Chronic inflammatory demyelinating polyradiculoneuropathy

From IDWiki

Background

  • Rare immune-mediated demyelinating polyneuropathy

Clinical Manifestations

  • Progressive or relapsing symmetric muscle weakness
    • Can be proximal or distal, upper or lower extremities
    • At least 8 weeks
    • Absent or decreased deep tendon reflexes
  • Variants:
    • Distal CIDP: distal sensory loss and muscle weakness predominantly in lower limbs
    • Multifocal CIDP: sensory loss and muscle weakness in a multifocal pattern, usually asymmetric, upper limb predominant, in more than one limb
    • Focal CIDP: sensory loss and muscle weakness in only one limb
    • Motor CIDP: motor symptoms and signs without sensory involvement
    • Sensory CIDP: sensory symptoms and signs without motor involvement

Diagnostic Criteria

Criterion Typical Distal Multifocal/Focal Motor Sensory
Clinical Criteria
Weakness symmetric in 4 limbs, proximal and distal Predominantly distal lower limbs 2 or more limbs (multifocal) or 1 limb (focal) symmetric in 4 limbs, proximal and distal none
Sensory disturbance in 2 or more limbs in 2 or more limbs in distribution of affected limbs none symmetric in 4 limbs
Electrodiagnostic Criteria
Motor conduction criteria + + + + normal
Sensory conduction abnormalities + + + normal
Sensory conduction criteria

Further Reading

  • EAN/PNS guideline on diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy: Report of a joint Task Force—Second revision. Eur J Neurol. 2021 ;28(11):3556-3583. doi: 10.1111/ene.14959.