Mitochondrial diseases are a group of disorders that primarily present during infancy with variable phenotypes.  While still rare, mitochondrial disease can present with stroke like episodes, lactic acidosis, deafness, diabetes mellitus, short stature, and myopathy, usually in association with a family history with maternal inheritance.  

We report a case of mitochondrial disease in a formerly healthy 53-year-old male who presented with seizures and encephalopathy after an exploratory laparotomy for a small bowel obstruction.  His history was significant for hearing loss and suggestive of gastric dysmotility. The patient’s clinical course was characterized by status epilepticus requiring a prolonged NCCU stay.  Initial MRI suggested an acute cerebellar CVA. Subsequent imaging studies showed extensive bicortical and subcortical diffusion restriction.  After an extensive workup, the patient was found to have 3 mitochondrial gene variants not previously described in a single patient: MT-TA, MT-TV, and MT-TS1.  The patient was started on L-carnitine and coenzyme Q10 supplementation and his potentially mitochondriotoxic medications, phenobarbital and phenytoin, were discontinued. Ultimately, he stabilized with seizure control and there was no further radiographic progression. However, clinically, he was unchanged and was discharged to a skilled nursing facility in an unresponsive state.