Rivas Eloy, Sanchez-Herrero Joaquin, Alonso Miguel, Alvarez Maria J, Teijeira Susana, Ballestín Claudio, Tardio Antoni, Navarro Carmen
Department of Pathology and Neuropathology, Meixoeiro Hospital, Vigo, Spain.
Neuropathology. 2005 Jun;25(2):153-8. doi: 10.1111/j.1440-1789.2005.00595.x.
We report the case of a 79-year-old woman who developed a rapidly progressive dementia (RPD) with severe memory impairment, early visual hallucinations and extrapyramidal signs. Symptoms started suddenly after hip replacement surgery following an accidental fall. Motor epileptic seizures appeared at the end of the illness. Dementia worsened gradually leading to akinetic mutism. She died five and a half months after the onset of symptoms. MRI showed cerebral atrophy but failed to detect any other lesion. Results of all laboratory tests performed were negative. After the most frequent treatable diseases were excluded, the diagnosis of dementia with Lewy bodies was initially considered. CJD was also suggested based on the rapid evolution of the disease and the positivity of 14-3-3 protein in CSF. Neuropathological examination revealed an extensive miliary metastatic dissemination from an unknown primary adenocarcinoma. Pulmonary origin was suggested according to the immunohistochemical profile. Histopathological changes of Alzheimer's disease were also observed in the cerebral cortex and hippocampus. Neither Lewy bodies nor PrP deposits were found. The sudden onset of the dementia just after the hip replacement surgery raises the possibility of a pathological fracture with secondary tumoral microembolic dissemination. Despite its rarity, this entity should be included in the differential diagnosis of RPD. This case illustrates the definite importance of neuropathological post-mortem examination in order to elucidate the different types of dementia.
我们报告了一例79岁女性患者,该患者出现快速进展性痴呆(RPD),伴有严重记忆障碍、早期视幻觉和锥体外系症状。症状在一次意外跌倒后的髋关节置换手术后突然出现。病程末期出现运动性癫痫发作。痴呆逐渐加重,最终导致运动不能性缄默症。患者在症状出现五个半月后死亡。MRI显示脑萎缩,但未发现任何其他病变。所有实验室检查结果均为阴性。在排除了最常见的可治疗疾病后,最初考虑路易体痴呆的诊断。基于疾病的快速进展以及脑脊液中14-3-3蛋白阳性,也怀疑为克雅氏病(CJD)。神经病理学检查发现有广泛的粟粒状转移灶,原发灶为不明来源的腺癌。根据免疫组化结果提示为肺源性。在大脑皮层和海马体中也观察到了阿尔茨海默病的组织病理学变化。未发现路易小体和朊蛋白沉积。髋关节置换手术后痴呆突然发作,提示可能存在病理性骨折继发肿瘤微栓塞播散。尽管这种情况罕见,但在RPD的鉴别诊断中应考虑到。该病例说明了尸检神经病理学检查对于明确不同类型痴呆的重要性。