DOI: 10.5061/dryad.7sqv9s4nx
title: Nerve ultrasound improves detection of treatment-responsive chronic inflammatory neuropathies

description: Objective To examine the diagnostic accuracy of nerve ultrasound in a
 prospective cohort of consecutive patients with a clinical suspicion of
 chronic inflammatory neuropathies, including chronic inflammatory
 demyelinating polyneuropathy, Lewis Sumner syndrome and multifocal motor
 neuropathy, and to determine the added value in the detection of
 treatment-responsive patients. Methods Between February 2015 and July
 2018, we included 100 consecutive incident patients with a clinical
 suspicion of chronic inflammatory neuropathy. All patients underwent nerve
 ultrasound, extensive standardized nerve conduction studies (NCS) and
 other relevant diagnostic investigations. We evaluated treatment response
 using predefined criteria. A diagnosis of chronic inflammatory neuropathy
 was established when NCS were abnormal (fulfilling criteria of
 demyelination of the EFNS/PNS) or when the degree of nerve enlargement
 detected by sonography was compatible with chronic inflammatory neuropathy
 and there was response to treatment. Results A diagnosis of chronic
 inflammatory neuropathy was established in 38 patients. Sensitivity and
 specificity of nerve ultrasound and NCS were 97.4% and 69.4%, and 78.9%
 and 93.5%, respectively. The added value of nerve ultrasound in detection
 of treatment-responsive chronic inflammatory neuropathy patients was 21.1%
 compared to NCS alone. Conclusions Nerve ultrasound and NCS are
 complementary techniques with superior sensitivity in the former and
 specificity in the latter. Addition of nerve ultrasound significantly
 improves the detection of chronic inflammatory neuropathies. Therefore, it
 deserves a prominent place in the diagnostic workup of chronic
 inflammatory neuropathies. Classification of Evidence This study provides
 Class I evidence that nerve ultrasound is an accurate diagnostic tool to
 detect chronic inflammatory neuropathies.
description: File contents overview <b>Table e-1
 </b>Final diagnoses <b>Legend</b> The final diagnoses of 100 patients in whom there is a clinical suspicion of chronic inflammatory neuropathy; data are shown as number of patients. <b>Abbreviations</b> ALS = amyotrophic lateral sclerosis, CIAP = chronic idiopathic axonal polyneuropathy, CIDP = chronic inflammatory demyelinating polyneuropathy, Classical = classical phenotype of CIDP, GBS = Guillain-Barré syndrome, HNLPP = hereditary neuropathy with liability to pressure palsies, MGUS = monoclonal gammopathy of undetermined significance, MMN = multifocal motor neuropathy, LSS = Lewis Sumner syndrome, PSMA = progressive spinal muscular atrophy, Pure motor = pure motor phenotype of CIDP, Pure sensory = pure sensory phenotype of CIDP <b>Table e-2 </b>Patient characteristics per subgroup <b>Abbreviations</b> Anti-GM1 = anti-GM1 antibodies, CIAP = chronic idiopathic axonal polyneuropathy, CIDP = chronic inflammatory demyelinating polyneuropathy, CSF = cerebrospinal fluid, GBS = Guillain-Barré syndrome, MGUS = monoclonal gammopathy of undetermined significance, MMN = multifocal motor neuropathy, NCS = nerve conduction studies, PNP = polyneuropathy, PSMA = progressive spinal muscular atrophy, Ultrasound = nerve ultrasound NA= not applicable, NP = not performed; “-”  = normal test result, “+”  = abnormal test result <b>Table e-3 </b>Treatment response per subgroup <b>Abbreviations</b> CIAP = chronic idiopathic axonal polyneuropathy, CIDP = chronic inflammatory demyelinating polyneuropathy, HHD = Hand Held Dynamometry, ISS = INCAT Sensory Sum Score, MCID = minimal clinically important difference, MMN = multifocal motor neuropathy, MRC = Medical Research Council, ODSS = Overall Disability Sum Score, PSMA = progressive spinal muscular atrophy, RODS = Rasch-built Overall Disability Scale, Treatment response = treatment response according to predefined criteria NA = not applicable, NP = not performed, NT = not treated, “-”  = no improvement or no response, “+” = improvement or response