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Phantom limb syndrome

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Phantom limb
ICD-10 G54.6-G54.7
ICD-9 353.6
OMIM {{{OMIM}}}
DiseasesDB {{{DiseasesDB}}}
MedlinePlus {{{MedlinePlus}}}
eMedicine {{{eMedicineSubj}}}/{{{eMedicineTopic}}}
MeSH {{{MeshNumber}}}


This article is about the syndrome. For the fictional character, see Phantom Limb

A phantom limb is the sensation that an amputated or missing limb is still attached to the body and is moving appropriately with other body parts (Mitchell 1871; Melzack 1992; Ramachandran & Hirstein 1998). Approximately 50 to 80% of amputees experience these phantom sensations in their amputated limb, and the majority of these people report that the sensations are painful (Sherman, Sherman & Parker 1984). Phantom sensations and phantom pain may also occur after the removal of body parts other than the limbs, e.g. after amputation of the breast, extraction of a tooth (phantom tooth pain) or removal of an eye (phantom eye syndrome).

Phantom pains can also occur in people who are born without limbs and people who are paralyzed (Ramachandran 1993; Saadah & Melzack 1994). Phantom pains occur when the missing limb causes discomfort. Other induced sensations include warmth, cold, itching, squeezing and burning (Ramachandran & Blakeslee 1998; Ramachandran & Hirstein 1998). 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.

Clinical description

Although not all phantom limbs are painful, patients will sometimes feel as if they are gesturing, feel itches, twitch or even try to pick things up. For example, Ramachandran and Blakeslee describe that some people's representations of their limbs don't actually match what they should be, for example, one patient reported that her phantom arm was about "6 inches too short" (Ramachandran & Blakeslee 1998).

Some people with phantom limbs find that the limb will gesticulate as they talk. Given the way that the hands and arms are represented on the motor cortex and language centers, this is not surprising. Some people find that their phantom limbs feels and behaves as though it is still there, others find that it begins to take on a life of its own, and doesn't obey their commands.

I placed a coffee cup in front of John and asked him to grab it [with his phantom limb]. Just as he said he was reaching out, I yanked the cup away.
"Ow!" he yelled. "Don't do that!"
"What's the matter?"
"Don't do that", he repeated. "I had just got my fingers around the cup handle when you pulled it. That really hurts!"
Hold on a minute. I wrench a real cup from phantom fingers and the person yells, ouch! The fingers were illusory, but the pain was real - indeed, so intense that I dared not repeat the experiment.

— Ramachandran, Phantoms in the Brain, p. 43. (Ramachandran & Blakeslee 1998)

Neurological basis

Until recently, the dominant theory for cause of phantom limbs was irritation in the severed nerve endings (called "neuromas"). When a limb is amputated, many severed nerve endings are terminated at the remaining stump. These nerve endings can become inflamed, and were thought to send anomalous signals to the brain. These signals, being functionally nonsense, were thought to be interpreted by the brain as pain.

Treatments based on this theory were generally failures. In extreme cases, surgeons would perform a second amputation, shortening the stump, with the hope of removing the inflamed nerve endings and causing temporary relief from the phantom pain. But instead, the patients' phantom pains increased, and many were left with the sensation of both the original phantom limb, as well as a new phantom stump, with a pain all its own (Ramachandran & Blakeslee 1998). In some cases, surgeons even cut the sensory nerves leading into the spinal cord or in extreme cases, even removed the part of the thalamus that receives sensory signals from the body.

In the early 1990s, Tim Pons, at the National Institutes of Health (NIH), showed that the brain can reorganize if sensory input is cut off (Pons et al. 1991). Hearing about these results,V. S. Ramachandran realized that phantom limb sensations could be due to "crosswiring" in the somatosenory cortex, which is located in the postcentral gyrus (Ramachandran & Blakeslee 1998; Ramachandran & Hirstein 1998), and which receives input from the limbs and body. Input from the left side of the body goes to the right hemisphere and vice versa. The input from extremities comes into the somatosensory cortex in an ordered way, the representation of which is referred to as the somatosensory homonculus. Input from the hand is located next to the input from the arm, input from the foot is located next to input from the hand, and so on. One oddity is input from the face is located next to input from the hand.

Ramachandran reasoned that if someone were to lose their right hand in an accident, they may then have the feelings of a phantom limb because the input that normally would go from their hand to the left somatosensory cortex would be stopped. The areas in the somatosensory cortex that are near to the ones of the hand (the arm and face) will take over (or "remap") this cortical region that no longer has input. Ramachandran and colleagues first demonstrated this remapping by showing that stroking different parts of the face led to perceptions of being touched on different parts of the missing limb (Ramachandran, Rogers-Ramachandran & Stewart 1992). Through magnetoencephalography (MEG), which permits visualization of activity in the human brain (Yang et al. 1994), Ramachandran verified the reorganization in the somatosensory cortex.

Treatment

Some treatments include drugs such as antidepressants. Spinal cord stimulation (SCS) can be effective treatment for phantom pain. An electrical stimulator is implanted under the skin, and an electrode is placed next to the spinal cord. The nerve pathways in the spinal cord are stimulated by an electric current. This interferes with the impulses travelling towards the brain and lessens the pain felt in the phantom limb (Melzack 1992). Instead, amputees feel a tingling sensation in the phantom limb.

Vibration therapy, acupuncture, hypnosis and biofeedback may all be used to treat phantom pain but are often of little help. The pain can sometimes be helped by keeping busy and focusing attention on something else. Massaging the stump can sometimes help.

For planned amputation, phantom pain can reduced by preoperative pain management, effective control of pain by analgesic or neuroleptic is required. The brain seems to implant the sensations from the preoperative state.

One particularly novel treatment for phantom limb pain is the mirror box developed by Ramachandran and colleagues (Ramachandran, Rogers-Ramachandran & Cobb 1995). Through the use of artificial visual feedback it becomes possible for the patient to "move" the phantom limb, and to unclench it from potentially painful positions. Repeated training in some subjects has lead to long-term improvement, and in one exceptional case, even to the complete elimination of the phantom limb between the hand and the shoulder (so that the phantom hand was dangling from the shoulder).

More recently, virtual reality has been used to combat the discomfort caused by phantom limb syndrome, [1]. Scientists from the University of Manchester have shown that phantom limb pain can be relieved by attaching the sufferer's real limb to an interface that allows them to see two limbs moving in a computer-generated simulation. This works on a similar principle to the mirror box technique in that the somatosensory cortex is being ‘tricked’, except that the illusion is stronger.

See also

References

  • Halligan, P.W. & Berger, A. (1999), "Phantoms in the brain", British Medical Journal, vol. 319, pp. 587-588
  • Halligan, P.W. (2002), "Phantom limbs: The body in mind", Cogntive Neuropsychiatry, vol. 7, no. 3, pp. 251-268
  • Halbert, J., Crotty, M. & Cameron, I.D. (2002), "Evidence for the optimal management of acute and chronic phantom pain: a systematic review", Clin J Pain, vol. 18, no. 2, pp. 84-92, PMID 11882771
  • Melzack, R. (1992), "Phantom Limbs", Scientific American, no. April, pp. 120-126
  • Mitchell, S. W. (1871), "Phantom limbs", Lippincott's Mag Popular Literature & Science, vol. 8, pp. 563-569
  • Pons, T. P., Garraghty, P. E. & Ommaya, A. K., et al. (1991), "Massive cortical reorganization after sensory deafferentation in adult macaques", Science, vol. 252, no. 5014, pp. 1857-1860
  • Ramachandran, V. S. , Rogers-Ramachandran, D. C. & Stewart, M. (1992), "Perceptual correlates of massive cortical reorganization.", Science, no. 258(5085), pp. 1159-1160
  • Ramachandran, V. S. (1993), "Behavioral and magnetoencephalographic correlates of plasticity in the adult human brain.", Proc. Natl. Acad. Sci., vol. 90, pp. 10413–10420
  • Ramachandran, V. S. , Rogers-Ramachandran, D. C. & Cobb, S. (1995), "Touching the phantom.", Nature, no. 377, pp. 489-490
  • Ramachandran, V. S. & Rogers-Ramachandran, D. C. (1996), "Synaesthesia in phantom limbs induced with mirrors.", Proceedings of the Royal Society of London, no. 263(1369), pp. 377-386
  • Ramachandran, V. S. & Blakeslee, S. (1998), Phantoms in the brain: Probing the mysteries of the human mind., William Morrow & Company, ISBN 0-688-15247-3
  • Ramachandran, V. S. & Hirstein, W. (1998), "The perception of phantom limbs: The D.O. Hebb lecture.", Brain, vol. 9, no. 121, pp. 1603-1630
  • Saadah, E. S. & Melzack, R. (1994), "Phantom limb experiences in congenital limb-deficient adults.", Cortex, no. 30(3), pp. 479-485
  • Sherman, R. A., Sherman, C. J. & Parker, L. (1984), "Chronic phantom and stump pain among American veterans: results of a survey.", Pain, no. 18, pp. 83-95
  • Yang, T. T., Gallen, C. C. & Ramachandran, V. S., et al. (1994), "Noninvasive detection of cerebral plasticity in adult human somatosensory cortex.", Neuroreport: An International Journal for the Rapid Communication of Research in Neuroscience, no. 5(6), pp. 701-704

External links

fr:Membre fantômezh:幻肢

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