Fujishiro Hiroshige, Iritani Shuji, Sekiguchi Hirotaka, Habuchi Chikako, Torii Youta, Matsunaga Shinji, Ozaki Norio, Yoshida Mari, Fujita Kiyoshi
Department of Sleep Medicine, Graduate School of Medicine, Nagoya University, Nagoya, Japan.
Department of Psychiatry, Graduate School of Medicine, Nagoya University, Nagoya, Japan.
Psychogeriatrics. 2016 Mar;16(2):139-44. doi: 10.1111/psyg.12128. Epub 2015 Apr 27.
Discrepancies between clinical and pathological diagnoses of dementia with Lewy bodies (DLB) may occur because the full disease progression remains unclear, especially during the early stage. Herein, we report the case of a 78-year-old Japanese man with hypochondriasis who had autopsy-confirmed limbic-type DLB pathology. He exhibited no core clinical features of DLB. We attempted to identify the clinicopathological correlations in the early stages of DLB. At the age of 77, he became hypochondriacal and exhibited progressive cognitive decline after the death of his wife. He was concerned about his poor physical condition, but hospital examinations did not identify any overtly abnormal findings. At 78 years of age, he consulted a neurologist with complaints of facial numbness and irritability. Neurological examination revealed no overt abnormality, and he scored 21 points on the Mini-Mental State Examination. Magnetic resonance imaging of the brain showed mild bilateral ventricular enlargement. The patient was clinically diagnosed as having possible Alzheimer's disease. Approximately 1 month after his consult, he died of acute pneumonia in a psychiatric hospital to which he had been admitted for severe aggressive behaviour. He exhibited no core clinical features pointing towards a clinical diagnosis of DLB. Neuropathological investigation revealed limbic-type Lewy body disease with concurrent minimum Alzheimer-type pathology, which corresponds to high-likelihood DLB pathology based on the Third Consortium DLB pathological criteria. The patient had minimum nigral degeneration, which is consistent with the absence of parkinsonism. This autopsied case suggests that some DLB patients exhibit hypochondriasis in the early stage of the disease, even if they lack the core clinical features of DLB.
路易体痴呆（DLB）的临床诊断与病理诊断之间可能存在差异，因为疾病的完整进展尚不清楚，尤其是在早期阶段。在此，我们报告一例78岁患有疑病症的日本男性病例，其经尸检确诊为边缘型DLB病理。他未表现出DLB的核心临床特征。我们试图确定DLB早期阶段的临床病理相关性。77岁时，他在妻子去世后变得疑病，并出现进行性认知衰退。他担心自己身体状况不佳，但医院检查未发现任何明显异常。78岁时，他因面部麻木和易怒症状咨询了神经科医生。神经检查未发现明显异常，他在简易精神状态检查表上得分为21分。脑部磁共振成像显示双侧脑室轻度扩大。该患者临床诊断为可能患有阿尔茨海默病。咨询后约1个月，他在因严重攻击性行为而入住的精神病院死于急性肺炎。他未表现出指向DLB临床诊断的核心临床特征。神经病理学调查显示为边缘型路易体病，同时伴有最低限度的阿尔茨海默型病理改变，根据第三次DLB联盟病理标准，这符合高度疑似DLB的病理特征。该患者黑质变性程度最低，这与无帕金森症相符。这例尸检病例表明，一些DLB患者在疾病早期表现出疑病症，即使他们缺乏DLB的核心临床特征。
