Table 2.
Neuropathological evaluation of SuS patients and Slco1c1-HA/EC-HA mice.
| SuS | Slco1c1-HA/EC-HA mice | |
|---|---|---|
| Frequent location of lesions | ||
| Leptomeningesa,b,c | Leptomeninges | |
| ND | Choroid plexus | |
| Snow ball lesions in corpus callosuma,b,c | Corpus callosum | |
| Subcortexa,b,c | Subcortex | |
| Cerebelluma,b,c | Cerebellum | |
| ND | Spinal cord | |
| Retinab,c | Retina | |
| Inner earb | Inner ear | |
| Target cells | Brain ECsa,b,c | Brain ECs |
| Neuropathological changes | ||
| Binding of CTLs to luminal side of BVs, invasion into vessel wallsa, moderate parenchymal infiltration of CD8+ T cells, rare number of CD20 B cells, and the absence of plasma cellsa,b,c | Binding of CTLs to luminal side of BVs, parenchymal infiltration of CD8+ T cells | |
| Occlusion of BVs, swollen ECs, thickened vessel wallsb,c, apoptosis of ECsa, focal disruption of the BBB, microhemorrhages/microinfarctsa,b,c | Occlusion of BVs, apoptosis of ECs, focal disruption of the BBB, microhemorrhages/microinfarcts | |
| Ischemic lesions with local loss of neurons, astrocytes, and oligodendrocytesa,c | Ischemic lesions with local loss of neurons, astrocytes, and oligodendrocytes | |
| Immune mechanism | ||
| Targeting of ECs by CD8+ T cells against unknown EC antigen(s)a | Targeting of ECs by MHC-restricted HA-specific CD8+ T cells | |
| No detection of deposition of Ig and complementa,c | No detection of deposition of Ig and complement | |
Table comparing the neuropathological findings between SuS patients and the EC-HA mouse model
BBB blood–brain barrier, BV blood vessel, CTL cytotoxic T cell, EC endothelial cell, Ig immunoglobulin, ND not detected
aThis manuscript
bDörr et al., based on MRI findings7
cAgamanolis et al. and Hardy et al.: based on neuropathological evaluation9,16