Characterization of the spectrum of Korean inflammatory demyelinating diseases according to the diagnostic criteria and AQP4-Ab status.
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.
Sung-Min
SM
Kim
Patrick
P
Waters
Mark
M
Woodhall
Ji Won
JW
Yang
Hyeran
H
Yang
Jee-Eun
JE
Kim
Jung-Joon
JJ
Sung
Department of Neurology, Seoul National University, College of Medicine, Seoul, Korea. pks1126@chol.com.
Kyung Seok
KS
Park
Kwang-Woo
KW
Lee
D000328:Adult; D000368:Aged; D051401:Aquaporin 4 / Q000276:immunology / Q000378:metabolism*; D001323:Autoantibodies / Q000032:analysis; D015331:Cohort Studies; D003711:Demyelinating Diseases / Q000175:diagnosis / Q000453:epidemiology* / Q000378:metabolism*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007249:Inflammation / Q000175:diagnosis / Q000453:epidemiology* / Q000378:metabolism*; D008297:Male; D008875:Middle Aged; D056910:Republic of Korea / Q000453:epidemiology
D051401:Aquaporin 4; D001323:Autoantibodies