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Contains fulltext : 203073.pdf (Publisher’s version ) (Closed access) INTRODUCTION: Neuralgic amyotrophy (NA) can often be difficult to diagnose. Nerve ultrasound (US) is potentially useful, but it is operator-dependent, especially for small nerves. METHODS: Fifty-one consecutive patients with NA (mean duration 16 months) and 50 control subjects underwent US of the brachial plexus and major nerves of the upper extremity at predefined sites. We compared cross-sectional areas (CSAs) of affected and unaffected sides with controls and sides within patients. RESULTS: The median nerve and radial nerve at the level of the upper arm were enlarged on the affected sides compared with controls and the unaffected sides of patients. Enlargement was most pronounced for affected sides vs. controls (median 44%, radial 67%). DISCUSSION: NA patients showed increased CSAs, especially in the major nerves of the upper limb, even after longer disease duration. This could make US a useful adjunct in diagnosing NA. Muscle Nerve 59:55-59, 2019.

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Item does not contain fulltext INTRODUCTION: In this exploratory study we investigated whether ultrasound can visualize the neonatal cervical roots and brachial plexus. METHODS: In 12 healthy neonates

Contains fulltext : 169859.pdf (Publisher’s version ) (Closed access) Myalgia, fatigue, and exercise intolerance are cause for referral to a neurologist. However, the diagnostic value of history, neurological examination, and ancillary investigations in patients with these symptoms is unknown. This study provides a sound footing for deciding which ancillary investigations should be conducted. A prospective observational study of the diagnostic approach in 187 patients with myalgia, exercise intolerance, or fatigue as their predominant symptom was performed. The primary outcomes were independent contribution of referral letter, history, examination, and ancillary investigations to a myopathy diagnosis. The secondary outcome was diagnostic value of combined ancillary investigations. 27% of patients had a myopathy. Positive family history (OR 3.2), progressive symptoms (OR 2.2), atrophy (OR 9.7), weakness (OR 10.9), and hyporeflexia (OR 4.4) were associated with a myopathy. Positive predictive values for myopathy were calculated for CK (0.32), EMG (0.66), ultrasound (0.47), and muscle biopsy (0.78). All contributed significantly in predicting myopathy. Multivariate analysis yielded a diagnostic algorithm facilitating a more efficient work-up in future patients. CK levels, EMG, ultrasound, and muscle biopsy independently contribute to predicting a myopathy. The diagnostic algorithm shows which combination of ancillary investigations should be employed in different subgroups and when to omit invasive techniques. This algorithm may drastically improve diagnostic efficiency.

Item does not contain fulltext INTRODUCTION: In this study we describe a protocol for quantitative ultrasound of facial muscles (procerus, zygomaticus major, levator labii superior, depressor anguli oris, mentalis, orbicularis oris pars labialis, orbicularis oris pars marginalis). Methods: Muscle thickness (MT) and echo intensity (EI) were measured in 12 healthy subjects and a myotonic dystrophy type 1 patient. Results: MTs ranged from 0.15 to 0.30 mm, except for the procerus muscle (0.06 mm). EIs ranged from 1 to 34, except for the procerus muscle. MT reproducibility was fair for the orbicularis oris pars labialis, excellent for the procerus and levator labii, and good for the other muscles. The myotonic dystrophy type 1 patient showed high EIs, outside the range in healthy subjects in 6 of the 7 muscles. MT was lower than the range seen in healthy subjects in 4 muscles. Conclusion: Quantitative muscle ultrasound of the facial muscles is feasible and shows moderate to excellent reproducibility. Muscle Nerve 48: 375-380, 2013.

Item does not contain fulltext INTRODUCTION: No longitudinal data on the normal development of muscle thickness (MT), quantitative muscle ultrasound echo intensity (EI), and muscle force (MF) in healthy children are yet available. METHODS: Reference values of MT, EI, and MF of 4 muscles from infancy to age 16 years were established during a 4-year follow-up period and correlated with age and growth. RESULTS: For most muscles, MT and MF correlated with growth and aging, with almost equal influences of weight and height. EI increased only slightly (1% per year) with height, weight, and age. CONCLUSIONS: To use these reference values for repeated measurements, MF and MT can be corrected for either weight or height. It does not seem necessary to correct EI for these factors during follow-up of a few years. These results provide a basis for more precise detection of changes in muscle structure or force in neuromuscular disorders. Muscle Nerve 47: 856-863, 2013.

Item does not contain fulltext Muscle ultrasound is an ideal imaging modality that allows for noninvasive, radiation-free point-of-care neuromuscular imaging. There are many potential applications of muscle ultrasound, including identification of abnormal muscle movements such as fasciculations, evaluation of muscle trauma, identification of physiologic parameters such as pennation angle, accurate performance of chemodenervation, and improved accuracy of challenging electrodiagnostic studies such as phrenic nerve conduction studies or needle electromyogram (EMG) of the diaphragm. Tissue Doppler imaging can be used to help identify inflammatory myopathies. With computer-assisted quantification, muscle ultrasound has high sensitivity and specificity in the diagnosis of pediatric neuromuscular disease and amyotrophic lateral sclerosis, and is a valuable addition to other diagnostic techniques for neuromuscular disease. When used as a first-line screening tool it can obviate the need for more invasive procedures such as EMG or muscle biopsy in certain patients. This chapter provides an overview of the fundamentals, clinical applications, and validation of muscle ultrasound for patients with neuromuscular disorders.

Item does not contain fulltext BACKGROUND: Ultrasound is a non-invasive method to quantitatively measure various muscle parameters. Purpose of this study was to assess the feasibility of ultrasound of lower leg and foot muscles and to obtain reference values for muscle thickness (MT) and echo intensity (EI). METHODS: Ultrasound measurements of leg and foot muscles were performed in 60 healthy adults. MT and EI were quantitatively determined for the abductor hallucis (AH), extensor digitorum brevis (EDB), extensor hallucis longus (EHL) and peroneus longus (PER) muscles. Influence of age, height, weight and sex was determined using a multiple linear regression analysis. RESULTS: All muscles except the AH could easily be visualized with ultrasound. EI tended to be increased above 60 years and MT was significantly higher in men compared to women, necessitating age- and sex-dependent reference values. CONCLUSIONS: This study shows that muscle ultrasound is capable of visualizing lower leg and foot muscles and reference values for MT and EI can be obtained. Future research will focus on the use of these reference values to evaluate muscle abnormalities caused by neuromuscular disorders like hereditary motor and sensory neuropathy.

Item does not contain fulltext We prospectively investigated the diagnostic accuracy and potential pitfalls of dynamic muscle ultrasound in the detection of fibrillations. The presence of fibrillations on both electromyography (EMG) and ultrasound was evaluated in 102 muscles of 38 patients with suspected fibrillation potentials, based on history and physical examination. Sensitivity and specificity were calculated. False-positive and false-negative results are described to identify pitfalls. Ultrasound detected fibrillations with a sensitivity of 45% and a specificity of 66%. Specificity improved to 85% when measurements were evaluated online. Proximal muscles showed higher sensitivities (63%) than distal muscles (33%). Ultrasound currently has a poor sensitivity and moderate specificity for detecting fibrillations. Several pitfalls were identified that, when avoided, could help improve diagnostic accuracy. Ensuring a distal limb temperature of at least 34 degrees C and identifying external movement artifacts as well as voluntary contractions can improve efficacy and open the way for diagnostic application of this technique.

Contains fulltext : 89861.pdf (Publisher’s version ) (Closed access) Ultrasonography can detect structural muscle changes caused by neuromuscular disease. Quantitative analysis is the preferred method to determine if ultrasound findings are within normal limits, but normative data are incomplete. The purpose of this study was to provide normative muscle ultrasonography data for muscle thickness and echo intensity for five different muscle groups in adults. Bilateral scans of the sternocleidomastoid, biceps brachii/brachialis, forearm flexor group, quadriceps femoris, and tibialis anterior were made in 95 volunteers, aged 17-90 years. Both muscle thickness and echo intensity showed gender differences and a muscle-specific non-linear correlation with age. The muscles of the upper extremities showed right-left differences. These data demonstrate the effect of age on muscle characteristics and provide normative values that can be used in clinical practice. 01 januari 2010

Contains fulltext : 70571.pdf (Publisher’s version ) (Closed access) Localized scleroderma is characterized by idiopathic fibrosis of the skin and adjacent structures, and muscle involvement occurs predominantly in deep morphea. We report a patient with linear scleroderma who presented with slowly progressive atrophy, muscle weakness, and loss of function of her right arm, mimicking a neuromuscular disorder. Muscle biopsy eventually revealed zones of myositis, compatible with morphea profunda. Morphea profunda may thus present as a neuromuscular mimic, even in case of nonprogressive skin sclerosis.Myositis in morphea profunda is generally limited to one region, whereas inflammatory myopathies generally cause diffuse proximal muscle weakness and atrophy. Furthermore, skin changes in morphea profunda differ from those seen in dermatomyositis, and histological features of muscle biopsy can further distinguish between morphea profunda and inflammatory myopathies. Muscle biopsy in morphea profunda implies the risk of sampling error, whereas results of electromyography and muscle imaging might better represent the extent of muscle inflammation.

Contains fulltext : 53398.pdf (Publisher’s version ) (Open Access)

Item does not contain fulltext INTRODUCTION: We explored the use of quantitative muscle ultrasonography (QMUS) for follow-up of juvenile dermatomyositis (JDM). METHODS: Seven JDM patients were evaluated at diagnosis and 1, 3, 6, 12, and 24 months using the Childhood Myositis Assessment Scale (CMAS) and QMUS. Muscle thickness (MT) and quantitative muscle echo intensity (EI) were assessed with QMUS in 4 muscles. RESULTS: Six patients experienced a monocyclic course. At diagnosis EI was slightly increased, and MT was relatively normal. After start of treatment MT first decreased and EI increased, with normalization of EI within 6-12 months (n = 4). One patient had higher EIs at diagnosis and slower normalization, indicating fibrosis, despite early normalization of CMAS. One patient experienced a chronic course, with high EIs and atrophy during follow-up. CONCLUSIONS: QMUS can provide additional information for follow-up of JDM regarding disease severity and residual muscle damage, particularly after normalization of CMAS. Muscle Nerve 52: 540-546, 2015.

Contains fulltext : 153146.pdf (Publisher’s version ) (Closed access) INTRODUCTION: We investigated the use of ultrasound to detect fascial thickening of the deltoid, vastus lateralis (VL), and rectus femoris (RF) muscles in dermatomyositis (DM) and polymyositis (PM). METHODS: Fascial thickness of 7 DM and 5 PM patients was compared with that of healthy controls (n = 54). RESULTS: The deltoid fascial thickness was 2-fold greater in the DM/PM group (1.15 mm vs. 0.54 mm; P < 0.001) compared with healthy controls. Eight patients had markedly thickened deltoid fascia (>5 SD). Only 1 patient with a mild clinical presentation had normal deltoid fascial thickness. Four patients also had fascial thickening of the VL and/or RF. CONCLUSIONS: Deltoid fascial thickness was increased significantly in DM and PM patients, whereas the quadriceps muscle only showed thickened fascia in severely affected patients. This study suggests that a concomitant fasciitis may be present more often than previously believed. Muscle Nerve, 2015 Muscle Nerve 52: 534-539, 2015.

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Item does not contain fulltext Background: Ultrasound and MR imaging are non-invasive methods that can be performed repeatedly and without discomfort. In the assessment of neuromuscular disorders it is unknown if they hold complementary information. In this study we tested this for patients with facioscapulohumeral muscular dystrophy (FSHD). Methods: We performed quantitative muscle ultrasound (QMUS) and quantitative MRI (QMRI) of the legs in 5 men with FSHD. Results: The correlation between QMUS determined z-scores and QMRI determined muscle fraction and T1 signal intensity (SI) was very high. QMUS had a wider dynamic range than QMRI, whereas QMRI could detect inhomogeneous distribution of pathology over the length of the muscles. Discussion: Both QMUS and QMRI are well suited for imaging muscular dystrophy. The larger dynamic range of QMUS can be advantageous in follow-up of advanced disease stages, whereas QMRI seems preferable in pathologies such as FSHD that affect deep muscle layers and show inhomogeneous abnormality distributions. © 2014 Wiley Periodicals, Inc.

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Contains fulltext : 80494.pdf (Publisher’s version ) (Closed access)

Contains fulltext : 81858.pdf (Publisher’s version ) (Open Access)

Contains fulltext : 74405.pdf (Publisher’s version ) (Open Access) RU Radboud Universiteit Nijmegen, 26 mei 2009 Promotor : Zwarts, M.J. Co-promotores : Verrips, A., Alfen, N. van 245 p.

Contains fulltext : 51752.pdf (Publisher’s version ) (Closed access)

Achtergrond Het Kleine-Levin-syndroom (KLS) is een zeldzame aandoening met periodieke extreme slaapzucht als belangrijkste symptoom. Daarnaast worden vraatzucht en hyperseksualiteit als klassieke symptomen genoemd, maar uit recent onderzoek blijkt dat deze volledige trias bij de meerderheid van de patiënten afwezig is. Casus Een 14-jarige jongen ontwikkelde na een periode met griepachtige verschijnselen KLS. In 3 jaar had hij 7 episodes van een week met extreme slaapzucht. Hij sliep daarbij 18 uur per dag, had last van depersonalisatie, apathie, angst, achterdocht, verwardheid en hallucinaties, en vertoonde seksueel ontremd gedrag. Hij at weinig. Aanvullend onderzoek toonde geen afwijkingen. Tussen deze episodes had hij geen klachten. Conclusie Uit deze casusbeschrijving en een samenvatting van de symptomen van 12 andere patiënten met KLS blijkt dat neuropsychiatrische symptomen veel typerender zijn voor KLS dan vraatzucht en hyperseksualiteit. Het is belangrijk om het beeld van de ‘standaard KLS-patiënt’ bij te stellen, zodat de aandoening tijdig wordt herkend en patiënten adequaat begeleid kunnen worden.