Iwasaki Yasushi, Deguchi Akira, Mori Keiko, Ito Masumi, Kawai Yoshinari, Mimuro Maya, Yoshida Mari
Department of Neuropathology, Institute for Medical Science of Aging, Aichi Medical University, Nagakute, Japan.
Department of Internal medicine, Oyamada Memorial Spa Hospital, Yokkaichi, Japan.
Psychogeriatrics. 2018 Sep;18(5):421-426. doi: 10.1111/psyg.12342. Epub 2018 Jul 11.
This case report describes a Japanese man who presented with slowly progressive memory disturbances that began at the age of 79 years. The man also displayed conspicuous behaviour and psychological symptoms in the early stage of dementia. Computed tomography revealed atrophy of the amygdala and severe hippocampal deterioration, particularly in the anterior portion. Lateral ventricular dilatation, mainly affecting the anterior and inferior horns, was also observed. Interestingly, cerebral neocortical atrophy in the frontal and temporal lobes was considerably mild for the patient's age. Apolipoprotein E gene analysis showed epsilon 3 homozygosity. The patient died at the age of 96 years, and his clinical diagnosis was Alzheimer's disease with severe behavioural and psychological symptoms of dementia. In addition to indicating considerable hippocampal atrophy, an autopsy revealed numerous neurofibrillary tangles and argyrophilic grains in the brain, as well as extensive senile plaques. Cerebral amyloid angiopathy was also recognized. The pathological findings were suggestive of both Alzheimer's disease and argyrophilic grain dementia; other neurodegenerative disorders were not apparent. The clinicopathologic findings of the present case suggest significant consideration should be made when determining the clinical diagnosis and pathogenesis of senile dementia.
本病例报告描述了一名日本男性,他在79岁时开始出现缓慢进展的记忆障碍。该男子在痴呆症早期还表现出明显的行为和心理症状。计算机断层扫描显示杏仁核萎缩以及严重的海马体退化,尤其是前部。还观察到侧脑室扩张,主要影响前角和下角。有趣的是,对于该患者的年龄而言,额叶和颞叶的大脑新皮质萎缩相当轻微。载脂蛋白E基因分析显示ε3纯合子。患者于96岁时死亡,其临床诊断为伴有严重痴呆行为和心理症状的阿尔茨海默病。尸检除显示相当严重的海马体萎缩外,还发现大脑中有大量神经原纤维缠结和嗜银颗粒,以及广泛的老年斑。还发现了脑淀粉样血管病。病理结果提示同时存在阿尔茨海默病和嗜银颗粒痴呆;未发现其他神经退行性疾病。本病例的临床病理结果表明,在确定老年痴呆症的临床诊断和发病机制时应予以充分考虑。