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Table of Contents
CASE REPORT
Year : 2019  |  Volume : 16  |  Issue : 1  |  Page : 79-81

Intravenous Immunoglobulin Therapy in Postpoliomyelitis Syndrome


Department of Neurology and Stroke, Azadi Teaching Hospital, Duhok, Kurdistan Region, Iraq

Date of Web Publication18-Mar-2019

Correspondence Address:
Dr. Saad Kazim Karim
Department of Neurology and Stroke, Azadi Teaching Hospital, Nakhoshkhana Road 9, 1014, AM, Duhok, Kurdistan Region
Iraq
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/MJBL.MJBL_116_18

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  Abstract 


Postpoliomyelitis syndrome is a new neurological disorder seen in individuals who survive remote poliomyelitis. An adequate diagnosis is important for treatment and prognosis. The diagnosis of postpoliomyelitis syndrome might be difficult as the diagnosis mostly depends on the patient's subjective description of the symptoms. The diagnostic criteria primarily depend on new and progressive muscle weakness, muscle fatigue, general fatigue, and pain in the absence of other disorders which may be similar to this syndrome. Furthermore, the postpoliomyelitis syndrome patients have reached an age when concomitant diseases are common. The possible etiology is that this entity of paralysis can be exploited by slowly progressive distal neuronal degeneration. Interestingly, this patient had primary upper limb polio paralysis and then new slowly progressive asymmetrical, muscle weakness in the contralateral lower limb. Few clinical trials using human intravenous immunoglobulin (IVIG) and pyridostigmine gave controversy about a specific treatment. In this case, we tried an IVIG therapy, but the weakness was progressively worsening and there was more atrophy in the muscle bulk of the affected muscle group. However, supportive treatment is also important to be planned. The aim of this case report is to describe the complexity of the diagnosis and treatment of such cases of the postpoliomyelitis syndrome in comparison to relevant literature.

Keywords: Intravenous immunoglobulin in postpoliomyelitis, postpoliomyelitis syndrome, weakness


How to cite this article:
Karim SK. Intravenous Immunoglobulin Therapy in Postpoliomyelitis Syndrome. Med J Babylon 2019;16:79-81

How to cite this URL:
Karim SK. Intravenous Immunoglobulin Therapy in Postpoliomyelitis Syndrome. Med J Babylon [serial online] 2019 [cited 2019 Aug 22];16:79-81. Available from: http://www.medjbabylon.org/text.asp?2019/16/1/79/254361




  Introduction Top


Postpoliomyelitis syndrome is a neuromuscular syndrome, usually characterized by new insidious symptoms of increased muscle weakness, pain, and fatigue at least 15 years following the paralysis of poliomyelitis, with a mean interval roughly around 35 years.[1],[2],[3] Postpoliomyelitis syndrome might be difficult to diagnose depending on the patient's own subjectivity since the complexity of the postpoliomyelitis syndrome necessitates taking into consideration and excluding other concomitant disorders such as disc herniation, diabetes mellitus, trauma, and other neuropathic disorders.

The goal of this presentation was to show the complexity of the postpoliomyelitis syndrome diagnosis in exclusion of concomitant disorders and to report that the weakness could affect the lower limb in upper limb-prior polio patient and to use intravenous immunoglobulin (IVIG) to minimize neuronal degeneration.


  Case Report Top


A 45-year-old male from Iraqi Kurdistan, a hospital-laboratory clerk, had polio at the age of 2 years, affecting the right upper limb. On April 2018, he noted to develop new insidious weakness of the left lower limb over 3 months with no sensory symptoms, also associated with asymmetry of the bulk of the thigh muscle. There was no history of trauma or injury or lumbar pain. His right lower limb was not affected. Physical examination revealed atrophy of his left pelvic and thigh muscle. There was fasciculation in the quadriceps and hamstring muscles. The tone was generally hypotonic. Further examination revealed the weakness of Medical Research Council (MRC) grade four out of five. The sensory function to pinprick sensation was intact. The examination of deep tendon reflexes disclosed reduced responses in the right upper limb and the left lower limb. Electromyography (EMG) revealed ongoing denervation neurogenic units in the left lumbosacral roots. Magnetic resonance imaging (MRI) of the lumbosacral spines did not reveal any neurological compression to the spinal cord or to the nerve roots, but MRI of the pelvis shows atrophy of the left pelvic muscle group. There was no medical history of diabetes mellitus, thyroid disease, or alcohol dependence. Blood investigations of free thyroxine level, thyroid-stimulating hormone, venereal disease research laboratory, full blood count, and serum electrolytes were normal.


  Discussion Top


A careful evaluation to diagnose postpoliomyelitis syndrome is mandatory since other concomitant disorders may mimic postpoliomyelitis syndrome. Hence, correct analysis of clinical features and then relevant investigatory tools were applied in this patient to apply a proper treatment. The United States National Institute of Neurological Disorders and Stroke lists the plausible criteria for the diagnosis of postpoliomyelitis syndrome as shown in [Table 1].[4]
Table 1: Diagnostic criteria for Postpoliomyelitis syndrome

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Patients with polio weakness in their legs were twice as likely to complain of the postpoliomyelitis syndrome compared to patients with weakness in their arms. The postpoliomyelitis syndrome can occur in muscles previously affected or clinically unaffected during the acute attack but is more likely to occur in the muscles that were originally affected. Interestingly, this patient had upper limb polio paralysis and then new slowly progressive asymmetrical, muscle weakness in the contralateral lower limb.[3],[5]

Certain risk factors for the development of the postpoliomyelitis syndrome have been identified, including the degree of severity of acute polio paralysis, advanced age of onset of acute polio paralysis, the number of permanent deficits present after recovery, female gender, recent weight gain, and a greater amount of physical activity during stability.[6]

The exact etiology is unknown. Many theories have been proposed, including immunopathogenic mechanisms, neuronal aging, and viral reactivation, but the most accepted hypothesis would be an excessive metabolism demand on surviving giant-sized motor units, which in turn would be brought on by equally excessive use of muscles and then excessive metabolic stress on the remaining prematurely degenerated neurons, thus resulting in a reduction in axonal “sprouting” in muscle fibers.[7]

Electromyogram studies indicate that >50% of the muscle's anterior horn cell population death must occur before clinical weakness can be detected.[3],[8]

The evidence of poliovirus in cerebrospinal fluid in patients with the postpoliomyelitis syndrome has been encountered. Sharief et al.'s study show the presence of only poliovirus immunoglobulin M (IgM) antibodies and they failed to find poliovirus in those patients.[9] Although the cerebrospinal fluid analysis for this patient was normal, unfortunately, the laboratory tool for poliovirus IgM is not available in our hospital.

Apart from physiotherapy and analgesia, up to date, there is no specific medical treatment for the postpoliomyelitis syndrome, even though few clinical trials used human IVIG.[10] Other trials which used pyridostigmine have been conducted, although the controversy was the dominated issue.[11]

Farbu et al.'s pilot study suggested that IVIG could be a therapeutic option. Patients receiving an IVIG reported a significant improvement in pain during the first 3 months, but no change was noted for subjective fatigue and muscle strength.[12]

There is a controversy over the appropriate dose of IVIG and therapeutic interval.[13] In this case we tried to show the beneficial therapeutic effect of human IVIG in a dose of 2 g/kg of body weight (total dose of 130 g divided over 5 consecutive days). The results noted 3 months later that muscle atrophy was progressively worsening in the affected muscles, and the strength of the muscle was Grade 3 MRC scale. Furthermore, EMG finding was approximately the same finding as it was shown before IVIG treatment. The only positive effect experienced by the patient was no more complaint of pain in his left lower limb. Hence, unfortunately, this line of immunotherapy is not promising. Similarly, one of a meta-analysis study stated that IVIG therapy to patients with postpolio syndrome (PPS) was unlikely to produce significant improvements in pain, fatigue, or muscle strength. Thus, based on randomized controlled trials, routinely administering IVIG to patients with PPS is not recommended as a standard treatment in PPS.[13],[14]


  Conclusion Top


Although there is no specific therapy, IVIG failed to give hope to treat the postpoliomyelitis syndrome. Hence, the goal of treatment is directed to physiotherapy, reassurance, and modification of lifestyle. In this case, the primary polio paralysis affected the upper limb, but the weakness of the postpoliomyelitis syndrome affected the lower limb.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Dalakas MC, Elder G, Hallett M, Ravits J, Baker M, Papadopoulos N, et al. Along-term follow-up study of patients with post-poliomyelitis neuromuscular symptoms. N Engl J Med 1986;314:959-63.  Back to cited text no. 1
    
2.
Trojan DA, Cashman NR. Post-poliomyelitis syndrome. Muscle Nerve 2005;31:6-19.  Back to cited text no. 2
    
3.
Jubelt B, Agre JC. Characteristics and management of postpolio syndrome. JAMA 2000;284:412-4.  Back to cited text no. 3
    
4.
March of Dimes Birth Defects Foundation. Post-Polio Syndrome:Identifying Best Practices in Diagnosis and Care. White Plains, NY:March of Dimes Birth Defects Foundation; 2000. Available from: http://www.post-polio.org/edu/pps.html. [Last cited on 2019 Feb 07].  Back to cited text no. 4
    
5.
Khadilkar SV, Yadav RS, Patel BA. Post-polio syndrome. In: Khadilkar SV, editor. Neuromuscular Disorders. Singapore: Springer; 2018. p. 77-82.  Back to cited text no. 5
    
6.
Klingman J, Chui H, Corgiat M, Perry J. Functional recovery. A major risk factor for the development of post-poliomyelitis muscular atrophy. Arch Neuro 1988; 45:645-7.  Back to cited text no. 6
    
7.
Wiechers DO, Hubbell SL. Late changes in the motor unit after acute poliomyelitis. Muscle Nerve 1981;4:524-8.  Back to cited text no. 7
    
8.
Marco Antonio ON, Mariana Pimentel DM, Viviane S, Osvaldo JM, Reny de SA, Gabriel Rodrigues DF, et al. Post poliomyelitis syndrome. Case report. Arq Neuropsiquiatr 2007;65:528-31.  Back to cited text no. 8
    
9.
Sharief MK, Hentges R, Ciardi M. Intrathecal immune response in patients with the post-polio syndrome. N Engl J Med 1991;325:749-55.  Back to cited text no. 9
    
10.
Bertolasi L, Frasson E, Turri M, Gajofatto A, Bordignon M, Zanolin E, et al. Arandomized controlled trial of IV immunoglobulin in patients with post-polio syndrome. J Neurol Sci 2013; 330:94-9.  Back to cited text no. 10
    
11.
Horemans HL, Nollet F, Beelen A, Drost G, Stegeman DF, Zwarts MJ, et al. Pyridostigmine in postpolio syndrome: No decline in fatigue and limited functional improvement. J Neurol Neurosurg Psychiatry 2003;74:1655-61.  Back to cited text no. 11
    
12.
Farbu E, Rekand T, Vik-Mo E, Lygren H, Gilhus NE, Aarli JA, et al. Post-polio syndrome patients treated with intravenous immunoglobulin: A double-blinded randomized controlled pilot study. Eur J Neurol 2007;14:60-5.  Back to cited text no. 12
    
13.
Elovaara I, Apostolski S, van Doorn P, Gilhus NE, Hietaharju A, Honkaniemi J, et al. EFNS guidelines for the use of intravenous immunoglobulin in treatment of neurological diseases: EFNS task force on the use of intravenous immunoglobulin in treatment of neurological diseases. Eur J Neurol 2008;15:893-908.  Back to cited text no. 13
    
14.
Huang YH, Chen HC, Huang KW, Chen PC, Hu CJ, Tsai CP, et al. Intravenous immunoglobulin for postpolio syndrome: A systematic review and meta-analysis. BMC Neurol 2015;15:39.  Back to cited text no. 14
    



 
 
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