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Incomplete Presentations in Typical Chronic Inflammatory Demyelinating Polyneuropathy: A Single-Center, Retrospective
Young Gi Min1, Irad Ahmed2, Christina Englezou2
1Department of Translational Medicine, Seoul National University College of Medicine, Seoul, Republic of Korea.
Nearly one-third of chronic inflammatory demyelinating polyneuropathy (CIDP) patients have incomplete forms, presenting milder disability. Prompt recognition of incomplete CIDP is crucial for effective treatment and management.
Area of Science:
- Neurology
- Clinical Electrophysiology
- Immunology
Background:
- Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare autoimmune disorder affecting peripheral nerves.
- Recently described incomplete phenotypes of typical CIDP lack defined frequency and clinical characteristics.
Purpose of the Study:
- To describe the clinical characteristics and frequency of incomplete typical CIDP.
- To compare incomplete CIDP phenotypes with the complete form.
Main Methods:
- Retrospective analysis of 64 treatment-naïve CIDP patients.
- Phenotype classification based on detailed motor examinations.
- Comparison of clinical, electrophysiological, and therapeutic characteristics.
Main Results:
- 30% of typical CIDP patients exhibited incomplete phenotypes (proximal arm-sparing, distal arm-sparing, pure paraparetic, pure proximal).
- Incomplete CIDP patients had milder pre-treatment disability and weakness compared to complete CIDP.
- Disability in incomplete CIDP at diagnosis did not correlate with muscle strength, unlike the complete form.
Conclusions:
- Incomplete CIDP forms are common, representing a milder variant of typical CIDP.
- Prompt recognition of incomplete CIDP is vital as treatment response is similar to the complete form.
- Proprioceptive loss may contribute to disability in incomplete CIDP.
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