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Table 3 Comparison of HAE with MS and EAE Models

From: Autoimmune encephalitis in humans: how closely does it reflect multiple sclerosis ?

MS Pathology

AcuteMS

Progr. MS

Case HAE

Marmoset MOG EAE

Mouse MOG EAE

Demyelination

     

DM Plaques

yes

yes

yes

yes

yesa

Perivenous / Confluent

yes

yes

yes

yes

yes

Periventricular

yes

yes

yes

yes

no

Slowly expanding lesions

no

yes

no

no

no

Dawson Fingers

yes

yes

yes

yes

no

Leukocortical Lesions

yes

yes

yes

yes

no

Intractortical Lesions

yes

yes

yes

yes

no

Subpial lesions

few

many

few

yes

no

Shadow plaques

few

var

no

yes

no

Pattern II DM

     

C9neo Deposition

var

few

yes

yes

no

Ig Deposition

var

var

yes

yes

no

Macrophage association

yes

yes

yes

yes

yes

Pattern III DM

     

Pre-phagocytic Lesions

var

few

no

no

no

Oligodendrocyte Apoptosis

var

var

no

no

no

Concentric DM Pattern

var

rare

no

no

no

Axonal / Neuronal Pathology

     

Relative axonal pres.

yes

yes

yes

yes

no

Acute injury in act. lesions

yes

yes

yes

few

yes

Diffuse axonal Injury

few

mod

few

few

few

Retrograde Neurodegeneration

few

yes

few

n.d.

yes

Astrocyte Pathology

     

Protoplasmic gliosis in active lesions

yes

yes

yes

mild

yes

Creutzfeldt Peters Cells

many

few

many

no

no

Fibrillary Gliosis in inactive lesions

yes

yes

yes

yes

yes

Aquaporin 4 loss

Var (Pat III)

no

no

n.d.

no

Inflammation

     

CD3

many

many

many

many

many

CD4

few

few

single

n.d.

many

CD8

many

many

many

n.d.

few

CD20

mod

mod

massive

mod

few

Plasma Cells

mod

mod

mod

mod

few

Follicle like structures

var

var

yes

yes

no

IgG PC

many

many

few

mod

n.d.

IgA PC

few

few

few

n.d.

n.d.

IgM PC

few

few

many

few

n.d.

PCNA CD8

yes

yes

yes

n.d.

n.d.

PCNA CD20

yes

yes

yes

n.d.

n.d.

Microglia / Macrophages

     

Iba1 (active lesions)

many

many

many

many

many

CD68 (active lesions)

many

many

many

n.d.

many

NADPH oxidase microglia

many

many

many

n.d.

no

Microglia nodules

many

mod

many

few

no

Diffuse activation NAWM/GM

mod

massive

mod

no

mod

Iron Related Pathology

     

Iron accumulation in NAWM/GM

Age related

Age related

Profound (age)

no

no

Iron loss in lesions

yes

yes

yes

no

no

Extracellular iron

yes

yes

yes

no

no

Uptake in Macrophages / MG

yes

yes

yes

few

no

Iron loss in PPWM

yes

yes

no

no

no

Oxidative Injury

     

Ox Lipids OG and Myelin

yes

yes

few (iron)

no

no

Ox lipids neurons

yes

yes

few (iron)

no

no

EBV

     

EBER positive B-cells

no

no

no

n.d.

n.d.

  1. Acute MS Marburg type of acute MS, Progr. MS primary or secondary progressive MS, Case HAE human autoimmune encephalitis, described in this study, Marmoset MOG EAE chronic EAE induced in marmosets by active sensitization with recombinant myelin oligodendrocyte glycoprotein in incomplete Freund’s adjuvant, Mouse MOG EAE chronic EAE in C57Bl6 mice induced by active sensitization with MOG35–55 peptide
  2. Yesa: white matter lesions in chronic mouse EAE are mainly due to massive axonal destruction and secondary demyelination
  3. Mod moderate, mild mild, few(iron) only few cells in lesions with high iron content