Case report: Effect of immunoglobulin on pain in Post-Polio Syndrome—Three case reports

doi:10.4236/pst.2013.11001

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Vol.1, No.1, 1-3 (2013) Pain Studies and Treatment

http://dx.doi.org/10.4236/pst.2013.11001

Case report: Effect of immunoglobulin on pain in

Post-Polio Syndrome—Three case reports

Lars Werhagen*, Kristian Borg

Division of Rehabilitation Medicine, Department of Clinical Sciences, Karolinska Institutet, Danderyd University Hospital,

Danderyd, Sweden; *Corresponding Author: [email protected]

Received 8 February 2013; revised 11 March 2013; accepted 3 April 2013

License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

ABSTRACT

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

1. INTRODUCTION

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.

2. METHODS

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-

orders.

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

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).

Patient

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. CASE REPORTS

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.

4. DISCUSSION

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

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

treatment

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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