Gertz H J, Stoltenburg G, Cruz-Sanchez F, Lafuente J, Schopol R
Institut für Neuropathologie, Klinikum Steglitz der Freien Universität Berlin.
Nervenarzt. 1988 Feb;59(2):110-2.
Creutzfeldt-Jakob disease (CJD) occurring in later life has a duration of about one year. Gait disturbance, myoclonic jerks, dementia and akinetic mutism occur. Post-mortem examination of the brain shows nerve cell loss, gliosis and spongiform changes of the grey matter. Clinical and neuropathological features of a case of the panencephalopathic type of CJD, with additional extensive degeneration of the cerebral white matter, are described. The panencephalopathic type of CJD is characterized clinically by a longer duration and a biphasic course.
晚发型克雅氏病(CJD)的病程约为一年。会出现步态障碍、肌阵挛性抽搐、痴呆和运动不能性缄默症。大脑尸检显示神经细胞丧失、胶质细胞增生和灰质海绵状改变。本文描述了一例全脑型CJD患者的临床和神经病理学特征,该病例还伴有大脑白质的广泛额外变性。全脑型CJD的临床特征为病程较长且呈双相病程。