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a severe exacerbation of Chronic inflammatory demyelinating polyneuropathy - post total knee replacement

Igor Policinski, Paul N Smith
Trauma & Orthopaedic Research Unit
Department of Surgery
The Canberra Hospital

Address for Correspondence:
Igor Policinski
Trauma & Orthopaedic Research Unit
Department of Surgery,The Canberra Hospital
Yamba Drive,Garran, ACT, 2605


Chronic inflammatory demyelinating polyneuropathy (CIDP) is a neurological disease affecting peripheral sensory and motor neurons about 2 per 100,000 adults.  We present a case of a woman with CIPD who underwent total knee arthroplasty and her postoperative course was complicated by a severe life threatening exacerbation of her neurologic condition. She required intubation and respiratory support in an intensive care unit before recovering post IV immunoglobulin and steroid treatment. Based on this case, we advocate preemptive dosing with either immunoglobulins or steroids to minimise the risk of a potentially life threatening exacerbation of CIPD post elective surgery. 

J.Orthopaedics 2009;6(2)e12


Chronic inflammatory demyelinating polyneuropathy; CIDP; total knee replacement; TKR



We present a case report of a woman with severe osteoarthritis and a chronic inflammatory demyelinating polyneuropathy. She underwent arthroplasty and her postoperative course was complicated by a severe life threatening exacerbation of her neurologic condition. Treatment with emergent respiratory support, intravenous immunoglobulins and corticosteroids was successful.


Case Report:

A 74-year-old lady presented to an orthopaedic surgeon with complaints of severe pain due to osteoarthritis of her right knee.  She had a past history of chronic inflammatory demyelinating polyneuropathy.

A neurologist had been consulted 3 years prior, because of the appearance of sensory disturbances in her fingers and toes, and impaired balance.  Her sensory symptoms had gradually worsened over several years. A previous urinary tract infection and an episode of diverticulitis had both led to transient exacerbation of symptoms. Post these illnesses she suffered multiple falls and noted proximal leg weakness, needing a walking frame to mobilise. Once these episodes had resolved, she regained strength and was able to discard the walking aid.  Apart from osteoarthritis and the above recent health events, her past medical history included mild hypertension, controlled ischemic heart disease and gastroesophageal reflux.

Her initial neurological examination revealed evidence of a motor and sensory polyneuropathy. Her gait was slightly ataxic. There was slight ptosis on the right, otherwise a normal cranial nerve examination.  Wasting and weakness was noted in the thenar, hypothenar and interosseous muscles bilaterally. Her toes were slightly clawed and she had prominent plantar arches. Upper limb reflexes were present but knee and ankle jerks were absent. The Babinski test was negative bilaterally. Sensation to pin prick, light touch and vibration was impaired in a glove and stocking distribution. There was evidence of dyscoordination with inaccurate “finger to nose” and “heel-knee-shin” tests.

Her screening electrolyte and haematologic blood tests were unremarkable. Nerve conduction studies showed that there was evidence of peripheral neuropathy, with velocity in the demyelinating range (range 29 – 39M/s).

She began treatment with a course of intravenous immunoglobulins (Intragam®, 0.4g per Kg), which led to a significant clinical improvement. Her balance and strength improved, she was walking normally and her sensation to her fingers and toes returned. Attempts were made to wean from the Intragam®, however she had a chronically relapsing clinical course and required monthly dosing to control the disease.

Assessment and investigation by the orthopaedic surgeon revealed severe degenerative joint disease of the right knee. As her pain was insufficiently relieved by analgesics and anti-inflammatories, she elected to undergo right total knee replacement.

The elective procedure went smoothly without any technical or anaesthetic problems.  After the operation she had a severe flare of her CIDP. She progressively developed slurred speech but no obvious dystonia of her tongue or oropharyngeal muscles. Cranial nerves were normal to examination. She had an intermittently flaccid four limb paresis and her lower limb reflexes were absent.  Sensory examination was difficult and inconsistent.

Three days post surgery she developed difficulty breathing and a paradoxical respiratory pattern.  An arterial blood gas showed that she was hypoxic (60.9 mmHg) and hypocapnic (24.9 mmHg), with a negative base excess of 11.0 and a pH of 7.375.  She was not significantly anaemic and Troponin was negative. A CT pulmonary angiogram was normal.  Unable to maintain spontaneous breathing, she was transferred to the intensive care unit where she was sedated and intubated.  Nerve conduction studies revealed a marked worsening of her peripheral neuropathy (range 20 – 36M/s).  She was investigated for myasthenia gravis, with acetylcholine receptor antibody of <0.20nmol/L.  Serum protein analysis showed a decreased total protein 57 g/L (n: 60 – 80g/L), albumin level of 27.5g/L (n: 35.0 – 50.0g/L) and a slight increase in Alpha 1 protein 4.6g/L (n: 1.0 – 3.0g/L).

She was administered an Intragam® infusion (0.4g /kg) and commenced on prednisolone 75mg daily.  She received a total of 12 doses of prednisolone. Her symptoms recovered dramatically with return of strength in upper and lower limbs and she was able to spontaneously and independently breathe. She was extubated after four days and was continued on a monthly infusion of Intragam®. Seventeen days after her procedure she was discharged home. At six week review she had returned to her pre-operative neurological status, had achieved an excellent range of knee motion and was mobilising satisfactorily.

Discussion :

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a neurological disease affecting peripheral sensory and motor neurons, causing progressive sensory loss and weakness, secondary to a cellular and humoral autoimmune medicated reaction towards peripheral nerve antigens1.  The prevalence is about 2 per 100,000 in adults with a mean age of onset at around 47 years of age, increasing with age1.  The course of the disease can be monophasic, relapsing (14%) or progressive (45%), over months or years 1,2.

Typical clinical presentation is a symmetrical, proximal and distal muscle weakness progressing for more than two months.  Pain, ataxia and facial weakness can also be encountered.  There are absent or diminished deep tendon reflexes, signs of demyelination on nerve conduction studies, increased levels of protein in cerebrospinal fluid (CSF) and nerve biopsies show axonal demyelination3.

Differential diagnosis include Guillain-Barré syndrome (GBS), inherited demyelinating polyneuropathies, metabolic neuropathies (associated with diabetes, uraemia acromegaly, amyloidosis and hypothyroidism), paraneoplastic neuropathies, multifocal motor neuropathy, neuropathies associated with IgM, IgG or IgA monoclonal gammopathies, infections with the Human immunodeficiency virus, Lyme disease or Hepatitis C, Sjögren’s Syndrome and inflammatory bowel disease 1,2.

Diagnosis is based on a widely used criteria set out by Saperstein et al.1 and the Inflammatory Neuropathy Cause and Treatment Group 1, which incorporate clinical features and electrophysiological studies.  The American Academy of Neurology also recommends obtaining CSF and nerve biopsies to confirm the diagnosis. The fundamental clinical features are the duration of symptoms and progressive nature, extending for more than 2 months, differentiating this condition from GBS.

There is no definitive treatment in the current literature and a positive response to treatment is achieved in 70% at best6.  Recognised and successful treatments for CIDP that are currently being used are intravenous immunoglobulins, plasma exchange and corticosteroids.  Other therapy includes immunosuppressive medications, such as azathioprine, cyclophosphamide, cyclosporine, and interferon therapy1.

The occurrence of a sudden and life threatening neurologic deterioration in a patient with inflammatory neuropathy following arthroplasty (or other surgery) has to our knowledge not been previously reported. A fluctuating but progressive course is not uncommon in this condition. Despite considering the possibility of a relapse associated with the surgery we could find no reports of such in the published literature and hence elected to proceed with surgery in a standard fashion. It is conjectural as to whether preemptive dosing with either immunoglobulins or steroids would have prevented the episode. Given this near death experience for our patient we would advocate preemptive treatment for individuals with CIDP coming to elective surgery.

Reference :

  1. Kieseier  BC, Kiefer R, Gold R, et al.  Advances in understanding and treatment of immune-mediated disorders of the peripheral nervous system. Muscle Nerve 2004; 30:131-156.

  2. Mcleod JG, Pollard JD, Macaskill P, Mohamed A, Spring P, Khurana V.  Prevalence of chronic inflammatory demyelinating polyneuropathy in New South Wales.  Ann Neurol 1999; 46:910-913.

  3. Bouchard C, Lacroix C, Planté, et al.  Clinicopathologic findings and prognosis of chronic inflammatory demyelinating polyneuropathy.  Neurology 1999; 52(3):498-503.

  4. McCombe PA, Pollard JD, McLeod JG.  Chronic inflammatory demyelinating polyradiculoneuropathy.  A clinical and electrophysiological study of 92 cases.  Brain 1987; 110:1617-1630.

  5. Van Schaik IN, Winer JB, De Haan R, Vermeulen M. Intravenous immunoglobulin for chronic inflammatory demyelinating polyradiculoneuropathy. Cochrane Database Syst Rev 2002;2:CD001797

  6. Köller H, Kieseier BC, Jander S, et al. Chronic Inflammatory Demyelinating Polyneuropathy.  N Engl J Med 2005; 352(13):1343-1356.

  7. Saperstein DS, Katz JS, Amato AA, et al.  Clinical spectrum of chronic acquired demylinating polyneuropathies.  Muscle Nerve 2001;24:311-324.

  8. Hughes R, Bensa S, Willison H, et al.  Randomized controlled trial of intravenous immunoglobulin versus oral prednisolone in chronic inflammatory demyelinating polyradiculopathy.  Ann Neurol 2001;50:195-201

  9. Kuntzer T, Radziwill AJ, Lettry-Trouillat R, et al. Interferon-beta1a in chronic inflammatory demyelinating polyneuropathy. Neurology 1999;53:1364-1365.


This is a peer reviewed paper 

Please cite as: Igor Policinski: A severe exacerbation of Chronic inflammatory demyelinating polyneuropathy - post total knee replacement

J.Orthopaedics 2009;6(2)e12





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