hunger, or pain. Abdominal aura is typically associated with epilepsy, especially temporal lobe epilepsy, and it can also be used in the context of migraine. The term is used to distinguish it from other types of somatosensory aura, notably visual disturbances and paraesthesia. The abdominal aura can be classified as a somatic hallucination. Pathophysiologically, the abdominal aura is associated with aberrant neuronal discharges in sensory cortical areas representing the abdominal viscera.
Abdominal migraine(AM) is a functional disorder that usually manifests in childhood and adolescence, without a clear pathologic mechanism or biochemical irregularity. Children frequently experience sporadic episodes of excruciating central abdominal pain accompanied by migrainous symptoms like nausea, vomiting, severe headaches, and general pallor. Abdominal migraine can be diagnosed based off clinical criteria and the exclusion of other disorders.
Migraine treatment may be either prophylactic (preventive) or abortive (rescue) or acute. Prevention is better than cure, so the ideal treatment goal is to prevent migraine attacks. Because migraine is an exceedingly complex condition, there are various preventive treatments which have their effect by disrupting different links in the chain of events that occur during a migraine attack. As rescue treatments also target and disrupt different processes occurring during migraine, these are summarized, with their relative merits and demerits.
Menstrual migraine is the term used to describe both true menstrual migraines and menstrually related migraines. About 7%–14% of women have migraines only at the time of menstruation. These are called true menstrual migraines. Most female migraineurs experience migraine attacks throughout the menstruation cycle with an increased number perimenstrually, these are referred to as menstrually related or menstrually triggered migraine.
↑ Stovner, J.; Hagen, K.; Jensen, R.; Katsarava, Z.; Lipton, R.; Scher, A.; Steiner, T.; Zwart, A. (Mar 2007). "The global burden of headache: a documentation of headache prevalence and disability worldwide". Cephalalgia: An International Journal of Headache. 27 (3): 193–210. doi:10.1111/j.1468-2982.2007.01288.x. ISSN0333-1024. PMID17381554. S2CID23927368.
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