Rinsho Shinkeigaku (Clinical Neurology)

Symposium 3

Glucose intolerance in myotonic dystrophy type 1

Hiroto Takada, M.D.1), Yoshinobu Oyama, M.D.1), Seiko Kon, M.D.1) and Takashi Goto, M.D.2)

1)Department of Neurology, Aomori Hospital, National Hospital Organization
2)Department of Internal Medicine, Akita Red Cross Hospital

Insulin resistance is a characteristic feature of glucose intolerance in myotonic dystrophy type 1 (DM1). DM1 patients with dysglycaemia have liver insulin resistance as well as muscle insulin resistance, and also abnormality of insulin secretion. Insulin resistance in DM1 might result in multiple metabolic defects. Low level of fasting plasma glucose is a characteristic feature in the early stage of glucose intolerance in DM1, Early intervention against insulin resistance in DM1 is suggested because glucose intolerance could deteriorate in a certain degree of cases. Metformin treatment is useful to improve insulin resistance in DM1. Diabetic patients with DM1 usually show mild hyperglycemia. However, poorly controlled patients with hyperglycemic pattern tending to rise from morning to evening exist. Intensive insulin therapy might be necessary in such cases. We should pay attention to hypoglycemia due to hyperinsulinemia, pseudo improvement of glucose control according to exacerbated dysphagia, and acute aggravation caused by infections, at a bedside.
Full Text of this Article in Japanese PDF (214K)

(CLINICA NEUROL, 52: 1259|1260, 2012)
key words: myotonic dystrophy, glucose intolerance, insulin resistance, fasting plasma glucose, metformin

(Received: 25-May-12)