Table 1

Major phenotypic variants of CIDP

CIDP phenotypic variantEstimated prevalence within CIDPOnsetClinical symptomsDistributionReferences
Typical CIDP51%ChronicSensory and motorSymmetrical, proximal and distal8–10
Sensory CIDP4–35%ChronicSensory predominant; motor involvement may developAs per typical CIDP5, 9–11
Chronic immune sensory polyradiculopathy5–12%ChronicSensory ataxiaAs per typical CIDP8, 9, 12, 13
Lewis-Sumner syndrome/ MADSAM6–15%ChronicSensory and motorAsymmetrical; often upper limb onset5, 8, 9, 14
Focal CIDP1%ChronicSensory and motorFocal; may progress to diffuse CIDP over time9, 15
DADS2–17%ChronicSensory predominant, but may include motor involvementSymmetrical, distal5, 9, 10
Acute onset CIDP2–16%Acute onsetAs per typical CIDPAs per typical CIDP9, 16–18
Motor CIDP4–10%ChronicMotor predominantAs per typical CIDP5, 8, 9, 13
  • CIDP, Chronic inflammatory demyelinating polyradiculoneuropathy; DADS, distal acquired demyelinating symmetric; MADSAM, multifocal acquired demyelinating sensory and motor neuropathy.