PMC8597803_01
Axial T2-weighted (A and B) and T1-weighted (C and D) MR images show bilateral symmetric signal changes which were hyperintense on T2 and hypointense on T1-weighted images, involving brainstem dorsal tracts (black arrow, A), basal ganglia (black arrows, B), medial thalami (yellow arrows, B), with diffuse cerebral gyral swelling (more marked in fronto-temporal lobes) and cystic changes in bilateral putamina.
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PMC8597803_01_autopsy-11-e2021334-g01.jpg
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Magnetic resonance imaging (MRI) of the brain showed bilateral symmetric lesions which were hyperintense on T2 and hypointense on T1-weighted images (Figure 1) with diffusion restriction (Figure 2) in cerebral hemisphere gyri, deep grey structures, and dorsal brainstem with lactate peak (Figure 3).
Axial diffusion-weighted imaging (DWI) (A and B. b = 1000 s/mm2) and apparent diffusion coefficient (ADC) maps (C and D) show bilateral diffusion restriction involving brainstem dorsal tracts (white arrow, A), basal ganglia (white arrow, B), medial thalami (yellow arrow, B), cerebellum and diffuse cerebral gyral swelling (more marked in fronto-temporal regions).
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PMC8597803_01_autopsy-11-e2021334-g02.jpg
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Magnetic resonance imaging (MRI) of the brain showed bilateral symmetric lesions which were hyperintense on T2 and hypointense on T1-weighted images (Figure 1) with diffusion restriction (Figure 2) in cerebral hemisphere gyri, deep grey structures, and dorsal brainstem with lactate peak (Figure 3).
MR spectroscopy (TE=144ms) at basal ganglia (putamen) showing lactate peak (double peak at 1.3 ppm which is inverted) and decreased NAA peak.
autopsy-11-e2021334-g03.jpg
PMC8597803_01_autopsy-11-e2021334-g03.jpg
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Magnetic resonance imaging (MRI) of the brain showed bilateral symmetric lesions which were hyperintense on T2 and hypointense on T1-weighted images (Figure 1) with diffusion restriction (Figure 2) in cerebral hemisphere gyri, deep grey structures, and dorsal brainstem with lactate peak (Figure 3).