Department of Neurology and Neurobiology of Aging, Kanazawa University Graduate School of Medical Sciences, Kanazawa, Japan.
Department of Neurology, Hokuriku Brain and Neuromuscular Disease Center, National Hospital Organization Iou National Hospital, Kanazawa, Japan.
Neuropathology. 2019 Aug;39(4):319-323. doi: 10.1111/neup.12581. Epub 2019 Jun 26.
We describe an autopsy-proven case of Parkinson's disease with dementia showing early-onset delusions and hallucinations with limbic-type Lewy body pathology. A Japanese man 72 years old at time of death, developed hand tremor at the age of 45. On neurological examination at 47 years of age, parkinsonian symptoms and signs were present. Pergolide mesylate 1000 μg/day improved his motor symptoms. Then, delusional jealousy appeared and he consulted the psychiatric department in our hospital at the age of 50. Pergolide mesylate 2000 μg/day and trihexyphenidyl hydrochloride 6 mg/day were prescribed. His delusional jealousy made him hit his wife at the age of 63, and visual hallucinations were demonstrated. Brain magnetic resonance imaging (MRI) at the age of 65 revealed mild frontal lobe atrophy. At the age of 72, apparent dementia and dysphagia appeared. The total clinical course was 27 years. The brain showed mild frontal atrophy and weighed 1295 g before fixation. Depigmentation of the substantia nigra and locus ceruleus was macroscopically apparent. Neuronal loss with gliosis was noteworthy in the substantia nigra, locus ceruleus, dorsal vagal nucleus, nucleus basalis of Meynert (NBM), and intermediate lateral nuclei; however, cerebral neocortex and limbic systems were relatively preserved. Widespread occurrence of Lewy bodies with a few Lewy neurites were demonstrated (limbic-type). Noticeable Lewy body pathology in the NBM was shown in contrast to that in other limbic system structures, such as the amygdala and parahippocampal gyrus, and cerebral cortex. In vivo structural imaging studies revealed that cholinergic projections from the NBM could be responsible for generation of cholinergic deficiency syndrome, including delusions and hallucinations. Furthermore, basal forebrain volume is reduced in patients with Parkinson's disease showing visual hallucinations. Prominent Lewy body pathology in the NBM could be related to not only visual hallucinations but also delusions.
我们描述了一例尸检证实的帕金森病伴痴呆病例，该病例表现为早发性妄想和幻觉，并有边缘型路易体病理学。患者为日本男性，死亡时 72 岁，45 岁时出现手部震颤。47 岁时进行神经学检查，出现帕金森病症状和体征。甲磺酸培高利特 1000μg/天改善了他的运动症状。随后，出现妄想性嫉妒，并于 50 岁时到我院精神科就诊。给予甲磺酸培高利特 2000μg/天和盐酸苯海索 6mg/天治疗。他的妄想性嫉妒使他在 63 岁时打了他的妻子，并出现了幻视。65 岁时的脑磁共振成像（MRI）显示轻度额叶萎缩。72 岁时，出现明显痴呆和吞咽困难。总病程为 27 年。大脑显示轻度额叶萎缩，固定前重 1295g。黑质和蓝斑色素脱失在宏观上明显。黑质、蓝斑、迷走神经背核、Meynert 核基底（NBM）和中间外侧核中可见神经元丢失伴胶质增生；然而，大脑新皮质和边缘系统相对保留。广泛存在Lewy 体，伴有少量 Lewy 神经突（边缘型）。与杏仁核和海马旁回等边缘系统结构以及大脑皮质相比，NBM 中明显的 Lewy 体病理学显示。体内结构成像研究表明，来自 NBM 的胆碱能投射可能是产生胆碱能缺乏综合征的原因，包括妄想和幻觉。此外，帕金森病患者的基底前脑体积减少与出现视觉幻觉有关。NBM 中突出的 Lewy 体病理学不仅与视觉幻觉有关，也与妄想有关。
