Neuromyelitis optica: a demyelinating disease characterized by acute destruction and regeneration of perivascular astrocytes.
BACKGROUND
A serum antibody directed against astrocytes is present in a high proportion of patients with neuromyelitis optica (NMO). The pathogenicity of the antibody is uncertain because no consistent astrocyte lesion is known to occur in NMO.


OBJECTIVE
To determine whether there is an astrocyte lesion in NMO and if this differs from astrocyte changes in multiple sclerosis (MS).


METHODS
Astrocyte pathology in early (still-myelinated) lesions and subacute NMO and MS lesions was examined immunohistochemically and in sections stained for astrocytes using routine histological techniques.


RESULTS
Demyelination in early NMO lesions is accompanied by oligodendrocyte apoptosis in a pattern identical to that seen in MS and this is preceded by an abrupt destruction of perivascular astrocytes. Reparative astrogliosis is effected by a population of unipolar, new astrocytes. Evidence of a different type of astrocyte lesion was found in MS.


CONCLUSIONS
The findings add to experimental evidence that the antibody is pathogenic. They also raise the possibility that demyelination in MS may be a bystander effect of an astrocyte lesion, i.e. that MS is not a disease primarily of myelin and oligodendrocytes.
Parratt
John D E
JD
The Institute of Clinical Neurosciences, Department of Medicine, The University of Sydney, NSW, Australia.
Prineas
John W
JW
D000328:Adult; D000368:Aged; D051401:Aquaporin 4 / Q000276:immunology; D001253:Astrocytes / Q000473:pathology*; D001344:Autopsy; D001921:Brain / Q000473:pathology; D003165:Complement System Proteins / Q000032:analysis; D003711:Demyelinating Diseases / Q000473:pathology*; D005260:Female; D005904:Glial Fibrillary Acidic Protein / Q000378:metabolism; D006801:Humans; D007136:Immunoglobulins / Q000032:analysis; D007150:Immunohistochemistry; D007249:Inflammation / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009103:Multiple Sclerosis / Q000473:pathology; D009186:Myelin Sheath / Q000378:metabolism; D009416:Nerve Regeneration / Q000502:physiology; D009471:Neuromyelitis Optica / Q000473:pathology*; D009836:Oligodendroglia / Q000473:pathology; D009900:Optic Nerve / Q000473:pathology; D016612:Paraffin Embedding; D013116:Spinal Cord / Q000473:pathology; D055815:Young Adult
D051401:Aquaporin 4; D005904:Glial Fibrillary Acidic Protein; D007136:Immunoglobulins; D003165:Complement System Proteins