Chronic Inflammatory Demyelinating Polyradiculoneuropathy Workup

  • Author: Richard A Lewis, MD; Chief Editor: Nicholas Lorenzo, MD   more...
 
Updated: Apr 26, 2010
 

Laboratory Studies

  • Test CSF on patients in whom chronic inflammatory demyelinating polyradiculoneuropathy is suspected. Protein level is increased significantly in 80% of patients (usually between 50 and 200 mg/dL, but can be higher); 10% of patients also have mild lymphocytic pleocytosis (< 50 cells) and increased gamma globulin (usually associated with HIV infection).
  • CBC count, sedimentation rate, antinuclear antibody, biochemistry profile, and serum and urine immunoelectrophoresis are necessary to exclude important associated systemic disorders.
  • In certain instances, genetic testing may be helpful. Examples include patients with positive family history, very insidious symmetric course of the disease, or some atypical features, including lack of treatment effect. In such cases, genetic testing for Charcot-Marie-Tooth disease might be indicated. Hereditary neuropathy with predisposition to pressure palsies can be suspected and tested for in some patients. Charcot-Marie-Tooth disease type 1X and adult-onset Charcot-Marie-Tooth disease type 1B have been confused with CIDP.
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Imaging Studies

  • Imaging studies can provide supportive evidence of CIDP.
  • MRI of the spine with gadolinium enhancement may show enhancement of nerve roots.
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Other Tests

EMG is a critical test to determine whether the disorder is truly a peripheral neuropathy and whether the neuropathy is demyelinating. Findings of a demyelinating neuropathy are as follows:

  • Multifocal conduction block or temporal dispersion of compound muscle action potential, as shown in the image belowElectromyography of a patient with chronic inflammElectromyography of a patient with chronic inflammatory demyelinating polyradiculoneuropathy illustrating conduction block, temporal dispersion of compound muscle action potential, prolonged distal latencies, and slowed conduction.
  • Prolonged distal latencies and dispersion of the distal compound motor action potential
  • Variable conduction slowing to less than 70% of normal
  • Absent or prolonged F wave latencies, as shown belowProlonged F wave latencies (normal is < 31). Prolonged F wave latencies (normal is < 31).

As the disease progresses, patients tend to develop secondary axonal degeneration.

Reports exist of a predominantly axonal neuropathy with clinical course and response to treatment similar to those of CIDP. Most cases of axonal neuropathy are not immune or inflammatory. However, some patients with an aggressive axonal neuropathy have been treated effectively with immunosuppressive and/or immunomodulatory therapy, raising the question of whether a chronic axonal inflammatory neuropathy, akin to the acute axonal variants of GBS, may be present. The relationship of these chronic axonal variants to CIDP is unclear.

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Procedures

Peripheral (sural) nerve biopsy is considered as supportive evidence of CIDP.

  • Consider biopsy for those patients in whom the diagnosis is not completely clear, when other causes of neuropathy (eg, hereditary, vasculitic) cannot be excluded, or when profound axonal involvement is observed on EMG.
  • Some experts recommend biopsy for most patients prior to initiating immunosuppressive therapy, but more recent guidelines no longer recommend biopsy.
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Histologic Findings

Tissue collected on biopsy of the sural nerve may demonstrate evidence of interstitial and perivascular infiltration of the endoneurium with inflammatory T cells and macrophages with local edema. Evidence exists of segmental demyelination and remyelination with occasional onion bulb formation, particularly in relapsing cases, like the one in the image below.

Electron micrograph of the peripheral nerve of a pElectron micrograph of the peripheral nerve of a patient with chronic inflammatory demyelinating polyradiculoneuropathy. Note "onion bulb" formation in the myelin sheath of the nerve fibers due to continuous demyelination and remyelination. Courtesy of A. Sima, MD, Department of Pathology, Wayne State University.

Some evidence of axonal damage also is observed, with loss of myelinated nerve fibers. The inflammatory infiltrate with neutrophil infiltration is observed in only a minority of patients.

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Contributor Information and Disclosures
Author

Richard A Lewis, MD  Professor and Associate Chairman of Neurology, Department of Neurology, Wayne State University School of Medicine

Richard A Lewis, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, American Neurological Association, and Peripheral Nerve Society

Disclosure: GBS/CIDP FI Grant/research funds Other; Baxter Consulting fee Consulting; Baxter Grant/research funds None; CSL Behring Consulting fee Consulting

Specialty Editor Board

Dianna Quan, MD  Associate Professor of Neurology, Director, Electromyography Laboratory, University of Colorado School of Medicine

Dianna Quan, MD is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, and Phi Beta Kappa

Disclosure: e-medicine Honoraria Other

Francisco Talavera, PharmD, PhD  Senior Pharmacy Editor, eMedicine

Disclosure: eMedicine Salary Employment

Florian P Thomas, MD, MA, PhD, Drmed  Director, Spinal Cord Injury Unit, Saint Louis Veterans Affairs Medical Center; Director, National MS Society Multiple Sclerosis Center; Director, Neuropathy Association Center of Excellence, Professor, Department of Neurology and Psychiatry, Associate Professor, Institute for Molecular Virology, and Department of Molecular Microbiology and Immunology, Saint Louis University

Florian P Thomas, MD, MA, PhD, Drmed is a member of the following medical societies: American Academy of Neurology, American Neurological Association, American Paraplegia Society, Consortium of Multiple Sclerosis Centers, and National Multiple Sclerosis Society

Disclosure: Nothing to disclose.

Selim R Benbadis, MD  Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, University of South Florida School of Medicine, Tampa General Hospital

Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association

Disclosure: UCB Pharma Honoraria Speaking, consulting; Lundbeck Honoraria Speaking, consulting; Cyberonics Honoraria Speaking, consulting; Glaxo Smith Kline Honoraria Speaking, consulting; Ortho McNeil Honoraria Speaking, consulting; Pfizer Honoraria Speaking, consulting; Sleepmed/DigiTrace Speaking, consulting

Chief Editor

Nicholas Lorenzo, MD  Chief Editor, eMedicine Neurology; Consulting Staff, Neurology Specialists and Consultants

Nicholas Lorenzo, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Neurology

Disclosure: Nothing to disclose.

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Electromyography of a patient with chronic inflammatory demyelinating polyradiculoneuropathy illustrating conduction block, temporal dispersion of compound muscle action potential, prolonged distal latencies, and slowed conduction.
Prolonged F wave latencies (normal is < 31).
Electron micrograph of the peripheral nerve of a patient with chronic inflammatory demyelinating polyradiculoneuropathy. Note "onion bulb" formation in the myelin sheath of the nerve fibers due to continuous demyelination and remyelination. Courtesy of A. Sima, MD, Department of Pathology, Wayne State University.
 
 
 
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