[Occipital blindness in juvenile, spongiform glio-neuronal dystrophy (author's transl)]

A 17-year-old patient with spongiform glioneuronal dystrophy is described. Symptoms of a severe infection at the onset of this disease give an insight on the unknown etiology. Occipital visual disturbances were the main signs in this case. 5 years after the onset of the disease death occured. The pathological-anatomical findings were: necrosis of ganglion cells in the visual cortex, severe loss of Purkinje- and granular cells of the cerebellum with spongy changes and cavities in the occipital lobe. Slight loss of ganglion cells was to be seen in the striata area, thalamus, dentate nucleus, and in the inferior olive. In addition, there was a demyelinisation of the posterior funiculi.