Quantitative muscle ultrasound and muscle force in healthy children: a 4-year follow-up study.

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.

Monitoring disease progression using high-density motor unit number estimation in amyotrophic lateral sclerosis.

In amyotrophic lateral sclerosis (ALS), progressive motor neuron loss causes severe weakness. Functional measurements tend to underestimate the underlying pathology because of collateral reinnervation. A more direct marker of lower motor neuron loss is of significant importance. We evaluated high-density motor unit number estimation (MUNE), as compared with the ALS Functional Rating Scale (ALSFRS) and maximal compound muscle action potential (CMAP) amplitude, for monitoring and classifying disease progression. MUNE showed good reproducibility (intraclass correlation coefficient = 0.86). MUNE showed a significantly greater decrease than the ALSFRS, the Medical Research Council (MRC) scale, and CMAP amplitude. Patients could be stratified into groups with rapidly or slowly progressive disease based on a decrement in MUNE at 4 months from baseline; ALSFRS score at 8 months was significantly lower in the rapidly progressive group. MUNE was sensitive to motor neuron loss early in the disease course when compared to other clinical measures. Stratification of patients based on a decrease in MUNE seems feasible.

Quantitative muscle ultrasonography in amyotrophic lateral sclerosis.

In this study, we examined whether quantitative muscle ultrasonography can detect structural muscle changes in early-stage amyotrophic lateral sclerosis (ALS). Bilateral transverse scans were made of five muscles or muscle groups (sternocleidomastoid, biceps brachii/brachialis, forearm flexor group, quadriceps femoris and anterior tibialis muscles) in 48 patients with ALS. Twenty-five patients were also screened for fasciculations. Quantitative analysis revealed a significant increase in echo intensity in all muscles and a decrease in muscle thickness of the biceps brachii, forearm flexors and quadriceps femoris on both sides. Fasciculations were easy to detect in multiple muscles of all screened patients except one. We conclude that quantitative ultrasonography can be used to detect muscle changes caused by ALS in an early phase of the disease. (E-mail: m.zwarts@neuro.umcn.nl).

Measuring the cortical silent period can increase diagnostic confidence for amyotrophic lateral sclerosis.

We evaluated a modified measurement of the cortical silent period (CSP) as a simple procedure to add further confidence in the diagnostic work-up for ALS. Thirty-seven consecutive patients with a suspicion of having ALS were included together with 25 healthy volunteers, and followed until a final diagnosis (ALS versus 'ALS mimic') was reached. Using a CSP cut-off value of 200 ms for males and 150 ms for females, the following test characteristics were obtained for ALS versus ALS mimics together with controls: sensitivity for excluding ALS 0.83, specificity 0.56 (males) and sensitivity 0.81, specificity 0.82 (females). A CSP longer than the mentioned cut-off values should alarm the clinician for the presence of a disorder other than ALS.

Motor unit characteristics in healthy subjects and those with postpoliomyelitis syndrome: a high-density surface EMG study.

The purpose of this study was to identify optimal ways to detect neurogenic changes with high-density surface electromyography (HD-sEMG). For this purpose, we searched for the variables that most clearly discriminated between postpoliomyelitis and healthy subjects. We obtained HD-sEMG from the quadriceps muscle at different force levels in nine subjects with postpoliomyelitis syndrome and in matched healthy controls. Single motor unit action potentials (MUAPs), extracted from the HD-sEMG signal and the raw signal itself, were analyzed. Areas under the curve of the extracted MUAP waveform, indicating motor unit size, perfectly separated both groups. Raw signal analysis showed significant differences between groups for the monopolarly recorded amplitude up to 60% of maximal force and for the level of interference at higher force levels (40-100% force). We conclude that with HD-sEMG it is possible to detect neurogenic motor unit changes noninvasively, both by analysis of the raw signal itself and by analysis of extracted single MUAPs. The diagnostic yield of the single MUAP analysis is clearly higher. These findings point toward applications for clinical practice and invite further studies exploring the diagnostic value of HD-sEMG.