A distinctive type of infantile inflammatory myopathy with abnormal myonuclei.
Four infants developed progressive muscle weakness after a normal initial postnatal development. All patients had a moderate elevation of serum creatine kinase (CK) activity. Muscle biopsies revealed, in addition to myopathic features, endomysial and perivascular inflammation. Electron microscopy disclosed prominent myonuclear abnormalities. Corticosteroids in 3 patients were moderately beneficial. This appears to be a clinicopathologically distinct form of inflammatory myopathy of infants.
Sripathi
N
N
Neuromuscular Research Group, Montreal Neurological Institute and Hospital, Quebec, Canada.
Karpati
G
G
Carpenter
S
S
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D002467:Cell Nucleus / Q000648:ultrastructure*; D002675:Child, Preschool; D006801:Humans; D007223:Infant; D008297:Male; D008870:Microtubules / Q000648:ultrastructure; D018908:Muscle Weakness / Q000188:drug therapy / Q000473:pathology*; D018482:Muscle, Skeletal / Q000473:pathology* / Q000648:ultrastructure; D009135:Muscular Diseases / Q000188:drug therapy / Q000473:pathology*
D000305:Adrenal Cortex Hormones