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case_images: Post-operative magnetic resonance imaging (MRI) of brain with and without gadolinium and pathological findings at age 12. (A) FLAIR sequence. (B) T1 sequence. (C) T1 and gadolinium sequence (post lesion resection) with no evidence of contrast enhancement. (D-F) Perivascular lymphocytic infiltrates in the meninges and the cortex. There are geographic areas of dystrophic calcifications, cortical gliosis and degenerative changes in the white matter with cavitary features, and macrophage accumulation. Arrow points the area of perivascular infiltration and asterisks mark the parenchymal inflammation. Pathology images courtesy of Peter Pytel, MD, The University of Chicago.
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case_images: Magnetic resonance imaging (MRI) of brain obtained at the time of presentation demonstrated a right temporal mass lesion that was surgically resected soon after the first hospital admission (Figures 1A-C). Preliminary pathological report raised the question of low grade glioneuronal tumor based on morphologically abnormal neuronal cells, however, detailed subsequent examination revealed perivascular lymphocytic infiltrates in the meninges and cortex, with areas of dystrophic calcifications and gliosis, concluding that the lesion is due to an idiopathic inflammatory process (Figures 1D-F).
case_images: MRI brain with and without gadolinium at age 18. (A-C) Multifocal regions of white matter T2/FLAIR hyperintensity in both cerebral hemispheres and corpus callosum. (D) Gadolinium enhancement in corpus callosum.
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case_images: Repeat brain MRI showed multiple gadolinium-enhancing lesions involving both hemispheres (Figure 2).
case_images: MR spectroscopy of brain lesions at age 18. (A) Double lactate peaks in the dominant T2/FLAIR hyperintense lesions of the corpus callosum and left cingulate gyrus. (B) Areas of MRS analysis.
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case_images: MR spectroscopy (MRS) analysis of one of the largest lesions detected a double lactate peak suggestive of a mitochondrial disease process (Figure 3).
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