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Initiation of metformin in MELAS patient–a dangerous combination

J. Trebach, Danish H. Ghazali, Devin J. Burke, Sarah G. Mahonski, R. Hoffman

Clinical toxicology, 2021

3 citations

Abstract

Dear Editor, Mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) is a genetic mitochondrial disease that presents with progressive myopathy, headaches, and recurrent seizures associated with encephalopathy and neurologic deficits in childhood [1]. MELAS is usually caused by a mutation affecting mitochondrial protein translation, with direct pathogenic mutations in nicotinamide adenine dinucleotide (NADH) dehydrogenase (complex I of the electron transport chain) recently emerging as an important cause [2–4]. Metformin also inhibits complex I of the electron transport chain. Here, we report a patient with MELAS who developed an exacerbation of her MELAS pathology recently after being started on metformin. A 30-year-old woman with MELAS presented to the emergency department (ED) with at least three episodes of seizure activity prior to arrival. Fingerstick glucose on arrival was 248mg/dL. The patient’s weight was 50 kg. There was no return to her baseline mental status between the seizures. In the ED, despite levetiracetam (60mg/kg IV) and lorazepam (4mg IV), she continued to seize and was intubated for airway protection and ultimately admitted to the intensive care unit (ICU) for status epilepticus. Her initial blood lactate in the ED was 12.2mmol/L. Computed tomography angiogram (CTA) and magnetic resonance imaging (MRI) were unremarkable. Lumbar puncture was only notable for an elevated cerebrospinal fluid lactate to 9.7mmol/L (reference range…

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