Introduction: The aim of this study was to evaluate the muscle MRI pattern of 9 patients (median age: 6.5 ± 2.74 years) affected by classic infantile-onset Pompe disease who were treated with enzyme replacement therapy.
Methods: We performed and qualitatively scored T1-weighted (T1-w) sequences of the facial, shoulder girdle, paravertebral, and lower limb muscles and short-tau inversion recovery (STIR) sequences of the lower limbs using the Mercuri and Morrow scales, respectively.
Results: On T1-w images, mild (grade 1) or moderate (grade 2) involvement was found in the tongue in 6 of 6 patients and in the adductor magnus muscle in 6 of 9. STIR hyperintensity was detected in all areas examined and was categorized as limited to mild in 5 of 8 patients.
Conclusions: On T1-w sequences, mild/moderate adipose substitution in the adductor magnus and tongue muscles was documented. STIR edema-like alterations of thigh and calf muscles are novel findings. Correlations with biopsy findings and clinical parameters are needed to fully understand these findings. Muscle Nerve 55: 841-848, 2017.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1002/mus.25417 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Stable Gastric Pentadecapeptide BPC 157 as Therapy After Surgical Detachment of the Quadriceps Muscle from Its Attachments for Muscle-to-Bone Reattachment in Rats.
Pharmaceutics
January 2025
Department of Pharmacology, School of Medicine, University of Mostar, 88000 Mostar, Bosnia and Herzegovina.
Background: This is a novel rat study using native peptide therapy, focused on reversing quadriceps muscle-to-bone detachment to reattachment and stable gastric pentadecapeptide BPC 157 per-oral therapy for shared muscle healing and function restoration.
Methods: Pharmacotherapy recovering various muscle, tendon, ligament, and bone lesions, and severed junctions (i.e.
Evaluation of Perineal Descent Measurements on Pelvic Floor Imaging.
J Clin Med
January 2025
Department of Obstetrics and Gynaecology, Croydon University Hospital, Croydon CR7 7YE, UK.
The aim of this study is to validate a uniform method for measuring perineal descent which can be used for different imaging methods, to establish cut-off values for this measurement, and to assess diagnostic test accuracy (DTA) of imaging techniques using these cut-off values. Secondly, the study aims to correlate perineal descent to symptoms, signs and imaging findings in women with obstructed defaecation syndrome (ODS) to assess its clinical relevance. Cross-sectional study of 131 women with symptoms of ODS.
View Article and Find Full Text PDFThe Effectiveness and Safety of Tibial-Sided Osteotomy for Fibula Untethering in Lateral Close-Wedge High Tibial Osteotomy: A Novel Technique with Video Illustration.
Medicina (Kaunas)
January 2025
Seoul St. Mary's Hospital, The Catholic University of Korea, Seoul 06591, Republic of Korea.
: Despite its advantages, lateral close-wedge high tibial osteotomy (LCWHTO) requires proximal tibiofibular joint detachment (PTFJD) or fibular shaft osteotomy for gap closing. These fibula untethering procedures are technically demanding and not free from the risk of neurovascular injuries. Our novel fibula untethering technique, tibial-sided osteotomy (TSO) near the proximal tibiofibular joint (PTFJ), aims to reduce technical demands and the risk of injury to the peroneal nerve and popliteal neurovascular structures.
View Article and Find Full Text PDFComparative magnetic resonance imaging-based study of pelvic floor morphology and function before pregnancy and after primigravida vaginal delivery.
BMC Pregnancy Childbirth
January 2025
Department of Radiology, The Affiliated People's Hospital of Fujian University of Traditional Chinese Medicine, 602 Ba Yi Qi Zhong Road, Fuzhou, China.
Background: Vaginal childbirth is one of the main risk factors for pelvic floor dysfunction. Magnetic resonance imaging (MRI) can facilitate quantitative evaluation of the morphology and function of the pelvic floor in static and dynamic environments. The objective of this study was to investigate the changes in pelvic floor morphology and function in primigravida women before pregnancy (BP) and after vaginal delivery.
View Article and Find Full Text PDFSevere fever with thrombocytopenia syndrome complicated with aspergillus endocarditis and multiple organ infarctions after glucocorticoid treatment in an immunocompetent man: a case report.
BMC Infect Dis
January 2025
State Key Laboratory for Diagnosis and Treatment of Infectious Diseases, National Clinical Research Center for Infectious Diseases, National Medical Center for Infectious Diseases, Collaborative Innovation Center for Diagnosis and Treatment of Infectious Diseases, The First Affiliated Hospital, Zhejiang University School of Medicine, 79 Qingchun Rd., Hangzhou City, 310003, China.
Background: Severe fever with thrombocytopenia syndrome (SFTS) is an emerging infectious disease characterized by leukopenia and thrombocytopenia, and aspergillosis is a common complication in severe cases. Previous studies have reported cases of SFTS complicated with invasive pulmonary aspergillosis (IPA) and central nervous system aspergillosis. Here, we present the first case of an immunocompetent patient with SFTS who progressed to IPA and Aspergillus endocarditis after glucocorticoid treatment, and embolism of the vegetations from the left ventricle led to multiple infarctions in the brain, kidney, and spleen.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!