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Fig. 2 | Acta Neuropathologica Communications

Fig. 2

From: An AARS variant as the likely cause of Swedish type hereditary diffuse leukoencephalopathy with spheroids

Fig. 2

Postmortem examination of brain tissue from Case 1. a Transverse section of the brain at the level of the posterior part of the frontal lobe showing liquefaction of the white matter sparing the subcortical arcuate fibers (arrow). b Section through the inner part of the cerebral cortex and the subcortical white matter showing preserved cortical neurons and subcortical myelin with some increased cellularity in the white matter (Luxol). c Section through the inner part of the cerebral cortex and the subcortical white matter showing slight gliosis of the cortex with preserved neurons and marked gliosis in subcortical white matter. In the transition zone large astrocytes are present (Immunohistochemistry of GFAP). d Cerebral cortex showing preserved neurons and some activation of microglial cells (arrows; Immunohistochemistry of CD68). e-h Sections from the deep white matter just beneath the preserved subcortical parts. e There are numerous corpora amylacea (arrows; Hematoxylin and eosin). f Fragmented axons with occasional swellings compatible with spheroids in the border (rim region) between preserved cortical white matter and the deep liquified parts (arrow; immunohistochemistry of neurofilament protein). g Numerous large rounded pigmented macrophages in the rim (arrows; Hematoxylin and eosin). h The rounded large macrophages in the rim region are CD68 positive (Immunohistochemistry of CD68). i The macrophages in the rim region contain PAS positive granula (arrows). j The storage material in the macrophages is also autofluorescent (arrows). k Section from the cervical spinal cord demonstrating marked secondary demyelination of the lateral and anterior corticospinal tracts (arrows, Luxol fast blue-cresyl violet). l The anterior corticospinal tract (upper part) showing vascular changes with thickened walls as compared with the adjacent, less affected white matter (lower part) (van Gieson)

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