Adult nemaline myopathy with trabecular muscle fibers.
The term "trabecular myopathy" has been used to designate a syndrome resembling limb-girdle muscular dystrophy in which the predominant pathological feature is an abundance of lobulated or trabecular muscle fibers. However, the validity of this nosological entity has not been verified. Herein we describe a 63-year-old man with a severe, progressive myopathy who exhibited the typical pathological features of both trabecular myopathy and nemaline myopathy in association with a biclonal gammopathy. In this case, adult-onset nemaline myopathy was probably the primary disease process. The diagnostic significance of trabecular muscle fibers remains uncertain.
Irodenko
Viktoriya S
VS
Department of Neurology, University of California, School of Medicine, 505 Parnassus Avenue, / San Francisco, California 94143-0114, USA. viktoriya.irodenko@ucsf.edu
Lee
Han S
HS
de Armond
Stephen J
SJ
Layzer
Robert B
RB
D001706:Biopsy; D018450:Disease Progression; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D002479:Inclusion Bodies / Q000378:metabolism / Q000473:pathology; D008297:Male; D008875:Middle Aged; D018485:Muscle Fibers, Skeletal / Q000473:pathology; D018908:Muscle Weakness / Q000209:etiology; D018482:Muscle, Skeletal / Q000473:pathology* / Q000503:physiopathology; D009173:Mycophenolic Acid / Q000008:administration & dosage / Q000031:analogs & derivatives; D017696:Myopathies, Nemaline / Q000473:pathology* / Q000503:physiopathology; D010265:Paraproteinemias / Q000209:etiology / Q000473:pathology / Q000503:physiopathology; D011241:Prednisone / Q000008:administration & dosage
D007166:Immunosuppressive Agents; D009173:Mycophenolic Acid; D011241:Prednisone