phantom limb syndrome

▪ neurophysiology

      the ability to feel sensations and even pain in a limb or limbs that no longer exist. Phantom limb syndrome is characterized by both nonpainful and painful sensations. Nonpainful sensations can be divided into the perception of movement and the perception of external sensations (exteroception), including touch, temperature, pressure, vibration, and itch. Pain sensations range from burning and shooting pains to feelings of tingling “pins and needles.” While phantom limb syndrome occurs only in amputees (amputation), phantom sensations may be perceived in people who have survived strokes (stroke) but lost function of certain body parts or who have spinal cord injury or peripheral nerve injury.

      Phantom limb syndrome was first described in 1552 by French surgeon Ambroise Paré (Paré, Ambroise), who operated on wounded soldiers and wrote about patients who complained of pain in amputated limbs. The same syndrome was later observed and noted by French scientist, mathematician, and philosopher René Descartes (Descartes, René), German physician Aaron Lemos, Scottish anatomist Sir Charles Bell (Bell, Sir Charles), and American physician Silas Weir Mitchell (Mitchell, S. Weir), who tended to wounded soldiers in Philadelphia during the American Civil War. Scottish physician William Porterfield wrote a firsthand account of phantom limb syndrome in the 18th century, following the amputation of one of his legs. He was the first person to consider sensory perception as the underlying phenomenon of the syndrome.

      In the 1990s Indian-born American neuroscientist Vilayanur S. Ramachandran found that neuroplasticity—the ability of neurons (neuron) in the brain to modify their connections and behaviour—could explain this strange syndrome. In fact, the underlying phenomenon of phantom limb syndrome can be explained specifically by map expansion neuroplasticity, in which local brain regions, each dedicated to performing one type of function and reflected in the cerebral cortex as “maps,” can acquire areas of the unused phantom map. This was evidenced by several key experiments carried out by Ramachandran. For example, when he touched the side of an amputee's face with a cotton swab, he found that the amputee also felt a sensation in his or her phantom arm. Ramachandran also scanned an amputee's brain using magnetoencephalography (MEG), an imaging technique based on the detection of magnetic fields in the brain produced through brain activity. The MEG study revealed that the amputee's face brain map had taken over the adjacent area of the arm and hand brain maps. Ramachandran concluded that after the arm was amputated, the arm and hand brain maps were starved for input and sent out special cell growth-stimulating proteins (protein), called growth factors, that attracted neuronal sprouting from the face map stored in an adjacent area of the brain.

      Although the painful symptoms of phantom limb syndrome resolve on their own in some patients, other patients may experience severe, sometimes debilitating chronic pain. This may be controlled by drugs such as analgesics (analgesic) (e.g., aspirin, acetaminophen, and narcotics (narcotic)), sedative-hypnotics (sedative-hypnotic drug) (e.g., benzodiazepines), antidepressants (antidepressant) (e.g., bupropion and imipramine), and anticonvulsants (e.g., gabapentin). Certain drugs, such as ketamine (an anesthetic) and calcitonin (a hormone), administered prior to amputation surgery lessen the likelihood of development of later pain symptoms. Coping techniques, including hypnosis, progressive muscle relaxation, and biofeedback (a learned ability to regulate body functions), have helped some patients manage their symptoms. Nonsurgical treatments that may relieve phantom pain include shock therapy (or electroconvulsive therapy), acupuncture, and transcutaneous electrical nerve stimulation. Implantable treatments, generally used only after noninvasive treatments have failed, include deep brain stimulation, intrathecal drug delivery systems, and spinal cord stimulation.

      The mirror box, a novel therapy for phantom limb syndrome first conceived by Ramachandran in the mid-1990s, has been used by a small number of patients. The therapy has had some success in alleviating pain associated with “learned paralysis,” often experienced by patients whose missing limbs were paralyzed prior to amputation. The box, which does not have a roof, contains a mirror in the centre and usually has two holes, one through which a patient inserts his or her intact limb and one through which the patient inserts the phantom limb. When the patient views the reflection of the intact limb in the mirror, the brain is tricked into “seeing” the phantom limb. By moving the intact limb and watching its reflection in the mirror, a patient can train the brain to “move” the phantom limb and thus relieve the learned paralysis and associated pain. The changes in the brain that contribute to the success of this therapy are not completely understood, and information from long-term studies on the use of mirror box therapy is scant, although several patients have reported long-term relief.

Michael Rugnetta

 

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