[Neuromyelitis optica in children. Two case reports].
Devic neuromyelitis optica (NMO) is an inflammatory demyelinating disease of the central nervous system associating acute transverse myelitis and optic neuritis. This is a different disease from multiple sclerosis for which the presence of the NMO antibody directed against aquaporin 4 is a specific marker. Brain damage on MRI does not exclude the diagnosis; the location is superposable in the brain zones rich in aquaporin 4 channels. We report 2 cases of NMO with anti-aquaporin 4. One patient was not symptomatic of brain damage. In this patient, the affinity of anti-NMO for aquaporin 4, studied by flow cytometry, was particularly high. Both patients were treated with immunosuppressive agents (rituximab) due to the failure of or dependence on high-dose corticosteroids.
Service de neurologie pédiatrique, CHU La Timone, Assistance publique-Hôpitaux de Marseille, 264, rue Saint-Pierre, 13005 Marseille, France. barthelemy.tosello@ap-hm.fr
B
B
Tosello
C
C
Halbert
J
J
Mancini
B
B
Chabrol
J
J
Boucraut
M
M
Milh
D000293:Adolescent; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use; D051401:Aquaporin 4 / Q000276:immunology; D001323:Autoantibodies / Q000097:blood; D005260:Female; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use; D009471:Neuromyelitis Optica / Q000175:diagnosis* / Q000188:drug therapy; D000069283:Rituximab
C496030:AQP4 protein, human; D058846:Antibodies, Monoclonal, Murine-Derived; D051401:Aquaporin 4; D001323:Autoantibodies; D007155:Immunologic Factors; D000069283:Rituximab