An adult-onset myopathy characterized by a double ring appearance of muscle fibers.
We report a 33-yr-old man with an unusual neuromuscular disorder characterized by progressive generalized weakness of 3 yr duration whose muscle biopsy showed a double ring appearance in most muscle fibers. This double ring appearance was due to a peripheral outer sarcoplasmic mass and an inner ring of annular myofibrils surrounding a core of normal longitudinally oriented myofibrils. Nerve conduction studies were normal. Electromyography showed fibrillations, positive waves, and increased brief duration, low amplitude, polyphasic potentials.
Coulter
C L
CL
Department of Neurology, Creighton University Medical Center, Omaha, NE 68135.
Marks
W A
WA
Bodensteiner
J B
JB
Leech
R W
RW
Tonin
P
P
DiMauro
S
S
Brumback
R A
RA
D000328:Adult; D004576:Electromyography; D015444:Exercise / Q000502:physiology; D005260:Female; D006003:Glycogen / Q000378:metabolism; D006801:Humans; D008854:Microscopy, Electron; D009132:Muscles / Q000378:metabolism / Q000473:pathology*; D009210:Myofibrils / Q000648:ultrastructure; D009468:Neuromuscular Diseases / Q000473:pathology*; D012519:Sarcoplasmic Reticulum / Q000648:ultrastructure
D006003:Glycogen