Chronic inflammatory demyelinating polyneuropathy: Difference between revisions

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


==See Also==
==See Also==

Revision as of 18:34, 12 September 2020

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
  • Impaired sensation
  • Areflexia
  • Elevated CSF protein
  • EMG studies consistent with demyelination
  • 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

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

  • Requires Neurology consult
  • IVIG
  • Plasma exchange
  • Corticosteroids
  • Therapy is tailored to each patient, no difference in efficacy between the three treatment options
  • May be placed on azathioprine, cyclophosphamide, or cyclosporine to prevent relapse

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