Cognitive functioning and depressive symptoms in Fabry disease: A follow‐up study

Abstract

Patients with Fabry disease (FD) have a high prevalence of depressive symptoms and can suffer from cognitive impairment, negatively affecting their life. The course of cognitive functioning and depressive symptoms in FD is unknown. The aim of this prospective cohort study was to describe changes in cognitive functioning and depressive symptoms and to identify related variables in patients with FD over 1 year. Assessments were conducted twice, using a neuropsychological test battery and the Centre of Epidemiological Studies Depression scale (CESD). Eighty‐one patients were included of which 76 patients (94%) completed both assessments (age: 44 years, 34% men, 75% classical phenotype). A significant decrease in cognitive functioning was found in four patients (5%), with patients regressing from excellent to average/good. Changes were not related to sex, phenotype, stroke, IQ or CESD scores. CESD scores ≥16 were present in 29 patients (38%) at baseline. Using the reliable change index a decrease in CESD scores was found in six patients (8%). Decreased CESD scores were independently related to employing a positive and problem solving coping style and increased CESD scores to an avoiding and brooding coping style and worsening health perception. We found no major changes in cognitive functioning in patients with FD during 1 year follow‐up making it an unsuitable outcome in FD treatment trials. Considering the high prevalence of persistent depressive symptoms, assessment of depressive symptoms should be part of routine follow‐up. Altering coping styles and health perception may improve psychological well‐being in FD.

Neuropsychiatric Symptoms and Their Association With Sex, Age, and Enzyme Replacement Therapy in Fabry Disease: A Systematic Review

“…However, normal cognitive performance was reported in memory, naming, perceptual functioning, and global cognitive functioning ( 16 – 18 ). Recently, prospective longitudinal studies reported normal cognition at baseline and two follow-ups [2-8 years after the baseline ( 31 )], suggesting a stable disease course. Körver et al ( 32 ) reported no major changes in cognition of 76 FD patients on one-year follow-up.…”

“…One of the most important is male sex (OR, 6.8; 95%CI, 1.6-39.8; p = 1.6 *10-2) ( 32 ). Körver et al showed that male sex is associated independently with cognitive impairment at baseline, but there were no changes related to sex in neuropsychological assessment after one-year follow-up ( 31 ). The study of Sigmundsdottir et al ( 33 ) reported that males with FD had a slower speed of information processing, reduced performance in executive functions (verbal generation, reasoning, problem-solving, perseveration), and they were more likely to show clinically significant limitations.…”

Health‐related quality of life in Norwegian adults with Fabry disease: Disease severity, pain, fatigue and psychological distress

“…We saw significant correlations between HADS depression score and both BPI interference and fatigue in our cohort. Whatever the etiology may be, given the magnitude and implications of psychiatric symptoms in FD, an increased focus on diagnosis and treatment of depression is warranted 19,76 …”

Depression: The Hidden Problem in Fabry Disease. A Review

“…Concerning psychological interventions, a recent study assessing changes in depressive symptoms after a 1-year follow-up found no difference when comparing CES-D scores between patients that received a counselling intervention and those who did not [52]. On the contrary, a small study assessing the effects of a counselling intervention administered by a psychotherapist and utilizing cognitive-behavioural strategies and insight-oriented techniques tailored to each patient particular situation improved depression in FD patients for up to 6 months after concluding the intervention.…”