MONOAMINE METABOLITES IN THE CEREBROSPINAL-FLUID IN INFANTILE SPINAL MUSCULAR-ATROPHY

Analyses of tryptophan and tyrosine metabolites in cerebrospinal fluid were performed to examine an abnormality of the monoamine neurone sys tem in severe infantile spinal muscular atrophy (SMA type I). The leve ls of 5-hydroxyindoleacetic acid and homovanillic acid were significan tly lower than those in controls. Decreases in the concentration of 5- hydroxyindoleacetic acid and kynurenine seemed to be related to the se verity of SMA type I. These results suggest that the monoamine neurone system may play a role in the pathophysiology of SMA type I.