Short ReportGlycogen Debrancher Enzyme Deficiency MyopathySadeh, Menachem MD*; Yosovich, Keren PhD†; Dabby, Ron MD*Author Information *Department of Neurology, Wolfson Medical Center Holon, Affiliated with Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel; and †Molecular Genetic Laboratory, Edith Wolfson Medical Center, Holon, Israel. Reprints: Menachem Sadeh, Department of Neurology, Wolfson Medical Center, 62 Halochamim St, Holon, Israel 5822012 (e-mail: [email protected]). The authors report no conflicts of interest. Journal of Clinical Neuromuscular Disease: June 2021 - Volume 22 - Issue 4 - p 224-227 doi: 10.1097/CND.0000000000000339 Buy Metrics Abstract Glycogen storage disease type III is a rare inherited disease caused by decreased activity of glycogen debranching enzyme. It affects primarily the liver, cardiac muscle, and skeletal muscle. Pure involvement of the skeletal muscle with adult onset is extremely rare. We report on a patient with myopathy due to glycogen storage disease III, and describe the clinical features, and pathologic and genetic findings. Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.