ONCOCYTIC CARDIOMYOPATHY - A RARE CAUSE OF UNEXPECTED EARLY-CHILDHOODDEATH ASSOCIATED WITH FITTING

A 15-month-old girl died unexpectedly in hospital following a five-day history of intermittent cardiac arrhythmias and convulsions preceded by several weeks of occasional vomiting. Autopsy revealed subendocardi al nodules in the left ventricle, and tricuspid and mitral valves that were composed of aggregated large cells with foamy, pale pink cytopla sm characteristic of oncocytic cardiomyopathy. Fat stains were positiv e for neutral lipid and phospholipid and electron microscopic examinat ion revealed numerous irregular mitochondria within affected cells. Ex amination of the brain revealed no structural or histologic abnormalit ies, anoxic damage or thromboembolic material. Oncocytic cardiomyopath y, though rare, may cause unexpected death in previously well young ch ildren with quite variable preceding clinical symptoms and signs which include fitting. Although the aetiology is unknown there is evidence that mitochondrial dysfunction may be involved.