Laura Winikka2, Austin Thomas1*, Michael Huang2 and Jeffrey Chen1,2
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.
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