Takayama S, Hatsuda N, Matsumura K, Nakasu S, Handa J
Department of Neurosurgery, Shiga University of Medical Science.
No Shinkei Geka. 1993 Feb;21(2):167-70.
We report a case of Creutzfeldt-Jakob disease in a 38-year-old man, transmitted by a cadaveric dural graft. In August 1985, he underwent cranial nerve decompression for hemifacial spasm and received a cadaveric dural graft for dural closure. He had been well until he began to complain of blurred vision and headache in May, 1990. He developed dementia, myoclonus and urinary incontinence over the subsequent 3 months. He was admitted to our hospital in August, 1990. On admission, he was somnolent and showed gait disturbance, myoclonus in extremities and elevated deep tendon reflexes symmetrically. The results of analysis of blood, urinary and cerebrospinal fluid were normal. The initial computed tomography (CT) and magnetic resonance imaging detected no abnormality. Electroencephalography showed typical periodic synchronous discharge (PSD). There was progressive worsening of his neurological symptoms, and this developed into mutism in September, 1990. CT, 11 months after clinical onset, showed marked enlargement of the ventricles and the sulci. In view of his rapid worsening clinical course, PSD findings on electroencephalography, and delayed progressive changes of CT findings, the diagnosis of CJD disease was made. The cadaveric dural graft was suspected as the cause of the patient's condition. Since Thadani et al reported the first case of CJD transmitted by cadaveric dural graft in 1988, 3 other cases have been reported. This is most likely the 5th reported case of Creutzfeldt-Jakob disease transmitted by cadaveric dural graft.
我们报告一例38岁男性的克雅氏病,其由尸体硬脑膜移植传播。1985年8月,他因面肌痉挛接受颅神经减压术,并接受尸体硬脑膜移植以闭合硬脑膜。直到1990年5月开始出现视力模糊和头痛之前,他一直状况良好。在随后的3个月里,他出现了痴呆、肌阵挛和尿失禁。1990年8月他入住我院。入院时,他嗜睡,有步态障碍、四肢肌阵挛,双侧深腱反射亢进。血液、尿液和脑脊液分析结果均正常。最初的计算机断层扫描(CT)和磁共振成像未发现异常。脑电图显示典型的周期性同步放电(PSD)。他的神经症状逐渐恶化,1990年9月发展为缄默症。临床发病11个月后的CT显示脑室和脑沟明显扩大。鉴于其临床病程迅速恶化、脑电图上的PSD表现以及CT表现的延迟性进行性改变,作出了克雅氏病的诊断。尸体硬脑膜移植被怀疑是患者病情的病因。自1988年塔达尼等人报告首例由尸体硬脑膜移植传播的克雅氏病病例以来,又报告了另外3例。这很可能是第5例报告的由尸体硬脑膜移植传播的克雅氏病病例。