38. Is the frequency of biotinidase deficiency greater in children with unexplained developmental delay or neurologic abnormalities than in the general population? This question was investigated by Sutherland et al. (A-40). They studied 274 children seen at a large outpatient clinic over a four-year period who had one or more of these neurologic abnormalities and for whom no specific cause for their abnormalities could be found.

None of the patients with nonclassic biotinidase-deficiency findings had a deficiency of biotinidase activity.