An autopsy case of dementia with Levy bodies with vocal cord abductor paralysis.
An autopsy case of dementia with Levy bodies with vocal cord abductor paralysis.
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利维氏体痴呆伴声带展肌麻痹的尸检病例。
DOI：
10.1159/000441448
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发表时间：
2015
期刊：
影响因子：
2.4
通讯作者：
Kobayashi T.
中科院分区：
文献类型：
--
作者：
Toru S;Uchihara T;Hara M;Mae S;Toru M;Hirokawa K;Endo T;Sugawara E;Kitagawa M;Kobayashi T.
声带外展肌麻痹（VCAP）常见于多发性全身性萎缩（MSA）的晚期[1，2，3，4，5]，在帕金森病（PD）中罕见[5，8，9]，在路易体痴呆（DLB）中例外。本文报告首例经尸检证实的DLB伴VCAP病例，患者71岁时出现焦虑症状，就诊于我院精神科，诊断为轻度痴呆，给予轻度镇静剂治疗。75岁时，他在打网球时遇到了困难。渐渐地，他的脚步慢了下来。76岁时，他出现了视觉幻觉。我院神经科医生根据简易精神状态检查（MMSE 21/30）发现运动不能、面具样脸和小步步态以及明显的痴呆。根据临床标准，他被诊断为可能患有DLB [10]。给予左旋多巴，但他的症状逐渐进展。他在81岁时因吸入性肺炎接受了经皮内窥镜胃造口术。他83岁时卧床不起。在87岁时，他表现出吸气性喘鸣，喉内窥镜检查显示双侧VCAP。颈胸XP和CT显示没有影响复发和/或迷走神经的病变。没有征得家属同意进行气管切开术。89岁时，因发热住进我院。入院时，神经系统检查发现新的发现，包括右侧优势强直和迈尔森征。无静息性震颤。患者表现为重度痴呆（MMSE 0/30）。他的脑部MRI显示包括海马在内的额颞叶皮质中度至重度萎缩和缺血性变化（图1a）。但无脑干-小脑萎缩、热十字征或纹状体异常信号。在住院第34天，他死于不受控制的感染。
Vocal cord abductor paralysis (VCAP), frequent in the later stage of multiple systemic atrophy (MSA)[1, 2, 3, 4, 5], is rare in Parkinson's disease (PD)[5, 8, 9] and exceptional in dementia with Lewy bodies (DLB). Here we report the first autopsy-verified case of DLB with VCAP.The patient felt anxiety at the age of 71, and consulted the psychiatrist of our hospital; mild dementia was diagnosed and minor tranquilizer was prescribed. At the age of 75, he experienced difficulty in playing tennis. Gradually, his walking slowed down. At the age of 76, he had visual hallucination. A neurologist of our hospital noted akinesia, mask-like face and small steppage gait and apparent dementia based on mini-mental state examination (MMSE 21/30). He was diagnosed as having probable DLB based on the clinical criteria [10]. L-DOPA was administered, but his symptoms progressed gradually. He had a percutaneous endoscopic gastrostomy because of aspiration pneumonia at the age of 81. He became bedridden at the age of 83. At the age of 87, he exhibited inspiratory stridor, and endoscopic examination of the larynx disclosed bilateral VCAP. The cervical-chest XP and CT revealed no lesion affecting the recurrent and/or vagus nerves. The family's consent to perform tracheotomy was not obtained. At the age of 89, he was admitted to our hospital with pyrexia. On admission, neurological examination revealed new findings including right-sided dominant rigidity and Myerson's sign. Resting tremor was absent. He showed severe dementia (MMSE 0/30). Moderate-to-severe atrophy of fronto-temporal cortex including hippocampus and ischemic change were revealed on his brain MRI (fig. 1 a). But brainstem-cerebellar atrophy, hot cross ban sign or abnormal intensity of striatum were absent. On hospital day 34, he died with uncontrolled infection.