Sur une pseudo-myopathie ligneuse généralisée par amyloïdose primaire endomysio-vasculaire

Abstract

The authors report the case of a 50-year-old white female whose chief complaints were of lassitude and weakness of the proximal limb muscles. She showed a rather athletic type of musculature (at least proximally), a severe and diffuse induration of all the muscles (“wooden” consistency), and a marked muscular rigidity with extreme slowness of movement. Mastication and, towards the end of her 18 months' illness, deglutition, were markedly impaired. Loss of weight, dyspnoea with a steadily deteriorating general state of health contrasted with the apparent increase in volume of the abdominal, girdle and proximal limb muscles. The diagnosis of primary amyloidosis was made clinically by the examination of muscle and rectal biopsies, both positive for amyloid, while the liver and renal biopsies were negative. At autopsy, all the striated muscles showed a very severe endomysial infiltration with amyloid while the viscera were much less involved: only the walls of arteriolar and venular vessels were heavily infiltrated with amyloid deposits; the lungs were severely involved while the myocardium was almost completely spared. The classical sites of localisation of “secondary amyloidosis” were intact. Histochemical and ultrastructural studies confirmed the presence of amyloid. The authors discuss at length the clinical differential diagnosis. They classify their case as an example of sporadic generalized primary amyloidosis in which the degree of striated muscle involvement usually remains relatively slight. The diffuse endomysial infiltration in this case was of a degree not previously seen in reported cases although a few similar but less severely-affected cases have previously been reported.