Attack-related severity: a key factor in understanding the spectrum of idiopathic inflammatory demyelinating disorders.
Understanding the spectrum of idiopathic inflammatory demyelinating disorders (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 clinical course and lesion distribution. We compared the demographic features, clinical characteristics, laboratory findings, and genetic backgrounds between 193 Japanese patients with and without clinically or radiographically fulminant attacks who all satisfied the diagnostic criteria for multiple sclerosis (MS). "Fulminant attacks" in the current study represent attack-related clinically or radiologically severe relapses but do not necessarily mean severe disability. Patients with fulminant attacks were clinically and immunogenetically distinct from those free of such attacks, and the previously described characteristics of the opticospinal form of MS (OSMS) or neuromyelitis optica (NMO) were mostly shared by patients with fulminant attacks. HLA profiles were similar among patients with fulminant 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 significantly more common in patients with fulminant attacks than in those without. Attack-related severity may be an important factor if validated by prospective studies defining criteria and establishing relationships to disease course and treatment regimens.
Fukazawa
Toshiyuki
T
Hokuyukai Neurology Hospital, Niju-Yon-Ken 2-2-4-30, Nishi-ku, Sapporo 063-0802, Japan. fukazawa@my.email.ne.jp
Kikuchi
Seiji
S
Niino
Masaaki
M
Yabe
Ichiro
I
Miyagishi
Ryuji
R
Fukaura
Hikoaki
H
Hamada
Takeshi
T
Tashiro
Kunio
K
Sasaki
Hidenao
H
D000293:Adolescent; D000328:Adult; D015703:Antigens, CD; D000943:Antigens, Differentiation / Q000235:genetics; D060908:CTLA-4 Antigen; D016172:DNA Fingerprinting / Q000379:methods; D003710:Demography; D003711:Demyelinating Diseases / Q000134:cerebrospinal fluid / Q000145:classification / Q000175:diagnosis* / Q000235:genetics; D004185:Disability Evaluation; D005260:Female; D005500:Follow-Up Studies; D006682:HLA-DP Antigens / Q000235:genetics; D006684:HLA-DR Antigens / Q000235:genetics; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D008875:Middle Aged; D009103:Multiple Sclerosis / Q000134:cerebrospinal fluid / Q000175:diagnosis* / Q000235:genetics; D012189:Retrospective Studies; D020133:Reverse Transcriptase Polymerase Chain Reaction / Q000379:methods
D015703:Antigens, CD; D000943:Antigens, Differentiation; D060908:CTLA-4 Antigen; C556706:CTLA4 protein, human; D006682:HLA-DP Antigens; D006684:HLA-DR Antigens