Understanding the spectrum of idiopathic inflammatory demyelinating d isorders (IIDD) is a fundamental issue for the diagnosis and treatment of these disorders as well as for the approach to their pathogenesis. The spectrum of IIDD is usually classified according to clin ical course and lesion distribution. We compared the demographic features, clini cal characteristics, laboratory findings, and genetic backgrounds between 193 Ja panese patients with and without clinically or radiographically fulminant attack s who all satisfied the diagnostic criteria for multiple sclerosis (MS). “Fulmi nant attacks”in the current study represent attack related clinically or radio logically severe relapses but do not necessarily mean severe disability. Patient s with fulminant attacks were clinically and immunogenetically distinct from tho se free of such attacks, and the previously described characteristics of the opt icospinal form of MS (OSMS) or neuromyelitis optica (NMO) were mostly shared by patients with fulminant attacks. HLA profiles were similar among patients with f ulminant attacks irrespective of the lesion distributions. The GG homozygous and G alleles of the CTLA4 gene A/G coding SNP at position 49 in exon 1 were signif icantly more common in patients with fulminant attacks than in those without. At tack related severity may be an important factor if validated by prospective st udies defining criteria and establishing relationships to disease course and tre atment regimens.
展开▼
