Diagnostics

Research

14 pages, 2017 KiB

Open AccessArticle

Dorsal Root Ganglia Volume—Normative Values, Correlation with Demographic Determinants and Reliability of Three Different Methods of Volumetry

by Moritz Kronlage, Thomas David Fischer, Rouven Behnisch, Daniel Schwarz, Philipp Bäumer, Veronique Schwehr, Sabine Heiland, Martin Bendszus and Tim Godel

Diagnostics 2022, 12(7), 1570; https://doi.org/10.3390/diagnostics12071570 - 28 Jun 2022

Cited by 2 | Viewed by 1381

Abstract

Background: Dorsal root ganglia (DRG) volume assessment by MR-Neurography (MRN) has evolved to an important imaging marker in the diagnostic workup of various peripheral neuropathies and pain syndromes. The aim of this study was (1) to assess normal values of DRG volume and [...] Read more.

Background: Dorsal root ganglia (DRG) volume assessment by MR-Neurography (MRN) has evolved to an important imaging marker in the diagnostic workup of various peripheral neuropathies and pain syndromes. The aim of this study was (1) to assess normal values of DRG volume and correlations with demographic determinants and (2) to quantify the inter-reader and inter-method reliability of three different methods of DRG volumetry. Methods: Sixty healthy subjects (mean age: 59.1, range 23–79) were examined using a 3D T2-weighted MRN of the lumbosacral plexus at 3 Tesla. Normal values of DRG L3 to S2 were obtained after exact volumetry based on manual 3D segmentation and correlations with demographic variables were assessed. For the assessment of inter-reader and inter-method reliability, DRG volumes in a subset of 25 participants were measured by two independent readers, each applying (1) exact volumetry based on 3D segmentation, (2) axis-corrected, and (3) non-axis-corrected volume estimation. Intraclass correlation coefficients were reported and the Bland–Altman analysis was conducted. Results: Mean DRG volumes ranged from 124.8 mm³ for L3 to 323.3 mm³ for S1 and did not differ between right and left DRG. DRG volume (mean of L3 to S1) correlated with body height (r = 0.42; p = 0.0008) and weight (r = 0.34; p = 0.0087). DRG of men were larger than of women (p = 0.0002); however, no difference remained after correction for body height. Inter-reader reliability was high for all three methods but best for exact volumetry (ICC = 0.99). While axis-corrected estimation was not associated with a relevant bias, non-axis-corrected estimation systematically overestimated DRG volume by on average of 15.55 mm³ (reader 1) or 18.00 mm³ (reader 2) when compared with exact volumetry. Conclusion: The here presented normal values of lumbosacral DRG volume and the correlations with height and weight may be considered in future disease specific studies and possible clinical applications. Exact volumetry was most reliable and should be considered the gold standard. However, the reliability of axis-corrected and non-axis-corrected volume estimation was also high and might still be sufficient, depending on the degree of the required measurement accuracy. Full article

(This article belongs to the Special Issue The Diagnostic Value of Nerve and Muscle Ultrasound in Neuromuscular Disorders)

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11 pages, 1785 KiB

Open AccessArticle

Spatial Distribution and Long-Term Alterations of Peripheral Nerve Lesions in Schwannomatosis

by Tim Godel, Philipp Bäumer, Said Farschtschi, Barbara Hofstadler, Sabine Heiland, Mathias Gelderblom, Martin Bendszus and Victor-Felix Mautner

Diagnostics 2022, 12(4), 780; https://doi.org/10.3390/diagnostics12040780 - 23 Mar 2022

Viewed by 1442

Abstract

Purpose To examine the spatial distribution and long-term alterations of peripheral nerve lesions in patients with schwannomatosis by in vivo high-resolution magnetic resonance neurography (MRN). Methods In this prospective study, the lumbosacral plexus as well as the right sciatic, tibial, and peroneal nerves [...] Read more.

Purpose To examine the spatial distribution and long-term alterations of peripheral nerve lesions in patients with schwannomatosis by in vivo high-resolution magnetic resonance neurography (MRN). Methods In this prospective study, the lumbosacral plexus as well as the right sciatic, tibial, and peroneal nerves were examined in 15 patients diagnosed with schwannomatosis by a standardized MRN protocol at 3 Tesla. Micro-, intermediate- and macrolesions were assessed according to their number, diameter and spatial distribution. Moreover, in nine patients, peripheral nerve lesions were compared to follow-up examinations after 39 to 71 months. Results In comparison to intermediate and macrolesions, microlesions were the predominant lesion entity at the level of the proximal (p < 0.001), mid- (p < 0.001), and distal thigh (p < 0.01). Compared to the proximal calf level, the lesion number was increased at the proximal (p < 0.05), mid- (p < 0.01), and distal thigh level (p < 0.01), while between the different thigh levels, no differences in lesion numbers were found. In the follow-up examinations, the lesion number was unchanged for micro-, intermediate and macrolesions. The diameter of lesions in the follow-up examination was decreased for microlesions (p < 0.01), not different for intermediate lesions, and increased for macrolesions (p < 0.01). Conclusion Microlesions represent the predominant type of peripheral nerve lesion in schwannomatosis and show a rather consistent distribution pattern in long-term follow-up. In contrast to the accumulation of nerve lesions, primarily in the distal nerve segments in NF2, the lesion numbers in schwannomatosis peak at the mid-thigh level. Towards more distal portions, the lesion number markedly decreases, which is considered as a general feature of other types of small fiber neuropathy. Full article

(This article belongs to the Special Issue The Diagnostic Value of Nerve and Muscle Ultrasound in Neuromuscular Disorders)

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8 pages, 627 KiB

Open AccessArticle

Assessment of Operator Reliability in Ultrasound of the Median and Ulnar Nerve Using Bland-Altman Analysis

by Jörg Philipps, Hannah Mork, Maria Katz, Mark Knaup, Kira Beyer, Kristina Spies, Johannes Corbach and Peter D. Schellinger

Diagnostics 2021, 11(11), 2134; https://doi.org/10.3390/diagnostics11112134 - 17 Nov 2021

Viewed by 1562

Abstract

Currently, there is no standardized method to evaluate operator reliability in nerve ultrasound. A short prospective protocol using Bland–Altman analysis was developed to assess the level of agreement between operators with different expertise levels. A control rater without experience in nerve ultrasound, three [...] Read more.

Currently, there is no standardized method to evaluate operator reliability in nerve ultrasound. A short prospective protocol using Bland–Altman analysis was developed to assess the level of agreement between operators with different expertise levels. A control rater without experience in nerve ultrasound, three novices after two months of training, an experienced rater with two years of experience, and a reference rater performed blinded ultrasound examinations of the left median and ulnar nerve in 42 nerve sites in healthy volunteers. The precision of Bland–Altman agreement analysis was tested using the Preiss–Fisher procedure. Intraclass correlation coefficients (ICC), coefficients of variation, and Bland–Altman limits of agreement were calculated. The sample size calculation and Preiss–Fisher procedure showed a sufficient precision of Bland–Altman agreement analysis. Limits of agreement of all trained novices ranged from 2.0 to 2.9 mm² and were within the test’s maximum tolerated difference. Ninety-five percent confidence intervals of limits of agreement revealed a higher precision in the experienced rater’s measurements. Operator reliability in nerve ultrasound of the median and ulnar nerve arm nerves can be evaluated with a short prospective controlled protocol using Bland–Altman statistics, allowing a clear distinction between an untrained rater, trained novices after two months of training, and an experienced rater. Full article

(This article belongs to the Special Issue The Diagnostic Value of Nerve and Muscle Ultrasound in Neuromuscular Disorders)

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13 pages, 1446 KiB

Open AccessArticle

High-Resolution Nerve Ultrasound Abnormalities in POEMS Syndrome—A Comparative Study

by Marc Dörner, Mihai Ceanga, Frank Schreiber, Jan-Hendrik Stahl, Cornelius Kronlage, Julia Wittlinger, Magdalena Kramer, Sophia Willikens, Stefanie Schreiber, Alexander Grimm and Natalie Winter

Diagnostics 2021, 11(2), 264; https://doi.org/10.3390/diagnostics11020264 - 9 Feb 2021

Cited by 7 | Viewed by 2533

Abstract

Background: High-resolution nerve ultrasound (HRUS) has been proven to be a valuable tool in the diagnosis of immune-mediated neuropathies, such as chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes) is an important differential diagnosis of CIDP. Until now, [...] Read more.

Background: High-resolution nerve ultrasound (HRUS) has been proven to be a valuable tool in the diagnosis of immune-mediated neuropathies, such as chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes) is an important differential diagnosis of CIDP. Until now, there have been no studies that could identify specific HRUS abnormalities in POEMS syndrome patients. Thus, the aim of this study was to assess possible changes and compare findings with CIDP patients. Methods: We retrospectively analyzed HRUS findings in three POEMS syndrome and ten CIDP patients by evaluating cross-sectional nerve area (CSA), echogenicity and additionally calculating ultrasound pattern scores (UPSA, UPSB, UPSC and UPSS) and homogeneity scores (HS). Results: CIDP patients showed greater CSA enlargement and higher UPSS (median 14 vs. 11), UPSA (median 11.5 vs. 8) and HS (median 5 vs. 3) compared with POEMS syndrome patients. However, every POEMS syndrome patient illustrated enlarged nerves exceeding reference values, which were not restricted to entrapment sites. In CIDP and POEMS syndrome, heterogeneous enlargement patterns could be identified, such as inhomogeneous, homogeneous and regional nerve enlargement. HRUS in CIDP patients visualized both increased and decreased echointensity, while POEMS syndrome patients pictured hypoechoic nerves with hyperechoic intraneural connective tissue. Discussion: This is the first study to demonstrate HRUS abnormalities in POEMS syndrome outside of common entrapment sites. Although nerve enlargement was more prominent in CIDP, POEMS syndrome patients revealed distinct echogenicity patterns, which might aid in its differentiation from CIDP. Future studies should consider HRUS and its possible role in determining diagnosis, prognosis and treatment response in POEMS syndrome. Full article

(This article belongs to the Special Issue The Diagnostic Value of Nerve and Muscle Ultrasound in Neuromuscular Disorders)

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11 pages, 1449 KiB

Open AccessArticle

Assessment of Peripheral Nervous System Alterations in Patients with the Fabry Related GLA-Variant p.A143T

by Tim Godel, Katharina von Cossel, Reinhard E. Friedrich, Markus Glatzel, Sima Canaan-Kühl, Thomas Duning, Moritz Kronlage, Sabine Heiland, Martin Bendszus, Nicole Muschol and Victor-Felix Mautner

Diagnostics 2020, 10(12), 1027; https://doi.org/10.3390/diagnostics10121027 - 30 Nov 2020

Cited by 5 | Viewed by 2343

Abstract

The purpose of this study is to examine alterations of the peripheral nervous system (PNS) in oligo-symptomatic patients carrying the Fabry related GLA-gene variant p.A143T by Magnetic Resonance Neurography (MRN) and skin biopsy. This prospective study assessed dorsal root ganglia (DRG) volume [...] Read more.

The purpose of this study is to examine alterations of the peripheral nervous system (PNS) in oligo-symptomatic patients carrying the Fabry related GLA-gene variant p.A143T by Magnetic Resonance Neurography (MRN) and skin biopsy. This prospective study assessed dorsal root ganglia (DRG) volume L3 to S2, vascular permeability of the DRG L5, S1, and the spinal nerve L5 in five patients carrying p.A143T in comparison to patients with classical Fabry mutations and healthy controls. Moreover, skin punch biopsies above the lateral malleolus of the right foot were obtained in four patients and intraepidermal nerve fiber density (IENFD) was counted individually. Compared to controls, DRG volumes of p.A143T patients were enlarged by 30% (L3, p < 0.05), 35% (L4, p < 0.05), 29% (L5, p = 0.15), 36% (S1, p < 0.01), and 18% (S2, p < 0.05), but less pronounced compared to patients carrying a classical Fabry mutation. Compared to healthy controls, vascular permeability was decreased by 40% (L5 right), 49% (L5 left), 48% (S1 right), and 49% (S1) (p < 0.01–p < 0.001), but non-significant less than patients carrying a classical Fabry mutation. Compared to sex-matched 5% lower normative reference values per decade, IENFD was decreased in three of four patients. MRN and determination of IENFD is able to detect early alteration of the PNS segment in oligo-symptomatic patients with the disease-modifying GLA-variant p.A143T on an individual basis. This procedure might also help in further GLA-variants of uncertain significance for early identification of patients with single major organ manifestation. Full article

(This article belongs to the Special Issue The Diagnostic Value of Nerve and Muscle Ultrasound in Neuromuscular Disorders)

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Review

Jump to: Research, Other

23 pages, 8379 KiB

Open AccessReview

Nerve Ultrasound in Traumatic and Iatrogenic Peripheral Nerve Injury

by Juerd Wijntjes, Alexandra Borchert and Nens van Alfen

Diagnostics 2021, 11(1), 30; https://doi.org/10.3390/diagnostics11010030 - 26 Dec 2020

Cited by 37 | Viewed by 9110

Abstract

Peripheral nerve injury is a potentially debilitating disorder that occurs in an estimated 2–3% of all patients with major trauma, in a similar percentage of medical procedures. The workup of these injuries has traditionally been clinical, combined with electrodiagnostic testing. However, this has [...] Read more.

Peripheral nerve injury is a potentially debilitating disorder that occurs in an estimated 2–3% of all patients with major trauma, in a similar percentage of medical procedures. The workup of these injuries has traditionally been clinical, combined with electrodiagnostic testing. However, this has limitations, especially in the acute phase of the trauma or lack of any recovery, when it is very important to determine nerve continuity and perform surgical exploration and repair in the case of the complete transection or intraneural fibrosis. Ultrasound can help in those situations. It is a versatile imaging technique with a high sensitivity of 93% for detecting focal nerve lesions. Ultrasound can assess the structural integrity of the nerve, neuroma formation and other surrounding abnormalities of bone or foreign bodies impeding the nerve. In addition, this can help to prevent iatrogenic nerve injury by marking the nerve before the procedure. This narrative review gives an overview of why and how nerve ultrasound can play a role in the detection, management and prevention of peripheral nerve injury. Full article

(This article belongs to the Special Issue The Diagnostic Value of Nerve and Muscle Ultrasound in Neuromuscular Disorders)

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7 pages, 518 KiB

Open AccessReview

Neuromuscular Ultrasound in the Pediatric Population

by Trent A. VanHorn and Michael S. Cartwright

Diagnostics 2020, 10(12), 1012; https://doi.org/10.3390/diagnostics10121012 - 26 Nov 2020

Cited by 9 | Viewed by 2221

Abstract

The diagnosis and evaluation of neuromuscular disorders traditionally involves electrodiagnostic (EDx) testing, including nerve conduction studies (NCSs) and electromyography (EMG). These tools can cause pain and discomfort, an important consideration when performed on children. Neuromuscular ultrasound is noninvasive, cost-effective, and increasingly utilized for [...] Read more.

The diagnosis and evaluation of neuromuscular disorders traditionally involves electrodiagnostic (EDx) testing, including nerve conduction studies (NCSs) and electromyography (EMG). These tools can cause pain and discomfort, an important consideration when performed on children. Neuromuscular ultrasound is noninvasive, cost-effective, and increasingly utilized for the detection of neuromuscular pathology. Studies investigating the performance and clinical implementation of ultrasound have primarily been performed in adult populations. Ultrasound in children has the potential to guide EDx testing and ultimately improve diagnostic efficiency and accuracy. This review aims to describe key features of neuromuscular ultrasound in the pediatric population based on the available studies, including our own institutional experience. Full article

(This article belongs to the Special Issue The Diagnostic Value of Nerve and Muscle Ultrasound in Neuromuscular Disorders)

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Other

Jump to: Research, Review

11 pages, 1145 KiB

Open AccessCase Report

Peripheral Nerve Imaging Aids in the Diagnosis of Immune-Mediated Neuropathies—A Case Series

by Marc Dörner, Frank Schreiber, Heike Stephanik, Claus Tempelmann, Natalie Winter, Jan-Hendrik Stahl, Julia Wittlinger, Sophia Willikens, Magdalena Kramer, Hans-Jochen Heinze, Stefan Vielhaber, Thomas Schelle, Alexander Grimm and Stefanie Schreiber

Diagnostics 2020, 10(8), 535; https://doi.org/10.3390/diagnostics10080535 - 30 Jul 2020

Cited by 6 | Viewed by 3551

Abstract

Background: Diagnosis of immune-mediated neuropathies and their differentiation from amyotrophic lateral sclerosis (ALS) can be challenging, especially at early disease stages. Accurate diagnosis is, however, important due to the different prognosis and available treatment options. We present one patient with a left-sided dorsal [...] Read more.

Background: Diagnosis of immune-mediated neuropathies and their differentiation from amyotrophic lateral sclerosis (ALS) can be challenging, especially at early disease stages. Accurate diagnosis is, however, important due to the different prognosis and available treatment options. We present one patient with a left-sided dorsal flexor paresis and initial suspicion of ALS and another with multifocal sensory deficits. In both, peripheral nerve imaging was the key for diagnosis. Methods: We performed high-resolution nerve ultrasound (HRUS) and 7T or 3T magnetic resonance neurography (MRN). Results: In both patients, HRUS revealed mild to severe, segmental or inhomogeneous, nerve enlargement at multiple sites, as well as an area increase of isolated fascicles. MRN depicted T2 hyperintense nerves with additional contrast-enhancement. Discussion: Peripheral nerve imaging was compatible with the respective diagnosis of an immune-mediated neuropathy, i.e., multifocal motor neuropathy (MMN) in patient 1 and multifocal acquired demyelinating sensory and motor neuropathy (MADSAM) in patient 2. Peripheral nerve imaging, especially HRUS, should play an important role in the diagnostic work-up for immune-mediated neuropathies and their differentiation from ALS. Full article

(This article belongs to the Special Issue The Diagnostic Value of Nerve and Muscle Ultrasound in Neuromuscular Disorders)

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4 pages, 725 KiB

Open AccessInteresting Images

Compression of the Lateral Antebrachial Cutaneous Nerve due to Leakage of Iron after an Intravenous Iron Infusion

by Soyoung Kwak and Min Cheol Chang

Diagnostics 2020, 10(8), 516; https://doi.org/10.3390/diagnostics10080516 - 25 Jul 2020

Cited by 1 | Viewed by 5337

Abstract

Skin staining due to iron leakage into the subcutaneous tissue can sometimes occur during intravenous iron infusion. We describe a case of lateral antebrachial cutaneous nerve (LACN) entrapment due to extravasated iron after an intravenous iron infusion. A 41-year-old woman received an intravenous [...] Read more.

Skin staining due to iron leakage into the subcutaneous tissue can sometimes occur during intravenous iron infusion. We describe a case of lateral antebrachial cutaneous nerve (LACN) entrapment due to extravasated iron after an intravenous iron infusion. A 41-year-old woman received an intravenous ferric carboxymaltose infusion for iron deficiency anemia. However, during the infusion, extravasation of iron occurred and brown pigmentation developed on the lateral side of the cubital fossa. Sixteen months later, the patient still had some staining in her anterolateral elbow and proximal forearm. In addition, she complained of tingling pain over her left forearm. Ultrasonography (US) revealed a lateral antebrachial cutaneous nerve (LACN) under the stained area. When we swept the stained area with the US transducer, she reported a tingling pain on her left lateral forearm, the region innervated by the left LACN. Therefore, we considered that the pain resulted from the compression of the left LACN by the leaked iron during the intravenous infusion. Leaked iron can compress the cutaneous nerve and result in neuropathic pain and cosmetic problems. When patients with skin staining after iron infusion have neuropathic pain, clinicians should consider the possibility of entrapment of the cutaneous nerves. Full article

(This article belongs to the Special Issue The Diagnostic Value of Nerve and Muscle Ultrasound in Neuromuscular Disorders)

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