Abstract
BackgroundThe 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.MethodsIn 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.ResultsEighteen 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.ConclusionsAmong 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.
Highlights
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
Idiopathic inflammatory demyelinating disease of the central nervous system (IIDD) refers to a wide spectrum of disease entities that mostly consist of multiple sclerosis (MS) [1], neuromyelitis optica (NMO) [2,3], acute disseminated encephalomyelitis (ADEM) [4], acute transverse myelitis (ATM) [5], and optic neuritis (ON) [6]
Patients In total, 260 consecutive patients who were suspected as having IIDDs such as definite NMO, NMO spectrum disorder (NMOSD) [2,3], ADEM [4], MS [1], ATM [5], ON [6], or a clinically isolated syndrome (CIS) of the brain [15] and whose serum was tested at the John Radcliffe Hospital, Oxford, were screened [16]
Summary
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 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. The NMO/MS ratio, as well as the relative frequencies of other demyelinating diseases such as ADEM, ATM, and ON among Korean patients with IIDD, have not been sufficiently studied. The aim of this study was to describe a cohort of 203 patients from three centers in Korea with IIDD of the central nervous system, using international clinical and serological criteria The relative frequency of NMO to that of MS (NMO/MS ratio) was previously reported to be high in Thailand (1.4) [10] and Japan (0.29–0.59) [11,12], compared to that in Europe (0.024) [13] and Latin America (0.073–0.26) [14].
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