Heckmatt Scale Research Articles

Ultrasound visualization of Botulinum toxin injection: Identifying clinical correlates to optimize outcomes in spasticity management.

Botulinum toxin injections are well established and commonly used for spasticity management. Clinicians strive to optimize outcomes from toxin injections. One potential complication is toxin spread beyond the intended muscle, which can lead to unwanted weakness. The utilization of ultrasound allows direct visualization of target muscles and identification of toxin leakage from the target muscle. Ultrasound evaluation of clinical factors that correlate to toxin leakage have not been studied. To identify cases of botulinum toxin injectate leak beyond the targeted muscle during ultrasound-guided spasticity injections and associate cases of leak with predictive clinical factors, which include muscle size, fibroadipose changes, and number of previous injections. This was a prospective observational study. An outpatient clinic in an academic medical center. Patients who demonstrated wrist flexor spasticity warranting intervention were invited to participate. Patients received standard-of-care spasticity management with injection of onabotulinumtoxinA into the flexor carpi radialis muscle based upon clinical presentation and prescribing guidelines. Ultrasound video was recorded, and a blinded review was conducted by the study team. The primary outcome measure was visualized leak of injectate on recorded ultrasound video. Documented leak was then associated with clinical factors including diameter of the flexor carpi radialis, volume of injectate used, history of prior injections, and fibrotic change of the muscle. The study included 54 patients, 77.8% of whom had an underlying diagnosis of cerebrovascular accident. Injectate leak was observed in 18.5% of injections and could not be confirmed in 9.3% of injections. Multivariable analysis demonstrated increased odds of leak with higher Modified Heckmatt Scale score. No statistically significant increase in leak was noted with higher volume of injectate or smaller muscle diameter. Extramuscular leak of botulinum toxin injection may be associated with fibroadipose muscle change.

Can we differentiate patients with dysferlinopathies and inflammatory myopathies by ultrasound? A discriminant analysis study.

Idiopathic inflammatory myopathies (IIM) are a heterogeneous group of diseases that are characterized by inflammation and muscle weakness. Dysferlinopathies are autosomal recessive limb-girdle muscular dystrophies caused by mutations in DYSF, which share a similar clinical presentation and histopathological inflammatory changes. To determine the sonographic differences between dysferlinopathies and IIM and whether these differences allow their classification. This observational, cross-sectional, and analytical study evaluated 20 muscles from 11 patients with dysferlinopathies and 11 patients with IIM. The patients were matched for age, sex, and disease duration. Clinical and laboratory variables were analyzed. Semi-quantitative scales were used to weigh the gray scale and power Doppler muscle abnormalities. Descriptive statistics were computed and discriminant analysis was performed to determine the ultrasound variables that best predicted the final diagnosis. Forty muscles were evaluated. Atrophy and higher Heckmatt scale scores were observed in patients with dysferlinopathies. A set of three muscles (biceps/brachialis, quadriceps, and gastrocnemius) had a diagnostic accuracy of 100% (sensitivity, 100%; specificity, 100%; canonical coefficient, 0.733 p < 0.001). A set of two formulas was used to classify both diseases correctly. In the present study, scanning of three muscle groups showed high diagnostic accuracy in differentiating dysferlinopathies from MII. Ultrasound can be used as an initial test in patients with suspected muscle disease or as an additional tool to support diagnosis in controversial cases.

Assessing muscle architecture with ultrasound: implications for spasticity.

Botulinum Neurotoxin Type A (BoNT-A) injections using Ultrasound (US) guidance have led to research evaluating changes in muscle architecture. Controversy remains as to what constitutes increased Echo-Intensity (EI) in spastic muscles and whether this may affect outcomes. We aim to provide a narrative review of US muscle architecture changes following Central Nervous System (CNS) lesions and explore their relationship to spasticity. Medline, CINAHL, and Embase databases were searched with keywords: ultrasonography, hypertonia, spasticity, fibrosis, and Heckmatt. Three physicians reviewed the results of the search to select relevant papers. Reviews identified in the search were used as a resource to identify additional studies. A total of 68 papers were included. Four themes were identified, including histopathological changes in spastic muscle, effects of BoNT-A on the muscle structure, available US modalities to assess the muscle, and utility of US assessment in clinical spasticity. Histopathological studies revealed atrophic and fibro-fatty changes after CNS lesions. Several papers described BoNT-A injections contributing to those modifications. These changes translated to increased EI. The exact significance of increased muscle EI remains unclear. The Modified Heckmatt Scale (MHS) is a validated tool for grading muscle EI in spasticity. The use of the US may be an important tool to assess muscle architecture changes in spasticity and improve spasticity management. Treatment algorithms may be developed based on the degree of EI. Further research is needed to determine the incidence and impact of these EI changes in spastic muscles.

Establishing the role of muscle ultrasound as an imaging biomarker in facioscapulohumeral muscular dystrophy

Facioscapulohumeral muscular dystrophy (FSHD) is a hereditary muscle disease, that causes weakness and wasting of skeletal muscles. In this cross-sectional cohort-study on FSHD patients, we assessed muscle ultrasound findings and their relation to clinical outcome measures, evaluating the role of ultrasound as biomarker in FSHD. We included 115 genetically confirmed FSHD patients (52% males, age-range 22–80 years). They were subjected to a standardized muscle ultrasound protocol of seven truncal and upper- and lower extremity muscles bilaterally. Muscle images were scored using the Heckmatt scale. Muscle echogenicity was quantified using z-scores. Compound echogenicity and Heckmatt scores were calculated. Nearly all patients (94%) had one or multiple muscles with an increased echogenicity z-score. The trapezius muscle was most severely affected, followed by the rectus femoris muscle. Both compound ultrasound scores strongly with multiple clinical outcome measures (ρ 0.68–0.79, p < 0.001). While most muscles showed a high level of agreement between the echogenicity z-score and Heckmatt score (>95%), the tibialis anterior and gastrocnemius muscle showed lower levels of agreement (82 and 92%). In conclusion, our study confirms the use of muscle ultrasound as clinical severity biomarker and provides a solid base for future longitudinal studies to establish ultrasound as a monitoring biomarker in FSHD.

A comprehensive study of skeletal muscle imaging in FHL1-related reducing body myopathy.

FHL1-related reducing body myopathy is an ultra-rare, X-linked dominant myopathy. In this cross-sectional study, we characterize skeletal muscle ultrasound, muscle MRI, and cardiac MRI findings in FHL1-related reducing body myopathy patients. Seventeen patients (11 male, mean age 35.4, range 12-76 years) from nine independent families with FHL1-related reducing body myopathy underwent clinical evaluation, muscle ultrasound (n = 11/17), and lower extremity muscle MRI (n = 14/17), including Dixon MRI (n = 6/17). Muscle ultrasound echogenicity was graded using a modified Heckmatt scale. T1 and STIR axial images of the lower extremity muscles were evaluated for pattern and distribution of abnormalities. Quantitative analysis of intramuscular fat fraction was performed using the Dixon MRI images. Cardiac studies included electrocardiogram (n = 15/17), echocardiogram (n = 17/17), and cardiac MRI (n = 6/17). Cardiac muscle function, T1 maps, T2-weighted black blood images, and late gadolinium enhancement patterns were analyzed. Muscle ultrasound showed a distinct pattern of increased echointensity in skeletal muscles with a nonuniform, multifocal, and "geographical" distribution, selectively involving the deeper fascicles of muscles such as biceps and tibialis anterior. Lower extremity muscle MRI showed relative sparing of gluteus maximus, rectus femoris, gracilis, and lateral gastrocnemius muscles and an asymmetric and multifocal, "geographical" pattern of T1 hyperintensity within affected muscles. Cardiac studies revealed mild and nonspecific abnormalities on electrocardiogram and echocardiogram with unremarkable cardiac MRI studies. Skeletal muscle ultrasound and muscle MRI reflect the multifocal aggregate formation in muscle in FHL1-related reducing body myopathy and are practical and informative tools that can aid in diagnosis and monitoring of disease progression.

Evaluation of myotonometry for myotonia, muscle stiffness and elasticity in neuromuscular disorders

Neuromuscular disorders show extremely varied expressions of different symptoms and the involvement of muscles. Non-invasively, myotonia and muscle stiffness are challenging to measure objectively. Our study aims to test myotonia, elasticity, and stiffness in various neuromuscular diseases and to provide reference values for different neuromuscular disease groups using a novel handheld non-invasive myometer device MyotonPRO®. We conducted a monocentric blinded cross-sectional study in patients with a set of distinct neuromuscular diseases (NCT04411732, date of registration June 2, 2020). Fifty-two patients in five groups and 21 healthy subjects were enrolled. We evaluated motor function (6-min walk test, handheld dynamometry, Medical Research Council (MRC) Scale) and used ultrasound imaging to assess muscle tissue (Heckmatt scale). We measured muscle stiffness, frequency, decrement, creep, or relaxation using myotonometry with the device MyotonPRO®. Statistically, all values were calculated using the t test and Mann–Whitney U test. No differences were found in comparing the results of myotonometry between healthy and diseased probands. Furthermore, we did not find significant results in all five disease groups regarding myotonometry correlating with muscle strength or ultrasound imaging results. In summary, the myometer MyotonPRO® could not identify significant differences between healthy individuals and neuromuscular patients in our patient collective. Additionally, this device could not distinguish between the five different groups of disorders displaying increased stiffness or decreased muscle tone due to muscle atrophy. In contrast, classic standard muscle tests could clearly decipher healthy controls and neuromuscular patients.

Diaphragmatic and Rectus Femoris Muscles Ultrasonography in Relation to Motor and Respiratory Functions in Ambulatory Boys with Duchenne Muscular Dystrophy

AbstractOur aim was to establish correlations between GSGC (Gait, Stairs, Gower, Chair) scores and ultrasonographic (US) findings of rectus femoris muscle (RF) and to study correlation between pulmonary function tests (PFT) and diaphragmatic muscles thickness in ambulatory boys with Duchenne muscular dystrophy (DMD). Twenty-four ambulatory boys with DMD were included. Their motor functions were assessed using GSGC scale. All the participants underwent PFT. US was used to assess RF quantitatively (gray scale analysis) and semiquantitatively (modified Heckmatt score) besides assessment of diaphragmatic muscle thickness. Patients with grade IV modified Heckmatt scale had the worst functional performance compared with grade III and II evidenced by having the highest total GSGC score (p &lt; 0.01), worst gait, stairs climbing, chair rising scores, and the longest time for rising from floor (p &lt; 0.05). A significant positive correlation was detected between forced expiratory volume in 1s/ forced vital capacity and right diaphragmatic muscle thickness. GSGC score positively correlated with RF US findings (quantitative gray scale analysis). GSGC score is a successful tool that could be used for clinical evaluation of patients with DMD. Diaphragmatic US introduces an option for screening and monitoring of restrictive respiratory pattern in patients with DMD after determining the reference values of diaphragmatic muscle thickness in different ages.

Validity and reliability of visual assessment of orofacial muscle ultrasound images using a modified Heckmatt scale.

Orofacial muscle ultrasound images can be evaluated quantitatively or using a visual grading system. Quantitative muscle ultrasound (QMUS) is currently the most sensitive technique to detect pathology, but can be time-consuming. The aim of this study was to investigate the validity and reliability of two visual grading systems (the original Heckmatt scale or a modified 3-point version) for the optimal grading of orofacial muscle images. A retrospective, comparative, reliability and validity study was performed. Ultrasound images of the digastric, geniohyoid, masseter, temporalis muscles, and intrinsic muscles of the tongue of healthy participants and of patients (suspected of) having a neuromuscular disease were included. QMUS was used as the "gold standard." Two expert raters and one inexperienced rater rated all ultrasound images using both visual grading systems. A total of 511 ultrasound images were included. Criterion validity showed Spearman rho correlation coefficients of >0.59. Construct validity analysis showed strong to very strong associations between the visual grading systems and mastication and/or swallowing. Inter- and intrarater reliability of the original Heckmatt scale and the modified scale were good and comparable. Rater experience had a beneficial effect on the interrater reliability of both scales. Both the original Heckmatt and the modified Heckmatt scale are valid and reliable tools for the visual grading of orofacial ultrasound images. The modified Heckmatt scale, with only three grades and including an "uncertain" category, is considered easier to use in clinical practice.

Ultrasound Assessment of Spastic Muscles in Ambulatory Chronic Stroke Survivors Reveals Function-Dependent Changes.

To correlate ultrasound characteristics of spastic muscles with clinical and functional measurements in chronic stroke survivors. Ultrasound assessment and clinical and functional assessments were performed in 28 ambulatory stroke survivors (12 females, mean age 57.8 ± 11.8 years, 76 ± 45 months after stroke). Muscle thickness in the affected side was decreased compared with the contralateral side (p < 0.001). The decrease was more evident in the upper limb muscles. On the affected side, the Heckmatt scale score was lowest (closer to normal) in the rectus femoris (RF) muscle compared with other muscles (biceps brachii (BB), flexor carpi ulnaris (FCU) and medial gastrocnemius (MG)). Muscle thickness and echogenicity of spastic muscles did not correlate with spasticity, as measured with the modified Ashworth scale (MAS), Fugl-Meyer motor assessment scores, age, or time since stroke. There was a significant negative correlation between grip strength and percentage decrease in muscle thickness for the spastic FCU muscle (r = -0.49, p = 0.008). RF muscle thickness correlated with ambulatory function (Timed Up and Go test (r = 0.44, p = 0.021) and 6-metre walk test (r = 0.41, p = 0.032)). There was no significant correlation between echogenicity and functional assessments Conclusion: Ambulatory chronic stroke survivors had function-dependent changes in muscle thickness on the affected side. Muscle thickness and echogenicity of spastic muscles did not correlate with spasticity, Fugl-Meyer motor assessment scores, age, or time since stroke.

Hybrid reflected-ultrasound computed tomography versus B-mode-ultrasound for muscle scoring in spinal muscular atrophy.

Novel light- and sound-based technologies like multispectral optoacoustic tomography (MSOT) with co-registered reflected-ultrasound computed tomography (RUCT) could add additional value to conventional ultrasound (US) for disease phenotyping in pediatric spinal muscular atrophy (SMA). The aim of this study was to investigate the quality of RUCT compared to US for qualitative and quantitative assessment of imaging neuromuscular disorders. Subanalyzing the MSOT SMA study, 288 RUCT and 276 US images from 10 SMA patients (mean age 9.0±3.7) and 10 gender- and age-matched healthy volunteers (HV; mean age 8.7±4.3) were analyzed for quantitative (grayscale levels [GSLs]) and qualitative (echogenicity, distribution pattern, Heckmatt scale, and muscle texture) muscle changes. RUCT and US measures were further correlated with clinical standard motor outcomes. Quantitative agreement using GSLs revealed significantly higher GSLs in muscles of SMA patients compared to healthy muscles in both techniques (US mean GSL [SD] SMA vs. HV: 110.70 [27.8] vs. 68.85 [19.2], p<.0001; RUCT mean GSL [SD] SMA vs. HV: 91.81 [21.8] vs. 59.86 [8.2], p<.0001) with good correlation with motor outcome tests, respectively. Qualitative agreement between methods for muscle composition was excellent for differentiation of pathological versus healthy muscles, echogenicity, and distribution pattern, moderate for Heckmatt scale, and poor for muscle texture. The data suggest that RUCT may allow the assessment of basic qualitative and quantitative measures for muscular diseases with comparable results to conventional US.

Sonographic evaluations of the skeletal muscles in patients with Pompe disease.

Pompe disease usually has muscle weakness due to glycogen accumulation. Heckmatt scale is commonly used to grade the pertinent findings of ultrasound. Nonetheless, it is difficult to detect subtle changes of the muscle. Besides, no ultrasonographic parameter has been proposed to predict the motor functions of Pompe disease. Therefore, we aimed to find out an ultrasonographic parameter that can quantify the muscle involvement and correlate with the motor functions in Pompe disease. Eighteen patients with Pompe disease were enrolled. The echo heterogeneity index (standard deviation divided by mean echogenicity values by ImageJ analysis) and shear modulus were recorded from rectus femoris, biceps femoris, tibialis anterior, medial gastrocnemius, biceps brachii and triceps brachii muscles. Motor functions, including manual muscle strength, 6-min walk and four-limb stair climb tests were assessed. Correlations between ultrasonographic parameters and Heckmatt scale and motor functions were analyzed. The echo heterogeneity index, but not the shear modulus, was negatively correlated with the Heckmatt scale rating in all muscles. The echo heterogeneity indices of tibialis anterior (r=0.698, p=0.008) and medial gastrocnemius (r=0.615, p=0.025) muscles showed positive correlations with the walking distance. Besides, the echo heterogeneity indices of four lower limb muscles were negatively correlated with the duration of stair climbing. The echo heterogeneity index but not the shear modulus can be used to quantitatively describe the muscle involvement in Pompe disease. In addition, lower echo heterogeneity indices of lower limb muscles are associated with worse motor functions in these patients.

Sarcopenia in Patients After Severe Brain Injury

Introduction This article deals with the problem of sarcopenia in patients after severe brain injury. It presents the results of the comparative analysis of a group of patients and a group of volunteers with performed muscle fiber ultrasound. Relevance Sarcopenia is a serious complication in a critically ill patient. It appears early and progresses rather quickly during the patient’s critical condition. In order to diagnose sarcopenia, both radiation and ultrasound methods can be used. The use of ultrasound methods is less labor-intensive, energy-consuming, and economically costly and does not involve an increase in radiation exposure to the patient. The paper highlights the use and comparison of these methods in patients after severe brain injuries. Aim of study To assess the severity of sarcopenia in patients after severe brain injuries. Materials and methods 25 patients were included in this study with an average age of 56.75±19.84 years, ranging from 22 to 82 years, after severe brain injury in a minimally conscious state according to the FOUR (Full Outline of Unresponsiveness) scale, median 12 (12; 15) points. The assessment was carried out in the first 3 days from the moment of admission to the Federal Research and Clinical Center of Intensive Care Medicine and Rehabilitology. For comparison purposes, the study included 19 volunteers aged 35.63±7.02 years, ranging from 21 to 47 years. Results The data obtained indicate that patients after severe brain injuries had pronounced muscle fiber disorders affecting its thickness and echogenicity. The thickness of the biceps on the side of the brain injury was 0.93±0.27 cm (min 0.5; max 1.58) and the thickness of the biceps on the side opposite to the brain injury was 0.62±0.2 cm (min 0.27; max 0.93) with p=0.0007, statistically significant. In terms of echogenicity, the differences were not statistically significant (p=0.1). The thickness of the triceps on the side of the brain injury was 0.5±0.17 cm (min 0.25; max 0.82) and the thickness of the triceps on the opposite side to the brain injury was 0.38±0.14 cm (min 0.2; max 0.8) with p=0.028, statistically significant, while the degree of echogenicity according to the Modified Heckmatt scale on the side of the brain injury was 2.5 [2.0; 3.0] (min 2.0; max 4.0), and on the opposite side — 3.0 [3.0; 4.0] (min 2.0; max 4.0), p=0.01, statistically significant. The thickness of the brachioradialis on the side of the brain injury was 0.59±0.15 cm (min 0.39; max 0.92), on the opposite side — 0.50±0.17 cm (min 0.25; max 0.86), p=0.06, statistically significant; while the degree of echogenicity was 2.0 [2.0; 3.0] (min 1.0; max 4.0) on the side of the brain injury and on the opposite side to the brain injury — 2.5 [2.0; 4.0] (min 2.0; max 4.0), p=0.03, statistically significant. Pronounced statistically significant differences were also obtained in the thickness of the rectus femoris muscle (p=0.06) and its echogenicity (p=0.017). In comparing these indicators with the muscles of healthy volunteers for all indicated parameters p&lt;0.05, in most cases p=0.000001, statistically significant. Using computed tomography of the lumbar spine, a decrease in the cross-sectional area of the psoas muscle was revealed. The following values were obtained from the patients: psoas muscle cross-sectional area on the right side: 7.66±2.72 cm 2 (min 3.84; max 12.95), psoas muscle cross-sectional area on the left side: 7.85±2.64 cm 2 (min 3.7; max 12.6), Skeletal Muscle Index: 53.33±15.34 (min 28; max 81). Conclusion Diagnostic ultrasound methods to assess sarcopenia in patients after severe brain injuries have confirmed their effectiveness. In the present study, this method received a pronounced correlation with radiological techniques to identify patients affected by sarcopenia. We obtained statistically significant differences in the group of volunteers and patients, and some parameters differed by more than 2 times, which indicates the presence of severe sarcopenia in this group of patients.

Shear wave elastography combined with electromyography to assess the effect of botulinum toxin on spastic dystonia following stroke: A pilot study.

BackgroundShear wave elastography (SWE) is a method for carrying out a quantitative assessment of the mechanical properties of soft tissues in terms of stiffness. In stroke survivors, the paretic muscles may develop hypertonia due to both neural-mediated mechanisms and structural alterations with consequent muscular fibrous-fatty remodeling.MethodsFourteen adult patients with spastic dystonia following stroke were recruited. Muscle hypertonia was assessed using the modified Ashworth scale (MAS). Muscle activation was measured by surface electromyography (sEMG) with the selected muscle in shortened (spastic dystonia) and stretched (dynamic stretch reflex) positions. SWE was performed on a selected paretic muscle and on the contralateral non-paretic one to calculate shear wave velocities (SWV) along and across muscular fibers. The modified Heckmatt scale (MHS) pattern was also determined. All evaluations were performed shortly before BoNT-A injections (T0) and one month later (T1).ResultsAll SWV on paretic muscles were higher than contralateral non-paretic ones (p < 0.01). After BoNT-A injection, a significant reduction in MAS (p = 0.0018), spastic dystonia (p = 0.0043), and longitudinal SWE measurements, both in shortened (p = 0.001) and in stretched muscular conditions (p = 0.0029), was observed. No significant changes in SWV on non-paretic muscles were observed. Higher SWV resulted along the direction of muscular fibers vs. across them (p = 0.001). No changes resulted from the MHS evaluations after BoNT-A. There was a positive correlation between MHS scores and SWV values while the muscle was in the shortened position, but not with spastic dystonia recorded by sEMG.ConclusionsThis is the first study evaluating the effect of BoNT-A on muscle hypertonia following stroke, assessed by both SWE and sEMG. These findings support SWE as a useful method to disclose intrinsic muscular remodeling, independently of the effect of spastic dystonia, in particular, while muscles were assessed in a neutral position. SWE measurements of muscle stiffness cannot tell apart neural-mediated and intrinsic muscle hypertonia. Interestingly, when sEMG activity is very limited, as in spastic muscles kept in a shortened position, SWE can provide a measurement of stiffness due almost completely to intrinsic muscle changes. Alongside sEMG, SWE could aid clinicians in the assessment of responses to treatments.

P.33 Muscle ultrasound use in the initial diagnosis of childhood onset neuropathy and neuronopathy

Skeletal muscle ultrasound (MUS) is a non-invasive ancillary tool for evaluation of neuromuscular conditions that can enhance the physical exam and complement electrodiagnostic studies. While MUS is more extensively studied in primary myopathies, it can also enhance the diagnostic evaluation of patients with neuropathies. Neurogenic disease typically results in heterogenous "streaky" echogenicity in skeletal muscle while myopathic disease typically appears as homogenously increased echogenicity in a granular pattern. In addition, the superiority of MUS in detecting fasciculations compared to EMG is reported in amyotrophic lateral sclerosis, which is likely due to the higher volume of sampled muscle with MUS. Here, we describe MUS findings and EMG/NCS correlates in a cohort of 17 individuals with genetically confirmed childhood onset neuropathies and neuronopathies evaluated at the National Institutes of Health. MUS was graded using the modified Heckmatt scale (MHS) and a description of increased echogenicity as "streaky" or "granular" by three independent reviewers. In 17 patients evaluated, there was a length-dependent pattern of increased echogenicity. Tibialis anterior and gastrocnemius were most affected, and deltoid, triceps and biceps were most spared. "Streaky" appearance was seen in most muscles but was more difficult to appreciate in muscles with low echogenicity scores (grade 0 MHS) or in "end-stage" muscle (grade 3 MHS). MUS was more sensitive at detecting fasciculations than EMG in this setting. No clear pattern emerged to differentiate specific genetic etiologies of neuropathy/neuronopathy from one another via MUS. This study demonstrates the utility of muscle ultrasound in detecting neuropathic disease.

Sonographic Evaluation of Muscle Echogenicity for the Detection of Intensive Care Unit-Acquired Weakness: A Pilot Single-Center Prospective Cohort Study.

Qualitative assessment by the Heckmatt scale (HS) and quantitative greyscale analysis of muscle echogenicity were compared for their value in detecting intensive care unit-acquired weakness (ICU-AW). We performed muscle ultrasound (MUS) of eight skeletal muscles on day 3 and day 10 after ICU admission. We calculated the global mean greyscale score (MGS), the global mean z-score (MZS) and the global mean Heckmatt score (MHS). Longitudinal outcome was defined by the modified Rankin scale (mRS) and Barthel index (BI) after 100 days. In total, 652 ultrasound pictures from 38 critically ill patients (18 with and 20 without ICU-AW) and 10 controls were analyzed. Patients with ICU-AW had a higher MHS on day 10 compared to patients without ICU-AW (2.6 (0.4) vs. 2.2 (0.4), p = 0.006). The MHS was superior to ROC analysis (cut-off: 2.2, AUC: 0.79, p = 0.003, sensitivity 86%, specificity 60%) in detecting ICU-AW compared to MGS and MZS on day 10. The MHS correlated with the Medical Research Council sum score (MRC-SS) (r = −0.45, p = 0.004), the mRS (r = 0.45; p = 0.007) and BI (r = −0.38, p = 0.04) on day 100. Qualitative MUS analysis seems superior to quantitative greyscale analysis of muscle echogenicity for the detection of ICU-AW.

Assessment of the upper limb muscles in patients with Fukuyama muscular dystrophy: Noninvasive assessment using visual ultrasound muscle analysis and shear wave elastography

Fukuyama-type congenital muscular dystrophy (FCMD) is severe, childhood-onset muscular dystrophy. Recently, our group has discovered a potential treatment using antisense oligonucleotides. Therefore, an effective, reliable, and objective method of assessing muscle is needed. Ultrasound is a minimally invasive tool that can be applied without radiation exposure or pain. Evaluating tissue stiffness by shear wave elastography (SWE) has especially recently attracted attention. Here, we aimed to evaluate SWE value of the upper limb muscles: biceps brachii, triceps brachii, brachioradialis, abductor pollicis brevis, and abductor finger muscle in patients with FCMD. Upper extremity function was evaluated by visual muscle ultrasound analysis (VMUA) and SWE in 13 patients with FCMD and 20 healthy controls. The motor function evaluation tool was used to evaluate motor function, and the correlation with the dynamics of the SWE was determined. VMUA scaled using the Heckmatt scale was higher in patients with FCMD. SWE was also significantly higher and stiffer in the biceps brachii and brachioradialis in patients with FCMD. Furthermore, the severity of FCMD symptoms was correlated with muscle stiffness. We conclude that VMUA and SWE can be useful tools for monitoring muscle atrophy and upper limb function in patients with FCMD.

Is the Outcome of Diagnostic Nerve Block Related to Spastic Muscle Echo Intensity? A Retrospective Observational Study on Patients with Spastic Equinovarus Foot.

To investigate the relationship between spastic calf muscles echo intensity and the outcome of tibial nerve motor branches selective block in patients with spastic equinovarus foot. Retrospective observational study. Forty-eight patients with spastic equinovarus foot. Each patient was given selective diagnostic nerve block (lidocaine 2% perineural injection) of the tibial nerve motor branches. All patients were evaluated before and after block. Outcomes were: spastic calf muscles echo intensity measured with the Heckmatt scale; affected ankle dorsiflexion passive range of motion; calf muscles spasticity measured with the modified Ashworth scale and the Tardieu scale (grade and angle). Regarding the outcome of tibial nerve selective diagnostic block (difference between pre- and post-block condition), Spearman's correlation showed a significant inverse association of the spastic calf muscles echo intensity with the affected ankle dorsiflexion passive range of motion (p = 0.045; ρ = 00-0.269), modified Ashworth scale score (p = 0.014; ρ = -0.327), Tardieu grade (p = 0.008; ρ = -0.352) and Tardieu angle (p = 0.043; ρ = -0.306). These findings support the hypothesis that patients with spastic equinovarus foot with higher spastic calf muscles echo intensity have a poor response to selective nerve block of the tibial nerve motor branches.

Validity of Skeletal Muscle Ultrasound in Assessment of Suspected Limb Girdle Muscular Dystrophy Patients

Abstract Background Limb-girdle muscular dystrophies (LGMDs) are a clinically and genetically heterogeneous group of disorders characterized by progressive muscle weakness and degenerative muscle changes. Studies have shown that ultrasound can be useful both for diagnosis and follow-up of LGMDs patients. Objectives This study aims to measure the sensitivity and the specificity of muscle ultrasound in assessment of suspected limb girdle muscular dystrophy patients. Subjects and Methods This cross-sectional descriptive study was conducted on Fifty-five patients with suspected LGMD from neuromuscular unit, myology clinic, Ain Shams University hospitals and eight healthy subjects. Age was above 2 years. Both sexes were included in the study. They underwent real-time B-mode ultrasonography performed with using Logiq p9 General Electric ultrasound machine and General Electric 7-11.5 MHZ linear array ultrasound probe. All ultrasound images have been obtained and scored by a single examiner and muscle echo intensity was visually graded semiquantitative according to Heckmatt's scale. The examiner was blinded to the muscle biopsy results and clinical evaluations. Results Statistical analysis revealed that the diagnostic performance of muscle US (Heckmatt’s score) in LGMD is most sensitive when calculated in all examined upper limb and lower limb muscles, followed by lower limb muscles alone. US of upper limb was found to be the least sensitive. Conclusions Muscle ultrasound is a practical and reproducible and valid tool that can be used in assessment of suspected LGMD patients.

WS7.7. Overview of quantitative muscle ultrasound

The diagnosis and treatment of a patient with suspected neuromuscular disease requires an integrated approach that utilizes clinical, electrodiagnostic, pathologic and genetic data. Ultrasound complements the clinical and electrophysiology exam by showing the degree of abnormalities in myopathies, as well as spontaneous muscle activities in motor neuron diseases and other disorders. US is particularly well suited for evaluation of neuromuscular disorders as it can be performed at the bedside, has no need for sedation, can rapidly evaluate multiple body regions, and is well tolerated by both children and adults including patients in wheelchairs or those who cannot lay flat or still. Muscle ultrasound can be used as a biomarker for Duchenne and FSH muscular dystrophy and spinal muscular atrophy, and the combination of electromyography and ultrasound increases the diagnostic certainty of amyotrophic lateral sclerosis. Qualitative visual analysis of muscle can be graded using the Heckmatt scale, based on the degree of echogenicity and visualization of the deep boney structures. Quantification of the gray scale levels can be performed by importing the ultrasound image into an image analysis software program such as ImageJ, selecting a region of interest within the muscle belly, and measuring the mean gray scale pixel values within the region of interest. Quantitative analysis allows for more sensitive and specific determinations of muscle pathology than qualitative analysis, with reported sensitivities for neuromuscular pathology around 95%. A drawback of quantitative muscle ultrasound is that it requires that imaging settings be held constant and that normative values have been obtained using identical imaging parameters. A strong advantage of ultrasound over other imaging modalities is that evaluation in real time permits the identification of pathologic muscle movement, particularly fasciculations.

Predicting the surgical reparability of large-to-massive rotator cuff tears by B-mode ultrasonography: a cross-sectional study.

PurposeThis study aimed to compare the ability of B-mode ultrasonography and magnetic resonance imaging (MRI) to predict the repairability of large-to-massive rotator cuff tears (RCTs).MethodsThis cross-sectional study included participants with large-to-massive RCTs who underwent arthroscopic repair. B-mode ultrasonography and MRI were conducted prior to arthroscopic repair. B-mode ultrasonography was used to evaluate the echogenicity of the rotator cuff muscle using the Heckmatt scale. Intra-rater and inter-rater reliabilities were examined for two independent physicians. MRI was used to evaluate the degrees of tendon retraction, fatty infiltration of rotator cuff muscles, and muscle atrophy. Finally, two experienced orthopedic surgeons performed surgery and decided whether the torn stump could be completely repaired intraoperatively.ResultsFifty participants were included, and 32 complete repairs and 18 partial repairs were performed. B-mode ultrasonography showed good intra-rater reliability and inter-rater reliability for assessment of the muscle echogenicity of the supraspinatus and infraspinatus muscles. The correlation coefficients between B-mode ultrasound findings and MRI findings showed medium to large effect sizes (r=0.4-0.8). The Goutallier classification of the infraspinatus muscles was the MRI predictor with the best discriminative power for surgical reparability (area under the curve [AUC], 0.89; 95% confidence interval [CI], 0.81 to 0.98), while the Heckmatt scale for infraspinatus muscles was the most accurate ultrasound predictor (AUC, 0.85; 95% CI, 0.74 to 0.96). No significant differences in AUCs among the MRI and ultrasound predictors were found.ConclusionB-mode ultrasonography was a reliable examination tool and had a similar ability to predict surgical reparability to that of MRI among patients with large-to-massive RCTs.