Chronic inflammatory demyelinating polyneuropathy

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Background

  • Prevalence of 1 to 2 per 100,000 adults
  • Autoimmune condition leading to cellular and humoral immune response to proteins on myelin sheath likely due to molecular mimicry from infectious process
  • Acute variant is Guillain-Barre syndrome
    • GBS is self limited whereas CIPD is progressive for more than two months

Clinical Features

  • Symmetrical proximal and distal muscle weakness that is progressive for GREATER than two months
    • Muscle weakness more predominant feature than sensory symptoms
  • Reduced or absent deep tendon reflexes in upper and lower extremities
  • Elevated CSF protein without pleocytosis
  • Electrodiagnostic evidence of a demyelinating neuropathy
  • Segmental nerve demyelination on nerve biopsies
  • Can be relapsing or chronic and progressive

Differential Diagnosis

Weakness

Evaluation

  • Clinical diagnosis with electrophysiological studies
  • 3 separate criteria can be used to diagnose CIPD
    • Only the American Academy of Neurology requires a lumbar puncture and nerve-biopsy specimen
  • Closest differential is Guillain-Barre syndrome
    • The onset of GBS is usually easily identified, while the precise onset of CIDP is typically less clear
    • Antecedent events are more frequent with GBS than in CIDP
    • ~70% of GBS cases are preceded by an infectious illness, vaccination, or surgery by three to four weeks prior to the onset of clinical symptoms.
    • By contrast, most studies have found an antecedent event prior to CIDP in no more than 30% of patients.
    • Prominent sensory signs (ie, sensory ataxia and impaired vibration and pinprick sensation) favor CIDP
    • The need for ventilator support favors GBS
    • Autonomic dysfunction (tachycardia, urinary retention) much more common in GBS than CIPD
    • Progression or relapse beyond eight weeks from onset suggests acute-onset CIDP rather than GBS
    • May be difficult to distinguish initially between CIPD and GBS if recognized early but relapsing-remitting course over months with identify CIPD

Workup

  • Lumbar puncture can be performed inpatient
    • Will show elevated protein without pleocytosis
  • Nerve biopsy will show demyelination and demyelination often with signs of inflammation
    • Often unnecessary and of low yield due to proximal nerve involvement, but still performed if diagnosis uncertain
  • MRI may show gadolinium enhancement and enlargement of proximal nerves or roots
  • Electrophysiological diagnostic procedures with show evidence of demyelination
' American Academy of Neurology Saperstein Criteria Inflammatory Neuropathy Cause and Treatment Group
Presentation Motor and sensory dysfunction in > 1 limb Major: symetric proximal and distal weakness; Minor: Exclusive distal weakness or sensory loss Progressive or relapsing motor and sensory dysfunction of > 1 limb
Time course in months ≥ 2 ≥ 2 > 2
Reflexes Reduced or absent Reduced or absent Reduced or absent
Electrodiagnostic Criteria Any 3 of the following 4 criteria: partial conduction block of ≥1 motor nerve, reduced conduction velocity of ≥2 motor nerves, prolonged distal latency of ≥2 motor nerves, or prolonged F-wave latencies of ≥2 motor nerves or the absence of F waves 2 of the 4 AAN electrodiagnostic criteria Partial conduction block of ≥2 motor nerves and abnormal conduction velocity or distal latency or F-wave latency in 1 other nerve; or, in the absence of partial conduction block, abnormal conduction velocity, distal latency, or F-wave latency in 3 motor nerves; or electrodiagnostic abnormalities indicating demyelination in 2 nerves and histologic evidence of demyelination
CSF WBC < 10/mm^3, negative VDRL, elevated protein Protein > 45mg/dL; WBC <10/mm^3 Recommended but not mandatory
Biopsy Demylelination and remyelination Demyelination Not mandatory

Management

Disposition

  • Often require admission for physical therapy, occupation therapy, treatment, and possible placement for rehab if unable to perform ADLs

See Also

External Links

References

  • Köller, H., Kieseier, B. C., Jander, S., & Hartung, H.-P. (2005). Chronic inflammatory demyelinating polyneuropathy. The New England Journal of Medicine, 352(13), 1343–1356.