Podcast
Questions and Answers
Which diagnostic method should be considered for CIDP only in specific circumstances, such as when other methods cannot confirm it or when there is little or no response to treatment?
Which diagnostic method should be considered for CIDP only in specific circumstances, such as when other methods cannot confirm it or when there is little or no response to treatment?
Which nerve biopsy is most common, but biopsy of a clinically affected nerve is more likely to provide useful information?
Which nerve biopsy is most common, but biopsy of a clinically affected nerve is more likely to provide useful information?
What are the findings suggestive of CIDP on nerve biopsy?
What are the findings suggestive of CIDP on nerve biopsy?
What should patients be informed of before undergoing nerve biopsy?
What should patients be informed of before undergoing nerve biopsy?
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What is the aim of the statement on nerve biopsy?
What is the aim of the statement on nerve biopsy?
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What are the possible complications associated with sural nerve biopsy?
What are the possible complications associated with sural nerve biopsy?
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What type of testing is strongly advised in adult patients with a clinical suspicion of CIDP?
What type of testing is strongly advised in adult patients with a clinical suspicion of CIDP?
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What should be included in the testing for monoclonal gammopathy?
What should be included in the testing for monoclonal gammopathy?
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What should be done if a gammopathy is found during monoclonal gammopathy testing?
What should be done if a gammopathy is found during monoclonal gammopathy testing?
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What is indicated in patients with a distal and painful CIDP phenotype, in whom a lambda light chain associated IgA or IgG paraprotein is found, when POEMS syndrome is suspected?
What is indicated in patients with a distal and painful CIDP phenotype, in whom a lambda light chain associated IgA or IgG paraprotein is found, when POEMS syndrome is suspected?
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What should be considered in patients with distal CIDP if no IgM paraprotein is found or anti-MAG antibody testing is negative?
What should be considered in patients with distal CIDP if no IgM paraprotein is found or anti-MAG antibody testing is negative?
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What is the contribution of nerve biopsies to the diagnosis of CIDP?
What is the contribution of nerve biopsies to the diagnosis of CIDP?
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When should nerve biopsy be considered for diagnosing CIDP?
When should nerve biopsy be considered for diagnosing CIDP?
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What should be observed when performing nerve biopsy?
What should be observed when performing nerve biopsy?
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Which nerve is most commonly biopsied for CIDP diagnosis?
Which nerve is most commonly biopsied for CIDP diagnosis?
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What are some findings suggestive of CIDP on nerve biopsy?
What are some findings suggestive of CIDP on nerve biopsy?
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What is the success rate of immunomodulating agents in patients diagnosed with CIDP through biopsy?
What is the success rate of immunomodulating agents in patients diagnosed with CIDP through biopsy?
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What are the limitations of nerve biopsies in CIDP diagnosis?
What are the limitations of nerve biopsies in CIDP diagnosis?
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What type of testing is strongly advised in adult patients with a clinical suspicion of CIDP?
What type of testing is strongly advised in adult patients with a clinical suspicion of CIDP?
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What should be included in monoclonal gammopathy testing for CIDP diagnosis?
What should be included in monoclonal gammopathy testing for CIDP diagnosis?
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What should be done if a gammopathy is found during monoclonal gammopathy testing for CIDP diagnosis?
What should be done if a gammopathy is found during monoclonal gammopathy testing for CIDP diagnosis?
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What should be considered in patients with distal CIDP regarding monoclonal gammopathy testing?
What should be considered in patients with distal CIDP regarding monoclonal gammopathy testing?
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When is VEGF serum level testing indicated in patients with a distal and painful CIDP phenotype?
When is VEGF serum level testing indicated in patients with a distal and painful CIDP phenotype?
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What are some complications associated with sural nerve biopsy?
What are some complications associated with sural nerve biopsy?
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Study Notes
Good Practice Points for Nerve Biopsy and Monoclonal Gammopathy Testing in CIDP Diagnosis
Nerve Biopsy:
- Nerve biopsy should not be a routine procedure for diagnosing CIDP.
- It should only be considered in specific circumstances, such as when CIDP cannot be confirmed with other diagnostic methods, or when there is little or no response to treatment.
- Skilled surgeons, neuropathologists, and specialized pathology laboratory facilities should be available for nerve biopsy.
- The low accuracy of nerve biopsy should be fully understood by the patient before undergoing the procedure.
- Current expert consensus on minimal standards for processing and evaluating nerve biopsies should be observed.
- Sural or superficial peroneal nerve biopsy is most common, but biopsy of a clinically affected nerve is more likely to provide useful information.
- Findings suggestive of CIDP on nerve biopsy may include thinly myelinated axons and small onion bulbs, demyelinated internodes in teased fibers, perivascular macrophage clusters, and supportive features of demyelination on electron microscopy.
- Clinical outcomes in patients with suspected CIDP, treated with immunomodulating agents after a biopsy-guided diagnosis of CIDP, have been successful.
- Nerve biopsies have poor sensitivity and specificity, and their contribution to the diagnosis is limited by these inaccuracies.
- The statement on nerve biopsy aims to reduce the number of unnecessary biopsies for suspected CIDP, given the low diagnostic accuracy and invasive nature.
- Sural nerve biopsy is associated with numbness in the area of innervation and other complications such as acute and chronic pain, allodynia, dysaesthesia, neuroma formation, infections, and wound dehiscence.
- Only a small number of carefully selected nerve biopsies will contribute to a more accurate diagnosis of CIDP and a lower probability of misdiagnosis.
Monoclonal Gammopathy Testing:
- Testing for serum monoclonal proteins is strongly advised in adult patients with a clinical suspicion of CIDP.
- Testing should include serum protein electrophoresis and immunofixation, spot urine immunofixation for light chains, and measurement of serum free light chains.
- If a gammopathy is found, further evaluation may be required, and haematology-oncology consultation should be strongly considered.
- Relevant monoclonal proteins may still have normal light chain and ratio measurements in SFLC assays.
- In patients with distal CIDP, repeat testing should be considered if no IgM paraprotein is found or anti-MAG antibody testing is negative.
- Vascular endothelial growth factor (VEGF) serum level testing is indicated in patients with a distal and painful CIDP phenotype, in whom a lambda light chain associated IgA or IgG paraprotein is found, when POEMS syndrome is suspected.
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Description
Test your knowledge on the good practice points for nerve biopsy and monoclonal gammopathy testing in the diagnosis of CIDP. This quiz covers important considerations such as when to consider nerve biopsy, the potential complications associated with sural nerve biopsy, and the recommended tests for monoclonal gammopathy. Keywords specific to the quiz topic include nerve biopsy, CIDP, monoclonal gammopathy, serum protein electrophoresis, and immunofixation.
