title: Elevation of tau protein levels in the cerebrospinal fluid of children with West syndrome.
abstract: OBJECTIVE
  West syndrome is an epileptic encephalopathy with a poor developmental outcome. Tau protein levels in the cerebrospinal fluid (CSF) are reported to be markers of axonal damage and neurodegeneration. This study aimed to investigate axonal damage and the effects of adrenocorticotropic hormone (ACTH) therapy on axons in West syndrome, as measured by tau protein levels in CSF.
  
  
  METHODS
  Tau protein levels in CSF before and after ACTH therapy were determined by an enzyme-linked immunosorbent assay in 26 children with West syndrome. Of these 26 children, 18 were symptomatic, and 8 had a cryptogenic form of West syndrome. A group of 41 unaffected children was included in the study as a control group.
  
  
  RESULTS
  The levels of tau protein in CSF were significantly higher in children with West syndrome than in the control group, and these levels remained high after ACTH therapy. ACTH therapy was effective for 20 of the 26 children with West syndrome, and their CSF tau protein levels were significantly higher after ACTH therapy than before therapy.
  
  
  CONCLUSIONS
  Our results suggest that axonal damage occurs in West syndrome, as judged by tau protein levels in CSF.
authors: Department of Pediatrics, Yamaguchi University Graduate School of Medicine, Yamaguchi, Japan. m003um@yamaguchi-u.ac.jp
authors: Hirofumi
authors: H
authors: Inoue
authors: Takeshi
authors: T
authors: Matsushige
authors: Shunji
authors: S
authors: Hasegawa
authors: Arisa
authors: A
authors: Abe
authors: Yasunori
authors: Y
authors: Iida
authors: Teruaki
authors: T
authors: Inoue
authors: Takashi
authors: T
authors: Ichiyama
mesh_terms: D000324:Adrenocorticotropic Hormone / Q000627:therapeutic use; D001369:Axons / Q000473:pathology*; D002675:Child, Preschool; D005260:Female; D006728:Hormones / Q000627:therapeutic use; D006801:Humans; D007223:Infant; D008297:Male; D009410:Nerve Degeneration / Q000473:pathology*; D013036:Spasms, Infantile / Q000134:cerebrospinal fluid* / Q000188:drug therapy / Q000473:pathology*; D016875:tau Proteins / Q000134:cerebrospinal fluid*
chemical_list: D006728:Hormones; C054369:MAPT protein, human; D016875:tau Proteins; D000324:Adrenocorticotropic Hormone