[Involvement of the optical system and ocular muscle nuclear in subacute necrotizing encephalomyelopathy (Leigh) from a pathomorphological point of view (author's transl)].

We report on the pathomorphological findings in the optical system, including the ocular muscles, of a girl who died at the age of 15 of subacute necrotizing encephalomyelopathy (Leigh). The first symptom of the disease, which was caused by respiratory insufficiency, was the alteration in visual function seven-and-a-half years previously. In addition, changes in pupillary innervation and disturbances of motility of the eyes were noted during the early stages of the disease and therefore represent an important sign in differential diagnosis. Clinical, pathomorphological and differential diagnosis, aspects of this rare disease are discussed.