CIDP Diagnosis

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Questions and Answers

When should nerve biopsy be considered for diagnosing CIDP?

  • Only when there is little or no response to treatment
  • Both B and C (correct)
  • As a routine procedure
  • Only when other diagnostic methods cannot confirm CIDP

What should be observed when performing nerve biopsy?

  • Maximal standards for processing and evaluating nerve biopsies
  • Minimal standards for processing and evaluating nerve biopsies (correct)
  • No specific standards for processing and evaluating nerve biopsies
  • Only the patient's consent

Which nerve is most commonly biopsied for CIDP diagnosis?

  • Sural nerve (correct)
  • Peroneal nerve
  • Median nerve
  • Radial nerve

What are some findings suggestive of CIDP on nerve biopsy?

<p>Demyelinated internodes in teased fibers (D)</p> Signup and view all the answers

What is the success rate of immunomodulating agents in patients diagnosed with CIDP through biopsy?

<p>High (A)</p> Signup and view all the answers

What are the limitations of nerve biopsies in CIDP diagnosis?

<p>Poor sensitivity and specificity (D)</p> Signup and view all the answers

What type of testing is strongly advised in adult patients with a clinical suspicion of CIDP?

<p>Monoclonal gammopathy testing (A)</p> Signup and view all the answers

What should be included in monoclonal gammopathy testing for CIDP diagnosis?

<p>All of the above (C)</p> Signup and view all the answers

What should be done if a gammopathy is found during monoclonal gammopathy testing for CIDP diagnosis?

<p>Haematology-oncology consultation should be strongly considered (A)</p> Signup and view all the answers

What should be considered in patients with distal CIDP regarding monoclonal gammopathy testing?

<p>Repeat testing should be considered if no IgM paraprotein is found (B)</p> Signup and view all the answers

When is VEGF serum level testing indicated in patients with a distal and painful CIDP phenotype?

<p>When POEMS syndrome is suspected (C)</p> Signup and view all the answers

What are some complications associated with sural nerve biopsy?

<p>Numbness and acute or chronic pain (D)</p> Signup and view all the answers

When should nerve biopsy be performed to diagnose CIDP?

<p>Only when CIDP cannot be confirmed by other tests (B)</p> Signup and view all the answers

Who should be available to perform nerve biopsies?

<p>Specialized pathology laboratory facilities (C)</p> Signup and view all the answers

What should patients be informed of before undergoing nerve biopsy?

<p>The accuracy of nerve biopsy is limited (B)</p> Signup and view all the answers

What type of nerve biopsy is more likely to provide useful information?

<p>Biopsy of a clinically affected nerve (A)</p> Signup and view all the answers

What are some findings suggestive of CIDP on nerve biopsy?

<p>Demyelination on electron microscopy (B)</p> Signup and view all the answers

What is the purpose of the statement on nerve biopsy?

<p>To discourage nerve biopsies for suspected CIDP (A)</p> Signup and view all the answers

What is strongly advised in adult patients with a clinical suspicion of CIDP?

<p>Testing for serum monoclonal proteins (A)</p> Signup and view all the answers

What tests should be included when testing for serum monoclonal proteins?

<p>Serum protein electrophoresis and immunofixation, spot urine immunofixation for light chains, and measurement of serum free light chains (C)</p> Signup and view all the answers

What should be done if a gammopathy is found during monoclonal gammopathy testing?

<p>Further evaluation may be required (A)</p> Signup and view all the answers

What is the potential complication associated with sural nerve biopsy?

<p>Numbness in the area of innervation (B)</p> Signup and view all the answers

What is the success rate of treating patients with immunomodulating agents after a biopsy-guided diagnosis of CIDP?

<p>High (D)</p> Signup and view all the answers

What is the accuracy of nerve biopsy?

<p>Limited (B)</p> Signup and view all the answers

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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 performed routinely to diagnose CIDP.
  • It should only be considered in specific circumstances, such as when CIDP cannot be confirmed by other tests or when alternative diagnoses are being considered.
  • Skilled surgeons, neuropathologists, and specialized pathology laboratory facilities should be available to perform nerve biopsies.
  • The accuracy of nerve biopsy is limited, and patients should be informed of this before undergoing the procedure.
  • Minimal standards for processing and evaluating nerve biopsies should be followed.
  • 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, small onion bulbs, perivascular macrophage clusters, and demyelination on electron microscopy.
  • Clinical outcomes in patients with suspected CIDP who are treated with immunomodulating agents after a biopsy-guided diagnosis of CIDP have been successful.
  • Nerve biopsy may reveal findings suggestive of a different or differential diagnosis, leading to appropriate therapy.
  • Sural nerve biopsy is associated with numbness in the area of innervation and other potential complications.
  • The statement on nerve biopsy is intended to reduce the number of unnecessary biopsies for suspected CIDP.

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.

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