Peripheral lymphocyte subset and serum cytokine profiles of patients with West syndrome.
OBJECTIVE
To clarify the immune pathophysiology of West syndrome (WS).


METHODS
We measured peripheral blood lymphocyte subset and serum cytokine profiles in 76 WS patients and 26 age-matched controls. Adrenocorticotropic hormone (ACTH) is one of the most effective therapy for WS and presumably immune-modulating; therefore, we compared the measured parameters between before ACTH (pre-ACTH) WS patients and controls, between cryptogenic and symptomatic WS patients before ACTH (pre-ACTH), and between before (pre-ACTH) and after (post-ACTH) ACTH WS patients. The post-ACTH group included those who received the last ACTH dose within 1 month of sampling.


RESULTS
CD3+ CD25+, CD19+, and CD19+ CD95+ cells were found to be significantly lower in the pre-ACTH group than in the controls. Interleukin (IL)-1 receptor antagonist (RA), 5, 6, and 15; eotaxin; basic fibroblast growth factor (bFGF); and interferon gamma-inducible protein (IP)-10 levels were higher in pre-ACTH group than in the controls. No significant differences were found between the pre-ACTH cryptogenic and symptomatic groups. CD4+ cells, CD3+ cells, CD4+/8+ ratio, IL-1 beta, IL-12, and macrophage inflammatory protein (MIP)-1 beta were significantly higher in pre-ACTH group than in the post-ACTH group.


CONCLUSIONS
Our study revealed immunological alterations in WS patients, and these responses were modified by ACTH therapy. Further study is needed to elucidate whether or how the immune system alteration is involved in the pathophysiology of WS.
Shiihara
Takashi
T
Department of Neurology, Gunma Children's Medical Center, 779 Shimohakoda Hokkitsu-machi, Shibukawa, Gunma 377-8577, Japan. shiihara-ind@umin.net
Miyashita
Momoyo
M
Yoshizumi
Masakazu
M
Watanabe
Mio
M
Yamada
Yoshiyuki
Y
Kato
Masahiko
M
D000324:Adrenocorticotropic Hormone / Q000378:metabolism; D016022:Case-Control Studies; D002675:Child, Preschool; D016207:Cytokines / Q000097:blood*; D005260:Female; D006801:Humans; D007223:Infant; D007962:Leukocytes / Q000473:pathology; D016131:Lymphocyte Subsets / Q000145:classification / Q000473:pathology*; D008297:Male; D012189:Retrospective Studies; D013036:Spasms, Infantile / Q000097:blood / Q000276:immunology / Q000473:pathology
D016207:Cytokines; D000324:Adrenocorticotropic Hormone