PMC4782535_01
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
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F1
MRI revealed increased brainstem hyperintensities with MR spectroscopy (MRS) of the lesion showing peak lactate [Figure 1].
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
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F2
Muscle biopsy revealed reduced COX and COX-succinate dehydrogenase (SDH) activity without any evidence of ragged red fibers [Figure 2].
Repeat MRI brain T2-weighted (a) and FLAIR (b) sequencesshowing disappearance of brainstem hyperintensities after treatment
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F3
Repeat MRI after 3 months revealed complete disappearance of the hyperintensities [Figure 3].