Yoshimura N, Hayashi S, Fukushima Y
Department of Pathology, Hirosaki University School of Medicine, Japan.
Acta Pathol Jpn. 1992 Nov;42(11):826-31. doi: 10.1111/j.1440-1827.1992.tb01884.x.
A 38-year-old male patient with the juvenile variant of Parkinson's disease, in whom onset had occurred at the age of 24 yr, was autopsied. There were no clear symptoms of pancreatic or hepatic insufficiency during the entire clinical course. The only notable features were a slightly delayed decrease of the blood glucose level in an oral glucose tolerance test, slightly elevated levels of serum alkaline phosphatase and serum lactate dehydrogenase, and episodic loose stools. Autopsy revealed uniform enlargement of the pancreas due to massive fat replacement (lipomatous pseudohypertrophy): the exocrine glandular elements showed marked atrophy and loss, while the islets of Langerhans were preserved. The liver exhibited a histology closely mimicking alcoholic hepatitis associated with the diffuse presence of Mallory bodies (MBs), possibly indicative of a disturbance of protein metabolism. The nervous system showed the diffuse presence of Lewy bodies (LBs) in the cerebrum in addition to the ordinary lesions of Parkinson's disease. Although the etiopathogenesis of none of these three lesions has been well elucidated, common epitopes of MBs and LBs have recently been demonstrated. Therefore, the present case study suggests that a specific underlying toxic agent may cause diffuse LBs in the brain on the one hand, and diffuse MBs in the liver and lipomatous pseudohypertrophy of the pancreas on the other.
一名38岁男性帕金森病青少年型患者接受了尸检,其发病年龄为24岁。在整个临床过程中,没有明显的胰腺或肝功能不全症状。唯一值得注意的特征是口服葡萄糖耐量试验中血糖水平略有延迟下降、血清碱性磷酸酶和血清乳酸脱氢酶水平略有升高以及偶发性稀便。尸检发现胰腺因大量脂肪替代而均匀肿大(脂肪性假肥大):外分泌腺成分显示明显萎缩和丧失,而胰岛得以保留。肝脏组织学表现与伴有马洛里小体(MBs)弥漫性存在的酒精性肝炎极为相似,这可能表明蛋白质代谢紊乱。神经系统除了有帕金森病的常见病变外,大脑中还弥漫性存在路易小体(LBs)。尽管这三种病变的病因发病机制均未完全阐明,但最近已证实MBs和LBs存在共同表位。因此,本病例研究表明,一种特定的潜在毒性物质可能一方面导致大脑中出现弥漫性LBs,另一方面导致肝脏中出现弥漫性MBs以及胰腺脂肪性假肥大。