Prophyrin precursors in blood, urine and cerebrospinal fluid in acute porphyria.

Four patients suffering from variegate prophyria were investigated during acute attacks. Porphyrin and prophyrin precursor concentrations were determined in the urine, serum, cereborspinal fluid (CSF) and stools. Levels of delta-aminolaevulinic acid (ALA) and porphobillinogen were found to be very much lower in CSF than in serum sampled concurrently, and were well below levels at which these substances have been shown to exert effects on neural tissue in vitro. These findings cast doubt on the possibility that ALA or porphobilinogen is responsible for the production of the neural manifestations of acute porphyria.