Case Report

J Korean Soc Pediatr Endocrinol. 1997; 2(2): 263-267.

A Case of Early Manifested Long-term Complications in MODY(Maturity Onset Diabetes in Young) Patient.

Mi Ran Kang, and Heon Seok Han

A 14-year old boy presented with myoclonic seizure with rightward deviation of eyeballs. Three years ago, he was diagnosed as diabetes necessitating insulin injection. At that time, his blood glucose was 448mg/dl, HbA1c 27.8%, serum C-peptide rose from 0.4 to 1.1ng/ml after glucagon, and 24 hour urine C-peptide was 6.7microg/day. Eye examination was normal. His maternal grandmother died of diabetes at 50 years old, and his mother's sister and his elder sister had NIDDM with oral hypoglycemics. But, he didn't control hyperglycemia himself since that time. On physical exam, his grasping power was decreased in right hand, and cataract was detected at the posterior pole of lenses in both eyes requiring surgery. EEG showed partial seizure disorder in left frontoparietal area, and MRI revealed cerebral infarction in left frontoparietal cortex. Sensory-motor polyneuropathy was noted in nerve conduction velocity. His neurologic symptom was improved gradually with insulin therapy, but nerve conduction velocity and MRI abnormalities did not improved after 6 months of follow-up. Although long-term diabetic complication is common in poorly controlled diabetes, very early manifested eye and nervous system complications like this case is extremely uncommon.

Keywords : MODY(Maturity onset diabetes in young), Long term complication