Perceptual and Motor Skills, 1990, 70, 1227-1232. O Perceptual and Motor Skills 1990

PHANTOM REPRESENTATIONS O F CONGENITALLY ABSENT LIMBS " ' PAUL SCATENA Universig of Rochester Stimmary.-It has been widely claimed that phantom limbs are not experienced by children born without the part or who have it amputated in early childhood. This review looks at some reported cases of such phantoms, as well as a lesser known account that shows that phantoms of aplasics can be vivid and dexterous. Reasons for the infrequency of such reports are examined, and some implications for understanding the development of the body schema are proposed.

The view that congenital amputees do not experience phantom limbs has been put forth in much of the literature on phantom phenomena (Cronholm, 1951; Hoffman, 1954; Kolb, 1954, 1982; Simmel, 1956, 1958, 1961, 1962, 1966; Weiss, 1956), phantom limb pain (White & Sweet, 1955; Nashold & Friedman, 1986), and the treatment of the handicapped (Frantz, 1959; Gillis, 1954; Lamb & Law, 1987; Mital & Pierce, 1971; Setoguchi & Rosenfelder, 1983). This absence of phantoms is usually attributed to a lack of experience of the body part. White and Sweet (1955) give a typical explanation: "Congenital absence of a limb never gives rise to awareness of a phantom, as the infant is born without a cortical schema of a body shape and therefore never developes a sensory representation of the missing limb in its brain." However, phantom limbs of congenital amputees have been reported in some well-documented cases, and this review examines some of these accounts and present some evidence gathered from studies of acquired amputees that can help evaluate these reports.

Weinstein and Coworkers Weinstein and Sersen's 1961 paper is the first modern English report of phantom limbs in cases of congenital amputees. They studied 30 subjects, aged 5 to 23 yr. and found five with apparent phantom limbs. With the younger subjects they used a "game" of asking whether their limbs ended at one point or another, finally indicating points beyond the stump. This method is supported by many reports that subjects with acquired amputations usually have a very definite sense of the length and position of their phantoms (Cronholm, 1951; Livingston, 1943). Weinstein, Sersen, and Vetter (1964) added more cases later, for a total of 18 cases of phantoms from 101 subjects studied. Should these subjects be believed? Weinstein and colleagues suggest sev'This paper profited from translation assistance from Wilhelm Braun, and from criticisms from ohn H. R. Maunsell and Ron Gerrard. 'Address for reprints Paul Latena, Ph.D., Cognitive Science Cluster/Dcpartment of Philosophy, University of Rochester, Rochester, New York 14627.

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eral compelling arguments. Some of the descriptions they collected match known properties of the phantoms of acquired amputees, such as the salience of the distal part. Two of the subjects in their later study (1964) reported intercalary phantoms, with gaps between the terminal phantom and the stump, which.are common in persons who lose limbs as adults. Some have attributed such reports to fantasy or a desire to be like others, but Weinstein and coworkers point out that some of their subjects have described very unappealing appendages. J. S., a 7-yt-old boys with bilateral missing arms, claimed to have two "blobs," 4 and 6 in. long protruding from his stumps, while K. H., a 10-yt-old girl born with an absence of her left forearm and hand, said she had an immobile phantom arm slightly shorter than her intact right limb (Weinstein & Sersen, 1961). Why haven't such cases been reported before? Although we'll refer to a few earlier reports later in this paper, the shortfalls in the literature have some lessons for us. Subjects with acquired amputations, virtually all of whom experience phantoms, are often reluctant to talk about them. Many papers cite Pick (1915), who discussed now well-established phenomena such as phantom hands perceived as starting at shoulder stumps, but who also claimed that phantoms were never experienced by individuals born without limbs, which he explained to the lack of experience of the missing part in the establishment of the body schema. Pick's conclusion has been cited by countless later workers, although Simmel (1966) remarked that "if he had ever examined a group of individuals so affected, he kept it a secret." Also in agreement with Pick (1915), many writers further state that children do not experience phantoms if surgical amputation takes place before a certain age such as three or six years, the latter figure often based on the report of Riese and Bruck (1950). Weinstein and Sersen (1961) noted that many researchers who did not observe phantoms in congenital or young amputees relied on reports from the parents (e.g., Simmel, 1958), and they point out that the parents of their own subjects were unaware of any phantoms. Some parents are more than unaware: Simmel (1959) interviewed a 16-yr.-old girl who had had a congenitally deformed leg amputated when she was five: "Whether or not this girl ever had a phantom I have never been able to find out, because on both occasions when I tried to interview her, her mother was present, and both times the mother immediately said 'She never had anything like that,' to which the girl rather meekly agreed." Sirmlarly, parents have also been known to discount statements of children with amputations about this subject. Eland and Anderson (1977) reported that "During an interview with the child, he told the investigator that his right foot (the amputated one) had hurt earlier in the day. His mother interrupted the child and reminded him that his foot could not hurt anymore because it was gone."

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Another case further illustrates the problem of relying on the reports of parents. Easson and Sask (1961) studied a 37-mo.-old boy who lost his left arm in an accident with some farm machinery and who displayed "a very definite phantom limb" while in the hospital. H e complained of "tickling" in his fingers, reached for objects with h s "left hand," and fell over when he tried to use his "arm" to get up. The boy's mother was very upset about his injury, refusing to visit him during his 16-day hospitalization, and, following his release, his parents denied seeing any signs of a phantom limb. Easson and Sask observed the phantom-limb behaviors from the second postoperative day until the boy left the hospital. They comment that "It is most unlikely that our patient's phantom limb disappeared abruptly when he returned home. We can surmise that his parents continued to deny, as far as possible, their son's deformity and thus any signs of phantom phenomena." Easson and Sask point out that young children d o not have the verbal ability to describe such sensations, and so researchers must rely on nonverbal cues. Children are thereby distinctly different from adult amputees, who often refrain from speaking of their phantom sensations for fear of being though crazy. Easson and Sask's case and the one reported by Eland and Anderson above show that children are often unable to report phantoms and are unhkely to be believed if they do. Weinstein and coworkers found the phantom of aplasics to be relatively infrequent (18%) and lacking in strength compared to those of acquired amputees. Weinstein and Sersen (1961) drew the conclusion that, while these cases show that the body schema is at least partly innate, the vagueness of these experiences may show that phantoms may be "proportional in vividness to the magnitude of stimulation and to the period of time during which the engrams had been deposited."

Poeck's Case-A Vivid Phantom Vetter and Weinstein published a review (1967) of their cases and a few brief reports found in literature dating back to the nineteenth century. Most of the papers acknowledging phantoms in congenital amputees have cited only Weinstein and Sersen's 1961 paper, with a few also noting the 1964 report. Many of these writers stress the lack of clarity in the aplasics' phantoms and continue to treat the body schema as largely a product of experience. Klaus Poeck (1964) published a report that contradicts this conclusion. H e interviewed an 11-yr.-old girl with congenital absence of both forearms and hands, who said that from the age of six she had felt two phantom hands hanging below her stumps. She claimed to feel these hands clearly and to be able to move them at will. Poeck writes that "in her first years in school she had learned to solve simple arithmetic problems by counting with

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her fingers. . . . O n these occasions she would place her phantom hands on the table and count the outstretched fingers one by one." Several details support the young girl's account. She described only hands, and claimed to never have felt either forearm-in agreement with many acquired amputees. Poeck reports that her hands would telescope when approaching a wall, and disappear when the stump made contact with the surface, which accords with Poeck's findings with surgical amputees (Poeck, 1969). While many reports contain descriptions of phantoms as passing through objects, others find much variation in the reaction to solid objects. Jalavisto (1950) found an age-related difference in wall-approaching behavior, with younger amputees (17 to 24 yr. of age) tending to have their phantoms disappear or move to avoid conflict, and older amputees (25 + yr.) tending to have their phantoms enter the wall. Poeck's subject had never worn prostheses. Several writers have claimed that prosthesis use can cause phantoms to fade (Henderson & Smyth, 1948; Herrmann & Gibbs, 1945), although some writers find a reinforcing- effect (Simmel, 1956), as well as some who say there is no relationship between prosthesis use and phantoms (Cronholm, 1951; Haber, 1958). Weinstein, et a/. (1964) found a significantly higher percentage of phantoms among congenital amputees who had prostheses fitted before the age of seven than in those fitted later or not at all, and they speculated that the modern tendency to fit children with prostheses early i n life may be responsible for their finding subjects with phantoms while earlier workers have not. Poeck claims that his case and two more of individuals reporting vivid phantoms after amputation at the age of five show that "there is not even necessarily a qualitative difference between phantoms in adult amputees and in children with congenital absence of limbs."

Pain in a Congenitally Absent Hand I have found only one report of a congenital amputee with pain in a phantom. Sohn (1914) published an account of a 19-yr.-old woman born with her left forearm and hand missing and who claimed to feel pain radiating from the stump down to a hand and fingers. Sohn quotes her as saying "If the end of the stump was opened, a hand would grow out of it, for I am sure there is something inside which wants to come out. I t feels as though a lump inside is struggling to get out, and then there is this sticking pain which I feel all the way down to my fingers." This patient's phantom pain began with her first menstruation at the age of 14, and Sohn found its etiology clear: she was worried about her marriage prospects. Sohn reports that she was helped by therapy, and he considered the phantom pain to be "equivalent to a wish fulfillment." We can d o little more than accept his diagnosis and consider this to be psychogenic pain projected to the phantom rather than true phantom limb pain.

CONGENITALLY ABSENT LIMBS: PHANTOMS

Implications for Theories of Phantom Phenomena The term "phantom limb" was first used by Civil War surgeon S. Weir Mitchell in an article in Lippincott's Magazine (1871). Mitchell concluded by saying that, "no one seems to have examined in these directions any of the cases of people both without limbs. . . . I t would be worth while to learn if these unfortunates possess any consciousness of their missing members." The existence of phantoms of congenitally absent limbs has some implications for our theories of phantom phenomena, especially since they appear never to involve the pain that afflicts most acquired amputees. Many workers have relied on Pick's 1915 report claiming that aplasics d o not experience phantoms, and individuals working with children with such birth defects have not sought evidence to the contrary or have dismissed reports by children. Thus Pick's paper and its second- and third-hand citations have led people in this field astray. Phantom representations of congenitally absent limbs indicate that the neurological substrate of the phantom is at least partly innate. Cutaneous, proprioceptive, and lunesthetic input may facihtate the development of this substrate and appear to be required for pain. Reports of adult amputees experiencing the phantom reproduction of preamputation ingrown toenails and slivers under fingernails show that peripheral stimuli can make an imprint on the phantom. While innate structures may suffice for phantom sensations, early experience may reinforce those mechanisms. Simmel (1966) reported that the incidence of phantoms in children increased as a function of age at amputation, from 20% of those under 2 years, to 100% at 8 years. Weinstein (1969) has found figures concerning congenital cases which fit those data. Poeck's 1964 report shows that congenital phantoms can be vivid and motile, supporting Ronald Melzack's (1989) recent proposal of an innate '6 neuromatrix" underlying the phantom, for which matrix synaptic contacts can be modified by experience. More widespread acknowledgement of phantom limbs in aplasics and young amputees might also lead to better rehabilitative care of such individuals, who may at present often feel they must conceal these perceptual events. REFERENCES

CRONHOLM,B. (1951)

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HABER,W. B. (1958) Reactions to loss of limb: physiological and psychological aspects. Annals ofthe New York Academy ofscience, 74, 14-24. W. R., & SMYTH,G. E. (1948) Phantom limbs. Journal of Neurology and PsyHENDERSON, chiatry, 11, 88-112. HERRMANN, L. B., & G ~ B s E. , W. (1945) Phantom limb pain: its relation to the treatment of large nerves at time of amputation. American Journal of Surgery, 67, 168-180. HOFFMAN,. (1954) Phantom limb syndrome. a critical review of literature. Journal of Nervous an Mental Disease, 119, 261-270. JALAVISTO, E. (1950) Adaptation in the phantom limb phenomenon as influenced by the age of the amputees. Journal of Gerontology, 5, 339-342. KOLB,L. C. (1954) The painful phantom: psychology, physiology and treatment. Springfield, IL: Thomas. KOLB,L. C., & BRODIE,H . K. (1982) Modern clinical psychiatry. (10th ed.) Philadelphia, PA: Saunders. LAMB,D. W., & LAW,H. T. (1987) Upper limb deficiencies in children: prosthetic, orthotic, and surgical management. Boston, MA: Little, Brown. LIVINGSTON, W. K. (1943) Pain mechanisms. New York: Macmillan. ~ ~ E U A CR.K (1989) , Phantom limbs, the self and the brain. Canadian Psychology, 30, 1-16. MITAL, M. A., & PIERCE, D. S. (1971) Amputees and their prostheses. Boston, MA: Little, Brown. MITCHELL,S. W. (1871) Phantom limbs. Lippincott's Magazine, 8, 563-569. NASHOLD, B. S., & FRIEDM~V, H. (1986) Neurosurgical relief of pain. In D. C. Sabiston (Ed.), Textbook of surgery. (13th ed.) Philadelphia, PA: Saunders. Pp. 1413-1418. PICK, A. (1915) Zur Pathologie des Bewussteins vom eigenen Korper: Ein Beitrig aus der Kriegsmedizin. Neurologisches Centralbhtt, 34, 257-265. POECK,K. (1364) Phantoms faowing amputation k early childhood and in congenital absence of limbs. Cortex, 1, 269-275. In A. L. Benton (Ed.), Contributions to POECK,K. (1969) Modern trends In neur~psycholog~. clinical neuronsvcholo~v . -, Chcaeo. 1L: Aldine. Po. 1-29. RESE, W., & BRUCK,G. (1950) Le Aembre chez l'enfant. Revue Neurologique, 83, 221-222. Y., & ROSENFELDER, R. (1983) The limb deficient child. Springfield, IL: Thomas. SETOGUCHI, SIMMEL,M. L. (1956) On phantom limbs. Archives of Neurology and Psychiatry, 75, 637-647. SIMMEL, M. L. (1958) The conltions of occurrence of phantom limbs. Proceedings of the American Philosophical Society, 102, 492-500. SWMEL, M. L. (1959) Phantoms, phantom pain and 'denial.' American Journal of Psychotherapy, 13, 603-613. SWMEL, M. L. (1961) The absence of phantoms for congenitally missing limbs. American Journal of Psychology, 74, 467-470. SIMMEL,M. L. (1962) Phantoms-experiences following amputation in childhood. Journal of Neurology, Neurosurgery and Psychiatry, 25, 69-78. S ~ L M., L. (1966) Developmental aspects of the body scheme. Child Development, 37,

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SOHN,D. L. (1914) The psychic complex in congenital deformity. New York Medical Journal, 100, 959-961. VETTEX,R. J., & WEINSTEM,S. (1967) The history of the phantom in congenitally absent limbs. Neuroprychologia, 5, 335-338. WEINSTEIN,S. (1969) Neuropsychological studies of the phantom. In A. L. Benton (Ed.), Contributions to clinical neuropsychology. Chicago, IL: Aldine. Pp. 73-106. WEINSTEIN,S., & SERSEN,E. A. (1961) Phantoms in cases of congenital absence of limbs. Neurology, 11, 905-911. WEINSTEIN, S., SERSEN,E. A,, & V E ~ RR., J. (1964) Phantoms and somatic sensation in cases of congenital aplasia. Cortex, 1, 276-290. WEISS,A. A. (1956) The phantom limb. Annals oflnternal Medicine, 44, 668-677. WHITE, J. C., & SWEET,W. H. (1955) Pain: its mechanisms and neurosurgical control. Springfield, IL: Thomas.

Accepted May 14, 1990.

Phantom representations of congenitally absent limbs.

It has been widely claimed that phantom limbs are not experienced by children born without the part or who have it amputated in early childhood. This ...
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