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

Abstract

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.

Occupational therapy in spinocerebellar ataxia type 3: an open-label trial

“…OT improved the depressive symptoms of SCA3 patients, which are an important clinical problem already shown to be common among these patients (12 incapacitation levels observed in previous studies was not found in the present sample (12). We speculate that our lack of association was due to the recruitment of patients with relatively shorter disease duration and lower impact on their capabilities.…”

“…All of these findings lead to a progressive burden and incapacitation. In addition, depressive symptoms are rather frequent and may be related to the inexistence of an effective treatment (12). In SCA3, depressive scores have been associated with the level of incapacitation, conditions that probably reinforce each other.…”

Bidirectional Connections between Depression and Ataxia Severity in Spinocerebellar Ataxia Type 3 Patients

“…First, BDI was the only tool available for the evaluation of the subjects’ depression. The degree of depression would be accurate when assessed by a series of tools related to patients’ psychiatric symptoms, although BDI had also been used solely in several studies on the depression in SCA3 patients [15, 16, 18-21]. Second, although in our cohort, the frequency of depression was extremely high, we lacked an age- and sex-matched control group to compare between the 2 groups.…”

“…The severity of the subjects’ depression in SCA3 patients was evaluated by Beck depression inventory (BDI), which was solely and widely used for evaluating the depression of SCA3 patients [15, 16, 18-21]. Since the participating subjects were Chinese, the adopted BDI in Chinese version was found to exhibit optimal sensitivity and specificity in recognizing the depression among the ­Chinese-speaking population [22].…”

“…BDI was a self-rating tool comprising of 21 questions ranging from 0 to 63 as well as higher scores reflecting a severe depression [21]. In the present study, BDI score of ≥19 was defined as clinically relevant according to a previous study [16]. The frequencies of depression of different populations were cited from references.…”