Neuralgia
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Neuralgia (Greek ''neuron'', "nerve" + ''algos'', "pain") is pain in the distribution of one or more nerves, as in intercostal neuralgia, trigeminal neuralgia, and
glossopharyngeal The glossopharyngeal nerve (), also known as the ninth cranial nerve, cranial nerve IX, or simply CN IX, is a cranial nerve that exits the brainstem from the sides of the upper medulla, just anterior (closer to the nose) to the vagus nerve. B ...
neuralgia.


Classification

Under the general heading of neuralgia are trigeminal neuralgia (TN), atypical trigeminal neuralgia (ATN), occipital neuralgia, glossopharyngeal neuralgia and
postherpetic neuralgia Postherpetic neuralgia (PHN) is neuropathic pain that occurs due to damage to a peripheral nerve caused by the reactivation of the varicella zoster virus (herpes zoster, also known as shingles). Typically, the nerve pain (neuralgia) is confined ...
(caused by
shingles Shingles, also known as zoster or herpes zoster, is a viral disease characterized by a painful skin rash with blisters in a localized area. Typically the rash occurs in a single, wide mark either on the left or right side of the body or fac ...
or
herpes Herpes simplex is a viral infection caused by the herpes simplex virus. Infections are categorized based on the part of the body infected. Oral herpes involves the face or mouth. It may result in small blisters in groups often called cold ...
). The term ''neuralgia'' is also used to refer to pain associated with
sciatica Sciatica is pain going down the leg from the lower back. This pain may go down the back, outside, or front of the leg. Onset is often sudden following activities like heavy lifting, though gradual onset may also occur. The pain is often described ...
and
brachial plexopathy A brachial plexus injury (BPI), also known as brachial plexus lesion, is an injury to the brachial plexus, the network of nerves that conducts signals from the spinal cord to the shoulder, arm and hand. These nerves originate in the fifth, sixth, ...
.


Atypical (trigeminal)

Atypical trigeminal neuralgia (ATN) is a rare form of neuralgia and may also be the most misdiagnosed form. The symptoms can be mistaken for
migraines Migraine (, ) is a common neurological disorder characterized by recurrent headaches. Typically, the associated headache affects one side of the head, is pulsating in nature, may be moderate to severe in intensity, and could last from a few h ...
, dental problems such as temporomandibular joint disorder, musculoskeletal issues, and
hypochondriasis Hypochondriasis or hypochondria is a condition in which a person is excessively and unduly worried about having a serious illness. An old concept, the meaning of hypochondria has repeatedly changed. It has been claimed that this debilitating cond ...
. 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. ATN pain can be described as heavy, aching, and burning. Affected individuals have a constant migraine-like headache and experience pain in all three trigeminal nerve branches. This includes aching teeth, ear aches, feeling of fullness in sinuses, cheek pain, pain in forehead and temples, jaw pain, pain around eyes, and occasional electric shock-like stabs. Unlike typical neuralgia, this form can also cause pain in the back of the scalp and neck. Pain tends to worsen with talking, facial expressions, chewing, and certain sensations such as a cool breeze. Vascular compression of the
trigeminal nerve In neuroanatomy, the trigeminal nerve ( lit. ''triplet'' nerve), also known as the fifth cranial nerve, cranial nerve V, or simply CN V, is a cranial nerve responsible for sensation in the face and motor functions such as biting and che ...
, infections of the teeth or sinuses, physical trauma, or past viral infections are possible causes of ATN. In the case of trigeminal neuralgia, the affected nerves are responsible for sensing
touch In physiology, the somatosensory system is the network of neural structures in the brain and body that produce the perception of touch ( haptic perception), as well as temperature ( thermoception), body position (proprioception), and pain. It ...
, temperature sensation and pressure sensation in the
facial A facial is a family of skin care treatments for the face, including steam, exfoliation (physical and chemical), extraction, creams, lotions, facial masks, peels, and massage. They are normally performed in beauty salons, but are also a comm ...
area from the jaw to the
forehead In human anatomy, the forehead is an area of the head bounded by three features, two of the skull and one of the scalp. The top of the forehead is marked by the hairline, the edge of the area where hair on the scalp grows. The bottom of the fo ...
. The disorder generally causes short episodes of excruciating
pain Pain is a distressing feeling often caused by intense or damaging stimuli. The International Association for the Study of Pain defines pain as "an unpleasant sensory and emotional experience associated with, or resembling that associated with, ...
, usually for less than two minutes and usually only one side of the face. The pain can be described in a variety of ways such as "stabbing", "sharp", "like lightning", "burning", and even "itchy". In the atypical form of TN, the pain presents as severe constant aching along the nerve. The pain associated with TN is recognized as one of the most excruciating pains that can be experienced. Simple stimuli—such as eating, talking, making facial expressions, washing the face, or any light touch or sensation—can trigger an attack (even the sensation of a cool breeze). Attacks may be lone occurrences, clusters of attacks, or constant episodes. Some patients experience
muscle spasm A spasm is a sudden involuntary contraction of a muscle, a group of muscles, or a hollow organ such as the bladder. A spasmodic muscle contraction may be caused by many medical conditions, including dystonia. Most commonly, it is a muscle ...
, which led to the original term for TN of "" ("", meaning " spasm", and "", meaning "painful", in
French French (french: français(e), link=no) may refer to: * Something of, from, or related to France ** French language, which originated in France, and its various dialects and accents ** French people, a nation and ethnic group identified with Franc ...
).


Glossopharyngeal

Glossopharyngeal neuralgia consists of recurring attacks of severe pain in the back of the throat, the area near the tonsils, the back of the tongue, and part of the ear. The pain is due to malfunction of the
glossopharyngeal nerve The glossopharyngeal nerve (), also known as the ninth cranial nerve, cranial nerve IX, or simply CN IX, is a cranial nerve that exits the brainstem from the sides of the upper medulla, just anterior (closer to the nose) to the vagus nerve. ...
(CN IX), which moves the muscles of the throat and carries information from the throat, tonsils, and tongue to the brain. Glossopharyngeal neuralgia, a rare disorder, usually begins after age 40 and occurs more often in men. Often, its cause is unknown. However, glossopharyngeal neuralgia sometimes results from an abnormally positioned artery that compresses the glossopharyngeal nerve near where it exits the brain stem. Rarely, the cause is a tumor in the brain or neck.


Occipital

Occipital neuralgia, also known as C2 neuralgia, or Arnold's neuralgia, is a medical condition characterized by chronic pain in the upper neck, back of the head and behind the eyes.


Mechanisms

By understanding the neuroplastic changes following nerve damage, researchers may be able to gain a better understanding of the mechanism of hyperexcitability in the nervous system that is believed to cause neuropathic pain.


Peripheral nerve injury

A neuron's response to trauma can often be determined by the severity of the injury, classified by Seddon's classification. In Seddon's Classification, nerve injury is described as either neurapraxia,
axonotmesis Axonotmesis is an injury to the peripheral nerve of one of the extremities of the body. The axons and their myelin sheath are damaged in this kind of injury, but the endoneurium, perineurium and epineurium remain intact. Motor and sensory functions ...
, or
neurotmesis Neurotmesis (in Greek tmesis signifies "to cut") is part of Seddon's classification scheme used to classify nerve damage. It is the most serious nerve injury in the scheme. In this type of injury, both the nerve and the nerve sheath are disrupted. ...
. Following trauma to the nerve, a short onset of afferent impulses, termed "injury discharge", occurs. While lasting only minutes, this occurrence has been linked to the onset of neuropathic pain. When an
axon An axon (from Greek ἄξων ''áxōn'', axis), or nerve fiber (or nerve fibre: see spelling differences), is a long, slender projection of a nerve cell, or neuron, in vertebrates, that typically conducts electrical impulses known as action p ...
is severed, the segment of the axon distal to the cut degenerates and is absorbed by
Schwann cells Schwann cells or neurolemmocytes (named after German physiologist Theodor Schwann) are the principal glia of the peripheral nervous system (PNS). Glial cells function to support neurons and in the PNS, also include satellite cells, olfactory en ...
. The proximal segment fuses, retracts, and swells, forming a "retraction bulb". The
synaptic terminal Chemical synapses are biological junctions through which neurons' signals can be sent to each other and to non-neuronal cells such as those in muscles or glands. Chemical synapses allow neurons to form circuits within the central nervous syste ...
function is lost, as axoplasmic transport ceases and no
neurotransmitters A neurotransmitter is a signaling molecule secreted by a neuron to affect another cell across a synapse. The cell receiving the signal, any main body part or target cell, may be another neuron, but could also be a gland or muscle cell. Neurot ...
are created. The nucleus of the damaged axon undergoes chromatolysis in preparation for axon regeneration. Schwann cells in the distal stump of the nerve and
basal lamina The basal lamina is a layer of extracellular matrix secreted by the epithelial cells, on which the epithelium sits. It is often incorrectly referred to as the basement membrane, though it does constitute a portion of the basement membrane. The bas ...
components secreted by Schwann cells guide and help stimulate regeneration. The regenerating axon must connect to the appropriate receptors to make an effective regeneration. If proper connections to the appropriate receptors are not established, aberrant
reinnervation Reinnervation is the restoration, either by spontaneous cellular regeneration or by surgical grafting, of nerve supply to a body part from which it has been lost or damaged. See also * Denervation * Neuroregeneration Neuroregeneration refers ...
may occur. If the regenerating axon is halted by damaged tissue, neurofibrils may create a mass known as a
neuroma A neuroma (; plural: neuromata or neuromas) is a growth or tumor of nerve tissue. Neuromas tend to be benign (i.e. not cancerous); many nerve tumors, including those that are commonly malignant, are nowadays referred to by other terms. Neuro ...
. If an injured neuron degenerates or does not regenerate properly, then the neuron loses its function or may not function properly. Neuron trauma is not an isolated event and may cause degenerative changes in surrounding neurons. When one or more neurons lose their function or begin to malfunction, abnormal signals sent to the brain may be translated as painful signals.


Diagnosis

When assessing neuralgia to find the underlying mechanism, a history of the pain, description of pain,
physical examination In a physical examination, medical examination, or clinical examination, a medical practitioner examines a patient for any possible medical signs or symptoms of a medical condition. It generally consists of a series of questions about the pati ...
, and experimental examination are required. Pain is subjective to the patient, but pain assessment questionnaires, such as the McGill Pain Questionnaire can be useful for evaluation. Physical examinations usually involve testing responses to stimuli such as touch, temperature, and vibration. Neuralgia can be further classified by the type of stimuli that elicits a response: mechanical, thermal, or chemical. Response to the course of treatment is the final tool used to determine the mechanism of the pain. Additional tools may be used, predominantly in research settings including Laser Evoked Potentials and Quantitative Sensory testing.


Laser evoked potentials

Laser evoked potentials (LEPs) are measurements of cortical responses using lasers to selectively stimulate thermonociceptors in the skin. Lasers can emit a radiant-heat pulse stimulus to selectively activate A-delta and C free nerve endings. LEP abnormalities may be indicative of neuropathic pain, while a normal LEP is often more ambiguous. LEPs can assess damage to both central and peripheral nervous systems.


Quantitative sensory testing

Another method for testing the proper function of a nerve is Quantitative sensory testing (QST). QST relies on analysis of a patient's response to external stimuli of controlled intensity. A stimulus is applied to the skin of the nerve area being tested in ascending and descending orders of magnitude. Clinicians can quantify the mechanical sensitivity of the tactile stimulus using
von Frey hair An esthesiometer (British spelling aesthesiometer) is a device for measuring the tactile sensitivity of the skin (or mouth, or eye, etc.). The measure of the degree of tactile sensitivity is called aesthesiometry. The device was invented by Edward ...
s or Semmes-Weinstein monofilaments (SWMFs). Also, weighted needles can be used to measure pin-prick sensation, and an electronic vibrameter is used to measure vibration sensitivity. Thermal stimuli are quantified by using a probe that operates on the Peltier principle.


Treatment

Treatment options include medicines and surgery. Neuralgia is more difficult to treat than other types of pain because it does not respond well to normal pain medications. Special medications have become more specific to neuralgia and typically fall under the category of membrane stabilizing drugs or antidepressants such as Cymbalta. The antiepileptic medication(AED)
Lyrica Pregabalin, sold under the brand name Lyrica among others, is an anticonvulsant, analgesic and anxiolytic medication used to treat epilepsy, neuropathic pain, fibromyalgia, restless leg syndrome, opioid withdrawal and generalized anxiety di ...
(pregabalin) was developed specifically for neuralgia and other neuropathic pain as a successor to
Neurontin Gabapentin, sold under the brand name Neurontin among others, is an anticonvulsant medication primarily used to treat partial seizures and neuropathic pain. It is a first-line medication for the treatment of neuropathic pain caused by diabeti ...
(gabapentin). High doses of anticonvulsant medicines—used to block nerve firing— and tricyclic antidepressants are generally effective in treating neuralgia. If medication fails to relieve pain or produces intolerable side effects, surgical treatment may be recommended.Stechison, Michael. Personal INTERVIEW. 18 November 2008. Neural augmentative surgeries are used to stimulate the affected nerve. By stimulating the nerve the brain can be "fooled" into thinking it is receiving normal input. Electrodes are carefully placed in the dorsal root and subcutaneous nerve stimulation is used to stimulate the targeted nerve pathway. A technician can create different electrical distributions in the nerve to optimize the efficiency, and a patient controls the stimulation by passing a magnet over the unit. Some degree of facial numbness is expected after most of these surgical procedures, and neuralgia might return despite the procedure's initial success. Depending on the procedure, other surgical risks include hearing loss, balance problems, infection, and stroke. These surgeries include
rhizotomy ::''This article deals with Selective Dorsal Rhizotomy (SDR) rather than the rhizotomy procedures for pain relief; for those procedures, which have begun to take the name "rhizotomy" in certain instances, see facet rhizotomy and similar. A face ...
(where select nerve fibers are destroyed to block pain) and Microvascular decompression (where the surgeon moves the vessels that are compressing the nerve away from it and places a soft cushion between the nerve and the vessels).


History

The earliest cited instance of the term is the French, ''névralgie'', which, according to Rowland, was coined by François Chaussier in his 1801 ''Table Synoptique de la Névralgie'', for "...an affection of one or more nerves causing pain which is usually of an intermittent but frequently intense character". The features and assumed
etiology Etiology (pronounced ; alternatively: aetiology or ætiology) is the study of causation or origination. The word is derived from the Greek (''aitiología'') "giving a reason for" (, ''aitía'', "cause"); and ('' -logía''). More completely, ...
found in the medical literature have varied significantly over time. Various locations were proposed for the primary
lesion A lesion is any damage or abnormal change in the tissue of an organism, usually caused by disease or trauma. ''Lesion'' is derived from the Latin "injury". Lesions may occur in plants as well as animals. Types There is no designated classif ...
during the nineteenth century, including nerve
roots A root is the part of a plant, generally underground, that anchors the plant body, and absorbs and stores water and nutrients. Root or roots may also refer to: Art, entertainment, and media * ''The Root'' (magazine), an online magazine focusing ...
,
ganglia A ganglion is a group of neuron cell bodies in the peripheral nervous system. In the somatic nervous system this includes dorsal root ganglia and trigeminal ganglia among a few others. In the autonomic nervous system there are both sympathe ...
, trunks and branches, as well as the brain and spinal cord. In 1828, JC Warren and TJ Graham placed the cause in the trunk or branch of the nerve innervating the perceived site of the pain, though Graham also attributed neuralgia to "morbid sensibility of the nervous system" due to "great disorder of the general health". Teale in 1830 and many after him argued that it may be located in the spinal cord or nerve root. Later in the century some proposed it may be an affliction of organs such as the uterus or liver, while others classed certain headaches as neuralgias, and proposed that emotional distress may promote the condition.


Society and culture

* In
R. C. Sherriff Robert Cedric Sherriff, FSA, FRSL (6 June 1896 – 13 November 1975) was an English writer best known for his play ''Journey's End'', which was based on his experiences as an army officer in the First World War. He wrote several plays, many nove ...
's play '' Journey's End'', the character Hibbert lies about having neuralgia to his commanding officer, and demands to be sent home. * In
Marcel Proust Valentin Louis Georges Eugène Marcel Proust (; ; 10 July 1871 – 18 November 1922) was a French novelist, critic, and essayist who wrote the monumental novel '' In Search of Lost Time'' (''À la recherche du temps perdu''; with the previous ...
's ''
Swann's Way ''In Search of Lost Time'' (french: À la recherche du temps perdu), first translated into English as ''Remembrance of Things Past'', and sometimes referred to in French as ''La Recherche'' (''The Search''), is a novel in seven volumes by French ...
'', the father of the narrator has neuralgia. * In the 1976 film '' Aces High'', a British ace in the
Royal Flying Corps "Through Adversity to the Stars" , colors = , colours_label = , march = , mascot = , anniversaries = , decorations ...
(played by Simon Ward) feigns neuralgia to escape the terrors of aerial combat. * The narrator of
Vladimir Nabokov Vladimir Vladimirovich Nabokov (russian: link=no, Владимир Владимирович Набоков ; 2 July 1977), also known by the pen name Vladimir Sirin (), was a Russian-American novelist, poet, translator, and entomologist. Bor ...
's novel ''
Look at the Harlequins! ''Look at the Harlequins!'' is a novel written by Vladimir Nabokov, first published in 1974. The work was Nabokov's final published novel before his death in 1977. Plot summary ''Look at the Harlequins!'' is a fictional autobiography narrated b ...
'' claims to have neuralgia of the jaw.


See also

*
Cranial nerves Cranial nerves are the nerves that emerge directly from the brain (including the brainstem), of which there are conventionally considered twelve pairs. Cranial nerves relay information between the brain and parts of the body, primarily to and f ...
*
Nerve A nerve is an enclosed, cable-like bundle of nerve fibers (called axons) in the peripheral nervous system. A nerve transmits electrical impulses. It is the basic unit of the peripheral nervous system. A nerve provides a common pathway for the ...
*
Neuritis Neuritis () is inflammation of a nerve or the general inflammation of the peripheral nervous system. Inflammation, and frequently concomitant demyelination, cause impaired transmission of neural signals and leads to aberrant nerve function. Neu ...
*
Neuropathic pain Neuropathic pain is pain caused by damage or disease affecting the somatosensory system. Neuropathic pain may be associated with abnormal sensations called dysesthesia or pain from normally non-painful stimuli (allodynia). It may have continuous ...
*
Neuropathy Peripheral neuropathy, often shortened to neuropathy, is a general term describing disease affecting the peripheral nerves, meaning nerves beyond the brain and spinal cord. Damage to peripheral nerves may impair sensation, movement, gland, or o ...


References


Further reading

* Shankland, Dr. Wesley E. ''Face the Pain - The Challenge of Facial Pain'', (Omega Publishing, 2001) Dr. Shankland is a former associate editor of ''The Journal of Craniomandibular Practice''.


External links

{{Authority control Pain Neurological disorders Symptoms and signs: Nervous system