[Refsum's disease. Apropos of 2 cases disclosed by myocardiopathy].

Refsum's disease is a polyneuropathy due to a hereditary error in the metabolism of a fatty acid, phytanic acid, usually leading to cardiac failure only at an advanced stage of the disease. The authors report the case of two brothers with Refsum's disease revealed by a heart failure before the clinical stage of the peripheral neuropathy. In the younger brother, the affection started at the age of 22 years by an acute pulmonary oedema which revealed a dilated, hypokinetic myocardiopathy, associated with retinitis pigmentosa, ptosis, anosmia and biological myolysis.

[Atrio-ventricular block in Steinert's disease. Electrophysioligic and histologic study of the conduction system].

An anatomical study of the conducting pathways was carried out in a case of Steinert's disease complicated by a complete atrio-ventricular block with syncope. Electrical study of the potentials in the bundle of His located the block within and below the bundle. When the patient died, an anatomical study of the conducting pathways was carried out.

[Aneurysms of the membranous septum. Apropos of 4 cases].

Three cases of aneurysm of the membranous septum were diagnosed in vivo. The fourth case was discovered at post-mortem examination in a patient who had a conductive disorder. These four cases give us reason to review the anatomical, clinical, radiological and haemodynamic features of this condition.