Dataset Information

Nerve ultrasound can identify treatment-responsive chronic neuropathies without electrodiagnostic features of demyelination.

ABSTRACT:

Introduction

We present a case series of six treatment-naive patients with clinical phenotypes compatible with chronic inflammatory demyelinating polyneuropathy and multifocal motor neuropathy without electrodiagnostic features of demyelination but with abnormal peripheral ultrasound findings who responded to treatment.

Methods

All six patients underwent a complete set of ancillary investigations, including extensive nerve conduction studies. We also performed standardized nerve ultrasound of median nerves and brachial plexus as part of a larger effort to evaluate diagnostic value of sonography.

Results

Nerve conduction studies did not show conduction block or other signs of demyelination in any of the six patients. Sonographic nerve enlargement was present in all patients and was most prominent in proximal segments of the median nerve and brachial plexus. Treatment with intravenous immunoglobulin resulted in objective clinical improvement.

Discussion

Our study provides evidence that nerve ultrasound represents a useful complementary diagnostic tool for the identification of treatment-responsive inflammatory neuropathies.

SUBMITTER: Goedee HS

PROVIDER: S-EPMC6771613 | biostudies-literature | 2019 Oct

REPOSITORIES: biostudies-literature

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Publications

Nerve ultrasound can identify treatment-responsive chronic neuropathies without electrodiagnostic features of demyelination.

Goedee H Stephan HS Herraets Ingrid J T IJT Visser Leo H LH Franssen Hessel H van Asseldonk Jan-Thies H JH van der Pol W Ludo WL van den Berg Leonard H LH

Muscle & nerve 20190724 4

<h4>Introduction</h4>We present a case series of six treatment-naive patients with clinical phenotypes compatible with chronic inflammatory demyelinating polyneuropathy and multifocal motor neuropathy without electrodiagnostic features of demyelination but with abnormal peripheral ultrasound findings who responded to treatment.<h4>Methods</h4>All six patients underwent a complete set of ancillary investigations, including extensive nerve conduction studies. We also performed standardized nerve u ...[more]

PMID: 31294858

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Project description:As reliable biomarkers of disease activity are lacking, monitoring of therapeutic response in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) remains a challenge. We sought to determine whether nerve ultrasound and electrophysiology scoring could close this gap. In CIDP patients (fulfilling EFNS/PNS criteria), we performed high-resolution nerve ultrasound to determine ultrasound pattern sum scores (UPSS) and predominant echotexture nerve conduction study scores (NCSS) as well as Medical Research Council sum scores (MRCSS) and inflammatory neuropathy cause and treatment disability scores (INCAT) at baseline and after 12 months of standard treatment. We retrospectively correlated ultrasound morphology with nerve histology when available. 72/80 CIDP patients featured multifocal nerve enlargement, and 35/80 were therapy-naïve. At baseline, clinical scores correlated with NCSS (r2?=?0.397 and r2?=?0.443, p?<?0.01), but not or hardly with UPSS (Medical Research Council sum scores MRCSS r2?=?0.013, p?=?0.332; inflammatory neuropathy cause and treatment disability scores INCAT r2?=?0.053, p?=?0.048). Longitudinal changes in clinical scores, however, correlated significantly with changes in both UPSS and NCSS (r2?=?0.272-0.414, p?<?0.0001). Combining nerve/fascicle size with echointensity and histology at baseline, we noted 3 distinct classes: 1) hypoechoic enlargement, reflecting active inflammation and onion bulbs; 2) nerve enlargement with additional hyperechogenic fascicles/perifascicular tissue in >?50% of measured segments, possibly reflecting axonal degeneration; and 3) almost no enlargement, reflecting "burned-out" or "cured" disease without active inflammation. Clinical improvement after 12 months was best in patients with pattern 1 (up to 75% vs up to 43% in pattern 2/3, Fisher's exact test p?<?0.05). Nerve ultrasound has additional value not only for diagnosis, but also for classification of disease state and may predict treatment response.

Dataset Information

Nerve ultrasound can identify treatment-responsive chronic neuropathies without electrodiagnostic features of demyelination.

Introduction

Methods

Results

Discussion

Publications

Nerve ultrasound can identify treatment-responsive chronic neuropathies without electrodiagnostic features of demyelination.

Similar Datasets

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