Current and Future Applications of Ultrasound Imaging in Peripheral Nerve and Muscle Disorders

A special issue of Medicina (ISSN 1648-9144).

Deadline for manuscript submissions: closed (25 March 2023) | Viewed by 13221

Special Issue Editors


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Guest Editor
Department of Neurology, Center for Vertigo and Dizziness, Jena University Hospital, Jena, Germany
Interests: peripheral nerves; clinical neurophysiology; ultrasound; polyneuropathy; vertigo and dizziness

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Guest Editor
Department of Neurology, Jena University Hospital, Friedrich Schiller University, 07747 Jena, Germany
Interests: peripheral nerves; muscle and nerve ultrasound; diabetes mellitus; polyneuropathy; diabetic neuropathy, carpal tunnel syndrome

Special Issue Information

Dear Colleagues,

Recently, many ultrasound applications for the diagnosis of neuromuscular disorders have emerged. Neuromuscular ultrasound protocols are increasingly being used in the work-up for the differential diagnosis of peripheral nerve diseases (such as entrapment neuropathies, traumatic nerve lesions, plexopathies, and polyneuropthies), muscle diseases (such as myopathies, muscular dystrophies, and myositis), neurodegenerative disorders (such as amyotrophic lateral sclerosis), among others. The number of research papers focussing upon neuromuscular ultrasound continues to grow, and the clinical applications of neuromuscular ultrasound continue to expand. In addition, there is a growing number of promising technical advancements in neuromuscular ultrasound, such as the evaluation of the echogenicity of nerves and muscles, the assessment of intraneural blood flow using Doppler ultrasound, or ultrasound elastography to assess changes in the stiffness of the nerve.

Ultrasound is a fast, comfortable, and widely available diagnostic tool that provides additional morphological information about the peripheral nerves and muscles. It is growing in importance in the field of neuromuscular diseases, and therefore, Medicina is launching this Special Issue.

We encourage you and your co-workers to submit your articles reporting on this topic. Reviews or original articles dealing with the clinical use of neuromuscular ultrasound are welcome in particular. In addition, we warmly invite you to submit articles reporting evidence and expectations from innovative ultrasound techniques.

Prof. Dr. Hubertus Axer
Dr. Bianka Heiling
Guest Editors

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Keywords

  • peripheral nerve ultrasound
  • muscle ultrasound
  • polyneuropathy
  • nerve trauma
  • entrapment syndromes
  • myopathy
  • myositis
  • ultrasound techniques

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Published Papers (5 papers)

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Research

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11 pages, 1152 KiB  
Article
Peripheral Nerve Ultrasound for the Differentiation between ALS, Inflammatory, and Hereditary Polyneuropathies
by Annkatrin Hildebrand, Frank Schreiber, Luisa Weber, Philipp Arndt, Cornelia Garz, Susanne Petri, Johannes Prudlo, Sven G. Meuth, Yannic Waerzeggers, Solveig Henneicke, Stefan Vielhaber and Stefanie Schreiber
Medicina 2023, 59(7), 1192; https://doi.org/10.3390/medicina59071192 - 24 Jun 2023
Cited by 5 | Viewed by 2170
Abstract
Background and Objectives: Ultrasound (US) is a non-invasive tool for the in vivo detection of peripheral nerve alterations. Materials and Methods: In this study, we applied nerve US to assist the discrimination between the spectrum of amyotrophic lateral sclerosis (ALS, n = 11), [...] Read more.
Background and Objectives: Ultrasound (US) is a non-invasive tool for the in vivo detection of peripheral nerve alterations. Materials and Methods: In this study, we applied nerve US to assist the discrimination between the spectrum of amyotrophic lateral sclerosis (ALS, n = 11), chronic inflammatory demyelinating polyradiculoneuropathy (CIDP, n = 5), and genetically confirmed Charcot–Marie–Tooth disease (CMT, n = 5). All participants and n = 15 controls without neurological diseases underwent high-resolution US of the bilateral tibial nerve. The nerve cross-sectional area (CSA) and nerve microvascular blood flow were compared between the groups and related to cerebrospinal fluid (CSF) measures, clinical symptoms, and nerve conduction studies. The analyses are part of a larger multimodal study on the comparison between US and 7 Tesla (7T) magnetic resonance neurography (MRN). Results: The patients and controls were matched with respect to their demographical data. CMT had the longest disease duration, followed by CIDP and ALS. CSA was related to age, weight, and disease duration. CSA was larger in CMT and CIDP compared to ALS and controls. The blood flow was greatest in CIDP, and higher than in CMT, ALS, and controls. In ALS, greater CSA was correlated with greater CSF total protein and higher albumin quotient. The US measures did not correlate with clinical scores or nerve conduction studies in any of the subgroups. Conclusion: Our results point towards the feasibility of CSA and blood flow to discriminate between ALS, CIDP, and CMT, even in groups of small sample size. In ALS, larger CSA could indicate an inflammatory disease subtype characterized by reduced blood–nerve barrier integrity. Our upcoming analysis will focus on the additive value of 7T MRN in combination with US to disentangle the spectrum between more inflammatory or more degenerative disease variants among the disease groups. Full article
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11 pages, 1042 KiB  
Article
Nerve Ultrasound of Peripheral Nerves in Patients Treated with Immune Checkpoint Inhibitors
by Katharina Kneer, Jan-Hendrik Stahl, Cornelius Kronlage, Paula Bombach, Mirjam Renovanz, Natalie Winter and Alexander Grimm
Medicina 2023, 59(6), 1003; https://doi.org/10.3390/medicina59061003 - 23 May 2023
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Abstract
Background and Objectives: Immune checkpoint inhibitors (ICIs) have enriched tumor therapy, improving overall survival. Immunotherapy adverse events (irAEs) occur in up to 50% of patients and also affect the peripheral nervous system. The exact pathomechanism is unclear; however, an autoimmune process is [...] Read more.
Background and Objectives: Immune checkpoint inhibitors (ICIs) have enriched tumor therapy, improving overall survival. Immunotherapy adverse events (irAEs) occur in up to 50% of patients and also affect the peripheral nervous system. The exact pathomechanism is unclear; however, an autoimmune process is implicated. Thus, the clinical evaluation of irAEs in the peripheral nervous system is still demanding. We retrospectively analyzed nerve ultrasound (NU) data of polyneuropathies (PNPs) secondary to checkpoint inhibitors. Materials and Methods: NU data of patients with PNP symptoms secondary to ICI therapy were retrospectively analyzed using the Ultrasound Pattern Sum Score (UPSS) as a quantitative marker. Our findings were compared with a propensity score match analysis (1:1 ratio) to NU findings in patients with chronic inflammatory demyelinating polyneuropathy (CIDP) and chemotherapy-associated PNP patients. Results: In total, 10 patients were included (4 female, mean age 66 ± 10.5, IQR 60–77), where NU was performed in 80%. The UPSS obtained ranged from 0 to 5 (mean 2 ± 1.6, IQR 1–2.5). The morphological changes seen in the NUs resembled sonographic changes seen in chemotherapy-associated PNP (n = 10, mean UPSS 1 ± 1, IQR 0–2) with little to no nerve swelling. In contrast, CIDP patients had a significantly higher UPSS (n = 10, mean UPSS 11 ± 4, IQR 8–13, p < 0.0001). Conclusions: Although an autoimmune process is hypothesized to cause peripheral neurological irAEs, NU showed no increased swelling as seen in CIDP. The nerve swelling observed was mild and comparable to ultrasound findings seen in chemotherapy-associated PNP. Full article
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9 pages, 950 KiB  
Article
Nerve Ultrasound Score in Chronic Inflammatory Demyelinating Polyneuropathy
by Cheng-Yin Tan, Mohd Azly Yahya, Khean-Jin Goh and Nortina Shahrizaila
Medicina 2023, 59(4), 747; https://doi.org/10.3390/medicina59040747 - 11 Apr 2023
Cited by 5 | Viewed by 2535
Abstract
Background and Objectives: Studies have suggested that, by applying certain nerve ultrasound scores, demyelinating and axonal polyneuropathies can be differentiated. In the current study, we investigated the utility of ultrasound pattern sub-score A (UPSA) and intra- and internerve cross-sectional area (CSA) variability [...] Read more.
Background and Objectives: Studies have suggested that, by applying certain nerve ultrasound scores, demyelinating and axonal polyneuropathies can be differentiated. In the current study, we investigated the utility of ultrasound pattern sub-score A (UPSA) and intra- and internerve cross-sectional area (CSA) variability in the diagnostic evaluation of demyelinating neuropathies. Materials and Methods: Nerve ultrasound was performed in patients with chronic inflammatory demyelinating polyneuropathy (CIDP) and acute inflammatory demyelinating polyneuropathy (AIDP) and compared to patients with axonal neuropathies. The UPSA, i.e., the sum of ultrasound scores at eight predefined measurement points in the median (forearm, elbow and mid-arm), ulnar (forearm and mid-arm), tibial (popliteal fossa and ankle) and fibular (lateral popliteal fossa) nerves, was applied. Intra- and internerve CSA variability were defined as maximal CSA/minimal CSA for each nerve and each subject, respectively. Results: A total of 34 CIDP, 15 AIDP and 16 axonal neuropathies (including eight axonal Guillain-Barré syndrome (GBS), four hereditary transthyretin amyloidosis, three diabetic polyneuropathy and one vasculitic neuropathy) were included. A total of 30 age- and sex-matched healthy controls were recruited for comparison. Significantly enlarged nerve CSA was observed in CIDP and AIDP with significantly higher UPSA in CIDP compared to the other groups (9.9 ± 2.9 vs. 5.9 ± 2.0 vs. 4.6 ± 1.9 in AIDP vs. axonal neuropathies, p < 0.001). A total of 89.3% of the patients with CIDP had an UPSA score ≥7 compared to the patients with AIDP (33.3%) and axonal neuropathies (25.0%) (p < 0.001). Using this cut-off, the performance of UPSA in differentiating CIDP from other neuropathies including AIDP was excellent (area under the curve of 0.943) with high sensitivity (89.3%), specificity (85.2%) and positive predictive value (73.5%). There were no significant differences in intra- and internerve CSA variability between the three groups. Conclusion: The UPSA ultrasound score was useful in distinguishing CIDP from other neuropathies compared to nerve CSA alone. Full article
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11 pages, 2230 KiB  
Article
Evaluating Diagnostic Ultrasound of the Vagus Nerve as a Surrogate Marker for Autonomic Neuropathy in Diabetic Patients
by Bianka Heiling, Adriana Karl, Nadin Fedtke, Nicolle Müller, Christof Kloos, Alexander Grimm and Hubertus Axer
Medicina 2023, 59(3), 525; https://doi.org/10.3390/medicina59030525 - 8 Mar 2023
Cited by 2 | Viewed by 2344
Abstract
Background and Objectives: Diagnostic ultrasound of the vagus nerve has been used to examine different polyneuropathies, and it has been suggested to be useful as a marker of autonomic dysfunction in diabetic patients. Materials and Methods: We analyzed the cross-sectional area [...] Read more.
Background and Objectives: Diagnostic ultrasound of the vagus nerve has been used to examine different polyneuropathies, and it has been suggested to be useful as a marker of autonomic dysfunction in diabetic patients. Materials and Methods: We analyzed the cross-sectional area (CSA) of the right vagus nerve of 111 patients with type 2 diabetes in comparison to 104 healthy adults and 41 patients with CIDP (chronic inflammatory demyelinating polyneuropathy). In the diabetes group, sympathetic skin response (SSR) was measured as an indicator for autonomic neuropathy. Carotid intima–media thickness (CIMT) was measured as a surrogate for atherosclerosis. Clinical symptoms of polyneuropathy were assessed using the Neuropathy Symptom Score and the Neuropathy Disability Score. Results: In total, 61.3% of the diabetes patients had clinical signs of polyneuropathy; 23.4% had no SSR at the feet as an indicator of autonomic neuropathy. Mean vagus nerve CSA did not differ in patients with and without diabetic polyneuropathy or in diabetic patients with and without SSR at the feet. No significant correlation was found between vagus nerve CSA and CIMT or SSR parameters in diabetic patients. Mean CSA of the right vagus nerve was slightly larger in diabetic patients (p = 0.028) and in patients with CIDP (p = 0.015) than in healthy controls. Conclusions: Effect sizes and mean differences were rather small so that a reliable diagnosis cannot be performed based on the vagus nerve measurement of a single person alone. Vagus nerve CSA seems not suitable as an indicator of autonomic dysfunction or cardiovascular risk in diabetic patients. Full article
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Review

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13 pages, 1382 KiB  
Review
Neuromuscular Ultrasound in Intensive Care Unit-Acquired Weakness: Current State and Future Directions
by Felix Klawitter, Uwe Walter, Hubertus Axer, Robert Patejdl and Johannes Ehler
Medicina 2023, 59(5), 844; https://doi.org/10.3390/medicina59050844 - 27 Apr 2023
Cited by 6 | Viewed by 3321
Abstract
Intensive care unit-acquired weakness (ICUAW) is one of the most common causes of muscle atrophy and functional disability in critically ill intensive care patients. Clinical examination, manual muscle strength testing and monitoring are frequently hampered by sedation, delirium and cognitive impairment. Many different [...] Read more.
Intensive care unit-acquired weakness (ICUAW) is one of the most common causes of muscle atrophy and functional disability in critically ill intensive care patients. Clinical examination, manual muscle strength testing and monitoring are frequently hampered by sedation, delirium and cognitive impairment. Many different attempts have been made to evaluate alternative compliance-independent methods, such as muscle biopsies, nerve conduction studies, electromyography and serum biomarkers. However, they are invasive, time-consuming and often require special expertise to perform, making them vastly impractical for daily intensive care medicine. Ultrasound is a broadly accepted, non-invasive, bedside-accessible diagnostic tool and well established in various clinical applications. Hereby, neuromuscular ultrasound (NMUS), in particular, has been proven to be of significant diagnostic value in many different neuromuscular diseases. In ICUAW, NMUS has been shown to detect and monitor alterations of muscles and nerves, and might help to predict patient outcome. This narrative review is focused on the recent scientific literature investigating NMUS in ICUAW and highlights the current state and future opportunities of this promising diagnostic tool. Full article
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