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This study investigates the relationship between objective cognitive impairment (OCI), subjective cognitive complaints and depressive symptoms in men and women with classical and non-classical Fabry disease (FD). Cognitive functioning was assessed using a neuropsychological test battery, subjective cognitive complaints using a structured interview and depressive symptoms using a depression scale (CESD). Eighty-one patients were included (mean age 44.5 ± 14.3, 35% men, 74% classical). Subjective cognitive complaints were reported by 64% of all patients. OCI was present in thirteen patients (16%), predominantly in men with classical FD. Thirty-one patients (38%) had a high score (≥16) on the CESD scale. Male sex (OR, 6.8; 95%CI, 1.6–39.8; p = 1.6 * 10 −2 ) and stroke (OR, 6.4; 95% CI, 1.1–41.0; p = 3.7 * 10 −2 ) were independently positively associated with OCI, and premorbid IQ (one IQ point increase: OR, 0.91; 95%CI, 0.82–0.98; p = 3.8 * 10 −2 ) was independently negatively associated with OCI. The CESD-score (one point increase: OR, 1.07; 95% CI, 1.02–1.13; p = 3.3 * 10 −3 ) and a history of depression (OR, 2.7; 95% CI, 1.1–7.3; p = 3.9 * 10 −2 ) were independently positively associated with subjective cognitive complaints. OCI is present in 16% of FD patients, warranting referral for neuropsychological assessment. Nevertheless, subjective cognitive complaints are related to depressive symptoms, emphasizing the importance of recognition and treatment of the latter.

Predictors of objective cognitive impairment and subjective cognitive complaints in patients with Fabry disease