[Leigh disease. Morphology and clinical aspects]

Morphologico-topographic differences, especially with regard to size and distribution of foci, as well as equalities of the histomorphological pattern are shown in an infantile and in a juvenile case of Leigh's disease. Almost identical microscopic findings in the area of the olives in the medulla oblongata seem to be of special importance. The typical symptoms at the beginning of the disease with predominance to muscular hypotonia and the bulbar paralytic final stage might at least enable a tentative diagnosis even without evidence to the basic enzymatic defect, mostly in pyruvate metabolism, and changes in mitochondria in muscles and the central nervous system. The morphological investigation should especially include the medulla oblongata.