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Deep Learning Technology: Sebastian Arnold, Betty van Aken, Paul Grundmann, Felix A. Gers and Alexander Löser. Learning Contextualized Document Representations for Healthcare Answer Retrieval. The Web Conference 2020 (WWW'20)
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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.
Tension-type headache pain is often described as a constant pressure, as if the head were being squeezed in a vise. The pain is frequently present on both sides of the head at the same time. Tension-type headache pain is typically mild to moderate, but may be severe.
According to the third edition of the International Classification of Headache Disorders, the attacks must meet the following criteria:
- A duration of between 30 minutes and 7 days.
- At least two of the following four characteristics:
- bilateral location
- pressing or tightening (non-pulsating) quality
- mild or moderate intensity
- not aggravated by routine physical activity such as walking or climbing stairs
- Both of the following:
- no nausea or vomiting
- no more than one of photophobia (sensitivity to bright light) or phonophobia (sensitivity to loud sounds)
Tension-type headaches may be accompanied by tenderness of the scalp on manual pressure during an attack.
Based on frequency, tension-type headaches can be sub-classified as
- Infrequent episodic: occurring less than once per month on average, or less than 12 episodes a year;
- Frequent episodic: occurring between 1-14 times per month on average for at least 3 months;
- Chronic: occurring 15 times a month for at least 3 months (CTTH - "chronic tension-type headache").
The pain occurs only on one side of the head (unilateral), around the eye (orbital), particularly above the eye (supraorbital), in the temple (temporal), or in any combination.
The pain of CH attack is remarkably greater than in other headache conditions, including severe migraine. The pain is typically described as burning, stabbing, boring or squeezing, and may be located near or behind the eye. As a result of the pain, those with cluster headaches may experience suicidal thoughts during an attack (giving the alternative name "suicide headache" or "suicidal headache"). It is reported as one of the most painful conditions.
Cluster headaches are recurring bouts of excruciating unilateral headache attacks of extreme intensity. The duration of a typical CH attack ranges from about 15 to 180 minutes. Most untreated attacks (about 75%) last less than 60 minutes.
The onset of an attack is rapid and most often without preliminary signs that are characteristic in migraine. Preliminary sensations of pain in the general area of attack, referred to as "shadows", may signal an imminent CH, or these symptoms may linger after an attack has passed, or even between attacks. Though CH is strictly unilateral, there are some documented cases of "side-shift" between cluster periods, or, extremely rarely, simultaneous (within the same cluster period) bilateral cluster headaches.
SUNCT often accompanies cranial autonomic symptoms, including lacrimation (tear flow), ipsilateral ptosis (drooping of the eyelid which is on the same side as the attacks), eyelid edema (swelling due to fluid accumulation), nasal blockage, and conjunctival injection (redness of eye). Attacks seldom occur without any of the cranial autonomic symptoms as in similar form of headache, short-lasting unilateral neuralgiform headache attacks with cranial autonomic features (SUNA).
The majority of SUNCT attacks occur chronically; however, some occur episodically. In chronic SUNCT, attacks can occur regularly or irregularly without a distinct refractory period, whereas in episodic SUNCT, attacks occur only for a certain period of time and then cease for another period. Episodes of attacks last from a few days to a few months, and up to 22 attack episodes in one year have been observed. SUNCT can go into remission even after a chronic period, and relapse either simultaneously or from some stimuli.
The average number of attacks per day is around 60, ranging from 3 to 200 times.
The attacks can be divided into three groups: single stabs, groups of stabs, and attacks with saw-tooth pattern, from the shortest to the longest duration respectively. The attacks usually last from five seconds to 240 seconds. Typically, longer attacks are more painful due to psychological effects, and patients often feel agitated before and during the attack. They occur mostly in the orbital, supraorbital, or temporal region, but can also occur in the retro-orbital (behind the orbit of the eye) region, side, top, and back of head, second and third trigeminal divisions, teeth, neck, and ear.
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.
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.
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.
Tension headache, also known as tension-type headache, is the most common type of primary headache. The pain can radiate from the lower back of the head, the neck, eyes, or other muscle groups in the body typically affecting both sides of the head. Tension-type headaches account for nearly 90% of all headaches.
Pain medication, such as aspirin and ibuprofen, are effective for the treatment of tension headache. Tricyclic antidepressants appear to be useful for prevention. Evidence is poor for SSRIs, propranolol, and muscle relaxants.
As of 2013 tension headaches affect about 1.6 billion people (20.8% of the population) and are more common in women than men (23% to 18% respectively).
The following diagnostic criteria are given for hemicrania continua:
1. Headache for more than 3 months fulfilling other 3 criteria:
2. All of the following characteristics:
- Unilateral pain without side-shift
- Daily and continuous, without pain-free periods
- Moderate intensity, but with exacerbations of severe pain
3. At least one of the following autonomic features occurs during exacerbations and ipsilateral to the side of pain:
- Conjunctival injection and/or lacrimation
- Nasal congestion and/or rhinorrhea
- Ptosis and/or miosis
4. Complete response to therapeutic doses of indomethacin, although cases of hemicrania continua that do not resolve with indomethacin treatment have been documented.
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.
Main features differentiating CPH from cluster headaches (migrainous neuralgia, above) are the higher frequency and shorter duration of attacks, higher incidence in women, and the response to treatment with indomethacin. CPH is not associated with cranial nerve palsies.
The International Headache Society's International Classification of Headache Disorders classifies hemicrania continua as a primary headache disorder.
Individuals with CPH suffer multiple short, severe headaches a day, often more than five, with most lasting between 5 and 30 minutes each. When compared to cluster headaches, CPH attacks are typically shorter. Each headache is centered around the eye, temple and forehead and is localized to one side of the head. While redness and watering of the eye are associated with CPH, patients typically do not experience nausea or vomiting.
Chronic headache, or chronic daily headache (CDH), is classified as experiencing fifteen or more days with a headache per month. It is estimated that chronic headaches affect "4% to 5% of the general population". Chronic headaches consist of different sub-groups, primarily categorized as chronic tension-type headaches and chronic migraine headaches. The treatments for chronic headache are vast and varied. Medicinal and non-medicinal methods exist to help patients cope with chronic headache, because chronic headaches cannot be cured. Whether pharmacological or not, treatment plans are often created on an individual basis. Multiple sources recommend "multimodal treatment", which is a combination of medicinal and non-medicinal remedies. Some treatments are controversial and are still being tested for effectiveness. Suggested treatments for chronic headaches include medication, physical therapy, acupuncture, relaxation training, and biofeedback. In addition, dietary alteration and behavioral therapy or psychological therapy are other possible treatments for chronic headaches.
Many secondary conditions have been reported to be possible causes of CPH, according to Mehta et al., most of which are arterial abrasions or tumors. These include aneurysms in the circle of Willis, middle cerebral artery infarction, parietal arteriovenous malformation, cavernous sinus and petrous ridge meningiomas, pituitary adenoma, Pancoast tumor, gangliocytoma of the sella turcica, and malignant frontal tumors. This accentuates the urgency for those diagnosed with CPH to receive an MRI head scan.
Medication overuse headache is a recognized ICHD (International Classification of Headache Disorders) classification. Over the years different sets of diagnostic criteria have been proposed and revised by the major experts of headache disorders. The term MOH first appeared in the ICHD 2nd edition in 2004. It was defined as a secondary headache, with the aim of emphasising excessive drug intake as the basis of this form of headache. The two subsequent revisions of the diagnostic criteria for MOH (2005 and 2006) refined and extended the definition of the condition on the basis of both its chronicity (headache on more than 15 days/month for more than three months) and drug classes, thereby identifying the main types of MOH. In the case of ergotamine, triptans, opioids and combination medications in particular, intake on > 10 days/month for > 3 months is required, whereas simple analgesics are considered overused when they are taken on > 15 days/month for >3 months.
Hypnic headaches are benign primary headaches that affect the elderly, with the average age of onset being 63 ± 11 years. They are moderate, throbbing, bilateral or unilateral headaches that wake the sufferer from sleep once or multiple times a night. They typically begin a few hours after sleep begins and can last from 15–180 min. There is normally no nausea, photophobia, phonophobia or autonomic symptoms associated with the headache. They commonly occur at the same time every night possibly linking the headaches with circadian rhythm, but polysomnography has recently revealed that the onset of hypnic headaches may be associated with REM sleep.
Lithium carbonate 200–600 mg at bedtime is an effective treatment for most patients but for those that can not tolerate Lithium, Verapamil, indomethacin or methylsergilide may be tried. Two patients have also responded to flunarizine 5 mg. It has also been shown that 1–2 cups of coffee or 100–200 mg of caffeine before bed can prevent hypnic headaches.
For diagnosis of hypnic headache syndrome, headaches should occur at least 15 times per month for at least one month. Included in the differential diagnosis of a new onset nighttime headaches in the elderly is drug withdrawal, temporal arteritis, Sleep apnea, oxygen desaturated, Pheochromocytoma, intracranial causes, primary and secondary neoplasms, communicating hydrocephalus, subdural hematoma, vascular lesions, migraines, cluster headaches, chronic paroxysmal hemicrania and hypnic headache. All other causes must be ruled out before the diagnosis of hypnic headache can be made.
Medication overuse headache (MOH), also known as rebound headache usually occurs when analgesics are taken frequently to relieve 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. Population-based studies report the prevalence rate of MOH to be 1% to 2% in the general population, but its relative frequency is much higher in secondary and tertiary care.
Lupus headache is a proposed, specific headache disorder in patients suffering from systemic lupus erythematosus (SLE).
Research shows that headache is a symptom commonly described by SLE patients —57% in one meta-analysis, ranging in different studies from 33% to 78%; of which migraine 31.7% and tension-type headache 23.5%. The existence of a special lupus headache is contested, although few high-quality studies are available to form definitive conclusions.
Lupus headache is an important item in the Systemic Lupus Erythematosus Disease Activity Index (SLEDAI), a scoring system often used in lupus research. The SLEDAI describes lupus headache as a "severe, persistent headache; may be migrainous, but must be nonresponsive to narcotic analgesia". A score of 8 is given to this item (items are given a relative weight of 1, 2, 4 or 8).
The 1999 American College of Rheumatology case definitions of neuropsychiatric syndromes in SLE do not define lupus headache, but rather propose several headache disorders loosely based on the International Headache Society (IHS) classification.
In the IHS scheme, headache due to lupus would be classified as "Headache attributed to other non-infectious inflammatory disease" (7.3.3). This label requires evidence of a disease flare accompanying the headache, and resolution of the headache with immunosuppressant treatment. However, a meta-analysis found no correlation between headaches and disease activity.
Atypical facial pain (AFP) is a type of chronic facial pain which does not fulfill any other diagnosis. There is no consensus as to a globally accepted definition, and there is even controversy as to whether the term should be continued to be used. Both the International Headache Society (IHS) and the International Association for the Study of Pain (IASP) have adopted the term persistent idiopathic facial pain (PIFP) to replace AFP. In the 2nd Edition of the International Classification of Headache Disorders (ICHD-2), PIFP is defined as "persistent facial pain that does not have the characteristics of the cranial neuralgias [...] and is not attributed to another disorder." However, the term AFP continues to be used by the World Health Organization's 10th revision of the International Statistical Classification of Diseases and Related Health Problems and remains in general use by clinicians to refer to chronic facial pain that does not meet any diagnostic criteria and does not respond to most treatments.
The main features of AFP are: no objective signs, negative results with all investigations/ tests, no obvious explanation for the cause of the pain, and a poor response to attempted treatments. AFP has been described variably as a medically unexplained symptom, a diagnosis of exclusion, a psychogenic cause of pain (e.g. a manifestation of somatoform disorder), and as a neuropathy. AFP is usually burning and continuous in nature, and may last for many years. Depression and anxiety are often associated with AFP, which are either described as a contributing cause of the pain, or the emotional consequences of suffering with unrelieved, chronic pain. For unknown reasons, AFP is significantly more common in middle aged or elderly people, and in females.
Atypical odontalgia (AO) is very similar in many respects to AFP, with some sources treating them as the same entity, and others describing the former as a sub-type of AFP. Generally, the term AO may be used where the pain is confined to the teeth or gums, and AFP when the pain involves other parts of the face. As with AFP, there is a similar lack of standardization of terms and no consensus regarding a globally accepted definition surrounding AO. Generally definitions of AO state that it is pain with no demonstrable cause which is perceived to be coming from a tooth or multiple teeth, and is not relieved by standard treatments to alleviate dental pain.
Depending upon the exact presentation of atypical facial pain and atypical odontalgia, it could be considered as craniofacial pain or orofacial pain. It has been suggested that, in truth, AFP and AO are umbrella terms for a heterogenous group of misdiagnosed or not yet fully understood conditions, and they are unlikely to each represent a single, discrete condition.
AFP has also been described as a medically unexplained symptom, which are thought by some to be largely psychogenic in nature. However, true psychogenic pain is considered to be rare. Some sources have assigned or categorized AFP as a psychosomatic manifestation of somatoform disorder, as defined in the Diagnostic and Statistical Manual of the American Psychiatric Association. Distinction should be made between somatoform disorder, where affected individuals are not inventing the symptom for some benefit, and other conditions like factitious disorder or malingering.
Recent evidence in chronic facial pain research appear to suggest that a proportion of individuals who have been diagnosed with AFP have neuropathic pain,
AFP is described as one of the 4 recognizable symptom complexes of chronic facial pain, along with burning mouth syndrome, temporomandibular joint dysfunction (TMD) and atypical odontalgia. However, there is a degree of overlap between the features of these diagnoses, e.g. between AFP and TMD and burning mouth syndrome.
Atypical odontalgia is similar in nature to AFP, but the latter term generally is used where the pain is confined to the teeth or gums, and AFP when the pain involves other parts of the face. Other sources use atypical odontalgia and AFP as synonyms, or describe atypical odontalgia as a sub-type, variant, or intra-oral equivalent of AFP. Sometimes "phantom tooth pain" is listed as a synonym for AO, and sometimes it is defined as toothache which persists after a tooth has been extracted. It has been suggested that it is likely that these terms do not represent a single, discrete condition, but rather a collection of misdiagnosed and as yet unidentified causes. This pain is often similar to pain from organic dental disease such as periapical periodontitis, or pulpitis (toothache), but unlike normal dental pain, it is not relieved in the long term by dental treatments such as endodontic therapy (root canal treatment) or tooth extraction, and it may even be worsened, return soon after, or simply migrate to other areas in the mouth following dental treatment.
Mollaret's meningitis is a recurrent or chronic inflammation of the protective membranes covering the brain and spinal cord, known collectively as the meninges. Since Mollaret's meningitis is a recurrent, benign (non-cancerous), aseptic meningitis, it is now referred to as benign recurrent lymphocytic meningitis. It was named for Pierre Mollaret, the French neurologist who first described it in 1944.
Although chronic meningitis has been defined as "irritation and inflammation of the meninges persisting for more than 4 weeks being associated with pleocytosis in the cerebrospinal fluid", cerebrospinal fluid abnormalities may not be detectable for the entire time. Diagnosis can be elusive, as Helbok et al. note: "in reality, many more weeks, even months pass by until the diagnosis is established. In many cases the signs and symptoms of chronic meningitis not only persist for periods longer than 4 weeks, they even progress with continuing deterioration, i. e. headache, neck stiffness and even low grade fever. Impairment of consciousness, epileptic seizures, neurological signs and symptoms may evolve over time."
Mollaret's meningitis is characterized by chronic, recurrent episodes of headache, stiff neck, meningismus, and fever; cerebrospinal fluid (CSF) pleocytosis with large "endothelial" cells, neutrophil granulocytes, and lymphocytes; and attacks separated by symptom-free periods of weeks to years; and spontaneous remission of symptoms and signs. Many people have side effects between bouts that vary from chronic daily headaches to after-effects from meningitis such as hearing loss and visual impairment, nerve pain and twitches. Symptoms may be mild or severe. Some cases may be short, lasting only 3–7 days, while others last for weeks to months.
While herpes simplex and varicella can cause rash, Mollaret's patients may or may not have a rash. Herpes simplex virus is likely the most common cause of Mollaret's meningitis.
Psychogenic pain, also called psychalgia, is physical pain that is caused, increased, or prolonged by mental, emotional, or behavioral factors.
Headache, back pain, or stomach pain are some of the most common types of psychogenic pain. It may occur, rarely, in persons with a mental disorder, but more commonly it accompanies or is induced by social rejection, broken heart, grief, lovesickness, or other such emotional events.
Sufferers are often stigmatized, because both medical professionals and the general public tend to think that pain from psychological source is not "real". However, specialists consider that it is no less actual or hurtful than pain from other sources.
The International Association for the Study of Pain defines pain as "an unpleasant sensory and emotional experience associated with actual or potential tissue damage, "or described in terms of such damage"" (emphasis added). In the note accompanying that definition, the following can be found about pain that happens for psychological reasons:
Many people report pain in the absence of tissue damage or any likely pathophysiological cause; usually this happens for psychological reasons. There is usually no way to distinguish their experience from that due to tissue damage if we take the subjective report. If they regard their experience as pain and if they report it in the same ways as pain caused by tissue damage, it should be accepted as pain.
Medicine refers also to psychogenic pain or psychalgia as a form of chronic pain under the name of "persistent somatoform pain disorder" or "functional pain syndrome". Causes may be linked to stress, unexpressed emotional conflicts, psychosocial problems, or various mental disorders. Some specialists believe that psychogenic chronic pain exists as a protective distraction to keep dangerous repressed emotions such as anger or rage unconscious.
It remains controversial, however, that chronic pain might arise purely from emotional causes. Treatment may include psychotherapy, antidepressants, analgesics, and other remedies that are used for chronic pain in general.