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

Sung Min Kim, Patrick Waters, Mark Woodhall, Ji W. Yang, Hyeran Yang, Jee Eun Kim, Jung Joon Sung, Kyung S. Park, Kwang Woo Lee

Research output: Contribution to journalArticlepeer-review

10 Scopus citations

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.

Original languageEnglish
Article number93
JournalBMC Neurology
Volume14
Issue number1
DOIs
StatePublished - 29 Apr 2014

Bibliographical note

Funding Information:
This work was supported by grant no. 2010–0024457 from the National Research Foundation of Korea Fund, grant no. A121118 from the Korea Health Industry Development Institute Research fund, and grant no. 02-2012-008 from the SNUBH Research Fund. PW acknowledges funding from the Oxford NIHR Biomedical Research Centre. PW and MW acknowledge funding from the NHS National Commissioning Group for rare diseases.

Funding Information:
Dr. Waters is a named inventor on a patent relating to assays for the detection of antibodies to Lgi1, Caspr2, and Contactin2, and may receive royalties for this technology. Dr. Waters receives research support from the Oxford NIHR Biomedical Research Centre and has received a speaker honorarium from Biogen Idec, Japan. Dr. Kim Sung-Min, Dr. Woodhall, Dr. Kim Jee-Eun, Dr. Yang Ji Won, Dr. Yang Hyeran, Dr. Sung Jung-Joon, Dr. Park Kyung Seok, and Dr. Lee Kwang-Woo declare that they have no competing interests.

Keywords

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

Fingerprint

Dive into the research topics of 'Characterization of the spectrum of Korean inflammatory demyelinating diseases according to the diagnostic criteria and AQP4-Ab status'. Together they form a unique fingerprint.

Cite this