Clinical features of opticospinal multiple sclerosis with anti-aquaporin 4 antibody.
BACKGROUND
We have followed 9 Japanese patients with opticospinal multiple sclerosis (OSMS), some of whom showed longitudinally extensive spinal cord lesions, deep sensory disturbances and resistance to treatment. We investigated the patients for anti-aquaporin 4 (AQP4) antibodies and related this to their neuroimaging, clinical and laboratory features.


METHODS
We studied the clinical course, neurological findings, cerebrospinal fluid (CSF), and electrophysiological findings, and determined the presence of anti-AQP4 antibody and human leukocyte antigen DPB1 and DRB1 alleles.


RESULTS
Five patients (56.6%) had anti-AQP4 antibody. Antibody-positive patients displayed female predominance, longitudinally extensive spinal cord lesions, higher frequency of exacerbations, severe disability, and higher cell counts and total protein content without IgG oligoclonal bands in the CSF. They also showed poor steroid responsiveness and poor therapeutic response to interferon beta(1b).


CONCLUSIONS
The presence of anti-AQP4 antibodies correlates with clinical severity and poor prognosis in OSMS.
Kanzaki
Mami
M
Division of Neurology, Department of Internal Medicine 3, National Defense Medical College, Tokorozawa, Japan.
Mochizuki
Hitoshi
H
Ogawa
Go
G
Tanaka
Keiko
K
Araki
Manabu
M
Motoyoshi
Kazuo
K
Hammond-Tooke
Graeme
G
Kamakura
Keiko
K
D000328:Adult; D000938:Antigen-Presenting Cells / Q000276:immunology; D051401:Aquaporin 4 / Q000276:immunology*; D001323:Autoantibodies / Q000097:blood*; D001921:Brain / Q000473:pathology; D004594:Electrophysiology; D005260:Female; D006801:Humans; D007167:Immunotherapy; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D020529:Multiple Sclerosis, Relapsing-Remitting / Q000453:epidemiology / Q000276:immunology* / Q000473:pathology / Q000503:physiopathology; D015995:Prevalence; D013116:Spinal Cord / Q000473:pathology
D051401:Aquaporin 4; D001323:Autoantibodies