Hyperglycemic crisis in patients with mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS)

Abstract Background Diabetes mellitus (DM) is the most commonly encountered endocrinopathy in patients with mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS), which manifests as multisystemic organ failure. Whether the management of DM in MELAS requires special consideration is not fully clarified. Patients and methods In this single-center study, we retrospectively reviewed the medical records of patients with MELAS to elucidate the clinical characteristics of MELAS-associated DM. Results Four patients among a total of 25 patients with MELAS treated in the study institution developed DM. One patient had well-controlled DM, whereas the remaining three patients experienced hyperglycemic crisis as the first manifestation of DM. Two of the three patients were children aged four and six years. The hyperglycemic events occurred after surgery, infection, and status epilepticus, respectively. None of the three patients had DM previously based on randomly measured serum glucose levels that were within the normal range before the hyperglycemic crisis. Glycated hemoglobin levels measured during the hyperglycemic crisis indicated prediabetes and DM in 2 and 1 patient, respectively. Two patients recovered, whereas one patient died after developing multiorgan failure. Conclusion Fulminant-onset DM observed in patients with MELAS underscore the importance of routine measurement for glycated hemoglobin and more intense evaluation of glucose intolerance regardless of the patient age and lack of symptoms. Clinicians should be aware of the potential acute onset of hyperglycemic crisis in patients with MELAS especially in the presence of aggravating factors.