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Objectives Diagnosis and treatment monitoring in CIDP is primarily based on clinical parameters. High-frequency ultrasound of peripheral nerves can reflect CIDP pathophysiology and changes with treatment. This project investigated potential diagnostic and prognostic biomarkers utilizing neuromuscular ultrasound. Methods We conducted a standardized clinical and ultrasonographic assessment of 50 CIDP patients (25 at WFBMC, 25 at Austin), comparing to 25 healthy controls and 25 axonal neuropathy subjects. Our protocol included whole-length assessment of both median and ulnar nerves, with unilateral assessment of other nerves. Results 25 of 25 CIDP patients studied at WFBMC had an abnormality on ultrasound (as determined by focal nerve enlargement determined by increased cross-sectional area), with 23 of 25 subjects having >=4 enlarged segments. 23 of 25 Austin CIDP patients had at least one enlarged segment, and 20 of 25 had >=4 enlarged segments. 46 of 48 had at least one abnormality in either median or ulnar nerve. Mild nerve enlargements were infrequently seen in healthy and disease controls. However, CIDP patients had clear difference in extent and pattern of enlargements, particularly with proximal upper limb enlargement. Specific markers differentiating CIDP patients will be presented. We analyzed our data in line with previously published diagnostic scores. We will discuss these findings for typical vs. atypical CIDP subtypes, and clinical correlations. Conclusions This cross-sectional study of neuromuscular ultrasound in patients with CIDP suggests assessing bilateral whole-length median and ulnar nerves may be adequate for diagnosis, and differentiating potentially treatment responsive immune-mediated neuropathies from axonal neuropathies and healthy controls.

Contains fulltext : 238533.pdf (Publisher’s version ) (Open Access) The addition of ultrasound (US) to electrodiagnostic (EDX) tests can significantly enhance the accuracy of testing for ulnar neuropathy at the elbow (UNE). We aimed to obtain expert consensus to guide clinicians on the combined use of EDX and US in UNE investigation. Consensus was achieved using the Delphi method. Two consecutive anonymised questionnaires were submitted to 15 experts, who were asked to choose their level of agreement with each statement. Consensus was pre-defined as ≥ 80% rating agreement. The experts concluded that all investigations of UNE should include both nerve conduction studies and US. There was consensus that US should include cross-sectional area measurement and assessment of nerve mobility at the elbow, and that the entire ulnar nerve should be imaged. This study defined expert opinion on the 'core' techniques that should be used routinely in the UNE investigation using EDX and US. Areas with lack of consensus highlighted some controversial issues in the current use of these diagnostic modalities and the need for future research. This document is an initial step to guide clinicians on the combined investigation of UNE using EDX and US, to be regularly updated as new research emerges.

We systematically identified and reviewed 29 studies of peripheral nerve ultrasound or magnetic resonance imaging (MRN) in amyotrophic lateral sclerosis (ALS). The majority of the ultrasound studies reported smaller nerves and nerve roots in ALS compared to healthy controls, but there was a large overlap of the cross-sectional nerve area between ALS and controls. Most of the MRN studies confirmed nerve abnormalities demonstrating slight T2 hyperintensities and, sometimes, mild enlargement of more proximal nerve segments (plexus, roots) in ALS. The size of the proximal nerve segments, i.e. nerve roots, is thus somewhat incongruent between nerve ultrasound and MRN in ALS. Peripheral nerve ultrasound has the potential to differentiate between ALS and multifocal motor neuropathy (MMN) in that patients with MMN have significantly larger nerves. Conversely, there is an overlap of MRN abnormalities in ALS and MMN, restricting the techniques' utility in the clinical setting. A subgroup of patients with ALS seems to reveal a sonographic nerve pattern suggesting peripheral nerve inflammation. In the future, combined imaging with nerve ultrasound and MRN assessing parameters such as blood flow or textural markers may aid in the understanding of the deep nerve microstructure down to the fascicle level, and thus, in the classification of the nerve state as more degenerative or more inflammatory in ALS. This systematic review provides evidence that nerve imaging abnormalities are common in ALS.

Eosinophilic fasciitis (EF) is a rare condition characterized by inflammation, thickening, and sclerosis of the skeletal muscle fascia.1 It can present with a variety of symptoms including induration and peau d’orange changes of the skin, stiffness in the distal limbs, arthralgias, myalgias, and joint contractures. Laboratory abnormalities may include elevated erythrocyte sedimentation rate (ESR), hypergammaglobulinemia, and peripheral eosinophilia.2-4 Electromyography (EMG) is either normal or demonstrates small voluntary motor unit potentials with early recruitment as can be seen in myopathic disorders. The diagnosis of EF typically relies on full thickness incisional biopsy, but interest in the application of imaging modalities including ultrasound (US) and magnetic resonance imaging (MRI) has increased in the past decade. We present three patients in whom imaging assisted in the diagnosis of EF. A systematic review of the literature identified additional cases.

Purpose of review:The use of ultrasonography for diagnosis of neuromuscular disorders is a relatively new but rapidly expanding field. This review covers key ultrasound findings in primary disease of muscle and nerve.Recent findings:Ultrasonography is used routinely for evaluation of entrapment neuropathies, hereditary and inflammatory neuropathies, polyneuropathies, myopathies, and motor neuron disease. When used in association with other electrodiagnostic testing, ultrasound has allowed physicians to increase the yield of clinical evaluation in diagnostic laboratories.Summary:While ultrasonography has not yet replaced the need for electrodiagnostic studies, it has become a useful accompaniment.

Neuromuscular ultrasound is an emerging technology for the evaluation of conditions affecting nerve and muscle, with the majority of research focusing on focal neuropathies. Despite this focus, researchers have also investigated the ultrasonographic changes that occur in the nerves and muscles of those with more diffuse polyneuropathies and motor neuron diseases, and this review will detail the findings in these conditions. Specific findings are discussed in this paper, but general themes will also be presented and include the following: hereditary polyneuropathies show diffuse nerve enlargement whereas immune-mediated polyneuropathies show more patchy involvement; nerve enlargement is more profound in demyelinating than axonal polyneuropathies; and muscle changes in motor neuron diseases include heterogeneous increases in echogenicity, atrophy, readily detectable fasciculations, and increased subcutaneous tissue thickness.

There is an inherent conflict between interpreting ultrasound imaging, which generates rich, highly textured complex representations of 3-dimensional space, and the rendering of these images into discrete measures for statistical analysis Reducing images to a small set of numbers, by necessity, ignores vast amounts of useful data, but the process is a prerequisite for generating objective information that can be shared by the clinical community. This article illustrates how ultrasound imaging is used in quantitative ways. The goal is not to supplant or diminish the importance of descriptive findings but to make the field amenable to statistical methods and standards for making diagnoses and designing therapeutic trials of new interventions. The discussion focuses first on muscle ultrasonography, a technique that was developed more than a decade earlier than nerve ultrasound, to illustrate key elements in determining how to best extract quantitative information from complex images. The discussion then applies some of these same principles to the study of 2 common nerve disorders.

Neuromuscular ultrasound is a painless, radiation-free, high-resolution imaging modality for assessment of the peripheral nervous system. The purpose of this study was to use neuromuscular ultrasound to assess the changes that occur in the median nerve after steroid injection for carpal tunnel syndrome (CTS).Ultrasound and nerve conduction studies were performed at baseline and 1 week, 1 month, and 6 months after steroid injection in 19 individuals (29 wrists) with CTS.Significant changes were noted in median nerve cross-sectional area (P0.001), mobility (P = 0.001), and vascularity (P = 0.042) at the distal wrist crease after steroid injection, and the nerve cross-sectional area correlated with symptom score and electrodiagnostic parameters. Changes in the ultrasonographic parameters were seen within 1 week of injection.These findings suggest neuromuscular ultrasound is potentially helpful for the assessment of individuals undergoing treatment for CTS, as typical changes can be expected after successful treatment injection.

Background Electrodiagnostic studies are traditionally used in the diagnosis of focal neuropathies, however they lack anatomical information regarding the nerve and its surrounding structures. The purpose of this case is to show that high-resolution ultrasound used as an adjunct to electrodiagnostic studies may complement this lack of information and give insight to the cause. Case presentation A 60-year-old male patient sustained a forearm traction injury resulting in progressive weakness and functional loss in the first three digits of the right hand. High-resolution ultrasound showed the presence of an enlarged nerve and a homogenous soft-tissue structure appearing to engulf the nerve. The contralateral side was normal. Surgery revealed fibrotic bands emanating from the flexor digitorum profundus muscle compressing the median nerve thus confirming the ultrasound findings. Conclusion A diagnostically challenging case of median neuropathy in the forearm is presented in which high-resolution ultrasound was valuable in establishing an anatomic etiology and directing appropriate management.