Vol.1, No.1, 1-3 (2013) Pain Studies and Treatment
Case report: Effect of immunoglobulin on pain in
Post-Polio SyndromeThree case reports
Lars Werhagen*, Kristian Borg
Division of Rehabilitation Medicine, Department of Clinical Sciences, Karolinska Institutet, Danderyd University Hospital,
Danderyd, Sweden; *Corresponding Author: lars.werhagen@ki.se
Received 8 February 2013; revised 11 March 2013; accepted 3 April 2013
Copyright © 2013 Lars Werhagen, Kristian Borg. This is an open access article distributed under the Creative Commons Attribution
License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Study design: Case reports. Setting: University
hospital setting. Objective: To analyze the effect
of intravenous immunoglobulin on neuropathic
and nociceptive pain in three patients with Post-
Polio Syndrome (PPS). Materials and Methods:
Three patients with PPS and pain who received
treatment with 90 g IvIg are described. Results:
Before treatment one of the patients had pure
neuropathic pain and the other two had a com-
bination of neuropathic and nociceptive pain.
There was no effect on pain in the patient with
pure neuropathic pain and only effect on the
nociceptive pain in the patients with a combina-
tion of neuropathic and nociceptive pain. Dis-
cussion: Pain is one of the most common sym-
ptoms in PPS. Previous studies have shown an
effect on pain in PPS patients receiving IvIg. The
results of the present study point to that the ef-
fect on pain is limited to nociceptive pain and
that there is no effect on neuropathic pain which
leads to increased knowledge of characteriza-
tion of responders of Iv Ig treatment. Conclusion:
IvIg treatme nt tr eatment reduce s no cicept iv e but
not neuropathic pain in PPS patients.
Keywords: Post-Polio Syndrome; Pain;
Immunoglobulin Treatment
Pain is common in patients with Post-Polio Syndrome
(PPS) and is present in a majority of patients [1-5]. The
pain in PPS is in most cases nociceptive to its character
but when neuropathic pain is present a concomitant dis-
ease must be suspected [3]. PPS patients in the western
world have reached an age where concomitant neurolo-
gical disorders more often occur. Thus, neuropathic pain
is anticipated to increase in PPS patients the coming
years. Furthermore, pain is more common among fe-
males than in males and the intensity of pain according
to the VAS scale, as reported by PPS patients, is often
high [3,6]. In a pr evious study we were able to show th at
treatment with intravenous immunoglobulin (IvIg) was
effective and reduced nociceptive pain in seven out of
ten PPS patients [7]. IvIg treatment, however, is costly
and it is therefore of importance to thoroughly character-
ize responders for the treatment.
In this case report we describe three patients with PPS
and pain of different origin, neuropathic and nociceptive,
who reacted differently to IvIg. Thus, this gives more
information to the background of the effect of IvIg in
PPS, particularly for pain, and increase the knowledge of
the different clinical features of responder s on IvIg.
A description of the patients appears in Ta bl e 1 . PPS
was defined according to the criteria of March of Dimes
[8]. Before acceptance for treatment all three patients
underwent a thorough neurological and general examina-
tion to confirm the PPS diagnosis. Medical investigation
revealed no diagnosis constitu ting exclusion of treatment
with IvIg in any of the patients. Immediately before and
six month after treatment the patients underwent an ex-
amination by the physician respon sible for the treatment,
one of the authors. At the same time they were inter-
viewed according to a specific questionnaire about the
presence of pain, its intensity, character and localization,
their social and working situation, walking aids, paresis
and its localization and the presence of concomitant dis-
Pain was classified according to the International As-
sociation for the Study of Pain (IASP). A pain was clas-
sified as neuropathic if present in an area with sensory
disturbances to pin-prick and light touch and without
relation to joint movements and/or infection. A pain was
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L. Werhagen, K. Borg / Pain Studies and Treatment 1 (2013) 1-3
Table 1. Gender, age at polio infection, actual age, marital and working status, pares at its localization, the use of walking aids and
concomitant diseases in the three PPS patients who underwent treatment with intravenous immunoglobulin (IvIg).
number Gender Age at
polio Age at
treatment Marital
status Working
status Paresis Walking
aids Concomitant disease
1 Female 1 year 58 years Married Retired Legs No Cervical disc herniation
2 Female 5 years 65 years Divorced Retired Legs 2 crutches Lumbar disc herniation
3 Female 5 years 63 years Married Retired Legs No Polyneuropathy
classified as nociceptive when aching in an area with
signs of inflammation and painful joint movements were
described. The intensity of the pain was measured with
the Visual Analogue Scale (VAS) 0 - 100 before and after
treatment. The patients received a total of 90 g of IvIg
(Xepol, Grifols, Barcelona, Spain) during three consecu-
tive days.
3.1. Case Number 1
A 58-year-old married and retired woman. She con-
tracted poliomyelitis at the age of one year. Initially she
suffered from paresis and weakness of her left leg. She
recovered and lived a normal life. Fifteen years previous
to this study she ex perienced weakness of her legs. Neu-
rological examination revealed a light to moderate pare-
sis of both legs but she was able to walk unaided. She
was diagnosed with PPS. In addition, she suffered from
severe pain in her right arm. This pain was classified as
neuropathic and the pain intensity before treatment was
80/100 (Table 2). Investigation with MRT revealed a
cervical disc hernia.
She had no effect on her pain after IvIg treatment and
was referred to a pain clinic.
3.2. Case Number 2
A 65-year-old retired and divorced woman. She con-
tracted poliomyelitis at the age of 5 years. She was ini-
tially paretic in both legs but was, however, able to live a
normal life and walked without aids. Around ten years
previous to this study she became weaker in her legs and
she was diagnosed with PPS. She walked with two crut-
ches and for longer distances she used a wheel-chair. In
1983 she underw e nt surgery due to a disc hernia at L4/L5
level and was re-operated in 1993. She suffered from
pain in her left leg classified as neuropathic and pain in
her feet, back and arms classified as nociceptive. After
treatment with IvIg the pain decreased from VAS 75 to
25. Her neuropathic pain was only slightly decreased.
3.3. Case Number 3
A 63-year-old retired and married woman. She con-
tracted poliomyelitis at the age of 5 years. She recovered
fully but 20 years previous to this study she experienced
problem with balance and weakness of the legs. However,
she was able to walk with out aids. At the post-polio o ut-
patient clinic she was diagnosed with PPS. 2002 she w as
diagnosed with polymyalgia rheumatica, and polyneuro-
pathy on a vascular basis. She underwent treatment with
prednisolone. When examined she suffered from a pain
in her lower legs classified as neuropathic and pain in
back and thighs classified as nociceptive. She had under-
gone treatment with Gabapentin for her neuropathic pain
without any effect. The intensity of pain according to
VAS was 40 - 90/100 before and 15 - 40/100 after IvIg
treatment. Six month after treatment her pain was classi-
fied as pure neuropathic pain.
The three patients of the present study demonstrate
that IvIg was effective when pain with classified as no-
ciceptive but not when classified as neuropathic. In all
three patients a concomitant disease was present, in two
patient’s disc herniation and in the third patient the back-
ground to the neuropathic pain was polyneuropathy. This
point to that the effect of IvIg in PPS is not primarily
directed to pain but to the background to the pain. The
background for the neuropathic pain is a direct effect on
nervous structures either due to an external pressure as in
disc herniation or due to metabolic factors as in poly-
neuropathy. However, the background for the nociceptive
pain is not clear. Pain from joint and muscles is a com-
mon complaint in PPS patients. However, the pain has
not been analyzed thoroughly. One possible background
for nociceptive pain in PPS may be inflammation. An in-
flammatory process in PPS has been described both in
cerebrospinal fluid and in peripheral blood [4,9-12]. IvIg
has been reported to decrease the inflammatory process
and in parallel a clinical effect including decrease of pain
was found [4,10-14]. Furthermore, Melin et al. (K. Borg
2012, personal communication) showed an increase of
prostaglandin antibodies in blood vessels of muscle in
PPS patients which may be one underlying cause of
muscle pain in PPS. Thus, one might speculate that the
finding of the present study, a decrease of nociceptive
pain as a result of IvIg treatent, is due to the dampen- m
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L. Werhagen, K. Borg / Pain Studies and Treatment 1 (2013) 1-3 3
Ta bl e 2 . Pain its character and intensity according to the Visual Analogue Scale (VAS) before and after treatment with intravenous
immunoglobulin (IvIg) in the three PPS patients.
Patient number Classification of pain before
treatment VAS before treatment Classification of pain after
treatment VAS after
1 Neuropathic 80 Neuropathic 80
2 Nociceptive neuropathic 75 Neuropathic 25
3 Nociceptive neuropathic 40 - 90 Neuropathic 15 - 40
ing of an inflammatory reaction. In order to confirm
these studies with larger patient materials are required.
Besides the low number of patients reported in th e pre-
sent study this study can be critized in other ways. The
classification of pain is difficult and the patients are in
age when concomitant diseases are commonly found.
However, the patients were before and after treatment
examined by the same physician and interviewed ac-
cording to a structured questionnaire and no other con-
comitant disorder was found. However, with the back-
ground of the results of this and other studies it is of im-
portance to further analyze pain in PPS adequately and to
further develop criteria for selection of PPS for treatment
with IvIg based on clinical and molecular data.
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