Rinsho Shinkeigaku (Clinical Neurology)

Original Article

Long-term treatment of diabetes mellitus in myotonic dystrophy with pioglitazone

Toshiyuki Yamamoto, M.D., Yasushi Oya, M.D., Tateo Isobe, M.D., Toshihiko Shirafuji, M.D., Katsuhisa Ogata, M.D., Masafumi Ogawa, M.D. and Mitsuru Kawai, M.D.

Department of Neurology, National Center Hospital for Mental, Nervous and Muscular Disorders, National Center of Neurology and Psychiatry

We report beneficial effects of pioglitazone on insulin resistance in diabetes mellitus accompanied with myotonic dystrophy (DM1). We studied eight DM1 patients with diabetes mellitus aged 32 to 60 (mean age 52.1±8.6 years). Three of them were under glibenclamide treatment, but their plasma glucose control was poor because of occasional hypoglycemia; others had not been treated with any hypoglycemic drugs. We administered a daily dose of 15 mg pioglitazone for 6-36 months (mean period 14.8±9.1 months). Plasma glucose control improved in all patients. In a 75 g oral glucose tolerance test, plasma glucose level at 120 min dropped from 203.3±41.7 mg/dl to 153.9±39.5 mg/dl (p=0.04); the area under the insulin curve up to 120 min (Σ IRI) dropped from 236.9±170.2 μU·hr/ml to 169.6±81.3 μU·hr/ml (p=0.12). Σ IRI decreased in four patients with pretreatment Σ IRI≥250 μU·hr/ml; it slightly increased in other patients with pretreatment Σ IRI≤150 μU·hr/ml. The homeostasis model assessment-insulin resistance (HOMA-IR) improved from 2.1±1.0 to 1.1±0.4 (p=0.04). Impairment of liver functions, cardiac failure, or hypoglycemia was not observed. Pioglitazone treatment is useful to improve insulin resistance and glucose control in DM1 patients with diabetes mellitus, especially patients with reactive hyperinsulinemia to glucose loading.

(CLINICA NEUROL, 45: 287|292, 2005)
key words: myotonic dystrophy, diabetes mellitus, insulin resistance, thiazolidinedione derivative, 75 g oral glucose tolerance test

(Received: 5-Apr-04)