Schmidt D, Noetzel H, Mühlhäuser J
Klin Monbl Augenheilkd. 1977 Oct;171(4):520-5.
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.
本文描述了一名患有海绵状神经胶质神经元营养不良的17岁患者。该病发病初期严重感染的症状为不明病因提供了线索。枕叶视觉障碍是该病例的主要体征。发病5年后患者死亡。病理解剖结果如下：视皮质神经节细胞坏死，小脑浦肯野细胞和颗粒细胞严重丢失，伴有海绵状改变及枕叶空洞形成。纹状体区、丘脑、齿状核及下橄榄核可见神经节细胞轻度丢失。此外，后索存在脱髓鞘改变。
