Phantom limb

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"Phantom limb syndrome" redirects here. Not to be confused with Alien limb syndrome.For other uses, see Phantom limb (disambiguation).
Phantom limb
A cat attempting to use its left foreleg to scoop litter several months after it has been amputated
Specialty Neurology

A phantom limb is the sensation that an amputated or missing limb is still attached. It is a chronic condition which is often resistant to treatment. [1] When the cut ends of sensory fibres are stimulated during thigh movements, the patient feels as if the sensation is arising from the non-existent limb. Sometimes the patient might feel pain in the non-existent limb. Approximately 80–100% of individuals with an amputation experience sensations in their amputated limb. However, only a small percentage will experience painful phantom limb sensation (phantom pain). These sensations are relatively common in amputees and usually resolve within two to three years without treatment. Research continues to explore the underlying mechanisms of phantom limb pain (PLP) and effective treatment options. [2]

Contents

Signs and symptoms

Most (80–100%) amputees experience a phantom limb, with some of them having non-painful sensations. [3] The amputee may feel very strongly that the phantom limb is still part of the body. [4]

People will sometimes feel as if they are gesturing, feel itches, twitch, or even try to pick things up. [5] The missing limb often feels shorter and may feel as if it is in a distorted and painful position. Occasionally, the pain can be made worse by stress, anxiety and weather changes. [6] Exposure to extreme weather conditions, especially below freezing temperatures, can cause increased sensitivity to the sensation. Phantom limb pain is usually intermittent, but can be continuous in some cases. The frequency and intensity of attacks usually declines with time. [6]

Repressed memories in phantom limbs could potentially explain the reason for existing sensations after amputation. Specifically, there have been several reports from patients of painful clenching spasms in the phantom hand with the feeling of their nails digging into their palms. The motor output is amplified due to the missing limb; therefore, the patient may experience the overflow of information as pain. The patient contains repressed memories from previous motor commands of clenching the hand and sensory information from digging their nails into their palm. These memories remain due to previous neural connections in the brain. [7]

Phantom limb syndrome

Despite the term "phantom limb" not being coined until 1871 by a physician named Silas Weir Mitchell, there have been earlier reports of the phenomenon. [8] One of the first known medical descriptions of the phantom limb phenomenon was written by a French military surgeon, Ambroise Pare, in the sixteenth century. Pare noticed that some of his patients continued reporting pain in the removed limb after he performed the amputation. [9] For many years, the dominant hypothesis for the cause of phantom limbs was irritation in the peripheral nervous system at the amputation site (neuroma). By the late 1980s, Ronald Melzack had recognized that the peripheral neuroma account could not be correct, because many people born without limbs also experienced phantom limbs. [10] According to Melzack the experience of the body is created by a wide network of interconnecting neural structures, which he called the "neuromatrix". [10]

Pons and colleagues (1991) at the National Institutes of Health (NIH) showed that the primary somatosensory cortex in macaque monkeys undergoes substantial reorganization after the loss of sensory input. [11]

Hearing about these results, V. S. Ramachandran hypothesized that phantom limb sensations in humans could be due to reorganization in the human brain's somatosensory cortex. Ramachandran and colleagues illustrated this hypothesis by showing that stroking different parts of the face led to perceptions of being touched on different parts of the missing limb. Later brain scans of amputees showed the same kind of cortical reorganization that Pons had observed in monkeys. [12] Ramachandran have also performed the world's first phantom limb amputation surgeries by asking patients to visualize the missing limb, which relieved pain, and in the long term completely removed the sensation of a phantom limb – the method is now known as the mirror therapy. [13]

Maladaptive changes in the cortex may account for some but not all phantom limb pain. Pain researchers such as Tamar Makin (Oxford) and Marshall Devor (Hebrew University, Jerusalem) argue that phantom limb pain is primarily the result of "junk" inputs from the peripheral nervous system. [14] Despite a great deal of research on the underlying neural mechanisms of phantom limb pain there is still no clear consensus as to its cause. Both the brain and the peripheral nervous system may be involved. [15]

Research continues into more precise mechanisms and explanations. [16]

Differentiation of limb sensations

Phantom limb syndrome (PLS) is a sensation that the amputated or missing limb is still attached to the body. This is different from residual limb pain (RLP) that is often experienced by people with amputations. While RLP occurs in the remaining or residual body part, the pain or sensation associated with PLS can be experienced in the entire limb or just one portion of the missing limb. Phantom limb can also present itself in two ways: phantom limb pain or phantom limb sensations. Phantom limb pain is a painful or unpleasant sensation experienced where the amputated limb was. Phantom sensations are any other, nonpainful sensations perceived in the amputated or missing limb area. [17]

Types of phantom sensations

There are 3 differentiated types of phantom sensations: kinetic, kinesthetic, and exteroceptive. Kinetic phantom sensations are perceived movements of the amputated body part (i.e., feeling your toes flex). Kinesthetic phantom sensations are related to the size, shape, or position of the amputated body part (i.e., feeling as if your hand is in a twisted position). Exteroceptive phantom sensations are related to sensations perceived to be felt by the amputated body part (i.e., feelings of touch, pressure, tingling, temperature, itch, and vibrations).

An additional sensation that some people with amputations experience is known as telescoping. Telescoping is when you feel as if your amputated limb is becoming more proximal to your body through progressive shortening. [17]

Neural mechanisms

Pain, temperature, touch, and pressure information are carried to the central nervous system via the anterolateral system (spinothalamic tracts, spinoreticular tract, spinomesencefalic tract), with pain and temperature information transferred via lateral spinothalamic tracts to the primary sensory cortex, located in the postcentral gyrus in the parietal lobe, where sensory information is represented somatotropically, forming the sensory homunculus. [18] Somatotopic representation seems to be a factor in the experience of phantom limb, with larger regions in the sensory homunculus typically experiencing more phantom sensations or pain. These areas include the hands, feet, fingers and toes.

In phantom limb syndrome, there is sensory input indicating pain from a part of the body that is no longer existent. This phenomenon is still not fully understood, but it is hypothesized that it is caused by activation of the somatosensory cortex. [9] One theory is it may be related to central sensitization, which is a common experience among amputees. Central sensitization is when there are changes in the responsiveness of the neurons in the dorsal horn of the spinal cord, which deals with processing somatosensory information, due to increased activity from the peripheral nociceptors. Peripheral nociceptors are sensory neurons that alert us to potentially damaging stimuli. [9]

There are theories that the phantom limb phenomenon may relate to reorganization of the somatosensory cortex after the limb is removed. When the body receives tactile input near the residual limb, the brain is convinced that the sensory input was received from the amputated limb because another brain region took over. Reorganization has been thought to be related to sensory-discriminative parts of pain as well as the affective-emotional parts of it (I.e., insula, the anterior cingulate cortex, and the frontal cortices). [19]

Phantom sensations can also occur when there has been a peripheral nerve injury resulting in deafferentation. This causes changes in the dorsal horn of the spinal cord, which normally has an inhibitory effect on sensory transmission. [17]

Treatment

Most approaches to treatment over the past two decades have not shown consistent symptom improvement. Treatment approaches have included medication such as antidepressants, spinal cord stimulation, vibration therapy, acupuncture, hypnosis, and biofeedback. [20] Reliable evidence is lacking on whether any treatment is more effective than the others. [21]

A mirror box used for treating phantom limbs, developed by V.S. Ramachandran Ramachandran-mirrorbox.svg
A mirror box used for treating phantom limbs, developed by V.S. Ramachandran

Most treatments are not very effective. [22] Ketamine or morphine may be useful around the time of surgery. [23] Morphine may be helpful for longer periods of time. [23] Evidence for gabapentin is mixed. [23] Perineural catheters that provide local anesthetic agents have poor evidence of success when placed after surgery in an effort to prevent phantom limb pain. [24]

One approach that has received public interest is the use of a mirror box. The mirror box provides a reflection of the intact hand or limb that allows the patient to "move" the phantom limb, and to unclench it from potentially painful positions. [25] [26]

Although mirror therapy was introduced by VS Ramachandran in the early 1990s, little research was done on it before 2009, and much of the subsequent research has been of poor quality, according to a 2016 review. [27] A 2018 review, which also criticized the scientific quality of many reports on mirror therapy (MT), found 15 good-quality studies conducted between 2012 and 2017 (out of a pool of 115 publications), and concluded that "MT seems to be effective in relieving PLP, reducing the intensity and duration of daily pain episodes. It is a valid, simple, and inexpensive treatment for PLP." [28]

Other phantom sensations

Phantom sensations may also occur after the removal of body parts other than the limbs, e.g. after amputation of the breast, [29] extraction of a tooth (phantom tooth pain) [30] or removal of an eye (phantom eye syndrome). [31] [32]

Phantom sensations have been noted in the transgender population. Some people who have undergone sex reassignment surgery (SRS) have reported the sensation of phantom genitals. The reports were less common among post-operative transgender women, but did occur in transgender men. Phantom penises in pre-SRS transgender men have been documented to be similar to the rate of phantom sensations in cis men post-penectomy. [33] Similarly, subjects who had undergone mastectomy reported experiencing phantom breasts; these reports were substantially less common among post-operative transgender men. [34]

See also

Related Research Articles

<span class="mw-page-title-main">Amputation</span> Medical procedure that removes a part of the body

Amputation is the removal of a limb by trauma, medical illness, or surgery. As a surgical measure, it is used to control pain or a disease process in the affected limb, such as malignancy or gangrene. In some cases, it is carried out on individuals as a preventive surgery for such problems. A special case is that of congenital amputation, a congenital disorder, where fetal limbs have been cut off by constrictive bands. In some countries, judicial amputation is currently used to punish people who commit crimes. Amputation has also been used as a tactic in war and acts of terrorism; it may also occur as a war injury. In some cultures and religions, minor amputations or mutilations are considered a ritual accomplishment. When done by a person, the person executing the amputation is an amputator. The oldest evidence of this practice comes from a skeleton found buried in Liang Tebo cave, East Kalimantan, Indonesian Borneo dating back to at least 31,000 years ago, where it was done when the amputee was a young child.

<span class="mw-page-title-main">Pain</span> Type of distressing feeling

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, actual or potential tissue damage."

<span class="mw-page-title-main">Complex regional pain syndrome</span> Array of painful conditions in humans

Complex regional pain syndrome, sometimes referred to by the hyponyms Reflex Sympathetic Dystrophy (RSD) or Reflex Neurovascular Dystrophy (RND), is a rare and severe form of neuroinflammatory and dysautonomic disorder causing chronic pain, neurovascular, and neuropathic symptoms. Although it can vary widely, the classic presentation occurs when severe pain from a physical trauma or neurotropic viral infection outlasts the expected recovery time, and may subsequently spread to uninjured areas. The symptoms of types 1 and 2 are the same except type 2 is associated with nerve injury.

<span class="mw-page-title-main">V. S. Ramachandran</span> Indian-American neuroscientist

Vilayanur Subramanian Ramachandran is an Indian-American neuroscientist. He is known for his wide-ranging experiments and theories in behavioral neurology, including the invention of the mirror box. Ramachandran is a distinguished professor in UCSD's Department of Psychology, where he is the director of the Center for Brain and Cognition.

<span class="mw-page-title-main">Sensory neuron</span> Nerve cell that converts environmental stimuli into corresponding internal stimuli

Sensory neurons, also known as afferent neurons, are neurons in the nervous system, that convert a specific type of stimulus, via their receptors, into action potentials or graded receptor potentials. This process is called sensory transduction. The cell bodies of the sensory neurons are located in the dorsal root ganglia of the spinal cord.

Cortical maps are collections (areas) of minicolumns in the brain cortex that have been identified as performing a specific information processing function.

<span class="mw-page-title-main">Mirror therapy</span> Treatment for some kinds of pain

Mirror therapy (MT) or mirror visual feedback (MVF) is a therapy for pain or disability that affects one side of the patient more than the other side. It was invented by Vilayanur S. Ramachandran to treat post-amputation patients who had phantom limb pain (PLP). Ramachandran created a visual illusion of two intact limbs by putting the patient's affected limb into a "mirror box," with a mirror down the center.

Phantom pain is a painful perception that an individual experiences relating to a limb or an organ that is not physically part of the body, either because it was removed or was never there in the first place.

Neuroplasticity, also known as neural plasticity or just plasticity, is the ability of neural networks in the brain to change through growth and reorganization. Neuroplasticity refers to the brain's ability to reorganize and rewire its neural connections, enabling it to adapt and function in ways that differ from its prior state. This process can occur in response to learning new skills, experiencing environmental changes, recovering from injuries, or adapting to sensory or cognitive deficits. Such adaptability highlights the dynamic and ever-evolving nature of the brain, even into adulthood.. These changes range from individual neuron pathways making new connections, to systematic adjustments like cortical remapping or neural oscillation. Other forms of neuroplasticity include homologous area adaptation, cross modal reassignment, map expansion, and compensatory masquerade. Examples of neuroplasticity include circuit and network changes that result from learning a new ability, information acquisition, environmental influences, pregnancy, caloric intake, practice/training, and psychological stress.

<span class="mw-page-title-main">Phantom eye syndrome</span> Medical condition

Phantom eye syndrome (PES) is a phantom pain in the eye and visual hallucinations after the removal of an eye.

Dysesthesia is an unpleasant, abnormal sense of touch. Its etymology comes from the Greek word "dys," meaning "bad," and "aesthesis," which means "sensation". It often presents as pain but may also present as an inappropriate, but not discomforting, sensation. It is caused by lesions of the nervous system, peripheral or central, and it involves sensations, whether spontaneous or evoked, such as burning, wetness, itching, electric shock, and pins and needles. Dysesthesia can include sensations in any bodily tissue, including most often the mouth, scalp, skin, or legs.

Body schema is an organism's internal model of its own body, including the position of its limbs. The neurologist Sir Henry Head originally defined it as a postural model of the body that actively organizes and modifies 'the impressions produced by incoming sensory impulses in such a way that the final sensation of body position, or of locality, rises into consciousness charged with a relation to something that has happened before'. As a postural model that keeps track of limb position, it plays an important role in control of action.

Tactile discrimination is the ability to differentiate information through the sense of touch. The somatosensory system is the nervous system pathway that is responsible for this essential survival ability used in adaptation. There are various types of tactile discrimination. One of the most well known and most researched is two-point discrimination, the ability to differentiate between two different tactile stimuli which are relatively close together. Other types of discrimination like graphesthesia and spatial discrimination also exist but are not as extensively researched. Tactile discrimination is something that can be stronger or weaker in different people and two major conditions, chronic pain and blindness, can affect it greatly. Blindness increases tactile discrimination abilities which is extremely helpful for tasks like reading braille. In contrast, chronic pain conditions, like arthritis, decrease a person's tactile discrimination. One other major application of tactile discrimination is in new prosthetics and robotics which attempt to mimic the abilities of the human hand. In this case tactile sensors function similarly to mechanoreceptors in a human hand to differentiate tactile stimuli.

Dejerine–Roussy syndrome or thalamic pain syndrome is a condition developed after a thalamic stroke, a stroke causing damage to the thalamus. Ischemic strokes and hemorrhagic strokes can cause lesioning in the thalamus. As initial stroke symptoms dissipate, an imbalance in sensation causes these later syndromes, characterizing Dejerine–Roussy syndrome. Although some treatments exist, they are often expensive, chemically based, invasive, and only treat patients for some time before they need more treatment, called "refractory treatment".

<span class="mw-page-title-main">Visual capture</span>

In psychology, visual capture is the dominance of vision over other sense modalities in creating a percept. In this process, the visual senses influence the other parts of the somatosensory system, to result in a perceived environment that is not congruent with the actual stimuli. Through this phenomenon, the visual system is able to disregard what other information a different sensory system is conveying, and provide a logical explanation for whatever output the environment provides. Visual capture allows one to interpret the location of sound as well as the sensation of touch without actually relying on those stimuli but rather creating an output that allows the individual to perceive a coherent environment.

Mirror-touch synesthesia is a rare condition which causes individuals to experience a similar sensation in the same part or opposite part of the body that another person feels. For example, if someone with this condition were to observe someone touching their cheek, they would feel the same sensation on their own cheek. Synesthesia, in general, is described as a condition in which a concept or sensation causes an individual to experience an additional sensation or concept. Synesthesia is usually a developmental condition; however, recent research has shown that mirror touch synesthesia can be acquired after sensory loss following amputation.

<span class="mw-page-title-main">Cortical remapping</span>

Cortical remapping, also referred to as cortical reorganization, is the process by which an existing cortical map is affected by a stimulus resulting in the creating of a 'new' cortical map. Every part of the body is connected to a corresponding area in the brain which creates a cortical map. When something happens to disrupt the cortical maps such as an amputation or a change in neuronal characteristics, the map is no longer relevant. The part of the brain that is in charge of the amputated limb or neuronal change will be dominated by adjacent cortical regions that are still receiving input, thus creating a remapped area. Remapping can occur in the sensory or motor system. The mechanism for each system may be quite different. Cortical remapping in the somatosensory system happens when there has been a decrease in sensory input to the brain due to deafferentation or amputation, as well as a sensory input increase to an area of the brain. Motor system remapping receives more limited feedback that can be difficult to interpret.

<span class="mw-page-title-main">Tactile hallucination</span> Hallucination involving perception of tactile input

Tactile hallucination is the false perception of tactile sensory input that creates a hallucinatory sensation of physical contact with an imaginary object. It is caused by the faulty integration of the tactile sensory neural signals generated in the spinal cord and the thalamus and sent to the primary somatosensory cortex (SI) and secondary somatosensory cortex (SII). Tactile hallucinations are recurrent symptoms of neurological diseases such as schizophrenia, Parkinson's disease, Ekbom's syndrome and delirium tremens. Patients who experience phantom limb pains also experience a type of tactile hallucination. Tactile hallucinations are also caused by drugs such as cocaine and alcohol.

During every moment of an organism's life, sensory information is being taken in by sensory receptors and processed by the nervous system. Sensory information is stored in sensory memory just long enough to be transferred to short-term memory. Humans have five traditional senses: sight, hearing, taste, smell, touch. Sensory memory (SM) allows individuals to retain impressions of sensory information after the original stimulus has ceased. A common demonstration of SM is a child's ability to write letters and make circles by twirling a sparkler at night. When the sparkler is spun fast enough, it appears to leave a trail which forms a continuous image. This "light trail" is the image that is represented in the visual sensory store known as iconic memory. The other two types of SM that have been most extensively studied are echoic memory, and haptic memory; however, it is reasonable to assume that each physiological sense has a corresponding memory store. For example, children have been shown to remember specific "sweet" tastes during incidental learning trials but the nature of this gustatory store is still unclear. However, sensory memories might be related to a region of the thalamus, which serves as a source of signals encoding past experiences in the neocortex.

<span class="mw-page-title-main">Limb telescoping</span>

Limb telescoping is the progressive shortening of a phantom limb as the cortical regions are reorganized following an amputation. During this reorganization, proximal portions of the residual limb are perceived as more distal parts of the phantom limb. Such effect is responsible for increased phantom pain due to the discrepancy between the patient’s body perception and their actual body. This effect may last from weeks up to years after post-amputation.

References

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  33. ↑ Ramachandran, V. S.; McGeoch, P. D. (January 2008). "Phantom Penises in Transsexuals". Journal of Consciousness Studies. 15 (1): 5–16.
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Hanyu-Deutmeyer AA, Cascella M, Varacallo M. Phantom Limb Pain. 2023 Aug 4. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan–. PMID: 28846343.

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