[Congestive cardiomyopathy associated with a stiff spinal column. "Rigid spine syndrome"]. / Cardiomyopathie congestive associée à un syndrome de la colonne raide. "Rigid spine syndrome".

The "rigid spine syndrome" is a rare form of myopathy characterised by rigidity of the dorso-lumbar spine, hyperextension of the neck and muscular retraction limiting mobility, especially of the upper limbs with elbows fixed in semi-flexion. This condition, which was first described relatively recently, has been little studied: only 8 cases have been published to this day. We report the case of a 20 year old man with this syndrome, associated with a severe diffuse non-obstructive hypertrophic cardiomyopathy. Electronic microscopy and histoenzymological studies were performed on the skeletal muscle. They confirmed myopathic changes, demonstrating a great variation in diameter of the fibres, but there was no evidence of a predominating histoenzymological form (type I muscular atrophy) as previously reported. Although there have been no other reports of cardiac involvement in the "rigid spine syndrome", a link between the two conditions would seem to be likely.

[Hypercalcemia and osteoclast activating factor (OAF) associated with acute lymphoblastic leukemia (author's transl)]. / Hypercalcémie et facteur d'activation des ostéoclastes au cours d'une leucémie aiguë lymphoblastique.

The occurence of hypercalcemia appears to be unusual in leukemia. The mechanisms are numerous: secretion of PTH of PTH like substance, prostaglandin E2 or vitamin D like sterols. The reported case is one of lymphoblastic acute leukemia and hypercalcemia associated with osteoclast activating factor secreted by leukemic cells. In this patient the serum levels calcium closely paralleled the course of acute leukemia.