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Abstract
Objective To validate the diagnostic accuracy of a previously described short sonographic protocol to identify chronic inflammatory neuropathy (CIN), including chronic inflammatory demyelinating polyneuropathy (CIDP), Lewis Sumner syndrome, and multifocal motor neuropathy (MMN), and to determine the added value of nerve ultrasound to detect treatment-responsive patients compared to nerve conduction studies (NCS) in a prospective multicenter study.
Methods We included 100 consecutive patients clinically suspected of CIN in 3 centers. The study protocol consisted of neurologic examination, laboratory tests, NCS, and nerve ultrasound. We validated a short sonographic protocol (median nerve at forearm, upper arm, and C5 nerve root) and determined its diagnostic accuracy using the European Federation of Neurological Societies/Peripheral Nerve Society criteria of CIDP/MMN (reference standard). In addition, to determine the added value of nerve ultrasound in detecting treatment-responsive patients, we used previously published diagnostic criteria based on clinical, NCS, and sonographic findings and treatment response (alternative reference standard).
Results Sensitivity and specificity of the sonographic protocol for CIN according to the reference standard were 87.4% and 67.3%, respectively. Sensitivity and specificity of this protocol according to the alternative reference standard were 84.6% and 72.8%, respectively, and of NCS 76.1% and 93.4%. With addition of nerve ultrasound, 44 diagnoses of CIN were established compared to 33 diagnoses with NCS alone.
Conclusions A short sonographic protocol shows high diagnostic accuracy for detecting CIN. Nerve ultrasound is able to detect up to 25% more patients who respond to treatment.
Classification of evidence This multicenter study provides Class IV evidence that nerve ultrasound improves diagnosis of CIN.
Glossary
- CIDP=
- chronic inflammatory demyelinating polyneuropathy;
- CIN=
- chronic inflammatory neuropathy;
- CSA=
- cross-sectional area;
- EFNS/PNS=
- European Federation of Neurological Societies/Peripheral Nerve Society;
- ETZ=
- Elisabeth-Tweesteden Hospital Tilburg;
- INCAT=
- Inflammatory Neuropathy Cause and Treatment;
- LSS=
- Lewis Sumner syndrome;
- MMN=
- multifocal motor neuropathy;
- NCS=
- nerve conduction studies;
- NPV=
- negative predictive value;
- PPV=
- positive predictive value;
- UMC=
- University Medical Center
Footnotes
Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
↵* These authors contributed equally to this work.
Class of Evidence: NPub.org/coe
- Received December 23, 2019.
- Accepted in final form April 6, 2020.
- © 2020 American Academy of Neurology
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