This exhibit illustrates the varied non-specific MR appearance of mitochondrial OXPHOS disease. The majority of patients suffering from mitochondrial OXPHOS disease will have either a normal MRI of the brain or non-specific neuroimaging findings. The results of the MRS analysis show that it is normal in the majority of cases. Abnormalities revealed on MRS included elevated lactate indicating acute metabolic derangement and decreased NAA and choline with or without elevated mI indicating neuronal loss. An abnormal MRS does not seem to add any more information than what was already neuropathologically evident on routine MRI. It is important for the neuroradiologist to consider mitochondrial OXPHOS disease as a possible diagnosis in patients exhibiting neuroimaging and neurospectroscopic findings that are illustrated in our exhibit.