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Prodromal or premonitory symptoms occur in about 60% of those with migraines, with an onset that can range from two hours to two days before the start of pain or the aura. These symptoms may include a wide variety of phenomena, including altered mood, irritability, depression or euphoria, fatigue, craving for certain food(s), stiff muscles (especially in the neck), constipation or diarrhea, and sensitivity to smells or noise. This may occur in those with either migraine with aura or migraine without aura.
Migraines typically present with self-limited, recurrent severe headache associated with autonomic symptoms. About 15–30% of people with migraines experience migraines with an aura and those who have migraines with aura also frequently have migraines without aura. The severity of the pain, duration of the headache, and frequency of attacks are variable. A migraine lasting longer than 72 hours is termed status migrainosus. There are four possible phases to a migraine, although not all the phases are necessarily experienced:
- The prodrome, which occurs hours or days before the headache
- The aura, which immediately precedes the headache
- The pain phase, also known as headache phase
- The postdrome, the effects experienced following the end of a migraine attack
Acephalgic migraines can occur in individuals of any age. Some individuals, more commonly male, only experience acephalgic migraine, but frequently patients also experience migraine with headache. Generally, the condition is more than twice as likely to occur in females than males. Pediatric acephalgic migraines are listed along with other childhood periodic syndromes by W.A. Al-Twaijri and M.I. Shevell as "migraine equivalents" (although not listed as such in the "International Classification of Headache Disorders"), which can be good predictors of the future development of typical migraines. Individuals who experience acephalgic migraines in childhood are highly likely to develop typical migraines as they grow older. Among women, incidents of acephalgic migraine increase during perimenopause.
Scintillating scotoma is the most common symptom which usually happens concurrently with Expanding Fortification Spectra. Also frequently reported is monocular blindness. Acephalgic migraines typically do not persist more than a few hours and may last for as little as 15 seconds. On rare occasions, they may continue for up to two days.
Acephalgic migraines may resemble transient ischemic attacks or, when longer in duration, stroke. The concurrence of other symptoms such as photophobia and nausea can help in determining the proper diagnosis. Occasionally, patients with acephalgic migraine are misdiagnosed as suffering epilepsy with visual seizures, but the reverse misdiagnosis is more common.
The prevention and treatment of acephalgic migraine is broadly the same as for classical migraine, but the symptoms are usually less severe than those of classic migraine, so treatment is less likely to be required.
Cluster headaches may occasionally be referred to as "alarm clock headache" because of the regularity of their recurrence. CH attacks often awaken individuals from sleep. Both individual attacks and the cluster grouping can have a metronomic regularity; attacks typically striking at a precise time of day each morning or night. The recurrence of headache cluster grouping may occur more often around solstices, or seasonal changes, sometimes showing circannual periodicity. Conversely, attack frequency may be highly unpredictable, showing no periodicity at all. These observations have prompted researchers to speculate an involvement, or dysfunction of the hypothalamus. The Hypothalamus controls the body's "biological clock" and circadian rhythm.
In episodic cluster headache, attacks occur once or more daily, often at the same time each day for a period of several weeks, followed by a headache-free period lasting weeks, months, or years. Approximately 10–15% of cluster headaches are chronic, with multiple headaches occurring every day for years, sometimes without any remission.
In accordance with the International Headache Society (IHS) diagnostic criteria, cluster headaches occurring in two or more cluster periods, lasting from 7 to 365 days with a pain-free remission of one month or longer between the headache attacks, may be classified as episodic. If headache attacks occur for more than a year without pain-free remission of at least one month, the condition is classified as chronic.
Chronic CH both occurs and recurs without any remission periods between cycles; there may be variation in cycles, meaning the frequency and severity of attacks may change without predictability for a period of time. The frequency, severity and duration of headache attacks experienced by people during these cycles varies between individuals and does not demonstrate complete remission of the episodic form. The condition may change unpredictably, from chronic to episodic and from episodic to chronic.
The typical symptoms of cluster headache include grouped occurrence and recurrence (cluster) of headache attack, severe unilateral orbital, supraorbital and/or temporal pain. If left untreated, attack frequency may range from one attack every two days to eight attacks per day. Cluster headache attack is accompanied by at least one of the following autonomic symptoms: drooping eyelid, pupil constriction, redness of the conjunctiva, tearing, runny nose, and less commonly, facial blushing, swelling, or sweating, typically appearing on the same side of the head as the pain.
Restlessness (for example, pacing or rocking back and forth), photosensitivity, aversion to light (photophobia) or sensitivity to noise (phonophobia) may occur during a CH. Nausea is a rare symptom, although it has been reported. Secondary effects may include inability to organize thoughts and plans, physical exhaustion, confusion, agitation, aggressiveness, depression and anxiety.
People with CH may dread facing another headache and adjust their physical or social activities around a possible future occurrence. Likewise they may seek assistance to accomplish what would otherwise be normal tasks. They may hesitate to make plans because of the regularity, or conversely, the unpredictability of the pain schedule. These factors can lead to generalized anxiety disorders, panic disorder, serious depressive disorders, social withdrawal and isolation.
Old headaches are usually primary headaches and are not dangerous. They are most often caused by migraines or tension headaches. Migraines are often unilateral, pulsing headaches accompanied by nausea or vomiting. There may be an aura (visual symptoms, numbness or tingling) 30–60 minutes before the headache, warning the person of a headache. Migraines may also not have auras. Tension type headaches usually have bilateral "bandlike" pressure on both sides of the head usually without nausea or vomiting. However, some symptoms from both headache groups may overlap. It is important to distinguish between the two because the treatments are different.
The mnemonic 'POUND' helps distinguish between migraines and tension type headaches. POUND stands for Pulsatile quality, 4–72 hOurs in length, Unilateral location, Nausea or vomiting, Disabling intensity. One review article found that if 4–5 of the POUND characteristics are present, migraine is 24 times as likely a diagnosis than tension type headache (likelihood ratio 24). If 3 characteristics of POUND are present, migraine is 3 times more likely a diagnosis than tension type headache (likelihood ratio 3). If only 2 POUND characteristics are present, tension type headaches are 60% more likely (likelihood ratio 0.41). Another study found the following factors independently each increase the chance of migraine over tension type headache: nausea, photophobia, phonophobia, exacerbation by physical activity, unilateral, throbbing quality, chocolate as headache trigger, cheese as headache trigger.
Cluster headaches are relatively rare (1–3 in 10,000 people) and are more common in men than women. They present with sudden onset explosive pain around one eye and are accompanied by autonomic symptoms (tearing, runny nose and red eye).
Temporomandibular jaw pain (chronic pain in the jaw joint), and cervicogenic headache (headache caused by pain in muscles of the neck) are also possible diagnoses.
For chronic, unexplained headaches, keeping a headache diary can be useful for tracking symptoms and identifying triggers, such as association with menstrual cycle, exercise and food. While mobile electronic diaries for smartphones are becoming increasingly common, a recent review found most are developed with a lack of evidence base and scientific expertise.
Vertigo is a medically recognized term for the symptom of vestibular system disturbance. It may include a feeling of rotation or illusory sensations of motion or both. The general term dizziness is used by nonmedical people for those symptoms but often refers to a feeling of light-headedness, giddiness, drowsiness, or faintness, all of which must be differentiated from true vertigo, since the latter symptoms might have other causes.
Motion sickness occurs more frequently in migraine patients (30–50% more than in controls). Benign paroxysmal vertigo of childhood is an example of migraine-associated vertigo in which headache does not often occur. Basilar artery migraine (BAM) consists of two or more symptoms (vertigo, tinnitus, decreased hearing, ataxia, dysarthria, visual symptoms in both hemifields or both eyes, diplopia, bilateral paresthesias, paresis, decreased consciousness and/or loss of consciousness) followed by throbbing headache. Auditory symptoms are rare. However, a study showed a fluctuating low-tone sensorineural hearing loss in more than 50% of patients with BAM with a noticeable change in hearing just before the onset of a migraine headache. The attacks of vertigo are usually concurrent with the headache and the family history is usually positive. The diagnostician must rule out: TIAs, and paroxysmal vestibular disorder accompanied by headache.
There is also a familial vestibulopathy, familial benign recurrent vertigo (fBRV), where episodes of vertigo occur with or without migraine headache. Testing may show profound vestibular loss. The syndrome responds to acetazolamide. Familial hemiplegic migraine (FHM) has been linked to mutations in the calcium channel gene. (Ophoff et al. 1966 cf. Lempert et al.)
90% of all headaches are primary headaches. Primary headaches usually first start when people are between 20 and 40 years old . The most common types of primary headaches are migraines and tension-type headaches. They have different characteristics. Migraines typically present with pulsing head pain, nausea, photophobia (sensitivity to light) and phonophobia (sensitivity to sound). Tension-type headaches usually present with non-pulsing "bandlike" pressure on both sides of the head, not accompanied by other symptoms. Other very rare types of primary headaches include:
- cluster headaches: short episodes (15–180 minutes) of severe pain, usually around one eye, with autonomic symptoms (tearing, red eye, nasal congestion) which occur at the same time every day. Cluster headaches can be treated with triptans and prevented with prednisone, ergotamine or lithium.
- trigeminal neuralgia or occipital neuralgia: shooting face pain
- hemicrania continua: continuous unilateral pain with episodes of severe pain. Hemicrania continua can be relieved by the medication indomethacin.
- primary stabbing headache: recurrent episodes of stabbing "ice pick pain" or "jabs and jolts" for 1 second to several minutes without autonomic symptoms (tearing, red eye, nasal congestion). These headaches can be treated with indomethacin.
- primary cough headache: starts suddenly and lasts for several minutes after coughing, sneezing or straining (anything that may increase pressure in the head). Serious causes (see secondary headaches red flag section) must be ruled out before a diagnosis of "benign" primary cough headache can be made.
- primary exertional headache: throbbing, pulsatile pain which starts during or after exercising, lasting for 5 minutes to 24 hours. The mechanism behind these headaches is unclear, possibly due to straining causing veins in the head to dilate, causing pain. These headaches can be prevented by not exercising too strenuously and can be treated with medications such as indomethacin.
- primary sex headache: dull, bilateral headache that starts during sexual activity and becomes much worse during orgasm. These headaches are thought to be due to lower pressure in the head during sex. It is important to realize that headaches that begin during orgasm may be due to a subarachnoid hemorrhage, so serious causes must be ruled out first. These headaches are treated by advising the person to stop sex if they develop a headache. Medications such as propranolol and diltiazem can also be helpful.
- hypnic headache: moderate-severe headache that starts a few hours after falling asleep and lasts 15–30 minutes. The headache may recur several times during night. Hypnic headaches are usually in older women. They may be treated with lithium.
The headaches can vary greatly in their clinical presentation and duration.
Quality of the headache has been described as dull and/or pressure-like sensation, and throbbing and/or pulsating sensation. The pain is usually on both sides of the head (in 88–93% of people with NDPH), but may be unilateral, and may be localized to any head region. The pain can fluctuate in intensity and duration, is daily, and lasts more than 3 months.
There may be accompanying photophobia, phonophobia, lightheadedness or mild nausea. Co-morbidity with mood disorders has been reported in a subset of patients.
Cranial autonomic nervous symptoms occur with painful exacerbations in 21%, and cutaneous allodynia may be present in 26%.
In 2002, Li and Rozen conducted a study of 56 patients at the Jefferson Headache Center in Philadelphia and published the following results:
- 82% of patients were able to pinpoint the exact day their headache started.
- 30% of the patients, the onset of the headache occurred in correlation with an infection or flu-like illness.
- 38% of the patients had a prior personal history of headache.
- 29% of the patients had a family history of headache.
- 68% reported nausea.
- 66% reported photophobia.
- 61% reported phonophobia.
- 55% reported lightheadedness.
Imaging and laboratory testing were unremarkable except for an unusually high number of patients who tested positive for a past Epstein-Barr virus infection.
Benign paroxysmal positional vertigo - Migraine is commonly associated with BPPV, the most common vestibular disorder in patients presenting with dizziness. The two may be linked by genetic factors or by vascular damage to the labyrinth.
Ménière's disease - There is an increased prevalence of migraine in patients with Ménière's disease and migraine leads to a greater susceptibility of developing Ménière’s disease. But they can be distinguished. Ménière's disease may go on for days or even years, while migraines typically do not last longer than 24 hours.
Motion sickness is more prevalent in patients with migraine.
Psychiatric syndromes Dizziness and spinning vertigo are the second most common symptom of panic attacks, and they can also present as a symptom of major depression. Migraine is a risk factor for developing major depression and panic disorder and vice versa.
The ICHD classification and diagnosis of migraine distinguish 6 subtypes of hemiplegic migraine. FHM can be loosely divided into two categories: with and without cerebellar signs. Cerebellar signs refer to ataxia, sometimes episodic and other times progressive, that can accompany FHM1 mutations and is caused by degeneration of the cerebellum. These cerebellar signs result in a phenotypic overlap between FHM and both episodic ataxia and spinocerebellar ataxia. This is unsurprising as subtypes of these disorders (FHM1, EA2 and SCA6) are allelic, i.e., they result from mutations in the same gene. The other forms of FHM seem to be distinguishable only on the basis of their genetic cause.
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 (within 3 days at most), 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 cause of NDPH is unknown, and it may have more than one etiology. NDPH onset is commonly associated with an infection or flu-like illness, stressful life event, minor head trauma, and extra cranial surgery. Infection or flu-like illness and stressful life event are most often cited. The pathophysiology of NDPH is poorly understood.
The syndrome is difficult to treat and may persist for years. The age of onset ranges from 6 to greater than 70 years old, with a mean of 35 years. It is found to be more common in females in both the adult and pediatric populations. NDPH is rare. The Akershus study of chronic headache, a population based cross sectional study of 30,000 persons aged 30–44 years in Norway, found a one-year prevalence of 0.03 percent in the population.
In 1986, Vanast was the first author to describe the new daily-persistent headache (NDPH) as a benign form of chronic daily headache (CDH). The criteria for the diagnosis of NDPH were proposed in 1994 (the Silberstein–Lipton criteria) but not included in the International Classification of Headache Disorders (ICHD) until 2004.
Familial hemiplegic migraine is a form of hemiplegic migraine headache that runs in families.
An epileptic aura is the consequence of the activation of functional cortex by abnormal, unilateral, and brief neuronal discharge. In addition to being a warning sign to an upcoming seizure, the nature of an aura can give insight into the localization and lateralization of the seizure or migraine.
Not everyone experiences an aura with a seizure, but the most common auras include motor, somatosensory, visual, and auditory symptoms. The activation in the brain during an aura can spread through multiple regions continuously or discontinuously, on the same side or to both sides.
Auras are particularly common in focal seizures. If the motor cortex is involved in the over excitation of neurons, motor auras can result. Likewise, somatosensory auras (such as tingling, numbness, and pain) can result if in the somatosensory cortex. When the primary somatosensory cortex is activated, more discrete parts on the opposite side of the body and the secondary somatosensory areas result in symptoms ipsilateral to the seizure focus.
Visual auras can be simple or complex. Simple visual symptoms can include static, flashing, or moving lights/shapes/colors caused mostly by abnormal activity in the primary visual cortex. Complex visual auras can include people, scenes, and objects which results from stimulation of the temporo-occipital junction and is lateralized to one hemifield. Auditory auras can also be simple (ringing, buzzing) or complex (voices, music). Simple symptoms can occur from activation in the primary auditory cortex and complex symptoms from the temporo-occipital cortex at the location of the auditory association areas.
An aura is a perceptual disturbance experienced by some with migraines or seizures before either the headache or seizure begins. It often manifests as the perception of a strange light, an unpleasant smell, or confusing thoughts or experiences. Some people experience aura without a subsequent migraine or seizure (see silent migraine). Auras vary by individual experience; some people experience smells, lights, or hallucinations. Less known symptoms of the eye include disturbances, where the eyes roll in the back of the head caused by photosensitivity. A sufferer of this type of aura may experience tearfulness of the eyes and uncontrollable sensations of light followed by reduced symptoms after approximately 20 minutes; it is the rarest type of aura.
When occurring, auras allow people who have epilepsy time to prevent injury to themselves and/or others. The time between the appearance of the aura and the migraine lasts from a few seconds up to an hour. The aura can stay with a migraine sufferer for the duration of the migraine; depending on the type of aura, it can leave the person disoriented and confused. It is not uncommon for migraine sufferers to experience more than one type of aura during the migraine. Most people who have auras have the same type of aura every time.
Auras can also be confused with sudden onset of panic, panic attacks or anxiety attacks creating difficulties in diagnosis. The differential diagnosis of patients who experience symptoms of paresthesias, derealization, dizziness, chest pain, tremors, and palpitations can be quite challenging.
Retinal migraine is associated with transient monocular visual loss (scotoma) in one eye lasting less than one hour. During some episodes, the visual loss may occur with no headache and at other times throbbing headache on the same side of the head as the visual loss may occur, accompanied by severe light sensitivity and/or nausea. Visual loss tends to affect the entire monocular visual field of one eye, not both eyes. After each episode, normal vision returns.
It may be difficult to read and dangerous to drive a vehicle while retinal migraine symptoms are present.
Retinal migraine is a different disease than scintillating scotoma, which is a visual anomaly caused by spreading depression in the occipital cortex at the back of the brain, not in the eyes nor any component thereof. Unlike in retinal migraine, a scintillating scotoma involves repeated bouts of temporary diminished vision or blindness and affects vision from both eyes, upon which sufferers may see flashes of light, zigzagging patterns, blind spots, or shimmering spots or stars.
A vascular headache is an outdated term to describe certain types of headache which were thought to be related to blood vessel swelling and hyperemia as cause of pain.
There is no doubt that "some" headaches are caused by vascular effects. However, it is no longer a recognized term and not mentioned in the Headache classification of the International Headache society (IHS), although it is still used by some physicians and still mentioned in some medical classification systems. There are many types of vascular headaches. Other types of vascular headaches include headaches produced by fever, cluster headaches, and headaches from a rise in blood pressure (OSU Wexner Medical Center, 2012).
Headaches that were described as being vascular headaches include:
- Cluster headache
- Migraine
- Toxic headache
Many variations occur, but scintillating scotoma usually begins as a spot of flickering light near or in the center of the visual field, which prevents vision within the scotoma area. The affected area flickers but is not dark. It then gradually expands outward from the initial spot. Vision remains normal beyond the borders of the expanding scotoma(s), with objects melting into the scotoma area background similarly to the physiological blind spot, which means that objects may be seen better by not looking directly at them in the early stages when the spot is in or near the center. The scotoma area may expand to completely occupy one half of the visual area, or it may also be bilateral. It may occur as an isolated symptom without headache in acephalgic migraine.
As the scotoma area expands, some people perceive only a bright flickering area that obstructs normal vision, while others describe seeing various patterns. Some describe seeing one or more shimmering arcs of white or colored flashing lights. An arc of light may gradually enlarge, become more obvious, and may take the form of a definite zigzag pattern, sometimes called a fortification spectrum (i.e. teichopsia, from Greek τεῖχος, town wall), because of its resemblance to the fortifications of a castle or fort seen from above. It also can resemble the dazzle camouflage patterns used on ships in World War I. Others describe patterns within the arc as resembling Widmanstätten patterns.
The visual anomaly results from abnormal functioning of portions of the occipital cortex at the back of the brain, not in the eyes nor any component thereof, such as the retinas. This is a different disease from retinal migraine, which is monocular (only one eye).
It may be difficult to read and dangerous to drive a vehicle while the scotoma is present. Normal central vision may return several minutes before the scotoma disappears from peripheral vision.
Sufferers can keep a diary of dates on which the episodes occur to show to their physician, plus a small sketch of the anomaly, which may vary between episodes.
Animated depictions
The medical exam should rule out any underlying causes, such as blood clot, stroke, pituitary tumor, or detached retina. A normal retina exam is consistent with retinal migraine.
General symptoms of migralepsy are:
- Flashes of light
- Geometric or animate forms
- Visual hallucinations
- Vomiting
- Headache
- Blindness
- Loss of consciousness
- Convulsions
Benign paroxysmal vertigo of childhood is an uncommon neurological disorder which presents with recurrent episodes of dizziness. The presentation is usually between the ages of 2 years and 7 years of age and is characterised by short episodes of vertigo of sudden onset when the child appears distressed and unwell. The child may cling to something or someone for support. The episode lasts only minutes and resolves suddenly and completely. It is a self-limiting condition and usually resolves after about eighteen months, although many go on to experience migrainous vertigo (or vertiginous migraine) when older.
Benign paroxysmal vertigo of childhood is a migrainous phenomenon with more than 50% of those affected having a family history of migraines affecting a first-degree relative. It has no relationship to benign paroxysmal positional vertigo which is a different condition entirely.
Symptoms typically appear gradually over 5 to 20 minutes and generally last fewer than 60 minutes, leading to the headache in classic migraine with aura, or resolving without consequence in acephalgic migraine. Many migraine sufferers change from scintillating scotoma as a prodrome to migraine to scintillating scotoma without migraine. The scotoma typically spontaneously resolves within the stated time frame, leaving few or no subsequent symptoms, though some report fatigue, nausea, and dizziness as sequelae.
Diagnostic criteria require motor symptoms and at least one visual, sensory, or speech symptom, resembling basilar migraine. They may also be associated with cerebellar signs.
The International Headache Society classifies ophthalmodynia periodica as a primary stabbing headache.