Kozian R, Kiszka T, Peter K
Klinik für Psychiatrie und Neurologie Hans Berger, Friedrich-Schiller-Universität Jena.
Psychiatr Prax. 1994 Nov;21(6):235-7.
Kufs' disease is a very rare type of neuronal ceroid lipofuscinosis. A case of a 52 year old man with dementia is described. The cause for patient's dementia was the adult type (Kufs' disease) of neuronal ceroid lipofuscinosis. The diagnosis based on the histopathological post mortem-examination of the brain-tissue. A brother of our patient became ill with the same symptoms and at the same age of onset. So we conclude that there is a accumulation in the family.
库夫斯病是一种非常罕见的神经元蜡样脂褐质沉积症。本文描述了一例52岁患有痴呆症的男性病例。该患者痴呆的病因是成人型（库夫斯病）神经元蜡样脂褐质沉积症。诊断基于对脑组织的死后组织病理学检查。我们患者的一个兄弟在相同年龄出现了相同症状。因此，我们得出结论，该家族中存在这种疾病的聚集现象。
