Clinical spectrum of CNS aquaporin-4 autoimmunity.

Abstract

OBJECTIVES
Traditionally, neuromyelitis optica (NMO) was known to involve only the optic nerves and spinal cord. However, the discovery of highly specific anti-aquaporin-4 (AQP4) antibody for NMO enabled us to identify more diverse clinical manifestations. Here, we describe the demographic and clinical characteristics of patients who were anti-AQP4-antibody positive, represented by CNS AQP4 autoimmunity.
METHODS
In total, 388 consecutive patients with inflammatory demyelinating CNS diseases were tested for the anti-AQP4 antibody and 106 seropositive patients who were positive by ELISA or cell-based assay were included.
RESULTS
Ninety-seven patients were women, and 9 men. The median age at onset was 32 years. The median annualized relapse rate was 1.14 during the median follow-up of 7.0 years. When the 2006 revised diagnostic criteria for NMO were applied, 72% of patients met the criteria, and 28% had a limited form of NMO. Brain symptoms were observed in 51% of patients, and 24% of patients presented with brain symptoms as their first manifestation. Severe residual visual impairment or ambulatory disability was observed in 42% of patients. The median intervals to Expanded Disability Status Scale (EDSS) 6 and severe visual impairment in at least one eye were 12 and 11 years, respectively. A multivariate analysis revealed a delay of more than 4 years before appropriate immunotherapy was independently associated with reaching severe disability of more than EDSS 6.
CONCLUSION
The spectrum of neurologic manifestations and the disease course associated with CNS AQP4 autoimmunity is broader than previously recognized.

Links

Publisher Full Text

Authors

Kim SH, Kim W, Li XF, Jung IJ, Kim HJ

Institution

Department of Neurology, Research Institute and Hospital of National Cancer Center, Goyang, Korea.

Source

Neurology 78:15 2012 Apr 10 pg 1179-85

MeSH

Adult
Antibodies, Monoclonal, Murine-Derived
Aquaporin 4
Autoantibodies
Autoimmunity
Azathioprine
Disabled Persons
Drug Administration Schedule
Early Diagnosis
Enzyme-Linked Immunosorbent Assay
Female
Follow-Up Studies
Humans
Immunosuppressive Agents
Interferon-beta
Kaplan-Meier Estimate
Male
Middle Aged
Mitoxantrone
Multivariate Analysis
Mycophenolic Acid
Neuromyelitis Optica
Predictive Value of Tests
Prednisolone
Prognosis
Republic of Korea

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

22459676