Vill, Katharina and Schessl, J. and Teusch, V. and Schroeder, S. and Blaschek, A. and Schoser, B. and Mueller-Felber, W. (2015) Muscle ultrasound in classic infantile and adult Pompe disease: A useful screening tool in adults but not in infants. NEUROMUSCULAR DISORDERS, 25 (2). pp. 120-126. ISSN 0960-8966, 1873-2364
Full text not available from this repository. (Request a copy)Abstract
A cohort of 4 infantile and 15 adult Pompe patients has been investigated regarding correlation between strength and ultrasound of skeletal musculature. In adults, muscle ultrasound is useful to assess clinical and subclinical involvement of muscles. In this study, visible sonographic changes were found in every clinically affected muscle, using a modified Heckmatt scale. In some muscles morphologic changes preceded weakness. Regarding the anatomical pattern of involvement, our findings do not support the hypothesis of a specific pattern with a higher vulnerability of vastus intermedius than rectus femoris, which has been postulated before. A frequent sparing of triceps brachii could be confirmed. Intramuscular abnormalities occurred in a focal, a diffuse, or an intermediate pattern, with characteristics of both. In contrast to muscular dystrophies, bone echogencity was not markedly decreased in Pompe disease even in an advanced stage. In infants, muscle ultrasound showed no distinct pathology even in clinically severely affected children and should not be used as a screening method for infantile Pompe disease. (C) 2014 Elsevier B.V. All rights reserved.
| Item Type: | Article |
|---|---|
| Uncontrolled Keywords: | ACID MALTASE DEFICIENCY; ALGLUCOSIDASE ALPHA; MRI; ULTRASONOGRAPHY; CHILDHOOD; DIAGNOSIS; GLYCOGEN; CHILDREN; Infantile Pompe disease; Adult Pompe disease; Acid maltase deficiency; GAA-deficiency; Muscle ultrasound; Quantitative grey scale analysis |
| Subjects: | 600 Technology > 610 Medical sciences Medicine |
| Divisions: | Medicine > Lehrstuhl für Strahlentherapie |
| Depositing User: | Dr. Gernot Deinzer |
| Date Deposited: | 26 Jul 2019 09:19 |
| Last Modified: | 26 Jul 2019 09:19 |
| URI: | https://pred.uni-regensburg.de/id/eprint/5987 |
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