Clinical Diagnostic Criteria (EFNS/PNS)

Carol L. Koski, M.D. and Richard A. Lewis, M.D., Principal Authors

Information adapted from Joint Task Force of the EFNS and the PNS, 2005

1. Inclusion Criteria

1. Typical CIDP

   Chronically progressive, stepwise, or recurrent symmetric proximal and distal weakness and sensory dysfunction of all extremities, developing over at least 2 months; cranial nerves may be affected; and absent or reduced tendon reflexes in all extremities

2. Atypical CIDP

   One of the following, but otherwise as in typical CIDP (tendon reflexes may be normal in unaffected limbs):

   • Predominantly distal weakness (distal acquired demyelinating symmetric, DADS)
   • Pure motor or sensory presentations, including chronic sensory immune polyradiculoneuropathy affecting the central process of the primary sensory neuron (Sinnreich et al, 2004)
   • Asymmetric presentations (multifocal acquired demyelinating sensory and motor, MADSAM, Lewis-Sumner syndrome
   • Focal presentations (e.g., involvement of the brachial plexus or of one or more peripheral nerves in one upper limb
   • Central nervous system involvement (may occur with otherwise typical or other forms of atypical CIDP)

2. Exclusion Criteria

• Diphtheria, drug or toxin exposure likely to have caused the neuropathy
• Hereditary demyelinating neuropathy, known or likely because of family history, foot deformity, mutilation of hands or feet, retinitis pigmentosa, ichthyosis, liability to pressure palsy
• Presence of sphincter disturbance
• Multifocal motor neuropathy
• Antibodies to myelin-associated glycoprotein