NR AKSL
AU Serratrice,G.
TI [Inclusion body myositis]
OT La myosite a inclusions
QU Presse Medicale 1996 Jun 15; 25(21): 985-8
PT journal article; review; review, tutorial
AB Inclusion body myositis has been recently recognized as a clinical entity although its exact definition remains uncertain. Initially considered to be an inflammatory dermatomyositis, inclusion body myositis can actually take on three specific forms: disseminated muscle atrophy and weakness, pseudopolymyositis, or pseudo-degenerative disease. Inclusion body myositis is different from non-inflammatory neuromuscular diseases with vacuoles. Abnormal deposits are seen within the muscle fiber may contain amyloid substance, beta-amyloid precursor, ubiquitin, antichymotrypsin, protein tau, apolipoprotein E and even prions. The signification of these deposits is unknown. Deletions in mitochondrial DNA have been demonstrated but do not appear to play a causal role. More and more hereditary forms are being recognized and certain may be related to an abnormality in chromosome 9.
ZR 23
MH Amyloid beta-Protein/*biosynthesis; Autoimmune Diseases/*complications; English Abstract; Female; Human; Male; Myositis, Inclusion Body/*complications/genetics/metabolism/pathology; Prions/biosynthesis; Ubiquitins/*biosynthesis; alpha 1-Antichymotrypsin/biosynthesis; tau Proteins/biosynthesis
AD Service de Neurologie et des Maladies neuromusculaires, CHU La Timone, Marseille.
SP französisch
PO Frankreich