Diffuse Mallory Bodies in the Liver, Diffuse Lewy Bodies in the Brain and Diffuse Fat Replacement (Lipomatous Pseudohypertrophy) of the Pancreas in a Patient with Juvenile Parkinson's Disease

Abstract

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 demonskated. 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. Acta Pathol Jpn 42: 826–831, 1992.

Exploring the metabolic syndrome: Nonalcoholic fatty pancreas disease

“…Chronic liver disease: Yoshimura et al[131] and Sasaki et al[132] suggested that lipomatous pseudohypertrophy of the pancreas might be caused by chronic advanced hepatic lesions, which lead to ductal obstruction. Thus now, only case reports of patients with chronic hepatitis B and liver cirrhosis support this hypothesis[49,132,133].…”

Fat replacement of the malignant pancreatic tissue after neoadjuvant therapy

“…Recent developments in screening methods give useful information for defining this disorder [15]. MRI is 100% effective at revealing lipomatosis [4,16]. An abdominal CT may show the fatty replacement in the pancreatic bed [10].…”

“…Despite insufficient information about its etiology, obesity, diabetes mellitus, chronic pancreatitis, hereditary pancreatitis, and conditions that obstruct the pancreatic duct, for example calculi and tumors, are regarded as predisposing factors [2,3]. Pancreatic lipomatosis is also known as ''lipomatous pseudohypertrophy'' [4]. Lipomatous pseudohypertrophy is characterized macroscopically by enlargement of the pancreatic tissue and microscopically by replacement of the exocrine system by fat cells [5,6].…”

Lipomatous Pseudohypertrophy of the Pancreas Taking the Form of Huge Massive Lesion of the Pancreatic Head

“…A literature search of Japana Centra Revuo Medicina and PubMed revealed only 27 other cases worldwide, including those in which the condition was diagnosed with imaging alone [1,2,3,4,5,6,7,8,9,10,11,12,13,14,15,16]. Unlike steatosis resulting from obstruction of the pancreatic duct by pancreatolithiasis or a tumor, the causal factors of this disease are unknown.…”