Open Access Research article

Characterization of the spectrum of Korean inflammatory demyelinating diseases according to the diagnostic criteria and AQP4-Ab status

Sung-Min Kim1, Patrick Waters2, Mark Woodhall2, Ji Won Yang1, Hyeran Yang3, Jee-Eun Kim4, Jung-Joon Sung1, Kyung Seok Park13* and Kwang-Woo Lee1

Author Affiliations

1 Department of Neurology, Seoul National University, College of Medicine, Seoul, Korea

2 Nuffield Department of Clinical Neurosciences, Neuroimmunology group, Oxford, UK

3 Department of Neurology, Seoul National University Bundang Hospital, 166 Gumi-Ro, Bundang-Gu, Seongnam-Si, Gyeonggi, Korea

4 Department of Neurology, Seoul Medical Center, Seoul, Korea

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BMC Neurology 2014, 14:93  doi:10.1186/1471-2377-14-93

Published: 29 April 2014

Abstract

Background

The relative frequencies of demyelinating diseases among Korean patients with idiopathic inflammatory demyelinating disease of the central nervous system (IIDD) have not been sufficiently studied. We therefore describe a cohort of 203 patients with IIDD from three centers in Korea whose syndromes were identified precisely according to international clinical criteria and autoantibody to aquaporin 4 (AQP4-Ab) status.

Methods

In total, 260 consecutive patients were screened and 203 were included from three hospitals in Korea. All were tested for AQP4-Ab by using a cell-based assay. Patients who met the criteria for definite neuromyelitis optica (NMO) or had a positive AQP4-Ab test result were defined as the NMO group. Among the others, patients were assessed if they had acute disseminated encephalomyelitis, multiple sclerosis (MS), acute transverse myelitis, optic neuritis, or other demyelinating disease as a clinically isolated syndrome of the brain.

Results

Eighteen percent of patients were classified as the NMO group, 2% as acute disseminated encephalomyelitis, 18% as MS, 41% as acute transverse myelitis, 11% as optic neuritis, and 8% as other clinically isolated syndrome of the brain. AQP4-Ab was positive in 18% of patients and the relative frequency of NMO to MS (NMO/MS ratio) was 1.06. The mean duration of follow up in our patients was 64 months.

Conclusions

Among Korean patients with idiopathic inflammatory demyelinating diseases, the incidence of NMO may be similar to that of MS, and the overall positivity of AQP4-Ab could be lower than previously reported. In addition, acute transverse myelitis that is not associated with MS or NMO can be relatively common in these patients. Further population-based studies with AQP4-Ab are needed to determine the exact incidence of NMO and other idiopathic inflammatory demyelinating diseases in Korea.

Keywords:
Neuromyelitis optica; Multiple sclerosis; Anti-aquaporin-4 antibody; Idiopathic inflammatory demyelinating disease of the central nervous system; Korean