A Case of Acquired Generalized Lipodystrophy with Cerebellar Degeneration and Type 2 Diabetes Mellitus

Article View

The Review of Diabetic Studies,2004,1,4,193-197.
Published:February 2005
Type:Case Report
Author(s) affiliations:

Pei-Jiuan Chao1, Jack C.-R. Tsai2, Dao-Ming Chang1, Shyi-Jang Shin3, Yau-Jiunn Lee2

1Department of Internal Medicine and 2Department of Clinical Research, Pingtung Christian Hospital, Pingtung, 90000 Taiwan.

3Graduate Institute of Medicine, Kaohsiung Medical University, Kaohsiung, 80307 Taiwan.


Acquired generalized lipodystrophy (AGL) is a rare disorder of adipose tissue characterized by loss of fat from large regions of the body, occurring after birth. Its etiology remains unknown. Most AGL patients have had fasting and/or postprandial hyperinsulinemia, diabetes mellitus, hypertriglyceridemia, and fatty liver. We describe the case of a 30- year-old woman with a progressively unsteady gait and a generalized loss of body fat beginning at the age of 7. Cerebellar degeneration was revealed by imaging study, and the patient was eventually bedridden at the age of 15, due to progressive ataxia. She developed diabetes at the age of 25 without the presence of any evidence of ketoacidosis. The glutamic acid decarboxylase antibody was negative, Cpeptide level 3.6 ng/ml, HbA1c 13%, triglyceride 412 mg/dl, total cholesterol 196 mg/dl, high-density lipoproteincholesterol 28 mg/dl, adiponectin 0.76 μg/ml, and resistin was 22.8 ng/ml at the initial state of diabetes. AGL accompanied by type 2 diabetes and cerebellar degeneration was diagnosed on the basis of the clinical features and metabolic derangements.