The authors screened 3,867 psychiatric inpatients for intermittent acute
porphyria by use of a spot test to detect diminished activity of the
erythrocyte enzyme porphobilinogen (PBG) deaminase. Eighteen individuals so
identified also had persistently diminished quantitative activity of PBG
deaminase. Eight of these appeared to have intermittent acute porphyria by
the added criteria of increased urinary delta- aminolevulinic acid or PBG
or a family history of intermittent acute porphyria. The overall prevalence
of intermittent acute porphyria was 0.21%, a considerably higher rate than
that in the general population. Most of the subjects with the disorder had
periods of agitated psychosis and apathetic or depressed withdrawal, with
signs of neuropsychological impairment. Neurologic abnormalities were not
prevalent.Abstract Teaser