Heckmann J G, Lang C J, Petruch F, Druschky A, Erb C, Brown P, Neundörfer B
Department of Neurology, University of Erlangen Nuremberg, Erlangen, Germany.
J Neurol Neurosurg Psychiatry. 1997 Sep;63(3):388-90. doi: 10.1136/jnnp.63.3.388.
A 45 year old woman is reported who initially presented with a cerebellar syndrome, severe ataxia, and dysarthria. She rapidly deteriorated to coma vigile with bilateral myoclonic jerks, flexion rigidity, and immobility necessitating complete nursing. Her EEG showed generalised slow activity and periodic biphasic and triphasic waves. The CSF concentration of neuron specific enolase was very high. Consequently the diagnosis of Creutzfeldt-Jakob disease was established. Eight months later she died of respiratory complications. Thirty years earlier the patient had undergone corneal transplantation for keratoconus. Review of the organ donor's hospital records showed that death was caused by intercurrent pneumonia subsequent to subacute spongiform encephalopathy confirmed by necropsy. In view of two previous case reports in the literature it is presumed that the cadaveric cornea was the source of transmission of Creutzfeldt-Jakob disease in this patient.
据报道，一名45岁女性最初表现为小脑综合征、严重共济失调和构音障碍。她迅速恶化至睁眼昏迷状态，伴有双侧肌阵挛性抽搐、屈曲性强直和肢体不能活动，需要完全护理。她的脑电图显示广泛性慢活动以及周期性双相和三相波。脑脊液中神经元特异性烯醇化酶的浓度非常高。因此，确诊为克雅氏病。八个月后，她死于呼吸并发症。30年前，该患者因圆锥角膜接受了角膜移植。查阅器官捐献者的医院记录显示，死亡原因是尸检证实的亚急性海绵状脑病继发的间质性肺炎。鉴于文献中之前的两例病例报告，推测该患者克雅氏病的传播源是尸体角膜。
