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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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Psychological and social support has found to play a key role in the management of chronic illnesses and chronic pain conditions, such as trigeminal neuralgia. Chronic pain can cause constant frustration to an individual as well as to those around them. As a result, there are many advocacy groups.
The trigeminal nerve is a mixed cranial nerve responsible for sensory data such as tactition (pressure), thermoception (temperature), and nociception (pain) originating from the face above the jawline; it is also responsible for the motor function of the muscles of mastication, the muscles involved in chewing but not facial expression.
Several theories exist to explain the possible causes of this pain syndrome. It was once believed that the nerve was compressed in the opening from the inside to the outside of the skull; but leading research indicates that it is an enlarged or lengthened blood vessel – most commonly the superior cerebellar artery – compressing or throbbing against the microvasculature of the trigeminal nerve near its connection with the pons. Such a compression can injure the nerve's protective myelin sheath and cause erratic and hyperactive functioning of the nerve. This can lead to pain attacks at the slightest stimulation of any area served by the nerve as well as hinder the nerve's ability to shut off the pain signals after the stimulation ends. This type of injury may rarely be caused by an aneurysm (an outpouching of a blood vessel); by an AVM (arteriovenous malformation); by a tumor; such as an arachnoid cyst or meningioma in the cerebellopontine angle; or by a traumatic event such as a car accident.
Short-term peripheral compression is often painless. Persistent compression results in local demyelination with no loss of axon potential continuity. Chronic nerve entrapment results in demyelination primarily, with progressive axonal degeneration subsequently. It is, "therefore widely accepted that trigeminal neuralgia is associated with demyelination of axons in the Gasserian ganglion, the dorsal root, or both." It has been suggested that this compression may be related to an aberrant branch of the superior cerebellar artery that lies on the trigeminal nerve. Further causes, besides an aneurysm, multiple sclerosis or cerebellopontine angle tumor, include: a posterior fossa tumor, any other expanding lesion or even brainstem diseases from strokes.
Trigeminal neuralgia is found in 3–4% of people with multiple sclerosis, according to data from seven studies. It has been theorized that this is due to damage to the spinal trigeminal complex. Trigeminal pain has a similar presentation in patients with and without MS.
Postherpetic neuralgia, which occurs after shingles, may cause similar symptoms if the trigeminal nerve is damaged.
When there is no [apparent] structural cause, the syndrome is called idiopathic.
ATN is usually attributed to inflammation or demyelination, with increased sensitivity of the trigeminal nerve. These effects are believed to be caused by infection, demyelinating diseases, or compression of the trigeminal nerve (by an impinging vein or artery, a tumor, or arteriovenous malformation) and are often confused with dental problems. An interesting aspect is that this form affects both men and women equally and can occur at any age, unlike typical trigeminal neuralgia, which is seen most commonly in women. Though TN and ATN most often present in the fifth decade, cases have been documented as early as infancy.
About 65% of persons with CH are, or have been, tobacco smokers. Stopping smoking does not lead to improvement of the condition and CH also occurs in those who have never smoked (e.g. children); it is thought unlikely that smoking is a cause. People with CH may be predisposed to certain traits, including smoking or other lifestyle habits.
Atypical trigeminal neuralgia (ATN), or type 2 trigeminal neuralgia, is a form of trigeminal neuralgia, a disorder of the fifth cranial nerve. This form of nerve pain is difficult to diagnose, as it is rare and the symptoms overlap with several other disorders. The symptoms can occur in addition to having migraine headache, or can be mistaken for migraine alone, or dental problems such as temporomandibular joint disorder or musculoskeletal issues. ATN can have a wide range of symptoms and the pain can fluctuate in intensity from mild aching to a crushing or burning sensation, and also to the extreme pain experienced with the more common trigeminal neuralgia.
Cluster headache may, but rarely, run in some families in an autosomal dominant inheritance pattern. People with a first degree relative with the condition are about 14–48 times more likely to develop it themselves, and between 1.9 and 20% of persons with CH have a positive family history. Possible genetic factors warrant further research, current evidence for genetic inheritance is limited.
In the United States each year approximately 1,000,000 individuals develop herpes zoster. Of those individuals approximately 10-18% develop postherpetic neuralgia.
Less than 10 percent of people younger than 60 develop postherpetic neuralgia after a bout of herpes zoster, while about 40 percent of people older than 60 do.
Approximately 64–77% of people have a headache at some point in their lives. During each year, on average, 46–53% of people have headaches. Most of these headaches are not dangerous. Only approximately 1–5% of people who seek emergency treatment for headaches have a serious underlying cause.
More than 90% of headaches are primary headaches. Most of these primary headaches are tension headaches. Most people with tension headaches have "episodic" tension headaches that come and go. Only 3.3% of adults have chronic tension headaches, with headaches for more than 15 days in a month.
Approximately 12–18% of people in the world have migraines. More women than men experience migraines. In Europe and North America, 5–9% of men experience migraines, while 12–25% of women experience migraines.
Cluster headaches are very rare. They affect only 1–3 per thousand people in the world. Cluster headaches affect approximately three times as many men as women.
Primary headache syndromes have many different possible treatments. In those with chronic headaches the long term use of opioids appears to result in greater harm than benefit.
A variety of surgeries have been performed including microvascular decompression (MVD) of the fifth, ninth, and tenth nerves; as well as partial cutting of the nervus intermedius, geniculate ganglion, chorda tympani and/or the ninth and tenth cranial nerves.
Allodynia is a clinical feature of many painful conditions, such as neuropathies, complex regional pain syndrome, postherpetic neuralgia, fibromyalgia, and migraine. Allodynia may also be caused by some populations of stem cells used to treat nerve damage including spinal cord injury. Static mechanical allodynia is a paradoxical painful hypoaesthesia, one etiology of which is lesions of A-beta fibers.
Geniculate ganglionitis or geniculate neuralgia (GN), also called nervus intermedius neuralgia, Ramsay Hunt syndrome, or Hunt's neuralgia, is a rare disorder characterized by severe paroxysmal neuralgic pain deep in the ear, that may spread to the ear canal, outer ear, mastoid or eye regions. GN may also occur in combination with trigeminal or glossopharyngeal neuralgia.
The pain of GN is sharp, shooting or burning and can last for hours. Painful attacks can be triggered by cold, noise, swallowing or touch, but triggers are usually unique to the sufferer. Other related symptoms that may be experienced include increased salivation, bitter taste, tinnitus and vertigo.
GN is rare, and only limited data is available regarding the incidence, prevalence, and risk factors associated with this condition. Middle-aged adults, however, seem to be predominantly affected, women more than men.
GN may be caused by compression of somatic sensory branch of cranial nerve VII which goes through the nervus intermedius. In sufferers of GN, signals sent along these nerves are altered and interpreted by the geniculate ganglion (a structure in the brain) as GN pain. GN may also develop following herpes zoster oticus (Ramsay Hunt syndrome), where cold sores occur on the ear drum or ear. This may also be associated with facial paresis (weakness), tinnitus, vertigo and deafness. Disorders of lacrimation, salivation and/or taste sometimes accompany the pain. There is a common association with herpes zoster.
There are different kinds or types of allodynia:
- Mechanical allodynia (also known as tactile allodynia)
- Static mechanical allodynia – pain in response when touched
- Dynamic mechanical allodynia – pain in response to stroking lightly
- Thermal (hot or cold) allodynia – pain from normally mild skin temperatures in the affected area
- Movement allodynia – pain triggered by normal movement of joints or muscles
Overall, the outcome for acute low back pain is positive. Pain and disability usually improve a great deal in the first six weeks, with complete recovery reported by 40 to 90%. In those who still have symptoms after six weeks, improvement is generally slower with only small gains up to one year. At one year, pain and disability levels are low to minimal in most people. Distress, previous low back pain, and job satisfaction are predictors of long-term outcome after an episode of acute pain. Certain psychological problems such as depression, or unhappiness due to loss of employment may prolong the episode of low back pain. Following a first episode of back pain, recurrences occur in more than half of people.
For persistent low back pain, the short-term outcome is also positive, with improvement in the first six weeks but very little improvement after that. At one year, those with chronic low back pain usually continue to have moderate pain and disability. People at higher risk of long-term disability include those with poor coping skills or with fear of activity (2.5 times more likely to have poor outcomes at one year), those with a poor ability to cope with pain, functional impairments, poor general health, or a significant psychiatric or psychological component to the pain (Waddell's signs).
Low back pain that lasts at least one day and limits activity is a common complaint. Globally, about 40% of people have LBP at some point in their lives, with estimates as high as 80% of people in the developed world. Approximately 9 to 12% of people (632 million) have LBP at any given point in time, and nearly one quarter (23.2%) report having it at some point over any one-month period. Difficulty most often begins between 20 and 40 years of age. Low back pain is more common among people aged 4080years, with the overall number of individuals affected expected to increase as the population ages.
It is not clear whether men or women have higher rates of low back pain. A 2012 review reported a rate of 9.6% among males and 8.7% among females. Another 2012 review found a higher rate in females than males, which the reviewers felt was possibly due to greater rates of pains due to osteoporosis, menstruation, and pregnancy among women, or possibly because women were more willing to report pain than men. An estimated 70% of women experience back pain during pregnancy with the rate being higher the further along in pregnancy. Current smokers – and especially those who are adolescents – are more likely to have low back pain than former smokers, and former smokers are more likely to have low back pain than those who have never smoked.
Anesthesia dolorosa or anaesthesia dolorosa or deafferentation pain is pain felt in an area (usually of the face) which is completely numb to touch. The pain is described as constant, burning, aching or severe. It can be a side effect of surgery involving any part of the trigeminal system, and occurs after 1–4% of peripheral surgery for trigeminal neuralgia. No effective medical therapy has yet been found. Several surgical techniques have been tried, with modest or mixed results. The value of surgical interventions is difficult to assess because published studies involve small numbers of mixed patient types and little long term follow-up.
- Gasserian ganglion stimulation is stimulation of the gasserian ganglion with electric pulses from a small generator implanted beneath the skin. There are mixed reports, including some reports of marked, some of moderate and some of no improvement. Further studies of more patients with longer follow-up are required to determine the efficacy of this treatment.
- Deep brain stimulation was found in one review to produce good results in forty-five percent of 106 cases. Though relief may not be permanent, several years of relief may be achieved with this technique.
- Mesencephalotomy is the damaging of the junction of the trigeminal tract and the periaqueductal gray in the brain, and has produced pain relief in a group of patients with cancer pain; but when applied to six anesthesia dolorosa patients, no pain relief was achieved, and the unpleasant sensation was in fact increased.
- Dorsal root entry zone lesioning, damaging the point where sensory nerve fibers meet spinal cord fibers, produced favorable results in some patients and poor results in others, with incidence of ataxia at 40%. Patient numbers were small, follow-up was short and existing evidence does not indicate long term efficacy.
- One surgeon treated thirty-five patients using trigeminal nucleotomy, damaging the nucleus caudalis, and reported 66% "abolition of allodynia and a marked reduction in or (less frequently) complete abolition of deep background pain."
In 1995, the Food and Drug Administration (FDA) approved the Varicella vaccine to prevent chickenpox. Its effect on postherpetic neuralgia is still unknown. The vaccine—made from a weakened form of the varicella-zoster virus—may keep chickenpox from occurring in nonimmune children and adults, or at least lessen the risk of the chickenpox virus lying dormant in the body and reactivating later as shingles. If shingles could be prevented, postherpetic neuralgia could be completely avoided.
In May 2006 the Advisory Committee on Immunization Practices approved a new vaccine by Merck (Zostavax) against shingles. This vaccine is a more potent version of the chickenpox vaccine, and evidence shows that it reduces the incidence of postherpetic neuralgia. The CDC recommends use of this vaccine in all persons over 60 years old.
There is strong evidence to show that chronic orofacial pain (including AFP) is associated with psychological factors. Sometimes stressful life events appear to precede the onset of AFP, such as bereavement or illness in a family member. Hypochondriasis, especially cancerophobia, is also often cited as being involved. Most people with AFP are "normal" people who have been under extreme stress, however other persons with AFP have neuroses or personality disorders, and a small minority have psychoses. Some have been separated from their parents as children.
Depression, anxiety and altered behavior are strongly correlated with AFP. It is argued whether this is a sole or contributing cause of AFP, or the emotional consequences of suffering with chronic, unrelieved pain. It has been suggested that over 50% of people with AFP have concomitant depression or hypochondria. Furthermore, about 80% of persons with psychogenic facial pain report other chronic pain conditions such as listed in the table.
Hemicrania continua (HC) is a persistent unilateral headache that responds to indomethacin. It is usually unremitting, but rare cases of remission have been documented. Hemicrania continua is considered a primary headache disorder, meaning that it is not caused by another condition.
This syndrome is predominantly found in young women, but also occurs in children, teenagers and octogenarians.
It most often occurs in the middle of the night and lasts from seconds to minutes, an indicator for the differential diagnosis of levator ani syndrome, which presents as pain and aching lasting twenty minutes or longer. In a study published in 2007 involving 1809 patients, the attacks occurred in the daytime (33 per cent) as well as at night (33 per cent) and the average number of attacks was 13. Onset can be in childhood; however, in multiple studies the average age of onset was 45. Many studies showed that women are affected more commonly than men. This can be at least partly explained by men's reluctance to seek medical advice concerning such a delicate case as rectal pain.
During an episode, the patient feels spasm-like, sometimes excruciating, pain in the anus, often misinterpreted as a need to defecate. The pain must arise de novo, that is in absence of clear cause. As such, pain associated with penetrative anal intercourse, trauma or rectal foreign body insertion preclude a diagnosis of proctalgia fugax. Simultaneous stimulation of the local autonomic system can cause erection in males. In some people, twinges sometimes occur shortly after orgasm. Because of the high incidence of internal anal sphincter thickening with the disorder, it is thought to be a disorder of the internal anal sphincter or that it is a neuralgia of pudendal nerves. It is recurrent and there is also no known cure. However, some studies show effective use of botulinum toxin, pudendal nerve block, and calcium channel blockers. It is not known to be linked to any disease process and data on the number of people afflicted vary, but prevalence may be as high as 8–18%. It is thought that only 17–20% of sufferers consult a physician, so obtaining accurate data on occurrence presents a challenge.
The pain episode subsides by itself as the spasm disappears on its own, but may reoccur.
Hemicrania was mentioned in 1881 in The Therapeutic Gazette Vol. 2, by G.S.Davis, and the incident has been cited in King's American Dispensatory (1898 and later editions) 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.
In newer times, Hemicrania continua was described in 1981; at that time around 130 cases were described in the literature. 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.
Many persons with AFP blame organic disease for their pain.
Research in individuals with AFP showed that there is increased cerebral activity (demonstrated during positron emission tomography), possibly suggesting that there is an overactive alerting mechanism to peripheral stimuli. It has been theorized that this can trigger neuropeptides to be released, and the formation of free radicals (capable of damaging cells), the release of eicosanoids (e.g. prostaglandins). There is some suggestion that AFP is an early form of trigeminal neuralgia.
Pain may be referred from other parts of the head or other parts of the body to cause facial pain. Nowhere in the body is referred pain more well illustrated than in the face, and this is due to the richly and complexly innervated nature of the head and neck.
Proctalgia fugax (a variant of levator ani syndrome) is a severe, episodic pain in the regions of the rectum and anus. It can be caused by cramp of the levator ani muscle, particularly in the pubococcygeal part.
It is difficult to determine the exact cause of Tarsal Tunnel Syndrome. It is important to attempt to determine the source of the problem. Treatment and the potential outcome of the treatment may depend on the cause. Anything that creates pressure in the Tarsal Tunnel can cause TTS. This would include benign tumors or cysts, bone spurs, inflammation of the tendon sheath, nerve ganglions, or swelling from a broken or sprained ankle. Varicose veins (that may or may not be visible) can also cause compression of the nerve. TTS is more common in athletes and other active people. These people put more stress on the tarsal tunnel area. Flat feet may cause an increase in pressure in the tunnel region and this can cause nerve compression. Those with lower back problems may have symptoms. Back problems with the L4, L5 and S1 regions are suspect and might suggest a "Double Crush" issue: one "crush" (nerve pinch or entrapment) in the lower back, and the second in the tunnel area. In some cases, TTS can simply be idiopathic.
Rheumatoid Arthritis has also been associated with TTS.