Laura Winikka, Austin Thomas*, Michael Huang and Jeffrey Chen
Mitochondrial Disorders (MTDs) are a group of rare, highly heterogeneous disorders most commonly involving genetic defects in the oxidation-phosphorylation pathway. Multiple organ systems can be affected, though endocrine related conditions are particularly common. Non-diabetic patients with MTD have been found to have significantly increased rates of hypoglycemic episodes compared to the general population. Here we present a case of recurrent nocturnal hypoglycemic episodes in a 29-year-old male with unspecified mitochondrial myopathy who underwent extensive hypoglycemia testing which came back negative. The patient was ultimately started on a cornstarch therapy adapted from the treatment protocol for glycogen storage disease type Ia, leading to a resolution of his nocturnal hypoglycemia. The stabilization of blood glucose levels in this case suggests that the cornstarch protocol of 1.6 g/kg body weight before bedtime may be a viable treatment option for non-diabetic hypoglycemia in MTDs.
Published Date: 2022-09-19; Received Date: 2022-08-17
