Depressive Symptoms in Machado-Joseph Disease (SCA3) Patients and Their Relatives

• Published in: Community genetics 2007-01, Vol.10 (1), p.19-26
• Main Authors: Cecchin, C.R., Pires, A.P., Rieder, C.R., Monte, T.L., Silveira, I., Carvalho, T., Saraiva-Pereira, M.L., Sequeiros, J., Jardim, L.B.
• Format: Article
• Language: English
• Subjects: Adult; Ataxin-3; Caregivers; Depression - diagnosis; Depression - psychology; Family; Female; Humans; Machado-Joseph Disease - physiopathology; Machado-Joseph Disease - psychology; Male; Middle Aged; Nerve Tissue Proteins - metabolism; Nuclear Proteins - metabolism; Original Paper; Repressor Proteins - metabolism; Severity of Illness Index; Variance analysis
• ISSN: 1422-2795; 1662-4246; 1422-2833; 1662-8063
• DOI: 10.1159/000096276

Objectives: It was the aim of this study to determine the depression scores of Machado-Joseph disease (MJD) patients, their spouses, and individuals at 50% risk for MJD, and second, to verify the existence of a correlation between depressive symptoms and the degree of motor incapacitation. Subjects and Methods: Two hundred and forty-six individuals aged ≧18 years were studied: 79 MJD patients (group 1), 43 spouses of MJD patients (group 2), 80 individuals at risk for MJD (group 3), and a control group (group 4) composed of 44 patients with multiple sclerosis (MS). The following two tools were applied: the Beck Depression Inventory and the Barthel index of physical incapacitation, both in an adapted Brazilian Portuguese version. Results: Moderate to severe depressive scores were found in 33.5% of patients in the MJD families, in 16.3% of the spouses, and in 6.3% of the individuals at risk. This linear reduction between MJD family members was statistically significant (p < 0.0001, ANOVA). Depressive scores were also associated with age and the female sex. A direct correlation between Beck Depression Inventory scores and motor incapacitation was found in MJD patients (r = 0.507, Pearson correlation, p < 0.0001). Although the depressive symptoms in the control group with MS were higher than those found in MJD patients (59% of MS patients showed moderate to severe scores), depression did not correlate with physical incapacitation, age, or education attainment in the MS group. Conclusions: Depressive symptoms are rather common in MJD patients and in their spouses (caregivers). In this condition, depression seemed to be more reactive than primarily related to the disease process itself.