Case Report

Ann Rehabil Med 2013;37(6):901-906

pISSN: 2234-0645 • eISSN: 2234-0653
http://dx.doi.org/10.5535/arm.2013.37.6.901 Annals of Rehabilitation Medicine

Atypical Supernumerary Phantom Limb and

Phantom Limb Pain in a Patient With Spinal
Cord Injury: Case Report
Ja Young Choi, MD1, Hyo In Kim, MD1, Kil Chan Lee, MD2, Zee-A Han, MD3
1
Department of Physical Medicine and Rehabilitation, National Health Insurance Corporation Ilsan Hospital, Goyang;
2
Department of Rehabilitation Medicine, Yonsei University College of Medicine, Seoul;
3
Department of Spinal Cord Injury Rehabilitation, National Rehabilitation Center, Seoul, Korea

Supernumerary phantom limb (SPL) resulting from spinal cord lesions are very rare, with only sporadic and
brief descriptions in the literature. Furthermore, the reported cases of SPL typically occurred in neurologically
incomplete spinal cord patients. Here, we report a rare case of SPL with phantom limb pain that occurred after
traumatic spinal cord injury in a neurologically complete patient. After a traffic accident, a 43-year-old man
suffered a complete spinal cord injury with a C6 neurologic level of injury. SPL and associated phantom limb pain
occurred 6 days after trauma onset. The patient felt the presence of an additional pair of legs that originated at
the hip joints and extended medially, at equal lengths to the paralyzed legs. The intensity of SPL and associated
phantom limb pain subsequently decreased after visual-tactile stimulation treatment, in which the patient visually
identified the paralyzed limbs and then gently tapped them with a wooden stick. This improvement continued
over the 2 months of inpatient treatment at our hospital and the presence of the SPLs was reduced to 20% of the
real paralyzed legs. This is the first comprehensive report on SPLs of the lower extremities after neurologically
complete spinal cord injury.

Keywords Phantom limb pain, Spinal cord injury, Tetraplegia, Neuropathic pain

INTRODUCTION described after amputation of the extremities [1]. In nu-

merous neurologic diseases including stroke, epilepsy,
Phantom limbs are persistent postural and motor ex- and spinal cord injury (SCI), a slightly altered form of the
periences of the limb after physical loss and are usually phantom limb with distinctive experiences of an addi-
tional limb can occur. This phenomenon is much less fre-
Received January 31, 2013; Accepted May 20, 2013 quent than phantom limb sensations that occur after am-
Corresponding author: Zee-A Han putation or various lesions of the neuraxis, and is called
Department of Spinal Cord Injury Rehabilitation, National Rehabilitation
Center, 58 Samgaksan-ro, Gangbuk-gu, Seoul 142-884, Korea supernumerary phantom limb (SPL). By definition, SPL
Tel: +82-2-901-1802, Fax: +82-2-901-1591, E-mail: [email protected] refers to the awareness of an illusory extra limb in addi-
This is an open-access article distributed under the terms of the Creative tion to the real regular limbs [1]. The symptom has been
Commons Attribution Non-Commercial License (http://creativecommons.
org/licenses/by-nc/3.0) which permits unrestricted noncommercial use, described in a variety of different neurologic disorders,
distribution, and reproduction in any medium, provided the original work is
properly cited. with right hemispheric stroke being the most common.
Copyright © 2013 by Korean Academy of Rehabilitation Medicine SPL resulting from spinal cord lesions are extremely
Ja Young Choi, et al.

rare. The first description of supernumerary limbs in Functional Independence Measure showed intact cogni-
traumatic SCI was made in 1975 and involved a C5 SCI tion. Other than a past history of type 2 diabetes mellitus,
patient who had illusory awareness of an extra pair of no other significant medical history including psychiatric
legs [2]. However, the mechanisms underlying SPL are history existed. The patient also had no history of drug or
unknown, and although the clinical significance is also alcohol misuse.
yet to be described, the subjective reality of the addi- According to the International Standards for Neurologi-
tional limbs cause considerable distress in the afflicted cal Classification of Spinal Cord Injury, the patient had
patients. Here, we report a rare case of SPL with phantom a complete lesion (American Spinal Injury Association
limb pain in a patient with SCI with no prior history of impairment scale A [ASIA A]) with a C6 neurologic level
psychiatric disease. This is the first comprehensive report of injury. According to neurologic exams performed 6
on a SPL of the lower extremities after complete SCI. days after injury, the initial upper extremity motor score
was 27 and the lower extremity motor score was 0. These
CASE REPORT scores improved to 31 and 0, respectively, at 115 days af-
ter injury when he was transferred to our hospital. Total
A 43-year-old man was admitted to our hospital for light touch scores were initially 21 for the right side and
intensive rehabilitation 115 days after a traffic accident. 21 for left side and improved to 28 and 29, respectively,
Cervical computed tomography (CT) and magnetic reso- at 115 days follow-up. Total pin prick scores were initially
nance imaging (MRI) on the day of the injury had shown 23 for the right side and 24 for the left side, but improved
a C6 vertebral body fracture as well as a cervical cord to 27 and 27, respectively on follow-up. There was no in-
signal change on T2-weighted MRI images (Fig. 1A). He terval change in the neurologic exam at 6 months follow-
had undergone a C5-6-7 lateral mass fusion on the day up. Electrodiagnostic studies performed 2 months after
of the injury. Six days after injury, he was transferred to injury revealed complete myelopathy and bilateral sym-
the rehabilitation department. A subsequent C5-6-7-T1 metric peripheral sensorimotor polyneuropathy. Due to
anterior and posterior fusion was additionally performed concurrent diabetes related peripheral polyneuropathy,
35 days after injury due to spinal instability detected on sensory nerve conduction studies were done, which
follow-up cervical spine X-ray (Fig. 2). There was no con- showed low amplitudes in bilateral ulnar, superficial pe-
comitant brain injury on brain CT scans and all cogni- roneal, and sural nerves. However, conduction velocities
tive evaluations including cognitive score subsets of the of all examined nerves and bulbocavernosus reflex laten-

Fig. 1. Neuroimaging of the spinal cord on the day of injury. Magnetic resonance imaging scan T2-weighted sagittal
view (A), showing cervical cord signal change (arrow). Computed tomography scan sagittal (B) and axial views (C), re-
vealing C6 vertebral body fracture.

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Fig. 2. Cervical spine X-ray anterior-posterior view (A) Fig. 3. Schematic representation of the phantom limb.
and lateral view (B) were taken after first operation, C5- Illusory lower limbs originate from the pelvic joints and
6-7 lateral mass fusion, performed on the day of injury. extending at normal length medially to the patient’s par-
Cervical spine X-ray anterior-posterior view (C) and lat- alyzed legs.
eral view (D) were taken after the second operation, C5-
6-7-T1 anterior and posterior fusion, performed 35 days
after injury. prominence in the supine position and waning during
sitting. The patient reported that he could feel but could
cies were within normal range. Median somatosensory not see the additional limbs and was aware that this
evoked potentials (SEP) stimulating the median nerves feeling was an illusion. Visualization of his legs did not
at the wrist bilaterally showed prolonged N20 latency in diminish or alter the intensity of SPL sensations. The su-
both sides. Tibial and pudendal SEPs were un-evoked pernumerary legs were also associated with severe lanci-
with stimulations to the tibial nerves at the ankle and pu- nating pain that measured 8 points on the numeric rating
dendal nerve at penile shaft. scale. The patient also complained of severe neuropathic
SPL sensations appeared 6 days after trauma onset. pain of the paralyzed legs that worsened when the SPLs
The patient felt the presence of an additional pair of legs were in the flexed position. However, he reported that he
that originated at the hip joints and extended medially, had no voluntary control over the phantom limbs and the
at equal lengths to the paralyzed legs (Fig. 3). The patient movements of the SPLs were unintentional. Psychiatric
described the presence of both knee and ankle joints in evaluation showed no evidence of delusion, hallucina-
an extended position. SPL perceptions persisted through- tion or any other specific psychiatric abnormality, and
out the day but its intensity fluctuated with more vivid- the patient showed good insight regarding his various
ness during the afternoon and evening. Body posture clinical status related to his SCI. The intensity of the SPLs
also altered the intensity of SPL sensations with more was equal to his paralyzed limbs.

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Combinations of oral gabapentin, pregabalin, baclofen, conducted 13 months post-trauma, the patient reported a
tramadol, and duloxetine improved the neuropathic pain telescoping pattern of the SPL with total length shorten-
in both the real and supernumerary limbs that measured ing to levels half of the original. However, although short-
6 points on the numeric rating scale, but failed to affect er in length, the knee and ankle joints were still present
changes in the intensity of the SPLs. and the limb continued to remain in the extended posi-
The patient underwent visual-tactile stimulation treat- tion. Although SPL sensation and subsequent total motor
ment where he would first visually identify his paralyzed and sensory scores at 6 months follow-up improved, mo-
limbs and then gently tap his legs with a wooden stick tor and sensory scores pertaining to the lower extremities
(Fig. 4). Although his legs were anesthetic to both pain as well as the ASIA A neurologically complete injury state
and light touch, tactile stimulation evoked painful tin- remained unchanged. Additional lower limb SEP studies
gling sensations in his paralyzed legs. Thus, he was able and imaging studies were not preformed due to patient
to receive both visual and sensory feedback and recon- refusal. However, considering the lack of change in both
firm the existence of his paralyzed legs. Wooden stick motor and sensory scores as well as ASIA grades, these
stimulation also evoked painful tingling in the SPLs but studies did not appear to be clinically significant or es-
visually he was able to recognize the absence of the SPLs sential.
and thus differentiate the sense as erroneous. Visual-
tactile stimulation was performed three times daily for DISCUSSION
10–15 minutes per session with approximately one tap
per second. The patient was then asked to rate the inten- Phantom limb phenomenon occurs in as many as
sity of the SPLs on a 10-point numeric analog scale rela- 90%–100% of amputation cases [3]. However, such sen-
tive to the paralyzed real limb. The presence of the SPLs sory alterations of deafferented limbs, which also occur
were felt at an equal intensity to the real limbs initially in SCI patients, rarely manifest as a phantom limb. The
but improved to half the intensity of the real paralyzed SPL phenomenon is a subjective sensation of additional
limbs after 8 days of daily visual-tactile stimulation. This limbs that have occasionally been reported in central
improvement continued over the 2 months of inpatient nervous system disorders [4]. Cortical damage or subcor-
treatment at our hospital and the presence of the SPLs tical lesions have been associated with SPL, but SPL after
was reduced to 20% of the real paralyzed legs. spinal cord lesions have been extremely rare.
Nine months after the SCI, the patient reported persis- Supernumerary limbs in SCI was first mentioned in
tence of the illusory limb sensations, but the sensations 1975 [2], and only sporadic and brief descriptions have
had improved to an extent where it no longer affected the been made in the literature since [5]. The latter review
patient’s quality of life. Through a telephone interview reported four cases with three of the cases occurring in
cervical incomplete SCI patients [5].
Although phenomenologically similar, phantom sensa-
tions occurring after limb loss or after SCI and those felt
after cerebral lesions may differ in several aspects. Pain
is frequently associated with phantom limbs after ampu-
tation, but rarely accompanies SPLs [6]. Moreover, SPLs
after brain damage may be associated with cognitive
deficits, delusion or brief episodic seizures [7]. Although
amputation was not the triggering event for SPL in our
case, clinical manifestations were similar to amputation
related phantom pain in that there was debilitating pain
of the SPL in the absence of any consequences related to
brain damage.
Fig. 4. Visual-tactile stimulation treatment. The patient
would first visually identify his paralyzed limbs and then Head and Holmes [8] were the first to systematically
gently tap his legs with a wooden stick. study bodily perception or body schema and hypoth-

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esized that spatial perceptions of the body are achieved Despite the many hypotheses, the underlying neural
through central integration of incoming afferent infor- mechanisms of SPLs in spinal cord injury are not fully
mation from the periphery and updated through succes- understood.
sive changes in position. Thus, body schema is achieved Phantom limb pain is a challenging condition and stan-
through interplay between sensory feedback from the pe- dardized treatment protocols for SPLs are not available.
ripheral nervous system or periphery and image formu- Clinicians have outlined the fact that phantom limb pain
lation originating from the non-dominant parietal lobe is difficult to treat in comparison to conventional pain.
and other central brain structures [8]. The critical clue for treatment is considering possible
SPLs and phantom limb sensations are thought to be a roles of the visual system in the elicitation of this percep-
disruption of such normal bodily perceptions. SPLs are tive mismatch. Some SPLs are reported as not seen and
not infrequently reported after brain pathology and the not influenced, either by visual feedback of the actual
main postulation is thought to be due to reorganization limbs or direct touch of the actual limb. But, in certain
of the primary somatosensory cortex and subsequent de- patients, the SPL or their associated features such as pain
terioration of normal body schema [9]. were eliminated through visual feedback and tactile stim-
However, the literature suggests a large spectrum of ulation of the real limb [6]. Distressing intensities of SPL
features of various types of SPLs differing by the degree of sensation in our patient also improved through a regi-
motor controllability (impossible, automatic, intentional men of visual-tactile stimulation. Although neuropathic
movements), sensitivity to sensory feedback (visual, pain of the paralyzed and SPLs improved with medica-
tactile, proprioceptive), and patients’ mental framework tion only stimulation treatment was effective in improv-
(confused, delusional, indifferent) [9]. In this respect, it ing the intensity of the SPLs. Specific treatment protocols
appears inappropriate to expect a single explanation, and have not yet been developed. Therefore, visual-tactile
mechanisms may involve both psychological and organ- stimulation treatment may be effective in improving the
ic. intensity of the SPLs in future patients. This treatment
Other classic explanations, which may be more appro- tool has the advantage of being convenient to apply and
priate to our case, emphasize the dissociation between to carry out in both lower cervical SCI and paraplegia pa-
the previously established sensorimotor representa- tients. However, further studies need to be done to reveal
tions and the lesion-induced change in communication basic neurophysiological mechanisms of this interesting
between the brain and the paralyzed limb [7]. In other phenomenon and find effective measures to treat SPLs.
words, these illusory phenomena might be provoked by The patient reported here had the classic features of
loss of afferent information due to sensory impairment SPL. The phantom perception was vivid, continuous and
and failure to correctly integrate multimodal and mo- not visualized, and the symptoms developed without any
tor information to generate a normal experience of self- underlying cognitive or psychological dysfunction. Thus,
location [10]. Just as destruction of the sensory roots an organic cause rather than a psychodynamic explana-
often leads to the phenomenological experience of a tion was more appropriate in explaining the SPL in this
supernumerary limb, severe sensory and proprioceptive patient.
loss may erroneously re-program the innate body im- Though there have been other case reports of supernu-
age into constructing an illusory limb. In amputees and merary limbs in SCI patients, this case is prominent in
SCI patients, the ability to command motor function of many aspects. First, this case is the first to comprehen-
the limbs is either physically lost or neurologically dis- sively report on SPL after complete SCI. Almost all report-
connected. Hence, mismatch between central (cortical) ed case reports of SPL in SCI were associated with incom-
movement and sensory feedback from the peripheral plete injury, except one single sentence mentioning of a
nervous system makes it difficult to distinguish between case in 1975. Since the previous case was associated with
expected movements of the actual limbs with the unex- incomplete SCI injury, the SPL sensation vanished with
pected movement of illusory limbs. Another relatively subsequent neurologic recovery. However, the patient
simple mechanism to explain SPL in SCI may be through in this case experienced SPLs in both lower extremities
subcortical brain reorganization following SCI. despite the neurological completeness of the SCI lesion.

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those of amputees. Fourth, visual-tactile stimulation 2011;49:588-95.
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2002;94:476-8.
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Grossi, et al.’s (2002) spare thoughts on spare limbs.
No potential conflict of interest relevant to this article Percept Mot Skills 2003;97:3-10.
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