West syndrome: cerebrospinal fluid nerve growth factor and effect of ACTH.
West syndrome is a strictly age-limited encephalopathy of early infancy with unknown pathogenesis. It is often progressive, leading to mental retardation. Neurotrophic factors are important for the regulation of neuronal survival and differentiation, and their expression is influenced by hormones. Levels of beta-nerve growth factor in the cerebrospinal fluid were examined by two-site enzyme-linked immunosorbent assay method. Human antigen was used as a standard. We present data on largely normal levels of nerve growth factor in the cerebrospinal fluid of infants with cryptogenic etiology, but low or negligible levels in infants with symptomatic etiology, and very high levels in infants with symptomatic postinfectious etiology. Treatment with ACTH led to a greater increase in patients with a good response than in those with a poor response. Low nerve growth factor in patients with symptomatic infantile spasms possibly reflects massive neuronal death. The regression seen in these infants and their poor response to ACTH therapy may be due in part to lack of growth factors supporting neuron survival. This study, previously only demonstrated in animal models, is the first to depict nerve growth factor gene activity in humans as modulated by steroids.
Riikonen
R S
RS
Department of Child Neurology, Children's Hospital, University of Helsinki, Finland.
Söderström
S
S
Vanhala
R
R
Ebendal
T
T
Lindholm
D B
DB
D000324:Adrenocorticotropic Hormone / Q000627:therapeutic use*; D017668:Age of Onset; D006801:Humans; D007223:Infant; D009414:Nerve Growth Factors / Q000134:cerebrospinal fluid*; D013036:Spasms, Infantile / Q000134:cerebrospinal fluid* / Q000188:drug therapy
D009414:Nerve Growth Factors; D000324:Adrenocorticotropic Hormone