Abstract
A 48-year-old male had previously been diagnosed with Charcot-Marie-Tooth (CMT) disease. He had developed thirst, polyuria and polydipsia at age of 45, and was hospitalized for poorly controlled diabetes (HbA1c 10.8 % (NGSP) ) at age of 46. He had visceral obesity (body weight 84.0 kg, BMI 28.1 kg/m2 and waist circumference 101 cm) and muscle atrophy of the distal extremities at the time of admission. A hyperinsulinemic-euglycemic clamp study showed severe insulin resistance in the peripheral tissues (metabolic clearance rate (MCR) 4.38 ml/kg/min). A liver biopsy sample revealed nonalcoholic fatty liver disease (NAFLD) with hepatic steatosis and fibrosis (NAFLD class 2, steatosis 2, grade 0.5, stage 2). He started bolus insulin therapy together with diet and exercise therapy. He successfully lost 19.6 kg (body weight 64.4 kg, BMI 21.8 kg/m2 and waist circumference 75.5 cm) and obtaining good glycemic control with HbA1c about 5 % after 1.5 years. His insulin resistance had markedly improved (MCR 6.86 ml/kg/min), and the liver steatosis and fibrosis had disappeared (NAFLD class 0, steatosis 0, grade 0, stage 0). Lifestyle intervention may therefore be useful for achieving an improvement of insulin resistance and glycemic control in obese diabetic patients with CMT disease.
