PMC8414607_01
Initial MRI of the brain from outside hospital. It showed T2-weighted fluid-attenuated
inversion recovery (FLAIR) sequence hyperintensities in the bilateral basal ganglia and
diffuse increased signal in the white matter in the bilateral cerebral hemispheres (c),
with no structural abnormality, mass effect, hemorrhage, restricted diffusion, or
enhancement. (a) T1-weighted, (b) T1-weighted post-contrast, (d) diffusion-weighted
imaging, and (e) apparent diffusion coefficient.
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IgG, proteinase 3 AB IgG, amphiphysin AB, Purkinje cell cytoplasmic AB type 1, 2, Tr, antiglial AB type 1, collapsin response-mediator protein-5 (CRMP-5):immunoglobulin G (IgG), P/Q type and N type calcium channel AB, antineuronal Nu type 1, 2, 3 AB, and neuronal (V-G) K channel AB), and lumbar puncture with normal opening pressure (OP) and cerebrospinal fluid (CSF) analyses (i.e. glucose, encephalitis panel, VDRL, cytology, West Nile virus, and protein 14-3-3), except for elevated protein at 86 mg/dL. A computed tomography (CT) of the head, magnetic resonance imaging (MRI) of the brain with and without gadolinium, magnetic resonance angiogram (MRA) of the head and neck, and cerebral angiogram were also performed and were negative, except for T2-weighted image hyperintensities in the bilateral basal ganglia and increased T2-weighted image signal of the white matter in the bilateral cerebral hemispheres (Figure 1). Initial electroencephalogram (EEG) showed intermittent left-sided frontotemporal slowing and sharp waves, and a subsequent one a week later showed diffuse slowing.
Temporal sequence MR imaging of the brain comparing initial MRI from outside hospital
to those obtained at our institution. (a)-(c) Initial MRI from the outside hospital
obtained on 9 July 2018. Serial MRIs are shown from our institution obtained on 18 July
2018 (d)-(f) and 2 August 2018 (g)-(i). (a), (d), and (g) show temporal T2-weighted
FLAIR sequences at the different dates above. (b), (e), and (h) show T1-weighted
post-contrast images. (c) shows apparent diffusion coefficient image. (f) and (i) show
diffusion-weighted imaging. Images again demonstrate the temporal history of the
T2-weighted FLAIR sequence hyperintensities in the white matter in the bilateral
cerebral hemispheres ((a), (d), and (g)).
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MRI brain with and without contrast again demonstrated extensive white matter changes without abnormal enhancement (Figure 2).
Pathology of frozen sections from brain biopsy. (a) Low power demonstrating white
matter spongiform changes (40x, H&E). (b) High power demonstrating white matter
spongiform changes in a background of gliosis (400x, H&E). (c) CD68 IHC stain
highlighting macrophages (200x, CD68). (d) GFAP IHC stain highlighting reactive gliosis
(200x, GFAP).
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A right frontal brain biopsy was performed and pathology revealed white matter spongiform changes, with reactive gliosis, and absence of any signs of inflammation (Figure 3), suggestive of toxin exposure.