case_images: PMC4782535_01
case_images: Showing MRI brain T2-weighted (a) and FLAIR (b) showing brainstem and cerebellar peduncle hyperintensities with raised lactate peak in MRS (c). MRI = Magnetic resonance imaging, FLAIR = fluid attenuated inversion recovery, MRS = MR spectroscopy
case_images: AIAN-19-140-g001.jpg
case_images: PMC4782535_01_AIAN-19-140-g001.jpg
case_images: F1
case_images: MRI revealed increased brainstem hyperintensities with MR spectroscopy (MRS) of the lesion showing peak lactate [Figure 1].
case_images: Showing the COX-negative fibers (*) in the patient (b) in comparison with a normal muscle biopsy specimen showing COX-positive fibers (a). COX = Cytochrome C oxidase
case_images: AIAN-19-140-g002.jpg
case_images: PMC4782535_01_AIAN-19-140-g002.jpg
case_images: F2
case_images: Muscle biopsy revealed reduced COX and COX-succinate dehydrogenase (SDH) activity without any evidence of ragged red fibers [Figure 2].
case_images: Repeat MRI brain T2-weighted (a) and FLAIR (b) sequencesshowing disappearance of brainstem hyperintensities after treatment
case_images: AIAN-19-140-g003.jpg
case_images: PMC4782535_01_AIAN-19-140-g003.jpg
case_images: F3
case_images: Repeat MRI after 3 months revealed complete disappearance of the hyperintensities [Figure 3].