Lane K L, Brown P, Howell D N, Crain B J, Hulette C M, Burger P C, DeArmond S J
Department of Pathology, Duke University Medical Center, Durham, North Carolina.
Neurosurgery. 1994 Apr;34(4):737-9; discussion 739-40. doi: 10.1227/00006123-199404000-00026.
A 28-year-old woman with prior neurosurgery involving the placement of a cadaveric dural graft developed a rapidly progressive neurodegenerative disorder with prominent cerebellar dysfunction that was proven at autopsy to be Creutzfeldt-Jakob disease. She represents the second American to develop Creutzfeldt-Jakob disease in association with a dural graft. The unusual features of the case include the patient's initial clinical presentation with cerebellar ataxia in the absence of dementia, the widespread presence of kuru-type amyloid plaques on a histological examination of the brain, the development of clinical symptoms during pregnancy, and the subsequent delivery of a child who remains healthy at the age of 3 years.
一名28岁女性,曾接受过涉及植入尸体硬脑膜移植物的神经外科手术,后来患上了一种快速进展的神经退行性疾病,伴有明显的小脑功能障碍,尸检证实为克雅氏病。她是第二位因硬脑膜移植物而患上克雅氏病的美国人。该病例的不寻常特征包括患者最初以小脑共济失调为首发临床表现且无痴呆症状,脑组织组织学检查发现广泛存在库鲁病型淀粉样斑块,孕期出现临床症状,以及随后产下一名3岁时仍健康的孩子。