Related Experiment Video
Updated: Jun 21, 2026

Immunolabelling Myofiber Degeneration in Muscle Biopsies
Published on: December 5, 2019
Hereditary rimmed vacuole myopathy showing interstitial amyloid deposition in muscle tissue
Yukiko Kojima1, Kenji Sakai, Chiho Ishida
1Department of Neurology, Ishikawa Prefectural Central Hospital, 2-1 Kuratsukihigashi, Kanazawa 920-8530, Japan.
Abstract:
We describe a consanguineous family that had progressive myopathy with rimmed vacuole (RV) formation and amyloid deposition. Patient 1 is a 71-year-old woman with muscle atrophy in the lumbar girdle and lower extremities. Patient 2 is a 40-year-old man (the son of Patient 1) with fatty changes in the biceps femoris muscles. Muscle biopsies revealed myopathic and neurogenic degeneration with RV, necrotic fibers, and interstitial amyloid deposition. Amyloid deposition was detected only in the muscle tissue.
Related Concept Videos
Cardiomyopathy IV: Restrictive Cardiomyopathy
Amyloid Fibrils
Amyloid deposits were observed as early as 1639 in the liver and the spleen. In 1854, Rudolph Virchow performed iodine staining, normally used to...
Amyloid Fibrils
Amyloid deposits were observed as early as 1639 in the liver and the spleen. In 1854, Rudolph Virchow performed iodine staining, normally used to...
Cardiomyopathy III: Hypertrophic Cardiomyopathy
Lysosomal Hydrolases
Myasthenia Gravis ll: Pathophysiology

