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

BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.

Volume Matters in Ultrasound-Guided Perineural Dextrose Injection for Carpal Tunnel Syndrome: A Randomized, Double-Blinded, Three-Arm Trial.

Ultrasound-guided perineural dextrose injection (PDI) has been reported effective for carpal tunnel syndrome (CTS). Higher volume of injectate may reduce adhesion of median nerve from other tissues, but volume-dependent effects of PDI in CTS remain unknown. We aimed to investigate whether PDI with different injectate volumes had different effects for CTS participants. In this randomized, double-blinded, three-arm trial, 63 wrists diagnosed with CTS were randomized into three groups that received ultrasound-guided PDI with either 1, 2 or 4 ml of 5% dextrose water. All participants finished this study. Primary outcome as visual analog scale (VAS) and secondary outcomes including Boston Carpal Tunnel Questionnaire (BCTQ), Disability of the Arm, Shoulder and Hand score (QuickDASH), electrophysiological studies and cross-sectional area (CSA) of the median nerve at carpal tunnel inlet were assessed before and after PDI at the 1st, 4th, 12th and 24th weeks. For within-group analysis, all three groups (21 participants, each) revealed significant improvement from baseline in VAS, BCTQ and QuickDASH at the 1st, 4th, 12th and 24th weeks. For between-group analysis, 4 ml-group yielded better VAS reduction at the 4th and 12th weeks as well as improvement of BCTQ and QuickDASH at the 1st, 4th, and 12th weeks, compared to other groups. No significant between-group differences were observed in electrophysiological studies or median nerve CSA at any follow-up time points. There were no severe complications in this trial, and transient minor adverse effects occurred equally in the three groups. In conclusion, ultrasound-guided PDI with 4 ml of 5% dextrose provided better efficacy than with 1 and 2 ml based on symptom relief and functional improvement for CTS at the 1st, 4th, and 12th week post-injection, with no reports of severe adverse effects. There was no significant difference between the three groups at the 24th-week post-injection follow-up. Clinical Trial Registration: www.ClinicalTrials.gov, identifier NCT03598322.