Table 8. Pathological features of Cx43 astrocytopathy in active lesions of NMO and MS.
Pathological features | NMO and NMOSD (6 cases, n=26) | MS (3 cases, n=15) |
---|---|---|
Morphology of astrocytes | Highly degenerative (26/26) | Hypertrophic change (15/15), Creutzfeldt astrocytes (3/15) |
Immunoreactivity for | ||
Cx43 | Diffuse loss (26/26) | Diffuse loss (15/15) |
AQP4 | Diffuse loss (26/26) | Patchy loss (11/15), Diffuse loss (4/15) |
Cx47 & Cx32 | Preserved (8/26), Reduced (18/26) | Preserved (5/15), Reduced (7/15), Diffuse loss (3/15) |
Infiltration of neutrophils/eosinophils | 10/26 | 0/15 |
Complement deposition within | ||
Perivascular spaces | 12/26 | 0/15 |
Foamy macrophages | 11/26 | 3/15 |
Accompanied with distal oligodendrogliopathy | ||
MAG loss > loss of other myelin proteins | 8/26 | 12/15 |
Apoptotic nuclear change | 8/26 | 12/15 |
AQP4 = aquaporin-4, Cx = connexin, MAG = myelin-associated glycoprotein, MS = multiple sclerosis, NMO = neuromyelitis optica