Quantitative sonographic assessment of muscle thickness and fasciculations distribution is a sensitive tool for neuromuscular disorders.

INTRODUCTION: Loss of muscle thickness can be demonstrated in a wide spectrum of neuromuscular disorders, while fasciculations are more frequent in amyotrophic lateral sclerosis (ALS). In the current study, we aimed to determine the sensitivity and specificity of quantitative sonographic assessment of muscle thickness and the presence of fasciculations for diagnosing various neuromuscular disorders. METHODS: The thickness and the presence of fasciculations in eight muscles were determined by sonography in patients with myopathy (22), polyneuropathy (36), ALS (91), and spinal muscular atrophy (SMA) (31) and compared to normative values determined in 65 heathy control subjects. RESULTS: Reduced muscle thickness in at least one relaxed muscle showed 92-100% sensitivity for diagnosing a neuromuscular disease, with a specificity of 85% for differentiating patients from heathy controls (AUC = 0.90). Subtracting distal from proximal muscle thickness may differentiate between myopathy and polyneuropathy. Fasciculations in ≥1 proximal muscle showed good diagnostic accuracy (AUC = 0.87) for diagnosing ALS. DISCUSSION: Sonographic assessment of muscle thickness is a sensitive tool for diagnosing a wide spectrum of neuromuscular diseases, and may facilitate diagnosis even in patients with normal strength on neurological examination, while the presence of fasciculations in proximal muscles may facilitate ALS diagnosis.

A shortened surface electromyography recording is sufficient to facilitate home fasciculation assessment.

INTRODUCTION/AIMS: Fasciculations are an early clinical hallmark of amyotrophic lateral sclerosis (ALS), amenable to detection by high-density surface electromyography (HDSEMG). In conjunction with the Surface Potential Quantification Engine (SPiQE), HDSEMG offers improved spatial resolution for the analysis of fasciculations. This study aims to establish an optimal recording duration to enable longitudinal remote monitoring in the home. METHODS: Twenty patients with ALS and five patients with benign fasciculation syndrome (BFS) underwent serial 30 min HDSEMG recordings from biceps brachii and gastrocnemii. SPiQE was independently applied to abbreviated epochs within each 30-min recording (0-5, 0-10, 0-15, 0-20, and 0-25 min), outputting fasciculation frequency, amplitude median and amplitude interquartile range. Bland-Altman plots and intraclass correlation coefficients (ICC) were used to assess agreement with the validated 30-min recording. RESULTS: In total, 506 full recordings were included. The 5 min recordings demonstrated diverse and relatively poor agreement with the 30 min baselines across all parameters, muscles and patient groups (ICC = 0.32-0.86). The 15-min recordings provided more acceptable and stable agreement (ICC = 0.78-0.98), which did not substantially improve in longer recordings. DISCUSSION: For the detection and quantification of fasciculations in patients with ALS and BFS, HDSEMG recordings can be halved from 30 to 15 min without significantly compromising the primary outputs. Reliance on a shorter recording duration should lead to improved tolerability and repeatability among patients, facilitating longitudinal remote monitoring in patients' homes.

Assessment of Muscle Wasting in Long-Stay ICU Patients Using a New Ultrasound Protocol.

There is currently no standardized procedure to assess sarcopenia in long-stay catabolic patients. Our aim is to analyze a novel ultrasound muscle assessment protocol in these patients versus healthy controls, by carrying out a prospective observational study. We designed a new ultrasound protocol that assesses quadriceps rectus femoris (QRF) muscle quality in real-time B-mode, color-Doppler, and M-mode ultrasound, and evaluates QRF intramuscular central tendon thickness, cross-sectional area, and muscle thickness in ultrasound B-mode. Logistic regression was performed as a multivariable analysis on 29 cases and 19 controls. The QRF muscle area and thickness were shown to significantly decrease (p ≤ 0.001), and the central tendon thickness significantly increased (p = 0.047) in cases versus controls. The QRF muscle echogenicity and angiogenic activity fasciculations, subcutaneous edema, and intramuscular fluid were also significantly different between the two groups (p < 0.001). The selected variables in the multivariate logit analysis were the muscle area (OR per cm² = 0.07; 95% confidence interval (CI) = 0.012⁻0.41) and the central tendon thickness (OR per mm 1.887; 95% CI = 2.66⁻13.38).

[Needle electromyography features and values of paraspinal muscle parameters in assessment of respiratory function of patients with amyotrophic lateral sclerosis].

Objective: To analyze the features of needle electromyography (EMG) in patients with amyotrophic lateral sclerosis (ALS), and explore the correlation between EMG parameters of the tenth thoracic paraspinal muscle and disease duration, rate of disease progression, forced vital capacity (FVC) and revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R). Methods: Standard EMG was recorded from unilateral sternocleidomastoid muscle and/or tongue muscles, upper and lower limbs muscles and the tenth thoracic paraspinal muscle in 112 patients with definite ALS between March 2012 and June 2013 in the Department of Neurology at Chinese PLA General Hospital. Parameters studies included spontaneous potentials, duration and amplitude of motor unit potentials (MUP), pattern of recruitment. Results: EMG revealed diffuse neurogenic changes in each case. Fibrillation potential or positive sharp waves were found in some muscles in all of patients, and both of them were found in most of the cases. Fasciculation potentials (FPs) were found in 8 patients and accompanied with fibrillation potential and positive sharp waves in 5 of them. Complex repetitive discharges (CRDs) were found in only 1 patient. Duration and amplitude of MUP was prolonged and increased in ALS patients, and the rate of high amplitude and decreased recruitment pattern were 35.71% and 72.32%, respectively. Logistic regression analysis revealed an association of the FVC loss with the drop of ALSFRS-R and peak amplitude of the tenth thoracic paraspinal muscle. Conclusions: The abnormal spontaneous activity with high frequency are fibrillation potentials and/or positive sharp waves. However, FPs is observed in a small number of patients and CRDs is rarely seen in ALS. The ALSFRS-R and peak amplitude of thetenth thoracic paraspinal muscle may be of certain clinical value in estimating the severity of disease especially the respiratory dysfunction in ALS patients.

Infant neurologic assessment.

Infant neurologic assessment reflects the ongoing maturation of the central nervous system. Traditional approaches to assessment cannot be used. Key factors are accurate observation and flexibility in obtaining the data. A case example using a 4-month-old infant illustrates specific approaches to assessment.

Ultrasonographic assessment of the prevalence of fasciculations in lesions of the peripheral nervous system.

In previous studies, ultrasonography was a very sensitive method in detecting fasciculations. The present study was intended to examine the prevalence of ultrasonographically visible fasciculations in patients with neuromuscular diseases affecting the lower extremities. Ultrasonography of 9 muscles (rectus femoris, vastus lateralis, vastus intermedius, vastus medialis, sartorius, semitendinosus, tibialis anterior, gastrocnemius, and soleus muscles) was performed bilaterally in 70 adult healthy subjects and 172 patients with various neuromuscular diseases. Fasciculations were detected in 109 (63%) of 172 patients. The median value of affected muscles was 10 (range 1-18). Patients with spinal muscular atrophy (37/38, p < 0.001), hereditary motor and sensory neuropathy (24/25, p < 0.001), and lumbosacral radiculopathy with motor deficits (24/29, p < 0.001) exhibited fasciculations more frequently than did healthy volunteers. Radiculopathy with sensory deficits, lesions of either plexus or peripheral nerves, compartment syndrome, and myopathy were not associated with a significantly enhanced prevalence of fasciculations in the patient group compared to the control group. In summary, fasciculations are a frequent ultrasonographic sign in neuromuscular diseases. They are almost regularly found in patients with spinal muscular atrophy and those with hereditary motor and sensory neuropathy. Thus, the absence of fasciculations in ultrasonographic assessment should give cause for reconsidering these diagnoses.

Fasciculations: clinical, electromyographic, and ultrasonographic assessment.

Widespread fasciculations are an important clinical sign in, for example, degenerative lower motor neuron diseases (LMND). Usually they are detected by clinical inspection and electromyography. Recently myosonography has been proposed for the detection of fasciculations. This prospective study compares the value of these three modes of examination in patients with degenerative LMND. Seventy healthy control persons and 34 patients (11 women, 23 men; aged 43-78 years; median age 60.5) with LMND were included in the study. All participants were subjected to thorough visual screening for the presence of fasciculations. Fourteen muscles were examined bilaterally by myosonography and a median of 8 muscles were screened electromyographically (only in the patients); the investigators were blinded to the other findings. Clinical inspection and ultrasonography exhibited fasciculations in up to 5 and 8 muscles, respectively, in 8 healthy persons. Ultrasonography demonstrated fasciculations in all patients, clinical inspection in all but 2, and electromyography in 26 of 33 patients (1 patient was not examined electromyographically). Comparing the three methods, clinical observation revealed fasciculations in 42%, electromyography in 39%, and ultrasonography in 67% of all muscles. Thus, ultrasonography was significantly more sensitive than the other techniques (P < 0.001). The interrater agreement (correlation coefficient) r in respect of the presence or absence of fasciculation was 0.71 for the clinical, 0.85 for the electromyographic and 0.84 for the myosonographic examinations. Ultrasonography and electromyography were more reliable than the clinical examination (P < 0.001 and P < 0.01, respectively). Our study indicates that ultrasonography is more sensitive than clinical and electromyographic examination in visualizing fasciculations in patients with LMND. Additionally, it is more reliable than clinical examination.