Internal Medicine
Online ISSN : 1349-7235
Print ISSN : 0918-2918
ISSN-L : 0918-2918
A Case of Prader-Willi Syndrome with Slowly Progressive Insulin-dependent Diabetes Mellitus
Yuki TomodaYukiyoshi OkauchiArichika DeguchiYu TakenoshitaHiromi IwahashiIkuo Mineo
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JOURNALS OPEN ACCESS Advance online publication

Article ID: 5267-20


We report the case of a 52-year-old woman with Prader-Willi syndrome (PWS) and diabetes. Her diabetes was managed with sulfonylurea followed by premixed insulin; however, her glycemic control gradually worsened and became unstable. Her urine and blood C-peptide levels were undetectable. She tested positive for anti-GAD antibodies, and had a high-risk genotype-DRB1*09:01-DQB1*03:03-for slowly progressive insulin-dependent diabetes mellitus (SPIDDM) in the HLA-DR/DQ region, confirming the diagnosis of SPIDDM. Dysglycemia in PWS is thought to be attributable to hyperphagia and obesity. However, the possibility of SPIDDM might be considered if the insulin secretory capacity is almost lost in patients with PWS.

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© 2021 by The Japanese Society of Internal Medicine