Abstract: Patients receiving miglustat therapy, enzyme replacement treatment, and untreated individuals were assessed using psychologist-administered and computerized neuropsychological batteries. 55-60 exposed patients in Israel were evaluated. Miglustat recipients scored lower on 5 of 18 psychologist-administered subtests, yet performed comparably to controls on computerized assessments. The authors concluded that any dysfunction suggested is apparently subtle and of doubtful clinical relevance, and noted computerized testing offers language options, objectivity, brevity, and ease of use for longitudinal monitoring of substrate reduction therapy.
Computerized cognitive testing in patients with type I Gaucher disease: effects of enzyme replacement and substrate reduction
Elstein D, Guedalia J, Doniger GM, Simon ES, Antebi V, Arnon Y, Zimran A. Computerized cognitive testing in patients with type I Gaucher disease: effects of enzyme replacement and substrate reduction. Genet Med. 2005 Feb;7(2):124-30.